Confessions of a Cyster

Loss

2012-01-22T18:14:00.000-05:00

What a week...full of loss and mistakes on many different levels. This week has left me with a heavy heart and a questioning soul.

First, a quick health update. I am feeling really well. Last year I participated in a clinical trial for the COPD drug, Spireva. I was on the drug for 10 months. In December I was called by my research coordinator asking if I could come back to participate in another little study related to the first one. There were two arms of the initial Spireva study...one for people with CF and one for people with COPD. It turns out that several participants in the COPD arm of the study died of heart-related issues. Therefore, they wanted to bring several people back to go back on the drug for one more month, while monitoring the heart with EKG's at several intervals during the month. I spent part of my Monday at my CF center having repeated EKG's. I have a really low heart rate...always have and always will. However, it was even lower than normal. We didn't get a reading above 42 beats per minute on Monday. In an effort to not hurt my hearts feelings I choose to call it "highly efficient" instead of "slow".

I'm still trucking along with my 4 antibiotics. I'm still not seeing a great deal of side effects. I still HATE doing my nebulizer treatments twice a day. I'm still thankful beyond words that my health is stable!

On to the biggest mistake of the week. After years of begging, I finally gave in and got my kids a dog. If you read my previous post, you know that my kids have horrible lungs, asthma and allergies. However, I did my research. I looked for dogs that were the most hypoallergenic, we visited the puppy twice before we took her home to ensure that the allergies wouldn't be an issue and we took the dog with the caveat that this was a trial to see if our lungs agreed with this pup. We had the puppy, the most adorable yorkipoo ever, for a total of 24 hours. My eyes burned the entire time we had her, but more worrisome was that my son...whose health we just got stabilized a few weeks ago...started having asthma symptoms again within hours of bringing the dog home. I made the decision to give the dog back swiftly and she was back where we got her within hours. BUT not without tears. My son didn't seem to care much, but giving up the dog was equivalent to ripping my daughters heart out. She cried for hours on end. ***SOBBED*** This made my heart break. All I wanted to do was make my kiddos happy and I ended up making one sick and the other sad. This was a HUGE mommy-fail on my part. I should have never even gone through with the doggy trial. I'm left kicking myself and second-guessing my parenting choice in this instance. Emma is better and seems to understand why we couldn't keep the puppy, but my son is still not well. I had to start another prednisone burst. Why, Stacey, Why?!?!

Let's move on to Thursday. I got a call from my mother that the end was near for my grandfather. This is the only grandfather I have ever know. My maternal and paternal grandfather's both passed away before I was born. My grandmother got remarried to Merv at least 25 years ago. My memories of my Grandmother all include memories of Merv. Merv has had a long life of over 91 years. He has been deteriorating over the past few months. On Thursday he was rushed to the ER due to his inability to keep his blood-pressure up. I took off an afternoon of work and went to sit with him. I held his hand and told him that it was okay to let go. I reassured him that we would always take good care of Grandma. I listened to his stories...some of which made sense. Apparently, "they" are writing a story on the ceiling that he has been reading. It's kept him entertained the last several days. I honestly think this is lovely...that when his mind is deteriorating it's causing his to see stories on the ceiling. Merv is an author. He has written a few books, including "We Had a Shore Fast Line". Reading and writing have always been his thing. I am so glad they can still be his thing as his life winds down.

So I said my good-byes and Merv was moved to the ICU. It was determined in the ER that his white blood cell count was very high, so he had an infection of unknown etiology. He was started on IV Zosyn before I left. However, the prognosis isn't good. Both he and we want no extraordinary measures. It was explained to us that antibiotics are not considered an extraordinary measure. They are treating him, but there is still not much hope for recovery. It is very sad to see a person get to this point in life...where there is nothing left. I get peace in the fact that he is feeling no pain. So basically we are preparing for a loss to come, probably within the next few days.

I got a call from my mother last night telling me not to visit Merv in the hospital anymore. Apparently, the raging infection is c diff. CRAP! If you know my story you know that I have been hospitalized for c diff. That particular infection is not my friend. It's evil! Once you've had it, you are much more susceptible to getting it again. They are now requiring visitors to mask, gown and glove before entering Merv's room. CRAP! I kissed him when I arrived and I held his hand with my ungloved hand for an hour. I don't regret doing these things...I would do it all again, but I should have not been so lax in protecting myself. I should have masked, gloved or maybe even scrubbed. I did none of these things. So, we will wait and see what happens. Last time I got c diff the symptoms came on about a week after exposure. Now, we wait... CRAP!

Finally, just today one of my CF friends took her final breaths. Kristi was a beautiful friend. She frequently checked in on me, even though her health was so much worse off than mine. She was a kind soul. She was friendly to everyone. She was waiting for a new set of lungs. She didn't make it long enough to receive those lungs. Kristi and I talked about meeting one day when she was in Cleveland, since she was going to get her transplant at the Cleveland Clinic. That never happened. Kristi was 29 years old... My heart is heavy after hearing of this loss.

This week has been full of praying for guidance, praying for comfort, praying for healing...praying...praying...and praying some more.

What Would You Do?

2011-12-14T21:42:00.000-05:00

I've never really shared information about the health of my kids on here before, but I feel like I'm at a point where I need to make some decisions and I'm looking for some advice.

Both of my kids...Emma (age 9) and Jacob (age 5)...have had troublesome lungs their entire lives. Prior to having children my husband was genetically tested to see if he was a carrier of CF, which he was determines not to be. At that time they only had the test that identified 83 of the most common genes. Now, they have a test that can identify more than 120 genes. I had always been fine with the 83 gene test, especially after both of my kids were sweat tested and came back with relatively low scores. They are both carriers of CF, but they do not have CF. This is what I have always been told, so this is what I have been going with for years.

However, lately we have been less and less able to control their Asthma. Since October 1st my son has had 3 prednisone bursts, 2 ER visits and 4 rounds of antibiotics. We can't find the antibiotic that will clear his lungs. We're currently on Biaxin. He honestly sounds like a full-fledged CFer. My daughter has had one round of steroids and one round of antibiotics, but she has been needing to go to the nurses office during the school day almost every day for albuterol and needs her rescue inhaler from time to time at home.

I had been taking them to our general pediatrician who has some experience in Asthma for several weeks. More recently I was able to get them in with their pulminologist. I adore their pulmonologist. For many years he was a CF doctor at the CF clinic I go to... Unfortunately, my children can't go to my clinic, since they have insurance through my husband and he works for (and has insurance that only covers) the competing hospital system. I feel comfortable taking them to this pulmonologist, though, since he has a CF background. I feel like he would be able to see if there is ever a connection between their lung issues and my own.

So, we get to the appointment and the kids have their initial Pulmonary Function Tests (PFT's). The goal is that they reach 100% of the predicted value that was determined by their age and weight. My daughter went first and blew 70%. My son did worse, only blowing a 58%. I was SHOCKED. The little guy is only 5 years old...how can he only have 58% lung function.

The doctor explained that with Asthma they focus less on the numbers and more on the change that happens after albuterol is given and the tests are repeated. The change for my daughter was significant. She went from 70% to 100%! This pattern showed standard Asthma. We switched around some of her daily medications and increased the doses of others. I felt very comfortable with this plan.

My sons PFT following albuterol only increased a bit...he went from 58% to 62%. This frustrates me. The doctor explained that it is likely because he is going through an acute illness and when we can get that figured out with the right antibiotic he should improve. I don't know...

The doctor was limited in his time, but I asked him if my kids...Jake in particular...might have something I have read a little about call "atypical cystic fibrosis". This is a condition where someone displays CF symptoms when all tests reveal that they are only carriers of the disease or they have non-typical symptoms. My children are both carriers, this we are sure of. The doctor said that the lower sweat test results indicate that atypical CF is likely not the case. He said we could genotype them when I come back in a few months to determine the type of gene they got from me.

The other thing I have thought about is whether my kids (particularly my son) may be dealing with the mycobacterium that I've had for a while. I know non-CFer's can contract this infection. My kids have been exposed to the same environment as I have, so they likely have come in contact with myco. I'm kicking myself for not asking for a simple culture. My son certainly has enough of a "sample" to provide. My own CF doctor told me to get this done during my kids appointment and I completely forgot.

Since the appointment I haven't felt satisfied. I have been wondering if sweat tests are ever false negative. I remember the nurse during my sons sweat test commenting on how she was unsure if she got enough sweat for his sample...the cotton was pretty dry. Could this make the reading lower, or would it just turn out to be unreadable? Does it matter if they label what they have as atypical CF? Would their treatment plan be any different? Would they benefit from being treated more like a CFer? I'm just not sure...

I'm looking for advice on what you would do in this situation. I don't want to be one of those "difficult" mom's by continually bringing up this issue, if I've already been told it's not likely. However, my gut tells me there might be more to the story with Emma and Jakes' health. Johns Hopkins is doing a lot of atypical CF research. Do I even go that far to get another opinion?

What would you do???

Why I Fight...Blogger Challenge

2011-12-04T20:35:00.002-05:00

Why I Fight for a Cure for Cystic Fibrosis...

When I was born in 1975 the prognosis for Cystic Fibrosis was very bleak. I was always painfully aware of the current life expectancy and I always had a hard time envisioning life beyond that age.

Yet, I know that I have shared here before that I feel like one of the "lucky one's". I have worked hard at staving off CF and for the most part it has steered clear enough for me to reach milestones that my parents once thought would have been impossible. The current life expectancy is 37.4 years. I turn 37 next year. I no longer have trouble imagining a life beyond the life expectancy. I feel pretty certain that CF won't take me anytime soon.

So why do I fight for a cure? I fight for a cure so the next generation of kids with CF can suffer less and live longer. I fight so new parents of babies with CF don't have to worry about out-living their children. I fight so this nasty disease can be beaten into submission. I also fight for all of the amazing men and women I have met with CF who work so very hard every day to stay alive. I am hopeful that someday in the neat future we will all be able to live without having to work so hard at it...

Please take a look at this video that was put together by my friend, Emily's, family.

Emily's Entourage Video

Then take the challenge below!

Here are your official rules for participation:
1) View Emily's amazing video here, then consider making a donation of your own or "liking" her page on FB (neither is required, but both are encouraged!).

2) Create your own blog post, FB status update, or other form of social networking tool (letter, email, whatever). In the body of the message, place a small paragraph of why YOU fight for a cure for cystic fibrosis and why this cause matters to YOU. This can be your CF story, your wish for the holidays, your version of community -- whatever.

3) Link to Emily's Entourage Website and encourage your own readers to take up the challenge.

4) Comment on Piper's blog here with a link to your blog a message about how you shared this vision for an automatic entry into a drawing for a very special CF/transplant-awareness prize package, including gifts from iheartguts.com, apparel, and other fun goodies!

5) Re-post these rules on your own page.

100 Things

2011-11-23T20:11:00.000-05:00

I am thankful beyond measure and I want to shout it out for all the world to hear. Since I'm positive that there isn't a megaphone big enough to do the job, I figured I would just share with you the top 100 things I'm thankful for this year...in NO particular order.

1) The Golden Girls - I love falling asleep to the sound of Blanche, Rose, Dorothy and Sophia bickering every night.
2) My adorable Lego-obsessed son, Jacob
3) Chinese dwarf hamsters
3) Pad Thai
4) Hot baths
5) Deep breaths
6) That I have the daughter my mother always told me I would have...the one who would give me a run for my money ;-)
7) Wet kisses
8) My cozy home
9) Heartland Community Church
10) The friendship that my husband and I have had over the past 20+ years
11) Smooth flights
12) Working from home
13) Dr. Dazzle
14) Comfy boots
15) Friends that have lasted a lifetime (Lisa)
16) Friends that feel like they have been in my life forever (Elizabeth)
17) Friends that weather the storm (Vicki)
18) New friends
19) Forgiveness
20) Second chances
21) Books, books & more books
22) Cherry Coke Zero
23) Pumpkin Pie
24) Morning snuggles
25) My amazing mom
26) My kind father
27) The beach
28) The Comedy Central late night line-up
29) Cleveland sports teams...God love 'em
30) Sunday football
31) Family game night
32) Movie theater popcorn
33) Modern conveniences
34) My Grandmother...my likeness
35) King size beds
36) Antibiotics with minimal side effects
37) Portable nebulizers
38) Healthy hours...days...weeks...months
39) GG...my honorary niece
40) Ebay
41) Amazon Prime
42) Love
43) Medical researchers
44) My step-parents...for making my parents happy
45) Family movie night
46) Calculators
47) Good health insurance
48) Martinis with friends
49) The SiriusXM Channel "Lithium"...90's alternative music
50) Sunsets
51) Kind deeds
52) Soup made out of love...Cathy
53) A clean house...Lynn
54) Fun nail polish
55) Annoying brothers that grow up to be much less annoying
56) Allergy medications
57) Chocolate
58) Successful whale watching missions
59) Romantic weekends away
60) Family vacations
61) warm socks
62) Happy hugs
63) Facebook friends
64) Lazy Sunday's
65) Crisp fall days
66) Sand between toes
67) Understanding co-workers
68) Blogs
69) The ability to exercise
70) Miracles big and small
71) Almond Joy
72) Bullet-proof vests
73) Microwaves
74) Stickers
75) Hair dye
76) Fleece
77) Chap Stick
78) Birthdays
79) Target
80) Super-brilliant step-daughters
81) Honey Hut ice cream
82) Fireplaces
83) Avocados
84) Asic running shoes
85) Family gatherings
86) Puppies...cue the allergy medicine
87) Skype
88) Fantasy football
89) Good movies
90) Dinner dates with my hubby
91) Opportunities
92) Bike rides
93) Long walks with good friends
94) Sunglasses
95) Prayers
96) Thunderstorms
97) Cousins
98) My reliable car
99) Holidays shared with family
100) Breathtaking moments!

Happy Thanksgiving to you all!

MAC is Whack!

2011-11-01T20:08:00.000-04:00

I had a clinic appointment today. I went in wondering if I was going to be able to maintain my record of having a clean culture for both the m. abscesses and MAC. When I checked my cultures from my previous clinic visit in August they were negative for both bugs(for the first time). I figured I was home free. I figured wrong. Ever since I started culturing this tuberculosis-wannabe disease, my cultures have shown evidence of it within days. I guess it took the full 8 weeks for the MAC to grow this time around. This is a positive sign. It means that my disease burden is smaller. However, it is still there...

Why does this bum me out a bit? It's true that all CFer's culture bugs constantly. I already knew that I will never completely get rid of the mycobacterium. The goal is to suppress it until it's no longer visible in a culture. That means that it is being well controlled. I guess I'm frustrated, because I thought I saw the light at the end of the tunnel. I was hoping that the end of an era was within my sights. I was told that I could start weening off of some of the drugs I'm taking after one year of clean cultures from mycobacterium. I was under the impression that the year was going to start in August. I was wrong. The year has not yet begun.

The good news is that I feel really good and my PFT's are still holding steady. I really feel like this is a good treatment plan. The other good news is that a clinical trial will be starting at my center in a few months using inhaled Arikace to treated resistant MAC. Right now the FDA put a hold on this trial, but I'm really hoping it's not for long. Dr. Dazzle said that I am at the very top of the list to be contacted when that study opens up!

Lesson learned...don't assume your culture is clean before the final reading! I'm going to try the patience thing next time.

Nothing and Everything

2011-10-12T18:34:00.011-04:00

Two lovely people pointed out today that I no longer blog. It has been a while. I haven't quit. I've just run out of things to write about... This writers block seems to have coincided with my excellent health status as of late. This blog has always been my respite when I was feeling confusion or fear related to my CF. I can honestly say that I have not been having any of these feelings. I had clean margins following the pre-melanoma skin excision. At my August pulm appointment, my PFT's were higher than they had been for 10 years and I feel equally as excellent!

So what have I been filling my days with over the past two months, now that I have moved into a phase where I'm not being forced to obsess about CF day in and day out? I've been working hard, loving my family and enjoying my off time immensely. In the past 2 1/2 months I've traveled to New York City, Nova Scotia, Washington DC, Boston and Toronto. Half of this travel was for work and half was for play. My very favorite experience from these trips happened on my getaway with my husband to Boston. I got to knock Whale Watching off of my bucket list. It was absolutely breathtaking...amazing...wondrous!

Last year at this time I feared that my life and my health would never return to "normal". I was grieving the loss of the life I had before I had to incorporate CF into every hour of my existence. For the past 2 months I have gotten to experience life as it was again and I couldn't be more thankful. I don't want to waste a moment of it. I now do an hour of treatments a day, take a couple handfuls of pills and complete an hour workout...CF does not own the other 22 hours. So, I guess that's a big part of the reason I haven't been blogging. I really see this as a CF blog and for now, at least, CF has been put in its place.

I came across 'Desiderata' today, I thought it related and I thought I'd share...

Go placidly amid the noise and haste, and remember what peace there may be in silence.

As far as possible, without surrender, be on good terms with all persons. Speak your truth quietly and clearly; and listen to others, even to the dull and the ignorant, they too have their story. Avoid loud and aggressive persons, they are vexations to the spirit.

If you compare yourself with others, you may... become vain and bitter; for always there will be greater and lesser persons than yourself. Enjoy your achievements as well as your plans. Keep interested in your own career, however humble; it is a real possession in the changing fortunes of time.

Exercise caution in your business affairs, for the world is full of trickery. But let this not blind you to what virtue there is; many persons strive for high ideals, and everywhere life is full of heroism. Be yourself. Especially, do not feign affection. Neither be cynical about love, for in the face of all aridity and disenchantment it is perennial as the grass.

Take kindly to the counsel of the years, gracefully surrendering the things of youth. Nurture strength of spirit to shield you in sudden misfortune. But do not distress yourself with imaginings. Many fears are born of fatigue and loneliness.

Beyond a wholesome discipline, be gentle with yourself. You are a child of the universe, no less than the trees and the stars; you have a right to be here. And whether or not it is clear to you, no doubt the universe is unfolding as it should.

Therefore be at peace with God, whatever you conceive Him to be, and whatever your labors and aspirations, in the noisy confusion of life, keep peace in your soul.

With all its sham, drudgery and broken dreams, it is still a beautiful world.

Be cheerful. Strive to be happy.

Max Ehrmann c.1920

Freeing

2011-08-07T19:07:00.000-04:00

You Don't Need to be Happy or Positive

by Patty Sherry, author of Share Your Love Story

Ridding myself of ALL negativity,anger, fear, and sadness is not my goal. Getting over this is not something I need to do.

Don’t get me wrong, I love to be positive, happy, and to feel good, the key is I dont NEED to.

Being angry all of the time can make you sick; some say such negativity is toxic! I don’t completely agree with this belief. I say that anger, fear, and sadness is as much a part of my human experience as LOVE is.

Avoidance of these emotions is not necessary.

That kick in the stomach, pain in my heart, or that headache actually comes from the belief that these “negative” emotions are somehow wrong, bad, or a state of being I need to get myself out of.

Believing I should be over this is the hard part!

Fighting the fact that I am here in the first place, and thinking I need to be over there….feeling happy, becomes my struggle!

Positive thinking can make me sick? Well in a way it can…when positivity becomes a need.

So I am releasing the idea that happiness and being positive is a permanent state I must seek, and I am being more gentle with myself when I am in one of those “negative” places.

It’s so easy to get caught up in the belief that I am or YOU are doing something wrong or bad if we are not happy. Igniting that internal judgmental dialogue, ” I’m so stupid!” “Here we go again!” flows easily, even automatically.

Accepting what IS; I have no goal to get over anything! I remind myself that what I feel has to do with THIS moment. Change can come in the next moment.

I become more FREE in each moment, you do too. Running or hiding from any emotion YOU or I feel is not necessary, but rather we can embrace and shift it if we so CHOOSE.

Life is my party…and I can cry if I want to. Freeing isn’t it?

Compliance, Judgement & Progression

2011-07-29T16:45:00.001-04:00

A fellow blogger, Piper, over at A Matter of Life and Breath wrote an amazing blog post yesterday and extended a challenge to others to write about their thoughts on CF control and progression. So here goes...

I am an old CFer, who has by luck or biology or by the grace of God, had an easier road than most with this disease. It has limited me little up to this point and for that I am extremely fortunate. However, I don't ever feel like I have "control" over my disease. Instead, I feel like I do what I can to "manage" my disease(s) on a daily basis. It takes A LOT of work. It take enormous amounts of patience.

Sometimes, it even takes giving yourself a break. Allowing yourself to not be perfect. Treatments are crucial...compliance is so very important... Yet, sometimes, life gets in the way. My personal definition of compliance involves working hard and doing your best. Everybody's best is different. I think people in the CF community like to compare each other. If this one is running a marathon, while that one walks around the block for exercise, the one who walked is not doing "good enough". My wish is that people would stop making these comparisons. There will always be someone doing more than you. There will always be someone doing less. I think each of us should only judge ourselves when it comes to self-care.

I also think that sometimes a person can do everything by the book, yet still decline. This is the nature of the disease. I think judgement coming from others regarding this decline is harsh. That person who is declining is often judging themselves harshly enough already, even if they have done everything in their power to stave off the progression.

This is going to sound odd to some people, but I honestly feel judged for being "too healthy" within the CF community (no, I'm not complaining about being a too healthy CFer...I'm fortunate!). A lifetime of doctors, hospitals, pills and daily treatments doesn't make me a card-carrying CFer in some people's eyes. Yes, I have been told this directly. My lung function is not low enough to qualify. I haven't needed IV's enough times. I have been told that I shouldn't have feelings regarding my CF, because my road has been easier.

Here's the deal. This is MY experience. This is MY journey. These are MY feelings. I admit that my road has been easier, but I am entitled to my feelings, fears, worries. Just as I am entitled to celebrate successes without being judged. I wish more people were accepting of that fact that EVERYONE has a unique experience...all of them valid. Yet, I know that the judgement will continue to exist. Honestly, it's human nature. There are people who feel better about themselves when they put down others. I consider that the judges problem, not the problem of the judgee.
So, I have been feeling excellent lately! So much so, that I have had very little health-related news to blog about. Yet, the MAC infection persists. I have an appointment in a week in a half, where I will be cultured to see if the MAC has been suppressed at all. If not, we may need to switch things up again. I struggle with the unpredictability of this disease. I think I keep motivated to exercise regularly and be compliant with my treatments to give myself a sense of control. It makes me feel like even if nothing is going to change, at least I'm doing something.

These are my jumbled ramblings about compliance, judgement and progression. If you wish to take the challenge, read below...
1. Write a blog explaining your personal thoughts and experiences in dealing with CF control and progression. This could include your views on whether CF is in fact a "controllable" disease, your personal definition of compliance, your thoughts on whether (or how) someone with CF should be judged in terms of "good enough" self-care (what makes you feel judged? do you think those fears are justified? is judgment ever useful in this context?), your own struggles with control vs. unpredictability, and how you keep motivated in the face of so many questions. Or, you know, whatever you want to write about really. It's your blog.
2. Comment below with a link to your blog so that all of us can read your response. YOU DO NOT NEED TO LINK TO MY BLOG IN YOUR ANSWER. If you'd like to do so, please feel free, but this is about starting a discussion, not publicity.
3. Encourage your own readers to get in on the conversation by posting the same instructions on your blog. Remember, the more responses, the better the conversation. Let's see if we can get this one going as much as with past challenges.
4. If you don't have a personal blog (or just don't feel like going through steps 1-3), feel free to still make yourself heard by simply leaving a comment with your thoughts below.
5. Non-CFers are 100% welcome to participate, either by pulling from their own experiences or simply by offering their perspective as people, friends, and loved one's.

Fun Surgery Pictures

2011-07-06T17:59:00.001-04:00

I have my skin excised today. Besides some excess bleeding, all went very well! It was relatively pain-free. I did find out that what I have is pre-melanoma. This made me feel thrilled that I caught it and took care of it before it progressed. Now I just need to be more diligent about getting screened annually.

So here's how things went...

This is a picture of the eye-shaped cut that he made, prior to removing the actual skin. They remove down to the fat.

This is after they removed the skin. They remove all of the skin down to the fat. I have to wait to hear from pathology to ensure that they got clean margins. If not, I go back for them to take more.

This is after I was all stitched up. It required 8 internal stitches. They used steri-strips on to to avoid external stitches.

In all honestly, it really wasn't bad at all! It was WAY easier than a PICC placement. The best part is that I've got friends who have told me that they are now going to get their skin checked. You just never know with this stuff. I was certain that the spot was just a benign little freckle. Who knew? I hope this concludes all posts about skin issues.

In other news, we have been having a FABULOUS summer. We have had a few out of town visitors, we have been swimming quite often, we have been busy, busy, busy! We are really looking forward to a 4 day little trip to Washington DC with the kiddos. I loved my first trip to DC as a kid! A couple weeks later the hubby and I will be getting away to Boston ALONE for 5 days. I can't wait to eat my way through the city!

I hope all is right in your world...

Abnormal

2011-06-28T20:51:00.000-04:00

Don't you hate when you get a message from a doctor, that you have TONS of questions about, after hours? I do... About an hour ago I listened to my voicemail and had a message from my Derm.   It turns out that my biopsy showed abnormal cells in my skin lesion. They said it's not necessarily skin cancer, but that I will need to schedule a 30 minute surgery to have the area excised and I will need stitches. They will then do a more complete biopsy of the excised flesh.

So after I first listened to the message I thought...no big deal. It's just a little slicing and digging in my arm. It's only going to take 30 minutes. I've had 2 organs removed...this is NOTHING. Then I googled pictures of skin excisions. Icky! It looks like they take a big area and that that go pretty deep. Sometimes they have to put in an inner and outer row of stitches. What a pain in the ass!

So I've decided to shut my computer down for the night after posting to my blog so I don't freak myself out more than necessary. I tend to do that quite often when I start researching my own medical conditions. I refuse to think of this as anything but a harmless little freckle. I'm sure they are just being proactive in removing the area.

I do wish that my abnormal cells would have been found near one of the horrible tattoos that I got the day I turned 18...just because I could...   Better yet, it would be nice if the cells were found on my belly and the excision could double as a nice little tummy tuck. No such luck!

On the lung front, I feel AMAZING! I really feel like this combination of antibiotics, along with the Spireva are working wonders. After my appointment in May, Dr. Dazzle let me go back on a 3 month clinic schedule. I was going monthly for about 6 months, then every two months.   I've progressed to the point where quarterly will do :-)

I'll keep you all posted about this new skin stuff.

Have a fantabulous 4th of July weekend! Please wear your sunscreen!

I'll Take a Poker Face Please!

2011-06-22T20:03:00.001-04:00

I had an interesting Dermatologist appointment the other day. I've never seen a Dermatologist before, but after watching this video I decided it was time. Please, please, please take a few minutes to watch.

Dear 16 Year Old Me

So I very rarely go out without my SPF 100 sunscreen. Living in Ohio helps too, as we rarely get sun. However, I grew up getting burned quite often. As a teenager I sat in the sun with vegetable oil slathered on my skin. It was the thing to do in the late '80's and early 90's. In my early 20's I actually went tanning now and then. It was only after I started working with cancer patients that I started caring about my skin.

This video was enlightening. I never really made the connection that those of us with CF might be more prone to skin cancer. We are immuno-compromised by nature. That's why we pick up so many "bugs" that normal/healthy people don't... Dr. Dazzle said that this is how/why I picked up this nasty mycobacterium. Those who are immuno-compromised are at a greater risk of getting skin cancer too. That mean us guys!   We need to be more careful.

So I had this freckle on the back of my arm that was very dark compared to my alabaster skin. I have lots of freckles, but this didn't look like the rest. I'd love to take a picture and show you, but it no longer belongs to me. It's in a lab somewhere awaiting analysis. The appointment started off with the standard conversation about my my CF...the normal stuff I get when I list my meds and tell any doctor other than my Pulm that I have CF...the "Wow, shouldn't you be dead! or You're REALLY old for having CF! or Are you sure you have CF?"  I am still alive and kicking. I know I'm old. Yes, I'm sure I've got CF. We then moved on to my skin.  She looked over every inch of my skin, starting at my feet. At which point I told her that my real concern was this freckle. I lifted my arm to show her and I kid you not, she said "Oh man, that shouldn't look like that at all! That's way too dark! That's a grade 5 (something or other)". She then had her assistant take a picture of it, the needles came out to numb my arm, a blade sliced the lowly little freckle off and the biopsy was complete. I had no time to prepare. The entire process took about 45 seconds. I was bandaged up and told that my results would be back in about two to three weeks.

I left not really registering what happened. I left wishing the doctor had more of a poker face.  She looks at weird skin all day long. I'm pretty surprised about how much she reacted to my harmless little freckle. I kinda wish she would have said something reassuring, like that we're just going to do a little biopsy as a precaution, but it will likely come back normal.

I REALLY think this is going to be benign. Even if it is cancerous, it would VERY likely be basal cell, which is so easily dealt with. My father has basal cell carcinoma and it's no big deal. He has to get his skin checked and parts cut off here and there, but it's really not so life-threatening. I'm glad I finally went, so I could stop wondering.

So here's my plea for all of my CF friends out there...   PLEASE protect your skin! Not only to prevent the potential of getting cancer, but also because we're living much long and you don't want to have to deal with too many wrinkles in old age!

My Dad is Rad

2011-06-19T19:43:00.000-04:00

I adore my father. He is the nicest, most sincere man I've ever met. My dad is a hard worker, staying at the same job for 35 years. He's a golfer. He is kind. He is simple. He's a worrier. When I travel I still have to call him when my plane lands AND when I arrive at my destination. There is no doubt who I got my "worry" traits from... He adores my children. He adores his only daughter.

This is one of my favorite wedding pictures. My hands were freezing and my father was attempting to warm them up. He was taking care of me.

Happy Father's Day, Dad!!! I love you for now and always!

In Between

2011-06-12T21:50:00.000-04:00

I haven't posted for nearly a month. What could possibly have kept me out of the virtual world for so long? In all honesty, I've just been so busy with life that my computer time has been limited. These past couple of months I have been living in between. I am an official, card-carrying member of the sandwich generation.

I have a very small family in town. My mother was an army child and her family barely lived in the US during her childhood. They lived in Hawaii (before it was a state), China, the Philippians and she met my father as a teen in Nova Scotia. When it was time to return to the US, her parents, brothers and sister all scattered. Somehow we ended up in Ohio, which is where my mother and father have been since. We have no extended family locally. It's just my parents, step-parents and brother.

About a year and a half ago we encouraged my grandmother to move from Philly to Cleveland to be nearby. She agreed and moved here to an assisted living facility with her husband. At that point, my grandma had Stage 4 kidney disease. Over the past few months she has progressed to stage 5 disease. At the same time, my mother, who has dealt with severe back injuries and pain, was preparing for major surgery that would require rods, screws and a long, difficult recovery.

All of this background just to tell you that I have been wearing my caretaker hat over the past couple months. My mother hasn't needed much. I actually wish I could have been around for her more during this time. I made her some homemade soup and took her tons of DVD's, but what she needed most was sleep and rest...so staying away was actually better.

My grandmother, on the other hand, needed my help. I've spent a lot of time this past month taking her to doctors appointments, getting her admitted into the hospital and getting her dialysis started. It was a difficult transition for her, but she's now managing like a rocks star.

My kids have also had a lot of activities. The end of the school year is always full of stuff. Throw in a little bit of work travel and I was left juggling a lot of balls in the air. Trying to keep everything afloat. Just managing most days...

I haven't spent a lot of time thinking about Cystic Fibrosis. It's been nice! I've been feeling great! I've been as compliant as humanly possible. The Ethambutol seems to be having no side effects. I had to have an eye exam prior to taking the first dose. They also had to take pictures of my optic nerves. I guess optic nerve damage is a possible side effect of this drug. I'll go back to the Ophthalmologist every 3 months to ensure that all is going smoothly. So far, so good!

This past couple months have proven that I am officially part of the sandwich generation. I spent a great deal of time taking care of my older and younger family members. I am so thankful that I have been feeling well enough to take on this role. I love being needed. I love being able to help...and I love not being the patient myself!

Mom and grandma are doing great! This is a picture of my grandma, mom and aunt in Hawaii, where they lived for several years in the 1950's. My mother is wearing the striped dress. My grandmothers first name is Avalon. She is the reason I named my daughter Emma Avalon. I am more like my grandmother than any other family member.

I sure do love these women!

Miracles & Such

2011-05-14T19:31:00.001-04:00

As a new graduate with my Master's Degree I was working in the outpatient oncology department of Cleveland's largest hospital. As a large, specialize facility, we would get patients coming to us from all over the world. I remember her like it was yesterday. She was bald, skinny and her face could no longer hide her desperation. She was forty years old, at most. Her body was riddled with cancer. After conventional treatments failed to work, she went on a clinical trial in the US somewhere else in the country. When her cancer failed to respond to that clinical trial, she flew to Germany to try an innovative new treatment offered in that country. That treatment didn't reduce her cancer burden, which led her to Cleveland. She looked worn, exhausted, but absolutely determined to fight until the very end. After speaking with her I found out that she had little children, a husband, she was a hard worker, she had a lot of friends...and she was not willing to go gently into that good night. Yes, I did just throw a Dylan Thomas reference in here...

I was in my early twenties. I was naive. I thought I knew it all. In my head I was thinking... 'Go, be with your family. You've tried so hard...you've done so much. Spend the rest of your days loving and laughing and crying and holding your children close.' Of course, I never said this to the patient. This was her choice. This was her journey. She ended up dying within weeks of our meeting. That day I remember promising myself that I would never spend my last days searching for my medical miracle. If my miracle was not easily accessible, then I would be okay with my fate.

I guess13 more years of living, a husband and children of my own has changed my perspective. On Wednesday of last week I broke this promise to myself. No, these are not my last days...not even close. No, I have not exhausted every option and I am not searching for my medical miracle at this point. Yet, I did travel across the country to see an Infectious Disease expert at the National Institute of Health. Dr. Oh (not his real name) knows more than most physicians about mycobacterium. Dr. Dazzle has been encouraging me to make this trek to Maryland since last August. I finally decided to make the trip and I am so, so thankful that I made this choice. I left with a new treatment plan, a plan if that plan doesn't work, then yet another plan if we're still not seeing progress.

After I made it through being searched, having my belongings searched and having my car searched (this is a HUGE government facility and security was heightened) I made it to my appointment at my scheduled 6:30am time. I had a schedule jam packed with tests and consultations that would take until 5pm to complete. NIH is not a place that people go for every day treatment or health care. You have to go through a screening process and be accepted to be seen at NIH. They typically provide consultation and you almost always have to take part in a clinical trial to be seen. I signed two clinical trial consents while there.

This made for some very interesting people-watching.   Every patient at this facility was likely looking for their medical miracle. Most of them have likely had treatments fail. While waiting for my CT scan I sat in my little paper gown next to a man who was obviously battling cancer and a woman who was both deaf and blind. I felt strangely fortunate...overwhelmingly fortunate. Sure, I have to worry about my health and do things to take care of myself every day that my healthy, real-world friends don't.   Yet, it could be worse...it could always be worse.

I made a new best friend during this appointment. Dr. Cha Ching (not his real name either) is the Infectious Disease Fellow who spent a good two hours chatting with me about my history and present illness prior to my appointment with Dr. Oh. Dr. Cha Ching made understanding more about mycobacterium fun!   When reviewing my CT scan from earlier in the afternoon he made a little game out of locating the areas of "schmootz" hanging around in my lungs. Apparently, he likes saying schmootz better than infiltrates. Dr. Cha Ching gave me even more reason to adore him when he called me before 12 noon the day after the appointment with my AFB (acid fast bacilli) smear results. When the smears came back positive, meaning that my mycobacterium is still being resistant, he told me how sorry he was...and I could hear in his voice that he meant it. I was really, really impressed.

The very last thing on my schedule for the day was a one hour appointment with Dr. Oh. We basically reviewed the entire day and came up with a plan. My PFT's dropped by 14%, but this didn't bum me out. It was different equipment and I had done hypertonic saline about 2 hours earlier, so I'm thinking that may have irritated my airways. I have an appointment at my own clinic in a couple weeks, so then I will be able to really gauge where my lungs stand. My CT scan showed that I have some new nodules/infiltrations in my lungs, compared to my pre-treatment CT, but no cavities. I can deal with a few little nodules. This bacteria eats the lungs and can create huge holes/cavities in the lungs if unchecked. Sometimes, there is no change in PFT's when these cavities are present. The only way to determine the damage caused by this bug is through CT scans.

The plan is that I will be continuing my oral Azithromycin and Doxycycline, while doing the Amikacin every day now...instead of doing a month on and a month off. I am also adding a forth antibiotic called Ethanbutol. This is an old antibiotic used to treat Tuberculosis. If I still can't get clean cultures after a few months on this protocol, we may move on to IV Merepenum. Or there is a clinical trial starting at NIH for people like me, with refractory NTM (non-tuberculosis mycobacterium), so that may be yet another option.

While the lower PFT's and new nodules didn't bum me out, a couple of things were frustrating. The first was Dr. Oh's use of the word "refractory". When he said it my ears perked up and it's been bouncing around in my brain ever since. This word is used in the cancer world that I work in when someone's cancer won't go into or stay in remission. It usually doesn't end well.   The woman that I spoke of at the beginning of this post had refractory disease. I never thought of my NTM as being refractory, but then I realized that I have never had a clean culture since this was first discovered last year, despite gobs of antibiotics. I guess that makes it refractory.

The other thing that I wasn't expecting was hearing that my treatment is nowhere near being complete. I must be on this drug combination for at least a year following my first clean culture, which I haven't had yet. I was really hoping my belly would get a rest. I was hoping that Dr. Oh would say that my NTM wasn't so bad after all. I was hoping that I could take a little break starting in September. Instead, Dr. Oh said that he thinks I need to make my treatments a bit more aggressive, that I need to start them now and continue them longer.   As frustrating as these things were for me, the fact that we had multiple plans in place put me at ease.

I left feeling very encouraged. I left feeling confident that we are moving in the right direction. I left feeling completely smitten with this new medical team, who will be working hand in hand with my current medical team. I left and headed to an adorable little sushi place in Bethesda to meet with Josh and Melissa Sams. Josh is 35 and has CF and Melissa is his wife, who also has a child from a previous marriage who has CF. I can't tell you how much I adore these two amazing human beings! We laughed and had a grand time. It was a fantabulous ending to a reassuring day!

The Diagnosis

2011-05-01T19:11:00.003-04:00

May is Cystic Fibrosis Awareness Month. Several of my blogger friends, like Jen and Tara, have posted the story of their diagnosis or that of their child. I figured I would take this opportunity to share mine.

It was 1978 and I was 2 1/2 years old. I had been in and out of the hospital with pneumonia for more than a year. I would improve, go home, and decline. Each time my pneumonia was treated, but the cause of the repeated infection was never investigated. After being in the hospital more than not that entire second year of my life, my mother refused to take me home until someone found out what was causing my repeated illnesses. The doctors were reluctant to do a sweat test, because I was not a "failure to thrive" baby. I did not show signs of the digestive symptoms that CF often causes. At my mother's insistence they did a sweat test, which came back at a 95. They repeated it several times and got this very high number each and every time. My parents were told that I had Cystic Fibrosis. They knew what this meant. Our neighbor 2 doors down, Kim, was 12 years old and also lived with CF. She actually babysat me many times. My parents knew how complicated life was for Kim and her family with CF as an additional burden. The doctors told my parents that the life expectancy was 12 and that they would have to work hard to keep me alive. This was the knowledge that medical experts had about CF in the 70's.

My parents worked their butts off for me. I did nebulizer treatments and they did manual postural drainage on me every morning and night...never fail. Both of my parents worked full time, but they never skipped my treatments. They always made sure I took my pills and they missed a lot of work ensuring that I got to all of my well appointments and all of those unplanned appointments when I was ill. I know that my parents grieved. They both grieved differently, but they both grieved. When you have a child you have expectations for that child and their future. When a parent is told that the future of their child may be compromised it is a loss. I think that's one reason I worry so much about my future...not for my sake...but for the sake of my beautiful babies.

This story gets to the heart of what I think my parents may have felt:

WELCOME TO HOLLAND
by

Emily Perl Kingsley.

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

While I think my parents got more than they bargained for, they made my life full of wonderful things. They helped feed my determination. Caring for myself was necessary, but not limiting. They made sure I stayed active, putting me in dance and sports. I think that's part of the reason I continue to work out 4-6 times per week. When the going got tough, when they were tired and overworked, when their marriage failed...they did not give up on my health. They worked their asses off for me and taught me how to be an aggressive advocate for my care. I give them, along with medical advances, a lot of credit for getting me to where I am today.

I love you Mom Dad! I'm so sorry that my health made your world more complicated. I am forever thankful for all of your hard work and care over the past 33 years!

My Nemisis

2011-04-27T14:42:00.009-04:00

I had a little set back a couple weeks ago. Nothing too major, but enough to land me on more antibiotics.

As my disease has progressed I have come to despise stairs. All stairs frustrate me, but none more than the stairs that lead from the first floor to the second story of my home. In the olden days, I used to whip up the stairs with no problem. Now, my ability (or inability) to master the stairs has become an indicator how well my lungs are functioning.

Prior to the 4+ months of IV's that I had in 2010 I needed to take a good 2 minute break to catch my breath after navigating the 13 steps to the second floor of my house. If I was on the phone I had to discontinue my conversation and call the person back after I caught my breath enough to speak again. It was as if I had just finished a 100 meter dash. I would avoid unnecessary trips upstairs. My steps got piled high with things that needed to be taken upstairs, but had to wait until I was going up to stay. Once I was up, forget about asking to me to get something from downstairs...it wasn't happening.

A couple weeks ago I was feeling off. I barely got through my work day, then collapsed in bed each night. My belly felt horrible, my body felt horrible, my lungs felt horrible...I plain and simple felt horrible. The last straw was when I didn't have the energy to walk my son upstairs for a time-out after he misbehaved. While my son was enjoying this version of mommy, I was feeling rough.

A call to my doctor resulted in prescriptions for Flagyl and Levaquin. Dr. Dazzle thinks that this was an exacerbation of the H flu that I cultured in February, but we didn't treat. I took two doses of the Flagyl and experienced the worst peripheral neuropathy I've ever had. It was much worse than the neuropathy I got from the Zyvox I was taking a couple months ago. There goes another drug that I can no longer tolerate. The list of options decreases once again. Unfortunately, I am still experiencing neuropathy at night. This condition can become permanent. It's really annoying, so I hope this isn't the case for me.

The Levaquin, however, has done the trick. My lungs feel great! The stairs have become slightly less daunting. I have to admit that I have had THE conversation with my husband recently. The same conversation my parents had several years back. The conversation most people have when nearing 60 years of age. I had the "I think we should look into buying a ranch-style house soon" conversation. I've been dreaming about whisking freely about the house, punishing my children without a second thought, and feeling like less of a prisoner on whatever floor I happen to be on at any given moment. Easy access is my goal...

Breathe

2011-04-21T21:04:00.003-04:00

Four years ago today, Matt Scales, a musician from the UK with CF, died. He wrote an amazing song that was reproduced last year. This time it was sung by 3 amazing singers living with CF themselves. One of the singers, Josh, is a very dear friend of mine.

Click the link below to listen to this beautiful song and read about Matt...

Breathe Song Event

The Story of Us ~ 20 Years in the Making

2011-04-05T10:36:00.038-04:00

The year was 1991. It all started at a table in the back of the cafeteria at a very small private high school. The table in the back corner was where I sat, along with all of the other kids who were "rough around the edges". I remember our lunches together, but only vaguely. We bonded over french fries with ranch dressing. Ketchup was too ordinary for us. We threw caution to the wind and dipped our fries in a shared container of ranch dressing. That was our common bond at that time. I would like to say that it was love at first sight or that we knew we would always be together, but I can't and we didn't. M had steady girlfriends all through high school and I had the same boyfriend throughout. We were just two people who enjoyed our fries slathered in sauce.

Fast forward to 1997. I had just finished undergrad and was preparing to enter grad school at Case Western Reserve to pursue my dream of being a health care Social Worker. I worked hard at my studies, but I knew how to have a good time. Sometimes I was guilty of having too good of a time. I was always surrounded by friends and was the healthiest I had ever been in my life. Life was grand. Sorry, Jen, I couldn't resist posting this oldie but goodie... M was a full time punk musician and a part time college student. His band was busy touring the US and Europe. I've heard the stories and it's pretty obvious that a lot of oats were sewn during this time. It was in 1997 that I caught one of his shows when the band made it back to Cleveland. I have to admit that this wasn't my scene. I was never part of the punk culture...no purple hair and safety pin earrings for me. I did have fun at the show, though. Even though the music wasn't up my alley, I bought the bands latest CD, which M autographed for me after the show. It wasn't until I got home that I read what he wrote.... "French Fries with Ranch Dressing" followed by his stage name. Very clever, indeed.

I'd like to now write about how he had me at "french fries", however, it was not to be. I had a different serious boyfriend at the time, while M also had a long-term girlfriend. We were just two people passing in the night.

The year was 1999 when we would meet again. My best friend since 1st grade worked at the bank where M was a police officer. That's right, folks...my man morphed from a punk rocker to a police officer in a matter of a couple years. This time were were both single so we decided to actually go out on a sorta date kinda thing. It was pretty lame. From there we hung out when it was convenient, but we each were dating others along the way. We would go months without seeing one another, then hang out again on a whim.

What mattered at the time was that we were friends and it our relationship was easy. The love grew day by day. It wasn't until 2001 when I was offered a job in Baltimore at Johns Hopkins and I was preparing to move away that we both finally felt that what we had was right. I am not a big fan of the term "soul mates". Honestly, I think finding the one person for you has more to do with timing and circumstances than anything else. I know, this isn't a very romantic notion, but it works for us. Our timing and our circumstances finally allowed a real relationship to blossom.

M surprised me with a beautiful proposal in October of 2001. It was 9 years ago today, April 6th, 2002, that I wed M and became a bonus mom to then two year old, Sarah. The day was beautiful, maybe even perfect! Marriage is not easy. Marriage with a child is difficult. Marriage with three kids, two full time jobs and Cystic Fibrosis is tremendously challenging. I'm not going to lie to you, my esteemed readers, and tell you that we've had a fairy tale life. I won't tell you that we've always been supportive and appreciative of one another. That sometimes, maybe even often, gets lost in the chaos of this thing we call life. However, I will tell you that we are friends and our love has deep roots. Roots that are 20 years in the making. Lately I feel like M has gotten the short end of the stick when it comes to the in sickness and in health part of the vows that he spoke 9 years ago on this day. Yet, he has stepped up to the plate and kept this family functioning when I wasn't able to do my part. I am forever thankful for him for taking on the challenge known as "Stacey". Many others have tried, but failed miserably.

9 Years later...or 20 years in the making...and this is what we have become. An entire family! We even helped create entire other little human beings that didn't exist before. I am blessed beyond measure!

Happy 9 Years, M! I love you for always!

Distractions

2011-03-31T19:18:00.046-04:00

We just returned from a fabulous vacation to Florida! We spent 9 days away from home, in a different environment with fewer worries. Except for the fact that my son ran a fever on and off for 4 days and my step-daughter had to be seen at an Urgent Care to get antibiotics for a very painful inner ear infection. I guess no matter where we go, how many miles we travel, our worries are never really far away. We made the best of the healthy moments and had a blast!

I was excited to get away. The next couple months are going to be so busy with business travel, my mother's back surgery, my grandmother's new dialysis regimen, and medical appointments galore. This was the last bit of rest and relaxation that I will likely get for a while. I was looking forward to spending time with the family, the sun, the surf and the sand. However, when I walked into our rental unit I became as giddy as a little girl when I noticed this:

A fully stocked book case! I brought my Kindle, which is currently loaded with 57 books...but these were bonus books. More than half were titles I have not yet read. I have noticed over the past couple of years...the years when my health has become more complicated...I have been devouring books. I am a fiction girl. I have no interest in reading about real life experiences. I feel like I get my fix of real life from Facebook and blogs. I want to be swept away in a story. Something completely imagined...a fantasy. I have always been a reader, but never quite to this extent. I find that I have a hunger for books now.

The first book I read on my vacation was called "Home Safe", which was a little story about a mother/daughter relationship and loss. It was good, but it was one of those books that you move on from quickly. No lasting impression.

The next book was not an easy selection. I kept skimming the titles. Maybe I should read something fluffy like The Wedding by Nicholas Sparks. What about something silly, like one of the Shopaholic books? However, my eyes continually focused back on The Kite Runner by Kaled Hosseini. I know, I know...this book came out eons ago...2003 to be exact. I have wanted to read it and wanted to see the movie, but just never got around to it. I knew that the topic was heavy. Did I want to invest in a "heavy" book on my vacation? Of course, I did! I am a glutton for punishment. I have no regrets. The Kite Runner was by far one of the most amazing books I've read. I learned, I felt, I got angry, I cared and I learned some more... This book will stay with me for a long time to come.

Amir, the narrator of the book, was a young Afghan boy who often lost himself in the written word. He read and he wrote, which often helped him cope. In no way does my life parallel that of Amir, but this aspect of the book got me thinking about the role of reading in my life the past few years. Reading helps me fill all the quiet spaces...the times when negative thoughts and worries have the opportunity to creep into my mind. I find that I obsess less about my health when I'm engaged in a good story. When I'm reading a good book in a doctor's office waiting room I find that I obsess less about the germs invading my space. So here lies the correlation...as my health becomes more worrisome I rely more on my distraction...or shall we call it a coping mechanism. So when the going gets tough, you know you can find me somewhere in a corner with my nose deep in a book...er...make that my Kindle.

I decided to add a page to my blog with a list of all of the books I remember having read over the past couple years. I know I'm missing quite a few. This will be a record of all of the hours that I have spent coping...getting lost in a reality that doesn't include Cystic Fibrosis.

On the health front, I am pleased that I have been able to avoid getting any of the illnesses that all three of my kids have had over the past few weeks. I am still on three antibiotics, so maybe that's helping...who knows? I was finally able to schedule my appointment to see Dr. O at the National Institute of Health. I will be heading to Bethesda, Maryland for a couple days in early May for my work-up. Since I re-cultured the mycobacterium, I am really looking forward to hearing if my treatment protocol will change. The coordinator I spoke with today said that they typically treat their patients much more aggressively than other centers. I'm wondering if that may mean more months of IV's are in my future. I am hoping that we can find something to eradicate this beast... I have been having a difficult time following Facebook lately. I have met several people with CF on Facebook who also culture MAC and/or m. abscessus, and the majority of them are doing very poorly right now. I really hope for the best for them. At the same time, it's really hard not to compare and imagine that I may soon in a similar way.

My job over the next month is to not obsess over the possibility of a decline or more IV's. I guess more reading is in my future? Next up "Sarah's Key" followed by "A Thousand Splendid Suns".

Letter to Emma #1

2011-03-20T15:24:00.010-04:00

My Dearest Emma,

You are a beautiful soul. You are the little girl I never thought I'd have...my miracle baby. The first time I looked down at your wrinkly, red little face, I was madly in love. I used to dress you up like a baby doll. I was so proud of you and overjoyed that you were mine!

You have ALWAYS been a Daddy's Girl. I can't say that I always found this to be fair. After all, I'm the one who carried you in my body for nearly 10 months. I gained weight, endured pain and have permanent stretch marks as a reminder. I worried about you from the moment I found out that you existed. Your father, on the other hand, gained a few pounds of sympathy weight...but never endured quite the same experience. Yet, you always preferred Daddy. I've taken enough Psychology courses to know that this is common in little girls. So I never really worried that it meant anything significant...until recently.

Early last week you woke with a start very soon after falling asleep. You ran into me and Daddy's room sobbing, saying that you had a bad dream that Daddy died. You were inconsolable. We tried to find out more details, but you just cried and cried...hanging onto Daddy for dear life as I stroked your hair. You slept with us that night. Afraid that if you left something horrible would happen.

As I thought more about that incident, I tried to make sense of your tears. This was much more than the run of the mill reaction to a bad dream. Why were you afraid that Daddy would die? Might you have been afraid that I was going to die, but unable to express this fear? Might you have already come to terms with the fact that I may die soon, so you were fearful that if Daddy died too you would be left without a parent. Maybe I'm making more out of the situation than need be. I just want you to know that my heart hurt for you that night, if only because I hate the possibility that my disease makes these thoughts even enter your world. I truly hate that my disease complicates your otherwise normal childhood.

As you have grown it has become apparent how very similar you and I are to one another. Not only do we hear how much we look alike all the time, but I think you have picked up many of my traits...both good and bad. You're a smart girl...always preferring books over dolls, you know what you want and how to get it, you can be stubborn, you truly care even if you have difficulty showing it at times and you struggle with anxiety. Sometimes we butt heads. Even as I'm writing this you became upset with me when I told you that you couldn't put a K$sha temporary tattoo on your forehead. I don't care how temporary it is, the answer is still no.

As you've gotten older I can feel that you sometimes keep me at arms length. I often wonder if this is self-preservation. Might you be afraid to get closer, since the reality of losing me is there? When I was sick a lot, sometimes you even seemed a little angry with me. I want you to know that I understand. I honestly think I would do the same thing. I am a master at putting up walls to protect myself from devastation. I get it and it's OK. However, no matter how high or thick that wall gets, I will never stop saying and showing you that I love you to the end of the Earth and back again.

I know that you know that this song reminds me of you. I know that you roll your eyes every time I say that when it's playing on my ipod. It's true, though. Emma, I want you to listen to these words and please, ALWAYS know that I love you more than you may ever realize during my lifetime. You're my little M&M after all...

I love you and I love you and I love you!

Mommy

(Sorry I couldn't get the actual video, as it wouldn't allow me to embed...don't click on the screen if it doesn't play...like it says)

CF Denial Awareness

2011-03-14T13:52:00.014-04:00

I got my new "CF Denial Awareness" shirt in the mail today! Look...

I told my husband that I ordered the shirt as I was giggling about the message, which I find amusing. He just looked at me, dumbfounded, and said "I don't get it". Please tell me you get it.

CF denial is sometimes a wonderful thing. Not the type that leads to apathy and non-compliance. Instead, the kind where you unconsciously go through your daily CF routine, while not thinking or obsessing about the harsh realities of this beast we call Cystic Fibrosis. The kind where you're feeling sorta good for a day or two and you 'almost' forget. The kind where you're so busy or preoccupied with life that CF doesn't make it into your thoughts for minutes...sometimes hours. The kind where you sometimes feel like everyone else...all the other 'normal' people. This is my kind of CF denial and let me tell you...it is one comfortable place to be. So this is my effort to raise CF Denial Awareness!

This shirt was designed by Kelly Langs, a graphic designer with CF, for the late Paul Mooney aka "Q". I never got to know Q...he died just before I delved into the world of cyber-CF. From what I've heard, he was a funny guy, who wrote and sang music about CF in a very Bob Dillonesque fashion. I have all of his songs, with my favorite being "Cystic Dreams". Listening is a good time. These shirts were being sold for Q to help pay for his transplant. Following his death, Q's parents started an organization call The Cystic Dreams Fund and all shirt proceeds now go to this fund.

If you want a shirt like mine, you can order from here.

Ciao!

No Longer Who I Used To Be

2011-03-05T22:40:00.022-05:00

I work full time. My office is in New York and I live in Ohio, so I telecommute. It works for me, because most days I get the kids off to school head to my basement and do my thing for 8 hours. I'm able to do my nebs at my desk in front of my computer. It's really the ideal situation for me right now.

However, I manage/consult with 22 Chapters of my organization...18 in the US and 4 in Canada. That means I must travel. A few years ago I was traveling A LOT. I was reaping the frequent flyer rewards...being upgraded to first class from time to time...getting to pre-board and walk on the "blue carpet" that is reserved for "elite" members only. At the security checkpoint I knew just how far I had to strip down, what had to go in a zip lock bag and how to smile just right at the TSA agent to avoid a pat down. I used to zip through the airport in my suit and heels, checking my Blackberry, walking with purpose...because I had places to get to and people to see. I'd catch my flight, get to my destination, catch a cab and meet with colleagues for many more hours. I'd then meet up with any friends I have in that town/state/province, head back to my hotel late in the evening, get a HORRIBLE nights sleep in a bed that is not my own, then get up and do it all over again the next day. I'd fly home, being sure to buy some little trinket for each of my kids from a random airport gift shop. This always helped with my own guilt for leaving them for a few days. Then I would get up the next day and work a full day...never missing a beat.

I'm pretty certain those days are now over...

I set out for my first trip in many months last week with letters from my hospital explaining my medical devices and wearing sensible shoes. I knew before even leaving for this trip that the heels were going to be a no-go. I got through security with no problems and my flights to Oklahoma City were fine. All was on time and smooth. Besides the fact that my lungs felt like they were shriveling like raisins while on the planes, all was great. I was thinking...this isn't going to be hard after all.

I got a good nights sleep with a little help from my friend, Ambien. My work day went well. I even got to meet with a fellow CFer, Darby, that evening. We compared coping mechanism and micro organisms, listened to the music of a fellow CFer who lost his battle and laughed a bunch...all while abiding by the three foot rule. Good Times!

I had another OK night of assisted sleep, woke bright and early (4am) and made it to the airport by 5am for my flight. We all boarded the plane and pushed off from the gate. All was right with the world. It was going to be another smooth day of travel. NOT! It turns out Houston was not allowing planes in due to fog. Back to the gate we went to wait for our clearance. I bought one of those gynormous bags of trail mix and read for a good while, until it was time to board once again. Three hours later...off we went.

We arrive in Houston and have to exit the plane outside. It's 175 degrees outside, and being a born and raised Ohioan, I'm draped head to toe in North Face gear. I start to sweat. My skin starts to burn from the salt being left behind. I rub my eye and now I'm done for...salt in the eye is the worst!

I get into the airport and find a screen to get information about my next flight. Turns out it takes off in 15 minutes. I spot a cart with one of those holier than thou drivers who gets to decide who gets a prized seat on the vehicle. I contemplate asking...maybe even begging...for a ride. I even thought about pulling out the CF card for this one. Then I decided it wasn't worth the questions or the ridicule. I think I would have cried at that moment if the driver had said, "But you don't look sick".

So I run, only to find out after a few minutes of running that this is going to be a marathon. This is where I thank the Lord that I decided to wear sensible shoes. After more than a mile of running with 2 bags wearing some of the most insulated clothing there is, I finally make it to my gate a few minutes too late. I rebook on to the next flight to Cleveland and set out to find something other than the sunflower seeds and raisins that I have been munching on all morning. This, of course, means more walking...more lugging of bags...more sweating... The people...there were just so many of them. A lot of them were twice my age! They were all wearing their suits, checking their Blackberry's and walking with purpose. I was just sitting there...defeated...coming to terms with the fact that I was no longer part of that club. My body has been beaten up by this damn disease.

I finally drag myself to my new gate and board my new plane. When I got to my seat I pretended to struggle with getting my bag in the overhead bin, until a kind business man lifted it for me. When I was a "real" traveling business woman, I would have NEVER let anyone help me with my bag. That would have been a sign of weakness. This time I just didn't care.

I make my way to my window seat and put my headphones on to attempt to avoid any and all interaction with other human beings. The guy next to me doesn't get the hint. He bumps my arm and signals for me to remove my headphones. I humor him, only to hear him tell me how very glad he is that he's sitting next to me on this flight. He continues to tell me how his last flight was hell, because he was sitting next to a guy with the most disgusting cough. He was certain that the guy had Tuberculosis. It took every ounce of restraint I had to not tell him to F$#& Off. I should have told him that I have something similar to TB...I should have coughed on him. Instead, I just put on my headphones and pretended he didn't exist.

I got home in one piece. Even though every muscle, bone and join ached. I also brought a nice little sinus infection home with me. While I used this opportunity to bitch about how crappy business travel can sometimes be, I am happy to still feel well enough to work. I have just come the the realization that I am no spring chicken anymore, especially in CF years!

Getting My Grove Back

2011-02-23T15:23:00.011-05:00

Just a wee little update...

So, the news from last week knocked the wind out of my sails for a couple of days, but I picked myself back up and got my groove back. I can't live my life getting distraught about what a test result says...especially when I'm feeling so fine. AND, I must say, that I am feeling so, so fine!

After going off of the Zyvox I noticed a huge difference in my energy level. I actually have some energy now. Having been sick for so long, then being on a gajillion medications, I hadn't felt my typical manic levels of energy in eons. It's back and I am cherishing every little ounce of it. I know it may be fleeting...so I am making every effort to take full advantage.

I have to say, I feel a little guilty posting about feeling well for a change. So many of my fellow CF warriors are struggling right now. I have a friend in New Zealand who is picking up the pieces of her life after a devastating earthquake earlier this week. I have lots of friends right now who's lungs are bleeding, belly's are unbearably nauseous or are just recovering from hospital stays. All of this suffering, while I feel, dare I say it...healthy. There's a little bit of that survivors guilt creeping in again :-/

In other news, I started the Spireva phase 3 clinical trial yesterday. I did the phase 2 a little over a year ago, but didn't see much change in my health since I was in the midst of fighting off all of these bugs that I didn't even know I had yet. Yesterday I was at clinic for 6 hours. We did a pre-dosing PFT, then 4 post-dose PFT's. Once every hour for four hours. Here's the cool thing...my pulmonary function increased by 5% from my first PFT to the last!

Spireva is currently FDA approved for patients with COPD. Some CF patients currently take it off label. This study is intended to allow it to be used more liberally with CF patients. So far so good! I've had no side effect and my lungs feel fabulous! The plan is that I will be on the drug in the trial for 12 weeks, then I will go on it off label for up to 9 months longer. After that point, if I want to stay on it I will have to be prescribed it as part of my treatment plan at my expense.

We'll see where this takes us...

I want to thank all of my followers for your kind words and messages last week. Your support means more than you could ever imagine. I am convinced I have the best readers in the universe!

Guess I'm Not One of "Those" People

2011-02-14T19:15:00.009-05:00

When Dr. Dazzle and I first began our discussions about eradicating the mycobacterium abscessus, he told me of these people who have been diagnosed with this nasty bacteria...who received treatment...and who never cultured it again. They were able to basically render the abscessus dormant. I knew that this particular bug is difficult to treat. I knew that this bug can cause irreparable lung damage. I knew that the treatment itself was very risky. I decided to take the risk, because I KNEW that I was going to be one of "those" people that Dr. Dazzle told me about in our discussions. One of "those" people who would have nothing but positive results.

Today I found out that I am, in fact, not one of "those" people. The mycobacterium is back. I was able to suppress it the entire 4 months of my initial IV therapy. I finished my IV's in December. It waited less than 2 months to return. It looks like this is going to be one of those things I am just going to wrestle with for the rest of my life. Dr. Dazzle and I had a chat about it. He said that while we were hoping that it would not return, we knew it would be a possibility. For now, we are not going to change my treatment plan. We are now going to focus on treating based on symptoms. My PFT's are very stable, I'm not having fevers and I'm not having an increased cough. For now, we will just go with the flow.
After the phone call with Dr. Dazzle I joined my family in the kitchen to help my husband with dinner. I told him the treatment plan, saying that I will now be on Azithromycin and nebulized Amikacin for life. My daughter overheard this conversation and said, "You mean you'll even be on these medications when you're 96 years old?". *SILENCE* When I finally caught my breath sufficiently to respond I said, "Oh sweet pea, yes, hopefully I'll still be doing these medications when I'm 96 years old". Moments like these are the worst part of this damn disease...

So I'm not going to lie...I'm bummed. I feel like I put a lot of blood, sweat and tears into this treatment plan for the past 6 months, and we're right back where we started. Dr. Dazzle assures me that just because it's present, doesn't mean we didn't suppress it. This must be what it feels like to relapse from cancer. I not a big fan of this type of disappointment.

All that being said, I feel pretty good physically. That's a HUGE plus! I also have a lot to distract me from thinking too much about this crappy news. I think I'll go get myself a mani/pedi sometime this week...then all will be right with the world :-)

Clinic Update # Gajillion

2011-02-10T16:20:00.019-05:00

I had clinic again today. No surprises meant I left a happy girl. My PFT's were stable and now that I'm on Doxy I feel pretty good. Just a little fatigued here and there, along with intermittent belly pain, but that's to be expected when on 4 antibiotics. The only thing left is waiting the few days for the culture results to come back...

One thing that I have been experiencing lately is peripheral neuropathy. This can be a side effect of long term Zyvox use. Basically, neuropathy is nerve damage that can be permanent, that is sometimes caused by medication toxicity. This is manifesting itself in me by causing intense tingling in my hands. It feels like they're asleep, but no amount of moving them around takes the sensation away. Each episode that I've had has lasted from a couple minutes to an hour, and it most often occurs in me at night. Honestly, at this point it's just annoying. Not painful at all. However, neuropathy can get very severe. It can cause debilitating pain or lead to the loss of all feeling in the hands or feet. I was supposed to have 7 months left on the Zyvox. It is a very important component in abscessus treatment. However, Dr. Dazzle wants me to discontinue the drug immediately. He thinks the danger of having to deal with permanent nerve damage outweighs the benefits that I am getting from the drug right now. Some studies in South Korea show that abscessus can be treated sucessfully with Doxycycine, Amikacin and Azithromycin, so this will be my new plan. I tolerate the Doxy well, even though it makes it torturous to be in the sun. Guess I'll have to try to be a hermit again this summer :-/

I also signed my consents for a new study I will be starting on Monday. What's adding one more drug to the mix after all? I will be doing a double-blind Spireva study. I did a Spireva study a little over a year ago, but it was just testing the reactions of CF patients to the medication. This time they are looking for evidence that the drug can improve lung function. I have 2/3 chance of getting the drug versus the placebo. This is probably the 40th study I have participated in since childhood...no joke... I am the study queen!

So all in all it was a great appointment! Now, I'm just keeping my tingly fingers crossed that my cultures show that I have no new lung inhabitants...

Is it all about the attitude?

2011-02-03T12:58:00.008-05:00

I struggle with the notion that the only way to get through a devastating illness is through "Positive Attitude". I hear it all of the time from people, both those with illness and those who have never been sick a day in their lives. I have come to the conclusion that people often say this to protect themselves from having to deal with my emotions. Once I hear that "I've got to be positive" from someone, I will never be honest about my feelings with this person. They would see it as a failure. This person truly thinks that having emotions such as fear, anger and sadness are weaknesses. I have talked to so many cancer patients who have felt guilt for having feelings other than happiness/thankfulness and pressure to maintain a facade of positivity.

I sometimes feel this struggle when in public. Why share my burdens with others? However, within myself I have come to the conclusion that all of my feelings about my journey are OK. I am doing the best I can each and every day. I get sad...I get pissed off...I worry more than I should...I feel deep joy...I am appreciative... I feel such a range of emotions and all of them are acceptable. Maybe not acceptable to the eternal optimist, but acceptable to me.

I do, however, feel that being happy and enjoying life important. I think I am a generally positive person, if only because it's more fun to live life this way. Yet, I make room for other feelings too. I also try to never tell anyone else how they should feel.

The link below expresses how I feel about this topic... please take the time to read!

Positive Thinking vs. Realistic Thinking

Hope

2011-01-31T10:10:00.011-05:00

I have been a horrible blogger lately. I have been a very busy bee since I started back at work about a month ago. I'm back in the groove of raising my kids, working full time and trying to manage my CF. It is DIFFICULT...but I love it!

My health is OK. January was supposed to be an off month on my nebulized Amikacin. About 2 weeks ago I asked if I could go back on. My lungs were tight and I just felt like I needed the drug sooner rather than later. I went back on and felt relief for about a week. However, the sore and heavy lung feeling is back. I contacted Dr. Dazzle and he is putting me on Doxycycline, along with Ibuprofen for the inflammation. I guess the likely culprit for these lung issues is my Staph.

So the CF world was rocked with several deaths this month. First, Geneva...then Tom...then Tina... I am so sad for the suffering that they each endured, I am so sad for the families left behind, I am so sad that the lungs they got gave out or the lungs they needed never arrived...it's just all so sad. These beautiful souls were here one minute and gone the next. Geneva was vacationing and looked so alive in October. Tom was rooting on his Packers a couple Sunday's ago. Tina was grieving the loss of Tom just hours before word came of her own death. They were so alive...and then they just weren't.

Witnessing these lives taken form the disease we share certainly makes it difficult to live in the comfortable denial that I've become accustomed to. Early death is the part of CF that I try so hard to compartmentalize. I try to pack it away in a little box and put it in the far reaches of my brain. That is, until it smacks me in the face again. The reality is that 50% of people with CF will die before the age of 37. This is such progress, but it's not quite enough for the 35 year old writing this post.

I have always set goals for myself that were just beyond my life expectancy at the time. When I reached to goal I really felt like I was beating the odds. When I was a teen I said that I would be happy to live to get my diploma. When it got to that point, I wanted more life. Then I wanted to get married and have kids. This was a great accomplishment for me! I was so happy to have created a family...but then I wanted more. Now my goal is to see my kids graduate from high school. At least that's what I tell myself... While in reality I have always wanted so much more.

Maybe this sounds selfish. I probably sound like I'm whining. I have so much and have beaten this disease in so many ways already...but I want more. There's too much living left to do. Geneva, Tom and Tina had too much living left to do.

This is what I really want...

Maybe growing old is overrated? I still want it. Lord knows...we don't always get what we want. I guess there is always HOPE...

Collateral Damage

2011-01-18T16:42:00.008-05:00

I got a message from someone the other day that belonged to a mycobacterium forum that I visited. She had m. abscessus, but not CF. She was treated for the m. abscessus with IV Amikacin just like me. However, she wanted to warn me to be very careful with this treatment protocol, because the Amikacin ended up making her completely deaf.

Thankfully, Dr. Dazzle warned me about the grizzly side effects of each and every one of the drugs I've taken and am still taking. We got a baseline hearing and vision tests prior to beginning the Amikacin and I got a follow-up hearing test in December after having experienced occasional ringing and ear discomfort. I got the results from the December test last week. The report reads that "The only abnormality seen was a response at a severe hearing loss level at 12,000 Hz only in the right ear. " This is in the extremely high frequency range. I'm not surprised about this, because all of the ringing and discomfort was in my right ear. Honestly, though, I feel like I got off easy. I barely notice this change to my hearing. The Amikacin stays in the ears for 6 months after treatment is concluded, so I'm not completely out of the woods, yet. However, I have been experiencing far less ringing and discomfort since my treatment ended, so I am very optimistic.

This report and the message from the other patient got me thinking. For me, I would trade "some" of my hearing to keep my lung function. I guess in weighing the two, I conclude that you can live with bad hearing, but bad lungs can kill you. Sometimes it seems like CF is a real-life game of "Would You Rather".

"Would you rather go deaf and save your lungs or keep your hearing and experience lung deterioration?"

My family plays "Would You Rather" all the time, usually at the dinner table. The questions are usually silly and the answers don't have real life implications. "Would you rather eat earwax from a cat or lick between your fathers toes?" I hope to never truly be faced with this dilemma.

However, with all of the side effects and other possible things that can happen as a result of the treatments we need to save our lives, this "Would You Rather" scenario comes up a lot in the daily lives of those with CF. To save our lungs we sometimes end up hurting our kidney's or our liver. Sometimes our eyesight deteriorates and even our teeth can decay. In the case of transplant, sometimes it's a choice between diseases. It really is like treating one disease for another in some respects. The health concerns are different, but they don't diminish after transplant.

Making these "Would You Rather" decisions can be difficult. I think that some of us might even have chosen a different path in hindsight if a very negative consequence is experienced. For me, as far as the hearing was concerned, it was a no brainer. I felt comfortable that things were being monitored and that my lungs needed to be the priority at the time.

I would love to hear of any "Would You Rather" decisions you have had to make...

The Calm After the Storm...

2011-01-12T13:38:00.017-05:00

"Worry does not empty tomorrow of its sorrow; it empties today of its strength." Corrie Ten Boom

I've been struggling a bit the past couple of days. Not physically...only emotionally. Worry and anxiety have crept in and they are making themselves cozy. I first noticed the itch of anxiety a few days ago when I looked at my fingers. I don't bite my nails, but I chew the hell out of my cuticles. My fingernails look lovely, but they are attached to bloody stumps. I have chewed my fingers raw. I ONLY do this when I'm anxious, but I don't have time to focus on the problem and improve my thought process.

Post-Traumatic Stress Disorder...it's not just for soldiers. I'm not saying that I have PTSD in its true form...it is a very serious condition. However, I think there is something to be said about the stress and anxiety that can arise after the storm settles. I used to see this all the time when working one-on-one with oncology patients. A while after treatment has concluded and the craziness settles, the patient is left feeling abandoned and alone with their thoughts. The questions start... "Did we do enough?" "Does that ache mean I've relapsed?" "What are the long-term consequences of the the treatment I received?" "What if this comes back?" "What's next?".

Then there is the searching... Google is not my friend. I have been off of IV's for a month now. These types of questions have started filling all the little nooks and crannies of my brain. Whenever I have a question I google it. I have been looking up my symptoms, comparing my case to the cases of others, I have been searching out statistics...basically, I just want answers. I want answers where answers don't exist. Mostly what I find is more cause for concern.

Last night I stumbled across the website for an organization that's sole focus is conducting research and raising awareness for non-tuberculosis mycobacterium (NTM). I was impressed, since I now possess two strains of NTM. The very first thing I read on the home page of the site was that "NTM is a devastating chronic illness". I don't know why this statement has been suck in my head since I read it. I think I was under the impression that the m. abscessus and MAC were symptoms of my CF...just a little bump in my CF road. That statement made me feel like this is an entirely different deadly condition. I already have a "devastating chronic illness" with my CF. All of a sudden I feel like I have double the burden.

I think I was blindsided last night by the seriousness of what I went through over the last 6 months of 2010. I spent a lot of time and energy trying to convince myself and others that this was no big deal. Last night, when I found an entire organization devoted to the bacteria that has infected my body it was a reality check that this is serious business. The founder of this organization has been living with NTM for 15 years and this is seen as a huge success. Does that mean most people succumb more quickly? I am thrilled that this organization exists, yet it makes it all the more real for me. Deep down I knew that mycobacterium was a big deal...I guess my searching just provided absolute confirmation.

So, what am I doing about it? I am scheduling a consult with the CF/mycobacterium guru at the National Institute of Health (NIH) in Bethesda, Maryland. He is an infectious disease physician who is one of the leading minds in the impact of mycobacterium in CF patients. This was Dr. Dazzle's suggestion. I have so many questions to ask...mostly about my future...mostly questions that have no answers... Maybe if I ask enough people, eventually someone will give me the answers that I want to hear. I see scheduling this consult as being proactive. If these bugs come back and become resistant, I want to know the plan. It gives me a sense that I have some control over this/these diseases. My records are being sent to NIH, there will be a meeting about my case, then they will call and tell me when to head to Maryland to be seen in person.

I know without a shadow of a doubt that I need to keep things in perceptive. Corrie Ten Boom survived the Holocaust. Comparatively, I am blessed beyond measure. I know this and I live this...but constant reminders don't hurt. I have been enjoying my "health", my family, my job. Life is getting back to normal...whatever normal means for a CFer. Life is truly grand! If I could only eliminate this worry for good...if I could stop waiting for the other shoe to drop...then things would be golden...

Lung Exterminator Wanted!

2011-01-05T17:55:00.005-05:00

I have learned something very important about my lungs. I think they are very open and accepting. Although I really wish they would be a little bit more discriminating. They must have been paying attention all of those years I sat in Social Work school...learning to love and accept everyone.

Another day, another lung bug. Honestly, I have no idea how there is enough room in there for all of these germs. I now culture 5...that's FIVE...types of bacteria in my airbags. This is what I imagine is going on inside.

So let's review...

1) Staphylococcus - I have cultured staff my entire life.

2) Mycobacterium Abscessus - This is a very aggressive infection that led to the treatment protocol that I am still on.

3) Heamophilus Influenza - I cultured this in October, while I was on IV's. The IV's helped and I didn't need additional treatment.

4) Mycobacterium Avium Complex (MAC) - This was a new discovery that was shared with me in December. No additional treatment beyond that I'm getting for the m. abscessus is necessary at this point. This one derives from birds. Go figure...I don't even like birds.

5) Enteric Bacilli - This is my latest friend. Here's the scoop...

Enteric Bacilli is a gram negative rod that can grow in the lungs of people with CF and those who are neutropenic (low white blood count). BINGO...I have both. I am certain that the neutropenia played a role in me acquiring this new bug. I looked for answers about this Enteric Bacilli on google and freaked myself out. There was a whole bunch of talk about necrosis of the lungs. I also think it is related to E. Coli, which I know a friend of mine cultures in her lungs... Holy crap...literally! Dr. Dazzle always warns me to stay away from Dr. Google, as he/she tends to make people panic.

The good news is that Dr. Dazzle said I have as much of a chance of dying in a car accident on my way to work, than getting lung necrosis from Enteric Bacilli. Dr. Dazzle knows that I work from home, so that was very reassuring. He said that I got it from my nebulizer. I guess this germ just comes and goes as it pleases without treatment. Finally, a germ I don't have to worry about killing!

I am one of the 5% of adults with CF who has never, ever cultured pseudomonas. I would really like to keep it that way. I don't think I have taken all of the precautions necessary when cleaning/sterilizing my neb cups. I rinse them thoroughly and put them in the dishwasher, but I hear of people boiling them. I have never done such a thing. Since I have never cultured pseudomonas, I haven't needed to use my nebulizer all that much in my lifetime.

So, I'm looking for some advice. How do you sterilize your neb cups? Do you ever use them twice in the same day if you wash them out, or do you completely sterilize after every use? Inquiring minds want to know...

Lessons Learned ~ A Look Back at 2010

2010-12-23T11:18:00.024-05:00

Wow, 2010 has been quite a year. I've been reflecting a lot the past few days, as this year comes to a close. This was a year full of learning new things for me...new things about my body, new things about my disease, new things about the meaning of friendship, new things about faith and new things about love.

I have learned what my body needs to thrive. At the beginning of the year I was working out 5-7 times a week without fail. I pushed myself to the limit. I worked out when I was tired and sore. I worked out when I was sick. This was great for my muscles, but wreaked havoc on my lungs. My PFT's were the lowest they have been in my life when I was working out the most. When I started my latest treatment protocol in September I made a deal with myself. I needed to cut myself some slack. I needed to not push myself harder than my body was telling me it could handle. Sometimes, I just needed to rest and heal. Throughout my treatments there were 2 weeks that I never got to my workout class. There were several weeks that I only went once. I never pushed it and if I was tired I quickly made the decision to skip. I think I made the right choice for my body. My PFT's increased by 11% over the past 4 months, to a number I haven't seen in more than 5 years! I am back to trying to work out 4-5 times a week, all while making sure to listen to my body first and foremost. I think my body and I are starting to speak the same language. I will no longer push my lungs to the point of exhaustion.

This may be good or it may be bad, but I have learned a tremendous amount about Cystic Fibrosis this year. I learned tons about mycobacterium of all sorts from discussions with my health care team, talking with others who have it and doing my own research. So much so that I have burned myself out. When I cultured H flu in November I read a couple paragraphs about it and left it alone. After my appointment a couple weeks ago I found out that I cultured MAC, a new yeast and another bacteria that I never even got the name of. I was told not to worry about any of these latest bugs. It's not really my nature not to worry, but I am happily taking that route this time. I have reached information overload. I am feeling very well, my PFT's are up and I am in the maintenance phase of my treatment for the next 9 months. I have made a conscious effort to live more and worry less about what is growing in my lungs. Maybe that will be my New Year's resolution. "Live more...worry less"! Not an easy task for a neurotic like me. However, as long as I'm being closely monitored and I trust my health care team, I don't see why I have to focus so much on some stupid little bacteria. I'll let you know how that goes!

I learned so, so much about friendship this year. I received an amazing amount of support from many friends that I have never met...those who have walked this path before me. People who knew what it was like to live in this skin. I'm not certain what I would have done without the support and love of these kindred spirits...you all know who you are!

I think the biggest lessons learned came from my real life relationships. I feel like I became intimate very quickly with the idea that difficult times allow you to learn who your real friends are. I can freely admit now that I needed help. I was really, really hurting, overwhelmed and scared. I went from being someone thriving with CF, to someone desperately trying to understand where it all went wrong. I tried to put on a happy face, for everyone else's sake...mostly for my kids sake. I had a few very dear friends who saw through this facade and rescued my spirit...whether with soup (Cathy), weekly cards/gifts/phone calls (Lynn), with opportunities to vent and a HUGE hug at just the right time (Elizabeth) or with frequent emails from a neighbor asking what I needed (Alan)...among other beautiful friends... I will never, ever forget their kindness. I have learned that sometimes a small gesture when someone is hurting goes a very long way. I am going to work really hard to try to model myself after these truly remarkable women (and one guy) who made my world a little less scary.

Unfortunately, I also learned that when you are really sick several people that you think would be able or willing to lend a helping hand completely disappear. People I would have considered friends for several years pretend they have no idea what's been going on with my health. I know, I know...people have busy lives. Yet, sitting on this side of the fence I can assure you that the absence of a phone call, email or note felt like a slap in the face. At the same time, although hurtful, I am glad that I had these experiences. I would much rather surround myself with genuine people who can see beyond themselves. Especially, since I am trying hard to practice this myself. It turned out that this year was a great exercise in plucking out the true friends from the bunch.

I am a firm believer in "to each his/her own" when it comes to religion, faith, spirituality. You can believe, not believe or believe in a Higher Power that is different than my God...I'll still like you :-) I don't think everyone has to believe what I believe. I was raised strictly Lutheran and was sent to small Lutheran schools up until college. I believed, I'm just not sure in what... This year I found my church home. I found a soft place to land. I am learning a lot about faith and community by being a part of Heartland. For this I am thankful!

Love. That is how I would describe this holiday season. I felt it more deeply this year than any other. Love for my children, my husband, my family, my dear friends... I found myself so overwhelmed with peaceful and loving feelings over the holidays that I just wanted to freeze time. My friend Jenny was in the hospital just before Christmas and she wrote of

her gratitude for life and love in her beautiful post... I Came Here to Live . She hit the nail on the head with this one. Yes, this is how I feel! It's a must read!

My family went on our yearly trip to our cabin in Hocking Hills just before Christmas. With 3 kids, sometimes too much togetherness can be overwhelming. This year it was just right.

Everything about the holidays seemed just right for us this year. I have never felt more love and I have never felt more loved.

Here's hoping I don't need to learn nearly as much in 2011!!!
Have a safe, healthy and happy New Year!

A Final Gift for Jill

2010-12-19T21:18:00.008-05:00

I was very surprised to receive a Facebook email from my friend, Jill Svancara, a couple days ago. Jill died of CF in July during one of my hospital stays. Having seen and communicated with her one day and hearing of her death the next shook me to the core. Read about my thoughts regarding Jill's struggle and death in my post, Breathe Easy, Jill...

It turns out that Jill's husband wanted to communicate with me. He sent me the email in Jill's name. His message was very heartfelt and touching. He has been following my blog and asked if I could share an important message from him. I know that Jill would be proud knowing that even after her death, Clint is still working hard to raise awareness about the importance of organ donation. Clint's message is below:

A Christmas Gift for Jill

This Holiday season brings a change for my family. This will be our first Christmas without Jill.
Jill was a daughter, a Mother, and my wife.

In July- 2010 Jill died while on the waiting list for a double lung transplant, she was forty two. You see, Jill was born with Cystic Fibrosis, a genetic disorder that particularly affects the lungs and digestive system. Cystic Fibrosis is a disease that affects about 30,000 people in the United States. Years of lung damage had brought her to the point where she could no longer function without a new set of lungs.

Each day about 18 people die while waiting for the gift of life, unfortunately Jill was one of those people.

As many of us celebrate the birth of Christ, we know that his birth marked the beginning of the Christian religion. In his death, he gave us life. Shouldn't we follow this example from the man we called Teacher. In the unfortunate event of our life ending, we can and should give life back.
In Ohio about 50 percent of license drivers are registered to be organ donors. It only takes a moment to go on line http://www.donatelifeohio.org/ and register, or say yes when you re-new your drivers license. Most States have Organ Donation Registration on line, or you can visit http://www.organdonor.gov/ for more information. For parents, I encourage you to explain to your family the importance of this gift, and what it means to so many.

Please, I ask that you find it in your heart to give Jill this Christmas gift that will cost you nothing, but is priceless - become a registered organ donor. We cannot live for another person, but we sure can give life to others- but we must make that choice. While Jill faded, she gave the only gift she had left to give, and that was HOPE. Please accept her gift of HOPE, and return the gift, by becoming a registered organ donor.

Please e-mail my message to your trusted family, friends, and work associates – Lets use the power of the internet, or read it at your church, or maybe post it on a board somewhere. My hope is this Christmas we can get as many people as possible to become registered organ donors......that is the only gift I can now give to Jill – Hope for others. Will you help me?

Thank You, from the bottom of my heart!
Clint Svancara
Bay Village, Ohio

Top Ten Reasons I'm Thankful for Dr. Dazzle

2010-12-16T20:25:00.008-05:00

It's Thursday again. That means it's time for Thankful Thursday! Today I am Thankful for Dr. Dazzle. Here are the top ten reasons why:

10) We graduated from high school the same year. Yep, both from the class of '93. It took a while to get used to the fact that my doctor is a "young" as I am, because I am young...right?

9) We like much of the same music and we both have small kids, so there is never a lack of things to talk about after we finish talking about blood, snot and poop.

8) One of his little patients told him that he's cool, because he has good hair. I agree!

7) He is a perseverater...in that he perseverates. When a new symptom arises he mulls over the pro's and con's of the next move. You can actually see the wheels turning in that little head of his. Sometimes he thinks aloud and talks to himself. It shows me that he really puts a lot of thought into my care.

6) He is super duper aggressive in his approach to CF. Since I started seeing him in August my PFT's have risen by 10% from the baseline that I sat at for a couple years. That was lung function that I thought was gone forever. Today he told me not to worry much about the new MAC that I cultured. I am being treated for it now. He said that I will culture lots of bugs over the next 60 years. We'll beat them as they come. Yippee!

5) Dr. D ALWAYS emails or calls me back in a VERY timely manner. I appreciate this immensely, because I am one of the most impatient people to walk this Earth.

4) He doesn't treat me like I don't know what I'm talking about...even though I often don't know what I'm talking about. Basically, he doesn't treat me like I'm just a dumb patient.

3) Dr. Dazzle is both realistic and positive. He tells it to me straight, but then gives me the plan of action. When I was struggling with what to do with regards to the m. abscessus he told me that this was a very serious bug that is often responsible for very serious lung damage and sometimes death. However, we caught this early enough where we can treat, suppress and possibly eradicate this bug for good. I then got an email detailing the treatment plan and all potential side effects, along with what we were going to do monitor the side effects.

2) Dr. D texted my lab results to me on a Saturday during the Ohio State vs. Michigan football game. Seriously! My lab results were more important to him than that game! I have never gotten a text before that detailed my white blood count, platelet count and my last culture smear result...along with telling me to enjoy my weekend... It was good stuff!

1) He listens to me and actually hears what I'm saying. He remembers what I like and don't like, along with my thought process and he takes in all into account when offering a plan. I think we have a true partnership when it comes to my treatment plan. This is what I appreciate most!

It was really difficult for me to choose to change Pulmonologists over the summer. I was scared and nervous about what I might get, even though I knew it was time for the change. I truly feel like I've won the Pulmonologist lottery on this one... Yeah me! On this Thankful Thurday I am ever so thankful for Dr. Dazzle!

Bittersweet Appointment

2010-12-15T16:33:00.005-05:00

Today I finished my IV's. I had a clinic appointment and got my PICC pulled! Sweet relief! Today was 3 1/2 months in the making...the end of phase one of my treatment of Mycobacterium Abscessus (I am a HORRIBLE speller...and realize that I have spelled this wrong almost every time I've written it over the past several months...oops). The plan going forward is to continue nebulized Amikacin every other month for at least a year. I will continue the oral Zyvox for one year and I will continue the oral Azithromycin for life. I can certainly handle this plan!

I anticipated that I would be jubilant after today's clinic visit. I imagined that I would have stable PFT's, the m. abscessus would be gone and I would return home to my normal life. I knew it wasn't going to go as I imagined when the first PFT I blew was down 6% from last month. After three tries I was able to improve, so I actually only had a 3% decline overall. Not horrible, but I expected further improvement after all of the blood, sweat and tears I put into this treatment plan. Yet, it was not to be today.

Then I headed into my appointment with Dr. Dazzle. One of the first things I asked was about my last cultures. I really wanted to know if the H Flu was still present in my culture from last month. The look on his face when I asked told me immediately that all was not well with my cultures. I have not cultured m. abscessus since September, about a month after I started my treatment. This is great news! So what's new? I now can add Mycobacterium Avium Complex...also known as MAC...to the list of bugs living in my lungs. There must have been a buy one get one free deal going on when I contracted my mycobacterium. MAC is common in immunocompromised people, especially AIDS patients. It also tends to like living in the lungs of people with CF. This avium strain derives from birds. I don't even like birds!!! I guess it can also be found in dirt, dust and water. It is not as destructive as the Mycobacterium Abscessus can be, so it is often not treated until it progresses. This MAC was discovered after the full eight week examination of my late September culture. Dr. Dazzle thinks that the drugs I have been on over these last few months should have suppressed the MAC.

Yet, I'm bummed. My last 2 appointments have resulted in culturing 2 new bugs. This MAC is another one that will never go away completely. It can only be controlled. I did everything right. I have worked so hard all fall to get my lungs in tip top shape. In spite of my efforts, I still lost some lung function and picked up two new bugs. Today I learned that CF has a mind of its own. There are aspects of it that I can NOT control, despite my best efforts. Piss off CF!

Dr. Dazzle and I chatted a bit about my Brochiectasis. Mine is pretty much all in my upper lobes. My upper left lobe is the worst. That damn upper left lobe has been a thorn in my side for a while. I also met with one of the research nurses. They want me to participate in a quick one day study to test out a new way of doing Nasal PD's. It will involve getting another PFT, a sweat test, a nasal PD and nasal scrapes. So I'm heading out to the hospital again tomorrow for another all day round of procedures, this time for the sake of research. I'm bummed that I don't qualify for the Vertex study :-( Yet, they have a phase 3 Spireva study that I will start in January. I was involved in the phase 2 version of the same study.

Basically, I'm a bit frustrated that it always seems that there has to be some bad news to even out the good. I am going to work on focusing on the fact that I feel good and I am PICC free!!! I am heading out to a celebratory dinner with my family! I did 3 1/2 months of IV's without completely losing my mind... For this I think I deserve some sushi!

If you heard REALLY loud music and lousy singing coming from a car today, it was probably from me. This song makes me feel GOOD and represents how I feel about the end of this era!

Thankful Thursday

2010-12-09T18:24:00.007-05:00

I did a few Thankful Thursday's a while back, but fell out of the habit. Ronnie & Mandi just encouraged everyone to start again...so here I am writing on a Thankful Thursday!

Today I am thankful for soup.

It's the little things, isn't it? Today I spent the morning with my friend Cathy Hawk, a full-blooded Italian, learning to make pasta fagioli. We had a great time and I picked up some wicked soup-making skills! My friend Robin Davis joined in on the fun too! I love these ladies!!!

Even more important than the soup, I am thankful for Cathy's kindness over this past 4 months during my IV treatments. Cathy is a newer friend. I just met her at the beginning of 2010. Our girls were in Gymnastics together, then we found out that we went to the same work-out class. When Cathy found out about my illness from my blog she said that she didn't know what to say, but she knew how to make soup, so that would be how she would take care of me. Cathy kept up with my blog. Every single time I had a rough day or a turn for the worse I got an email from Cathy telling me that soup would be delivered the next day. It ALWAYS came at just the right time...when I was really down and out. Cathy took care of me. I will NEVER, EVER forget her kindness and love. I get teary thinking about it. I'm not sure I can ever repay her adequately.

So today, I am thankful for soup...Cathy's soup!

It's a CF Thing...

2010-12-06T19:59:00.027-05:00

Over this past year and a half I have "come out" of the CF closet. My family and closest friends knew about my illness, but many of my acquaintances or co-workers had no clue. It was quite easy to hide. Or, if they knew, they assumed that it must have not been so bad since I "looked" fine from the outside. This past 6 months of hospitalizations and almost constant IV treatments probably took some by surprise.

I have found that some people are still confused about my illness, since I still look the same on the outside. I think it's so easy for people to understand that someone is sick when they see outward proof of an illness or aggressive treatment. Much of the impact of CF is internal. So, I wanted to share some of the outward physical manifestations of Cystic Fibrosis, just to raise awareness.

Cough: A CF cough for most is very congested sounding. A coughing fit can last several minutes. Most people with CF cough every single day...I do. Some people with CF cough so hard that they open up a wound or blood vessel in their lungs and can cough up large amounts of blood. This is called Hemoptysis.

Salty Skin: In the middle ages in Europe they were informally diagnosing children with an unknown disease, simply based on salty skin. They used to say, "a child who tastes salty from a kiss on the brow...is hexed and soon must die". The disease that they were talking about was CF. My sweat is salty. I look like I have frosted skin when sweat dries on my face. Ick...sometimes it even burns my skin and eyes. On a positive note, I'm sure it exfoliates my skin at the same time!

Clubbed Fingernails: Due to a lack of Oxygen, most people with CF experience clubbed fingers. The nail beds and tips of the fingers are larger than what's considered normal. My pinkies and thumbs are OK so far, but my index, middle and ring fingers are clubbed...though not severely.

Barrel Chest: When people have illnesses that cause a chronic cough, they can actually change the shape of their rib cage. The rib cage takes on a more circular shape, then it's normal oval shape. This sometimes occurs in people with CF. I am pretty certain that this symptom doesn't impact me yet.

"CF Belly": I kinda made this one up... I haven't seen anything written on this topic, but I have spoken about it with several of my girlfriends with CF. That means it's definitely true! People with CF typically have difficulty keeping weight on. The CF pancreas does not digest fats. When the pancreas is deficient, it's called pancreatic insufficiency. CFer's who are PI take enzymes so that they can digest more of the foods and nutrients that they ingest. I am pancreatic sufficient. My pancreas still digests some of the fat and nutrients I take in, however not all of them. I am just not at the point yet where I need to take enzymes.

(Nope, this is not MY belly)

CF patients are encouraged to eat a lot of very high fat food. I have no problem with this part of the disease! My CF doctors have also encouraged me to keep a little layer of extra fat stored on me, since when I get sick it will melt away. Mission accomplished on that one! However, when us CF ladies get together we often feel the need to compare our CF belly's. Many of us have a similar body type, including skinnier arms and legs, along with the 4 month pregnant belly look. I'm sure the belly has to do with digestion...I don't know. I just know that no amount of crunches are going to flatten this girls CF belly :-/ I want to hear from my Cysters out there... Any of you have the "CF belly"?

A little health update. Not too much has changed in the last few weeks. I am still on the IV and nebulized Amikacin, along with the oral Zyvox and Azithromycin. My white blood cells and platelets have been behaving. I have a clinic visit next Wednesday, at which I will have my PICC pulled. No more IV's after Wednesday! Whew!

The Theory of Natural Selection

2010-11-24T19:45:00.007-05:00

'Tis the season to give thanks. This year, more than any of my previous years on this plant, I am thankful that Darwin's Theory of Natural Selection is no longer relevant in the human species. I am just sitting here, at age 35, laughing at the whole idea of "Survival of the Fittest". Nope, no longer the truth here in 2010. Today, sometimes the broken do survive.

What does this old fogey from the 19th century know anyways? People thought the Earth was flat back then...

I am thankful that I was born in 1975, at a time when medical science started advancing at a tremendous pace. Having no siblings or cousins with CF we were asked to think about where CF may have derived from in my family. My mother had an aunt die in childhood in the 1920's or 1930's of "whooping cough" ~ likely CF before the diagnosis existed. My father had 3 siblings, Earl, Eugene and Loraine who all died before the age of 8 of either "Whooping Cough" or "Consumption" ~ also likely CF.

If I were born a century, or even 20-30 years earlier I would have no doubt died at age 2 when I had horrible pneumonia for almost the entire year. If I were spared at the age of 2, I would certainly have died at age 16 when I had a majorly debilitating case of mono. If I miraculously survived the mono, I would have likely passed when I had a horrible case of C Diff that earned me a week in the hospital and a month long recovery. If that didn't get me, I am pretty sure that the lung infections that my medical team I have been working to control over the past 2 years would take me. Yet, I am still here! I am alive and thriving and mothering and working and wiving. I am so thankful to have been given what I see as 33 "bonus years". Years that would have never been mine to have if I had been alive in the days when Darwin's Theory was the word. Much of my life has been like icing on a cake...none guaranteed or even expected.

I am really, truly a lucky girl! When I am physically knocked down by my disease, which I have been much of the time over the past 6 months, I tend to forget how lucky I am. Yet, I know that I am almost as lucky a they come. I have been allowed to live in overtime. My wish is for the next generation of CFer's, the little one's being diagnosed now, to be even more lucky than I have been. I want them to be given many more "bonus years" than those I have been given. I want CF to be something that is managed and not deadly. Even better, I want a cure!

Today I am a thankful girl! I wish you all a Happy Thanksgiving! Count your many blessings...even if only for today.

Clinic Visit ~ The Good & The Bad

2010-11-18T14:02:00.010-05:00

I just got back from clinic. Do you want the good news or the bad news first? I'll go with the bad, so we can end on a positive note...

I got the H-Flu...AKA Haemophilus Influenzae. Yep, I cultured a new bug. This one is often seen in conjunction with meningitis. This is baffling to me since I have been on IV's for nearly 8 of the last 10 weeks. I know that having a depleted immune system makes me more susceptible to picking up new bugs, but I just imagined that being on 4 antibiotics would protect me. I guess that's not how it works :-/ Dr. Dazzle explained that H-Flu is often resistant to Cifoxitin, so that's why it still grew when I was on the IV's in Sept/Oct. This IV Amikacin that I started 2 weeks ago should suppress the H-flu. Dr. Dazzle is not at all worried, so I'm not going to worry either. This is another one of those bugs that will never go away. Instead we will work to keep it under control to avoid lung damage. So now, here's what the insides of my lungs look like...

H-Flu

Mycobacterium Abssessus

Staph

These are my enemies! It's a little frustrating to have new bugs to manage. It likely will make it a little more difficult in the future to figure out which bug is the culprit when I have an exacerbation.

In my last post I shamelessly bragged about how much energy I had, even though my blood counts were low. I forgot to knock on wood while writing that post. That energy evaporated on Sunday. On Monday I took a 6 hour nap, yesterday I took a 4 hour nap, and today I had a tremendously difficult time getting up and out the door to my clinic appointment. I have been tired when I wake up in the morning and no amount of napping replenishes my energy. I was relieved to hear that my counts have jumped a bit and are back to being low/normal. I was also relieved to hear Dr. Dazzle say that extreme fatigue is one of the main side effects of IV Amikacin. He compared it to the fatigue that cancer patients experience while undergoing chemotherapy. I know it's not a great symptom, but it was great to hear that this is normal. I no longer feel like a lazy slob. I feel like I can now give myself permission to rest when I need, knowing that no amount of fighting against it is going to take it away. So sleep I will do...

I brought up my hearing issues and Dr. Dazzle is scheduling another complete work-up from audiology. We'll see if my hearing has been compromised and if so, in what ways. I wouldn't be surprised if I've sacrificed a bit of the hearing in my right ear for the sake of this treatment.

That brings us to the great news! I blew AMAZING PFT's! I haven't had PFT's this high since 2005, prior to having my son. They were 3% higher than last time, which was 4% higher than my baseline. Basically, I'm 7% better than my baseline on my FEV1. Good stuff! I never share my PFT #'s on my blog...just a choice I made when I started blogging. Yet, believe me when I say that they were good :-)

I gotta go take a nap now. G'night all!

Can You Hear Me Now?

2010-11-14T16:16:00.006-05:00

I have had an amazing amount of energy this week and I'm not complaining. I got a call from my nurse on Friday saying that my blood counts are all out of whack again. Not surprisingly, my Hemoglobin was normal, hence the energy. However, my white count dropped back to 2. If they get to 1, which they probably are by now, I must give myself another Neupogen shot. My platelets are also giving me a run for my money right now. A normal platelet count is between 150,o00 and 300,000. Mine were 63,000 on Thursday when I had my last blood draw. My bone marrow better stop being lazy.

The other thing that I've noticed is that the hearing in my right ear feels different. It's a strange sensation. Twice this past week I have been stopped in my tracks from REALLY loud ringing in my right ear. It was so unpleasant that my immediate reaction was to grab my ear and shake my head until it stopped. It only lasted about 20 seconds, but it is making me a bit paranoid. Hearing loss is the biggest concern while I'm on this IV Amikacin. I feel like I am still able to hear well, though.

So I have a clinic visit on Thursday and I have some questions for Dr. Dazzle...surprise, surprise! I have 4 weeks left that I need to get in on these IV's. Will we continue this dance if my counts continue bouncing around? Could this turn into some type of permanent bone marrow condition? Is the ringing in my ears OK if it is only occasional? Could this become permanent?

I have been thankful for the burst of energy I had this past week! I had tons of fun. I kissed a camel named Charlie, I went out with my girlfriends, we refinanced the house and I finished my Christmas shopping. Life is Grand!

Minor Annoyances

2010-11-08T19:32:00.004-05:00

This week has been full of what I would call minor annoyances. I am very thankful that I was able to be released from the hospital last Wednesday. I had meals prepared and sent over from caring friends much of the week. I am tolerating the IV Amikacin wonderfully. I am just to the point where I think I'm done with the inconveniences associated with treating this stupid disease.

Annoyance #1 - Time Management
The progression of my CF has not changed the fact that I lead a busy life and stuff has got to get done. I really have no excuse when I am tolerating my medications and feeling relatively OK. Every day I have to meticulously plan my day to ensure that I get all 3 1/2 - 4 hours of my treatments in each day. I had a busy weekend with the family and fitting everything in was very tough. It was not impossible, just frustrating. There have been many times where I just can't fit everything in and I don't get done all that I wanted. I always end up having to give up something I love, like working out :-( I have been able to get to my workout class 3 times in the past 5 days, though. I just have to work extra hard and plan my time well so I can get there.

Annoyance #2 - Surveys/Letters from Insurance Companies
With 4 hospital stays and 2 ER visits in the past 4 months I have gotten MANY quality assurance surveys from these hospitals. To be honest, I have no idea which survey goes with which stay. I feel obligated to fill these out, so they sit in my pile of things I need to do...but they never make it to the top of the priority list for reasons such as "Annoyance #1". I also keep getting daily letters from my insurance company saying I have been approved for this or I have to use a different pharmacy for that. I am a Social Worker. It's my JOB to help people navigate their insurance plans, but when I think about the hoops I have to jump through it boggles my mind. The amount of information is overwhelming!

Today was the kicker... I got a form letter that said that I was "approved for home care or hospice services". I wish my insurance company would send me a survey like the one's I get from the hospitals. I would tell them where to shove their form letters. Couldn't they have one letter that goes to those who have been approved for home care and a second for people approved for hospice? It would have been nice if they could have even just circled, underlined or bolded the home care selection. Do they know something I don't? So either I am able to have a nurse come to the house twice a week to monitor my care or I have six months or less left to live! Crazy form letters...

Annoyance #3 - PICC lines
Yes, they are super, duper convenient. However, I am a nervous wreck with mine now, since my last one ended up infected. This one has been very sore and itchy so far. I think the adjustment that had to be done, the bleeding that caused and the 20 minutes of pressure to the PICC site as a result created one sore arm. I think it's itchy, because even though I've had a PICC most of the past 4 months, I have not yet mastered the art of keeping the site dry in the shower. Saran wrap does not work, nor does saran wrap taped into place with medical tape. I spend 15 minutes prior to every shower trying to wrap my arm myself, cursing at the saran wrap as it sticks to everything possible, except my arm. Yet, I still end up with a wet dressing. Grr. One day I will master this art.

Here is a HUGE don't for all my CF friends with PICC lines out there. If you have an extension, say like a 14 inch extension, and you finish your antibiotics leaving the extension dangling when you run to the bathroom...DO NOT allow your PICC extension to fall into the toilet. I'm not saying I did or anything, but I would suggest that you DON'T. If you do I would suggest running, not walking, to your supplies and immediately changing your cap and extension line! I mean, this sounds like it would be a good plan if it were to ever happen to anyone... Not me, of course.

Basically, I'm doing very well on this drug. My counts came back up with a jolt of Neupogen last week. I have some energy that I didn't seem to have on the other IV antibiotics I was on prior to this one. My hearing and vision seem to be OK so far, although I know that I may not notice those changes immediately. My cough it non-existent at this point. All good stuff! Even with these "annoyances" I really can't complain :-)

Events of Day #2

2010-11-02T20:03:00.003-04:00

This is my 50th blog post! Holy moly, I must have an awful lot to say...

So today wasn't horrible, but there were a couple of little surprises thrown in. First, I found out that my white blood count is back down to 1. I have 280 neutrophils. Basically, this is no where near enough. My body is not equipped to fight off ANY infection right now. I am neutropenic. The only puzzling thing this time around is that we don't really know why. Two weeks ago when my white count was depleted we knew it was the Cifoxitin. I went off of it at the time, took Neupogen shots, my counts recovered and all was right with the world. This time the only thing we are thinking is that the Zyvox must have caused this drop. This is not a common side effect of Zyvox, but it appears that I always get all of the fun side effects...so why not? So it's back on Neupogen for me. I will do 3 shots this week, get my blood draw twice a week, and continue with Neupogen as needed. The cruddy part is that I need to try to stay on the Zyvox for a full year. I've only been on it for 2 months. We'll see how long we can go along like this, but eventually, we may have to discontinue :-(

The second fun occurrence of the day was that the PICC team came in a few hours ago to tell me that they took another look at my Xray following my PICC placement and they realized that the line was too short. It wasn't far enough into my Superior Vena Cava. Therefore, I had to have an adjustment. Since they just needed to replace the line inside, not create a whole new hole, I turned down the drugs they offered me to take the edge off. Here's my advice! If you take anything away from this post, please let it be this. ALWAYS TAKE THE DRUGS THEY OFFER YOU TO "TAKE THE EDGE OFF". Oh my, the "adjustment" was not such a comfortable process. Nope, not at all. I sucked it up and didn't let it show, but I was in some pain :-/ I love the PICC nurses, though, so they made it more tolerable.

I am tolerating the IV Amikacin well and I think I'm still set to head home tomorrow so I can do all of this stuff for myself for the next 6 weeks. Yeah for home!!!

A "High" Hello from the Hospital

2010-11-01T19:31:00.009-04:00

A little "high" hello from the hospital...

Not nearly as bad as "David After Dentist"!

Sweet Dreams? (Warning ~ Gross Pics)

2010-10-31T13:23:00.018-04:00

It looks like my admit is a go for tomorrow. So while you are dreaming about ghosts, goblins and mini Snickers bars tonight...I will be dreaming about this...

These are pictures from my last PICC placement in September...

Yep, I'm getting another PICC. Since I need at least 6 more weeks of IV's and my last PICC got infected, I've got to go through this procedure again. While a port would be nice, my doctors think that once I eradicate this MA I will not need IV's enough in the near future to warrant a port. So another PICC it will be. I have NO idea why this procedure makes me so anxious? Maybe because it took three sticks to get it right the last time? I have had organs removed (gall bladder/appendix), I have had bronchs, I have been poked/prodded...heck I've even had two babies. Yet, no procedure makes me more nervous than this one. Once it's placed it's no big deal at all. It's just this tiny little 30 minute procedure that gives me chills. I'm not to the point where I need conscious sedation (Versed) yet. However, thank goodness for heavy doses of Xanax!

Preparing for Admit #4 in 4 Months...

2010-10-28T19:02:00.003-04:00

I'm getting really good at this... I am being admitted to the hospital on Monday (if there is a bed available) for three days to get my PICC placed and start on my 4th IV antibiotic in as many months. This will be 4 hospital admissions, 4 different IV antibiotics and 3 PICC lines...all in 4 months time. What a whirlwind. Throw in my 2 ER visits and I think I've made it onto some type of shit list with my insurance company.

I had an appointment with Dr. Dazzle and the team today. They were able to find 68 doses of IV Amikacin in Texas! The place in Texas will ship these to me weekly and my home care company will just come a couple times a week to do blood work and dressing changes. I'm excited that we now have what I need and I can get started on this last phase of IV's. I just want to get this part of the treatment plan over with. I'm ready to move on with my Mycobacterium Absessuss being history...until it rears it's ugly head sometime in the future. Hopefully, really, really far in the future!

Drug Shortages are Infuriating

2010-10-27T20:24:00.002-04:00

It is going on the third month that I have been dealing with the effects of the Amikacin drug shortage in the US. As a result I spent 4 weeks trying and failing both 2nd and 3rd line IV antibiotics for the treatment of my Mycobacterium Absessuss. The national media has finally picked up on this story. Here's an article my friend Marci posted on Facebook earlier today.

http://www.msnbc.msn.com/id/39798646/ns/health-health_care

I am one of the people whose treatment is dependent on drugs we have no way to obtain. My hospital did receive a shipment last week. The first shipment they have received of Amikacin in many months. However, we haven't been able to find a way to get the medication to the Home Care company to administer to me. People WILL die as a result of these shortages. People's cancer will not go into remission or they will relapse. People with MA will sustain permanent lung damage. The article reports that a couple of patients already died as a result of a morphine shortage.

I really don't want to get political, but I have been living this for several months and it's infuriating! We are supposed to have the best health care system in the world... However, these drugs, which are all very cheap to consumers, are not being produced, because there is not enough of an incentive for the drug companies. These drugs don't make the companies enough money to be a priority. This is rationing of care. Unfortunately, our system based on profit has decided that my care is OK to ration.

Fun While Waiting!

2010-10-25T20:15:00.008-04:00

Things have been going pretty well here. I have to say that it took me about 6 days after the IV's were discontinued and my PICC was pulled before I started have ANY energy at all. I had never felt this type of fatigue before. My cancer patients at work have talked to me about it all of the time, when the chemo depletes their entire immune systems. It is a fatigue deep down in your bones. It's not that your tired...it's that your body won't perform no matter how hard you will it to. I had no white blood cell count, no immune system and definitely no energy. I experienced bone pain, which was my bone marrow trying hard to restore my blood to normal. It was a bit scary and something I hope I never have to experience again. It also made me appreciate the symptoms that my patients have described to me on a whole different level! In the end, I was glad that the decision was made to pull my PICC and stop the IV Cifoxitin. Even though it means I will be starting over with a new PICC and 4-8 weeks of IV Amikacin soon.

So I have been feeling well, still tired, but OK overall, for the past 5 days. We've been making the most of this little "IV vacation"! Crazy to say that, since I am still on 3 antibiotics, one anti-fungal, a probiotic, a bunch of vitamins, Singulair and nebulized albuterol. However, the IV's are just so intense, that any break from them is reason to celebrate.

We had a blast at my dear friend Elizabeth's Halloween Party on Saturday!

My little guy, Jake, won for the scariest costume in the kids costume contest!

Tonight we went trick-or-treating at my grandmothers nursing home. I made Jake cute, not scary. He passed out in the car before we even started.

Oh, the weekend was great for other reasons too! It was 80 degrees and sunny here in Cleveland, Ohio State won against Purdue on Saturday and the Cleveland Browns won against the New Orleans Saints on Sunday! It doesn't get much better than that!

Basically, I'm enjoying this little reprieve. I've got an appointment with Dr. Dazzle on Thursday. I have a couple little things to discuss with him...like my right eye and left ear acting funky. My sinuses are also really trying to give me trouble. I think they are confused, because they are being blasted with antibiotics. They really want to be infected, but the drugs won't let them... Fun stuff! The Amikacin that I will be starting IV has been out of stock for months. Hospitals are just starting to get it back in stock. However, the home care companies do not have access to it yet. We are working on trying to get some in the hands of a home care company that can work with me. This may take a week or two. When we get it figured out I will go to RB&C to have my PICC placed, then have my first dose on IV's in the Dialysis Center. Then I can go home the same day! No hospital stay this time...Woo Hoo!

That's all for now... Off to enjoy more IV-free days before I'm hooked up again!

The Product of Mommy Guilt...

2010-10-19T16:58:00.005-04:00

You too could be the owner of 2 dwarf hamsters if you were lucky enough to be my children right now!
(Disclaimer: This is not our actual hamster. I swiped this photo off of the Internet. No, we are not planning on eating our hamsters!)

The guilt got the best of me. The guilt has been overwhelming. For over a month, during these intense treatments, I have probably spent nearly 75% of my time in bed. My body feels broken. I feel worthless. I think the most difficult thing has been the inability for me to carry on the roles that have made up my life for so many years. I have tried to be a good wife, a good mom, a good employee...now I feel good for, well, not much. The kicker happened last week during one of my high fevers. I was laying in bed, sleeping in fits and starts, when my kids got home from school. My daughter decided to craw into bed with me and watch TV. She stayed with me for hours. Every time I gained a bit of consciousness I found my dear little girl holding my hand, rubbing it ever so gently. So sweet...yet so wrong. It's just not supposed to work that way.

I know this will pass. At least I hope this will pass. Sometimes thoughts about possibly getting to the point where I can't maintain the life I built for myself creep in. I am just telling myself over and over that these treatments are going to make me even better able to fulfill all of my roles in life for a longer period of time. It's just going to take time. No pain, no gain...right? Hopefully, they will give me more energy when all is said and done.

This brings us back to the silly little hamsters. I mustered enough energy on Sunday afternoon to get the kids out for a couple hours. We went to Panera, which is right across from the pet store. My kids have been begging for a pet forever. The answer has always been, "We don't need another thing to take care of...we can barely take care of ourselves". The kids wanted to go to the pet store and I agreed that we could look, but that we were NOT buying anything. We walked out with two tiny little hamsters and all of the fixin's that we needed to give them a cozy little home with us. My heart just hurts for these kids right now. They seem to be doing great. Both are cruising along in school and are not having behavior issues that we have noticed. Yet, I still feel guilty that I can't be the mom they deserve right now. Hence, I bought them hamsters.

In working with kids with parents with cancer in the past, I know that having these kids work with animals is a great way for them to learn about caring for the sick loved one. Maybe they will learn a couple lessons from little "Lilly" and "Casey"? I just hope my little guy doesn't squeeze his too tight! We'll see...

Did I Do It? Was I Able to Give Myself a Shot???

2010-10-14T18:38:00.003-04:00

Two Posts in One Night...Oh My!

2010-10-13T20:58:00.003-04:00

Forget the previous post... It is completely null and void now. Dr. Dazzle just swung by...yes, at 9pm at night...to tell me that I have a white blood count of "0" now, do to my IV drug. I have 100 neutrophils total. Basically, I have absolutely no immune system. We are going to start Neupogen tonight, but Dr. Dazzle thinks my staying on the Cifoxitin is going to do more harm than good in the long run. I will have no PICC placed tomorrow and will likely start on about 4-8 weeks of Amikacin IV once it's available in the US again.

My, oh, my how quickly plans can change! So I'm disappointed that I failed yet another IV antibiotic. I have been on 3 and have failed all 3. Yet, Dr. Dazzle thinks the nebulized Amikacin is working well for me, so we'll stick with that and the oral antibiotics for a while until we can get a hold of the IV Amikacin. Then we'll try again and start over.

I guess I'm getting a little respite I wasn't planning on...

Being Poked and Prodded

2010-10-13T18:24:00.007-04:00

So I arrived at the hospital today at 2:30pm feeling, let's say, like shit. I had a fever, my bones hurt, my muscles hurt, my joints hurt, even my hair hurt. Due to rooming issues I had no room available for me when I arrived. I was given a blanket (since I had the chills) and a chair and sat in the alcove in the CF unit for a little over an hour. It pretty much sucked, but I didn't really have any other options. The staff were all fantastic, though. I do love them!

I felt so much better when I got to my room, but my excitement was dashed when the IV team arrived. Peripheral IV's are not my favorite. Especially since my veins ALWAYS roll and the phlebotomists always have to dig around for minute on end until they find the pesky little bugger. Yep, it happened this time too.

I noticed this morning that I have developed a rash all over my belly and arms. This is making it a bit more clear that all of my symptoms are a result of a reaction to the Cifoxitin, and not a virus or infection. So when I saw Dr. Dazzle I used all of the charm I could muster to try to convince him that my little PICCy friend should stay. I begged, I pleaded. I batted my eyelashes...but Dr. D convinced me that this was a necessary step we had to take just to remove all possibility that I have a PICC infection. Especially, since I now have just a teeny tiny immune system. So my PICC is now gone and I am grieving it's loss :-( Really, I am...because tomorrow I have to have a new one placed in the other arm. This is probably no big deal for some CFer's, but it takes me a hefty dose of Xanax and about and hour of mental preparation to get myself to a place where I can endure the procedure without being a complete basketcase. I think it's because it took them 3 tries of sticking that gynormous needle in my arm last time before they could thread it correctly. There are some gory pictures of what I went through a month ago when getting my sorely missed PICC.... I couldn't get them to load here due to the poor connection.

So, this PICC placement procedure is what I have to prepare for again tomorrow. This is what my dreams will be made of tonight. A few people have asked why I didn't get a port. Trust me, I've asked, several times. My docs just all agree that since I haven't needed IV's often in the past, that after this cycle of IV's they don't anticipate that I'll need them again for a good long time. I hope that's true, so I'm heading their advice for now.

If my fevers, aches and general sickness continue now that we removed my PICC, we know that the Cifoxitin is the culprit. However, we are going to try to mull through the IV and treat the symptoms. If they are nearly unbearable, we will just try to get 2 more weeks of the IV's in. If they are somewhat bearable, we will try for 4 more weeks. With needing to stop sooner than anticipated, we may end up revisiting IV Amikacin in the Spring.

I guess I was just sorta wishing that my body would be kind to an antibiotic for once. I was hoping that it would have been a good hostess, showing the good guys where to go and what to do to get rid of the bad guys...maybe without attacking me in the process...

Noooooo! Not the Hole!

2010-10-12T17:25:00.003-04:00

Yeppers, it looks like I'm being admitted again tomorrow. We CFer's often affectionately call the hospital "The Hole". Day 5 of fevers does not make Dr. Dazzle happy. I'm frustrated. This wasn't part of my plan. The fevers have continued. They are very sporadic and change quickly from low grade to pretty high. At 4pm today I was at 101.5 and felt like crud.

My blood cultures have not yet showed signs of infection. However, with such a low white blood count it may be tough to pick up an infection in me right now. I am going to mosey into the hospital tomorrow, they are going to pull my PICC and I am going to get my IV's though a peripheral IV for a day or two. Then they are going to place a new PICC, in the other arm, on Thursday. Hopefully, I'll be able to go home Thursday or Friday.

If we go along with this plan and the fevers stop, we know it was likely my PICC being a trouble-maker. If my fevers continue for a time, but go away eventually, than this is all probably a virus. If the fevers continue indefinitely, then we know that they are a side effect of the antibiotics. If that's the case, I will just have to work on managing the fevers for the next 2 months, while I'm on these IV's. That makes me a little nervous, because How will I know that I have an infection??? There's a question for Dr. D.

So this isn't all bad. At least I'm heading in feeling horrible. It will be nice to just lay back and be served for a couple days. One annoying this is that the CF floor is full...NO VACANCY! Therefore, I have to stay in the adult hospital...Boo! It's not quite as fun :-/

BLAH ~ Fevers

2010-10-11T15:47:00.004-04:00

I'll do my best to update, but I'm still feeling a bit groggy after my adventures in the ER last night. It started Friday night when I woke up soaking from head to toe with night sweats. I never, ever get night sweats, but I figured that I must have been warmer than usual due to this bit of Indian Summer that we have had here in Cleveland lately. I never took my temp. On Saturday I went to the farm and pumpkin patch with family for a fun-filled day! I was exausted when I arrived home and, once again, experienced night sweats in the middle of the night on Saturday.

It wasn't until Sunday afternoon, when my body started feeling achy, that I thought that it might be a good idea to take my temp. That's when I discovered my low grade fever...99.4. I always run low (in the 97's), so this was a legitimate fever. I checked my temp several more times during the day and the fever seemed to come and go. It's a very fickle little bugger.

So last night, after freezing my butt off all night and sleeping very little, I finally got up at 2am when the sweats started, to take my temp. It was 100.4. Not super alarming, but I immediately googled the PICC line procedures, knowing that fevers were of concern. In HUGE bold letters on the page I found it said that you must procede to the nearest emergency room immediately if you have a fever over 100. Ugh! All CF patients and CF momma's know that ER's are a huge waist of time for us. The staff don't typically understand our disease or needs. I called the pulminologist on call and made my best effort in convincing her that I really didn't need to go to the ER. It didn't work...so off to that germ-filled place I went...knowing that my job would be more educating them about what I needed them to do, then actually getting any information from them.

So Dr. NoPersonality walked in when I got to my room. He asked me what drugs I was on. When I told him about the 3 grams of Cifoxitin his eyes popped out of his head and he asked if I was sure that wasn't an error. I bought my next dose of IV's with me, so I pulled out the container and showed him the dosage information. By the way, all experienced CFer's know tha you need to bring your own drugs to the ER. Dr. NoPersonality then mumbled that he has never seen anyone on that high of a dose of IV Cefoxitin for this extended period of time. It was as if he was questioning my treatment plan. This guy, that I had to spell "Mycobacterium Abssessus" for was trying to make a judgement about my coarse of treatment. Maybe if he had a personality I would have thought this was just general conversation, but instead it came out more as judgement.

I proceeded to tell him that I needed labs and blood cultures drawn. I had to explain that I have a boarderline low white blood count, but that the cifoxitin has the potential to make this even lower. A normal white blood count is 4-11. If my white count is around 6 ot 7, that probably means I have a raging infection. So they drew blood cultures from the PICC and from a periferal vien so they could see if there is an infection in the line only or in the line and the blood. The blood cultures will take a day or two to grow, but the CBC showed that my white count is now 2. I am officially neutropenic and immuno-compromised. When is gets to 1.5 we start neupogen shots. I was hoping this wouldn't happen so quickly. There is a possiblilty it can bounce back, but it was frustrating to hear that it is at this point now. This does not mean that I don't have an infection, however.

They let me go around 6am and I followed up with Dr. Dazzle today. The ER doctor was absolutely convinced that I have a PICC infection, and that I need to get my line pulled ASAP. I'm not entirely convinced. I have little kids that are swarming with germs around me at all times. I think I probably have a virus. It makes more sense to me, since the boat load of antibiotics I'm on should really kill any infection I might get. Dr. Dazzle agrees with me. So, the plan is that we are going to wait for the blood culture results to come back. If my line is infected I will be admitted into the hospital, have my line pulled, get my IV's through a periferal IV for one day, then get a new PICC placed the next day. Oh, joy! How I love having PICC's place. Otherwise, I'm just going to ride out the fever...calling if it gets worse. If the fever does still last a few more days, I will get the PICC pulled anyway and go with the plan to have a new one placed. The fever has broken now, but I still feel like crud...fatiuge, muscle aches, etc... I'm just laying low for now.

CF is always an adventure!

The Good News!

2010-10-04T16:09:00.005-04:00

I had an appointment with Dr. Dazzle last Thursday. I was nervous about my PFT's. I have been REALLY junky since starting the Albuterol/Amikacin nebulizer treatments. My lungies are not used to nebulized treatments. I thought for sure this was a bad sign that I wasn't improving. Especially since I'm on enough antibiotics to keep a village infection-free. I was wondering where this junk was coming from. Well, it turns out it must have been hanging around for a while and it's finally working it's way out.

For those of you without knowledge about CF, it's as if my lungs are constantly infected with pneumonia and bronchitis. All of that infection clogs and scars the airways. Basically, mine are beginning to unclog! My FEV1 (large airways) increased by 4%, while my FEC (small airways) increased by 7%. It may not sound like a lot, but it's HUGE! I actually feel like this intense treatment plan is doing some good! I feel like I made the right choice by moving forward with the treatment now! It has given me even more motivation than I had before.

I'm still chugging along with my IV's. I've screwed up a couple times, gave myself double heparin or forgot to clamp when done, but I'm not dead yet, so the mistakes must have not been too major. I did however, freak Dr. D out a bit. I told him that my PICC got pulled out a couple centimeters during a dressing change and when the nurse turned her back I pushed it back in. Apparently, this is a no no. I actually knew it was a no no, since the nurse told me so just before I made the executive decision to push it back in. Apparently, the site still looks OK, but the real danger is sepsis. I'm not sure how I could become septic on this many antibiotics, but I'm sure it can happen. That was a week ago and I'm still doing OK, so I think the threat of infection is gone. Dr. D just made a point to reprimand me and I promised not to do it again :-/

All in all, things are going well. My bone marrow is holding up. We anticipate that it will start to fail in about 2 more weeks. At that point they are going to teach me how to give myself Neupogen shots. Nope, they can't go in my PICC. I actually have to inject them into my belly. Hey, if my cancer patients can do it, so can I! I'm thinking this would be a fun marital bonding activity, so I may actually ask my husband to give me the shots. I'm sure this is not what he had in mind when he vowed "in sickness and health"...Ha!

I've not had much motivation to open my laptop very often lately. I think that so much of my social networking world is tied to CF. Since I'm "living" CF for hours upon hours every day right now, I just think my brain needs a break. So please don't worry if you're not hearing from me much. I'm doing better than I expected!

Rockin' & Rollin'

2010-09-27T16:40:00.011-04:00

It's been a week on my newest IV drug and things seem to be going pretty smoothly. I am getting into a routine. I infuse my IV's at 10am, 4pm, 10pm and 4am. OK, so the 4am dose is pretty rough, but at least I've learned how to hook myself up in the dark, only half aware of what I'm doing. I'm finding that all of the spare time that I thought I would have, being off of work, is nowhere to be found. I have been one busy bee. The family and I went to a Cleveland Indians baseball game last Thursday, we went to Cedar Point Amusement Park's Halloweekends on Saturday and I went out to dinner with my girl friends on Wednesday night. I even went back to my exercise class twice last week! I was infusing my IV's all over town and feeling great about having the energy to get out and about... Until yesterday, when I crashed. I knew it would happen. I could feel my body starting to rebel. So yesterday was all about doing a bunch of nothing.

I think I overcompensated for having been completely out of it the week before. Nothing or nobody was going to stop me from doing everything that I set my mind to last week. I've decided that I am going to try t o pace myself for the remainder of my weeks left on IV's. I just can't get it all done every day without paying for it later. This will be a challenge for me, since I am typically and all or nothing type of girl.

So, I have a new attitude. I have got to limit myself and take care of myself. If I keep running myself ragged I will be no good for my family. I have to be kind to my body...I have to slow down...if I want to be available for my loved one's when they need me. My friend, Piper, reminded me of this when I was laboring over my treatment decision a month or so ago. She used the airplane oxygen mask analogy. I've flown a lot in my lifetime and over and over have heard the statement that "you must secure your own oxygen mask before helping others secure their masks". This is so true. I have also heard this time and time again as a Social Worker. We are always told to practice self-care to avoid burn-out. Again, you can't help others if you yourself are a wreck. This is what I need to constantly keep in the back of my mind when planning my day over the next few months. This is my new mission.

All in all I feel pretty good. The thrush is gone and so is the nausea. I continue to get my blood drawn twice a week to look for evidence of bone marrow suppression. This is the one thing that may derail this treatment plan. So far the bone marrow is looking strong. Good Stuff!

Thrush <- Nope, that's not the name of my new band...

2010-09-21T20:44:00.005-04:00

Of all of the side effects I was expecting, thrush was not one of them. It just never crossed my mind. Not until I woke up a few days back with a hell of a sore throat and fire in my belly. At least it's an easy fix and I'm already recovering from that little setback.

Today was interesting. After going off of the Tigercycline on Friday I had been IV free. Today we decided that I would start IV Cefoxitin. I guess since I am allergic to Penicillin I had a 30% chance of being allergic to Cefoxitin also. Therefore, I has to be observed while getting the first dose today. Off to the Dialysis Center I went, where I was by far the youngest person receiving treatment by at least 40 years. This process took FOREVER! When they tried to order my drug from the pharmacy, the pharmacist refused to fill it. She said that 3 grams was way too high of a dose and she did not feel comfortable releasing it. Dr. Dazzle stepped in and saved the day, as he always does. He's Dr. Dazzle after all! That just got me to thinking that I really must be getting some powerful stuff injected into my veins if the pharmacist is concerned...huh...

The bummer about Cefoxitin is that it will likely cause bone marrow suppression. This is concerning in my case, because I chronically have a low white blood count. The doctors have often wondered if this low white count was responsible for my lungs being scar-free for so many years even after enduring many lung infections. The by-product of the white cells is elastase, which is what causes the scarring. Without many white cells, there's not a lot of elastase and in turn there is less scarring. Interesting stuff, but it's just a theory. In the situation I now find myself, my low white blood count may be a hindrance. Having worked with cancer patients for 12 years I know that they are often given Nuepogen injections to boost their white counts after chemo. Therefore, I asked Dr. Dazzle if he could look into this option for me if the need presents itself. I just hate the idea that I keep failing on all of these medications. I really want this one to work-out. The only other crappy thing about this antibiotic is that I will have to infuse 4 times a day, which means I have to wake myself every day to infuse.

Soooooooo, I tolerated the Ceftoxin very well today and we're off on a new, different adventure!

In fun news, I finished "The Hunger Games" a couple weeks ago. I am almost finished with the second book in the series, "Catching Fire". By next week I should be able to finish the last in the series, "Mockingjay". These are amazing reads! Tons of action, a bit of love and some politics thrown in for good measure. I highly recommend this series!

Video Blog #2

2010-09-18T14:00:00.000-04:00

Needing to Vent

2010-09-16T20:37:00.006-04:00

UGH... This treatment sucks! I need to vent...if you don't want to listen to me rant you can stop reading now.

This treatment is brutal...absolutely brutal. The nausea is overwhelming. Nothing helps. Nothing takes it away. Nothing makes me feel better. I am now on 3 different anti-nausea meds. I can't function. Each day I have been home I have been able to take my kids to school and do NOTHING else. I come home and collapse. I stare at the TV. I watch whatever channel was left on from the night before. I have no interest in finding the remote to change the channel. I have no interest in reading. I have no interest in opening my laptop. I hope that someone will put some food in front of me at some point during the day. I am making no plans. I can barely get out of bed. I would love to "live life to the fullest", but right now my life involves me laying in bed...trying to talk myself into taking a shower...feeling guilty about the extra burden my husband has to deal with now...wishing that it would all go away.

I peel myself out of bed twice a day to follow bunch of complicated steps that involve measuring, dissolving, remeasuring, syringes, sharps containers, saline, vials...just to infuse myself with this vicious medication, that some people playfully call "tiger", that is eating away at my stomach lining. The thought of the medication, the sight of the medication and especially the smell of the medication give me a visceral reaction.

I am only one week in...7-11 weeks to go. Something has got to change. Please don't tell me that you know I can do it. I know I CAN do it too. It's just hard right now...it's really, really hard.

I Think I'll Take That Wheelchair Now...

2010-09-14T12:18:00.006-04:00

The good news! I'm home! We arrived home last night around dinner time.

I was doing so well with this treatment initially. It was all going so smoothly...until they got me up to the 50mg of Tigercycline. I had one really bad nausea day in the hospital, but now it's just annoying. It's very similar to morning sickness, but I have no little bundle of joy at the end of the rainbow to look forward to. I am on anti-nausea medications, which mask the nausea, but make me EXTREMELY exhausted. I guess it could be much worse, but this low-grade nausea and exhaustion is wiping me out! So much so that I have no interest in picking up a book or the computer. Writing this blog post is a stretch. So much so if they were to offer me a wheelchair now I would gladly accept.

I am also overwhelmed. I have so many medications...it's difficult to keep everything straight. I have to mix my own IV's before administering. I have to use a syringe and measure out sodium chloride and inject it into a vial of powder, which is the actual Tigercycline. I have to wait for it to dissolve, then measure it our with the syringe and inject it into the IV container with the saline. Then I move on to the SASH procedure. I then have to take a syringe and measure out a specific amount of Amikacin that I need to mix with albuterol and nebulize. A HUGE thanks to a fellow CFer who got me out of the Amikacin jam I was in!!! You know who you are! In total, I am on 4 antibiotics, two anti-nausea meds, nebulized albuterol, singulair, Vitamin D and a super-strong probiotic. I can't believe the body can tolerate this much medication...this many chemicals! I can't help but think about what my body is going to suffer, due to this invasion. I really hope the benefits outweigh the abuse to my body.

Basically, I'm just laying low. I'm sleeping tons and trying to spend any time awake with my family. I really wanted to be well enough to get back to working out, but it doesn't look like that's happening anytime soon. I'm just hoping my body gets used to these drugs and that I gain some energy over the next few weeks. It will be a huge bemmer if I feel like this for the next 8-12 weeks while on these IV's.

A huge thanks to my mom for watching my kiddos and doing all of my laundry while I was in the hospital. That was priceless. Another huge thank you to my amazing friend, Elizabeth, who brought dinner over for my family last night. It was such a whirlwind of an evening, but it was great to take some time out to eat yummy food at the table as a family without having to worry about cooking! These were the greatest gifts we could have been given yesterday :-)

The Before & The After ~ Thoughts on CF and 9/11

2010-09-11T08:56:00.004-04:00

Today I am reflecting on that most horrible of days 9 years ago. I remember every detail like it was yesterday. I was in my car driving on 90 West heading toward Toledo. I had a meeting at a hospital for work later that morning. Around 9am I got a call from my boyfriend, now my husband, saying that an airplane just crashed into the World Trade Center. Details were still sketchy. We thought it was just horrible that a plane crashed and it was into a major building. I had more than an hour left on my commute, so I listened to details on the radio. Soon, my husband called back to say that a second plane hit the other WTC tower and that a plane has been hijacked and is heading toward the White House. The visceral feeling that stirred in my gut was something I had never felt before. Now it was certain that the US was being attacked. I felt helpless. I felt unsafe. I felt panicked.

My husband, as a police officer, was called into work. He seemed to remain at work for days on end. Everyone was on high alert. They needed every force available. My father then called me during my commute. He had to work for days on end too. He worked for an airline and was dealing with stranded passengers for days. He was the one who told me about the path of the forth plane. The forth plane was known to be hijacked and made it to the Toledo, OH area, turned around and was heading back toward Cleveland. It was somewhere just overhead when my father called. He was concerned. This made me concerned. I couldn't see the plane, but I know that they were in contact with the Cleveland air traffic control when the plan crashed in Shanksville, PA. If it had crashed 15 minutes earlier it would have been in Cleveland. Only later did I start thinking about what was happening on that plane when it was right overhead. This would have been the time that the passengers were planning their attack on the hijackers. Some were already dead. People were desperately calling their loved one's to say their final goodbye's.

All of our the buildings in Cleveland were evacuated. Everyone was sent home. I was stuck in Toldeo. I finally arrived at the little community hospital in Toledo, OH. My first look at the destruction on TV was in a Cancer Center waiting room with many cancer patients waiting for their treatments. I was frightened and felt shaky. I went forward with my meeting, but cancelled the remainder of my meetings so I could get home. I needed to be home. I couldn't be in this city that was not my own, with people I did not know, at this scary time. When I arrived home I didn't feel any safer. I was no less confused. I was glued to the TV footage. I felt helpless.

Even today I automatically think of any event as being before 9/11 or after 9/11. If I am reminiscing with a friend and we talk about something that happened in 1995, my automatic first thought is that it happened before 9/11. If I see a picture from the year 2000, sometimes I examine the faces and think about how none of us knew what was to come, only a year in the future.

Often times tragedy creates a before and after situation...where things were one way before and they will never be the same after. It can happen in an instant. I think for many people the diagnosis of Cystic Fibrosis is their moment in time that changes everything. I don't doubt that for quite some time my diagnosis was my mother and fathers moment in time. I was diagnosed at the age of 2. In my mind, I never had a before when it comes to CF. My CF has been my always. I sometimes think about if it would have been better to have been diagnosed in adulthood. Would the lack of knowledge about CF have made growing up easier. I'm not sure. I know that my life would probably look very different. I selected my career as a result of my personal experience. I may be something completely different than a Social Worker had I not had my earlier experiences. I had my children before the age of 30...there was somewhat of an urgency. Maybe if I hadn't known I would have taken my time and have totally different children now, or none at all. It's just interesting to think about...

So today I am thinking a lot about 9/11, the people who were lost, the fear we felt as a nation and all that has happened in these nine quite interesting years...

Day 2... ZZZZZ's

2010-09-10T19:27:00.002-04:00

So we're coming to the end of day 2 and shockingly I am still tolerating all of the meds they have given me. The Tig that causes severe nausea is being given to me in phases. I need to get to the point where I can tolerate 50mg. Last night and this morning I was given 12.5mg. Tonight we will bump it up to 25mg. I have had NO nausea at all! I am thrilled! So much so that I had chili cheese fries and Resses Peanut Butter Cup pie for lunch! Maybe this is Operation Gain 20 Pounds, instead of Operation Kick Mycobacterium's Ass???

Today I had a complete hearing and vision screening. We needed a baseline so we know if I start losing or having changes with my vision or hearing along the way. An interesting thing happened on the way to these screenings. They came to pick me up in a wheelchair! I was horrified! I am NOT sick after all. I pleaded to please be permitted to walk...the appointment was several buildings away. They would not budge. I know it's for liability purposes, but I was still horrified. We have to wear masks whenever we leave our room on Rainbow 7, but we're allowed to take them off when we leave the floor. To top things off, the transporter made me wear my mask throughout the entire transport. I AM NOT SICK. I can walk...I can even run... I know this was a psychological thing for me. People stared in the hallways...people stared in the waiting room. I felt like I was having an out of body experience. Look, I was only going to get my hearing and vision checked. I'm sure these people thought I was on deaths door...PICC in my arm...mask on my face. As I was sitting there, angry, imagining what I must have looked like, I started thinking that maybe I really am sick. Maybe that's why I'm in the hospital, on IV meds along with a myriad of oral medications trying to kill the infection that has infested my lungs. Maybe I really am sick after all?

After the 3 hours it took to fully examine my ears and eyes...some tests I have never done before...I regained control. I again began pleading my case about not needing transport back to my room. They said that they could not release my chart to me, they could only release it to someone transporting me. I told them that I would be walking back to my room now and that they could send my chart back later. I left. Whew.

I have obviously had to be transported around hospitals in wheelchairs and beds in the past, but it just hit me differently this time. My friend Josh said he was happy I had this experience. He told me it sounded like a humbling experience. That it was... That Josh is a smart boy!

So the only really exciting thing that has come out of these few days is that I have gotten about 18 hours of sleep, including two big time naps both days. I guess that's more exciting for me than you, the reader. I think living on 3-5 hours of sleep for the past few weeks led to my body just shutting down as soon as all expectations of me lifted. I'm actually getting ready to get some more zzz's. Feels good and I feel great so far!

Day 1...Not So Bad!

2010-09-09T21:26:00.003-04:00

Well, I've made it to the end of day one. I got my PICC placed very soon after arriving. I posted some gory pictures they took of the procedure on Facebook. I wanted to see what they do, but I refused to watch the action live. This placement was not as easy peasy as I would have liked. They stuck me twice and tried to thread the line, only to get resistance. That was a crazy feeling. The third time was a charm. Even with all of the Xanax, my anxiety was increasing with each failed attempt. I could feel my face getting really hot. Now that I've had a PICC in the past I feel completely comfortable with this thing. I've already laid on that arm to sleep. It took weeks for me to do that last time! I am officially a PICC pro! I'm not sure this is a good thing?

Today we started the Tigecycline in a very small dose. This drug is know to cause horrible nausea, so I was given Zofran before even being given the med. Tig is the one drug that I will do for 8 weeks by IV. We also started the Linasolid and Azithromycin oral. I had no side effects at all! At least not yet... Tomorrow I will have my vision and hearing test completed. We need to get a baseline, since vision and hearing loss are side effects of using these drugs long-term. Since I will be on everything, except the Tig, for a year we really need to keep an eye on these things.

All in all it was a good day. I fell asleep at 5pm and slept until 9pm. Think I may have been tired??? Thank heavens for Ambien, which will likely keep me on a good sleep schedule! I plan to get out of here on Monday if all goes well. If it goes like it has so far, that should be no problem. I'll finish the 8 weeks of IV's from home. I think I'm pretty much a pro at that now too. I wonder if this is a good thing?

My First Shot at Video Blogging

2010-09-06T17:46:00.004-04:00

I'm thinking I may be video blogging more while I'm in the hospital. Just wanted to say "Hi" and make sure I know what I'm doing!

My Wish for the Upcoming Year

2010-09-03T15:17:00.008-04:00

I sometimes cry in the shower. It's private and there is no mess to clean up. I can also get the tears out of the way before I put on my happy face for the day. It works for me.

I don't cry for myself. I cry for my family, especially my beautiful children...especially my daughter, Emma. Emma has had a really rough 2010. Finishing her first year of elementary school, the 1st grade, proved to be very traumatic for her. Emma's 1st grade teacher was diagnosed with a rare brain disease in February and died on May 3rd. This was the teacher that Emma told me earlier in the school year, before she became ill, was like a second mother to her. What was supposed to be a typical right of passage for any child, completing first grade, became a lesson in grief and loss for Emma. Watching my daughter grieve and not being able to take away her pain was excruciating. Starting in February Emma started complaining of belly pain. Many visits to doctors and specialists later, we came to the conclusion that these were somatic symptoms. Her anxiety actually makes her physically ill. Whenever Emma said, "Mommy, my belly hurts", she's really saying, "Mommy, I'm scared" or "Mommy, I don't feel safe". I am certain I have the only 7 year old child who walks around with a pocket full of Tums everywhere she goes.

The school year ended and so did the belly aches. Until I was hospitalized in late June. We had a few belly aches and some behavior issues that were unusual, but nothing too bad. I was only in the hospital for 3 days and on IV's for 2 weeks. I felt like we got through it without too much trauma for the children. We told the kids the what's and why's of everything that was happening with mommy and have been honest in an age-appropriate way all along. A few weeks ago my husband told me that Emma asked him if he had a disease.

THAT BROKE MY HEART...

She's taking stock of her options. She knows that not all people with diseases live. She learned first hand that sometimes even being a really good person doesn't save you from the harsh realities of the cycle of life. She learned that anyone's time can come too soon. She learned all of this in first grade. She knows that Mrs. Williams had a disease and Mrs. Williams died. She knows that mommy has a disease, she's deducing that mommy may die. I have told her that the doctors are doing everything they can to make sure that I get and stay healthy for many years to come. I know from working with cancer patients that lying to children about disease is not recommended. I can't tell her I'm going to be OK. I don't know that I am going to be OK. The truth is that my disease is terminal. I have been aware of this truth since the age of 2. The truth is that most of us, more than half, with this disease will die before we reach our 40th birthday's. A lucky few...a handful...will reach their 50's. Things are trending in a positive direction, but who knows if we will find the illusive cure in my lifetime. I'm getting older.

So what do I tell my daughter? How do I tell her? How can I take away her fear and pain?

There will be no escape over the next year. I will start my treatment on Thursday and will be treated for ONE YEAR or longer. I will be on IV's, nebs and oral antibiotics for the first 8 weeks. Then about 4 months on nebs and the remainder of the year will just be easy oral medications. This is really powerful stuff. I will be getting millions and millions of milligrams of medication. It will likely make me sick. I may get worse, before getting better. There will be no hiding this from my children. I am going to do everything I can to normalize this experience for them. Similar to the approach we take with my Vest treatments. I let the kids put the Vest on and sing. They love it! To them it's just something mom's have to do every day. Yet, they know that normal mom's aren't hooking up their own IV's at the breakfast table. They know that normal mom's don't walk around with PICC lines. Normal mom's don't have a home health nurse come weekly to take labs and do dressing changes. I'm going to do my best to be strong for my kids. Maybe I'll let my daughter hook up my IV's sometimes. Let her feel involved. I'm going to smile through it and tough it out. As long as I can use my blog to express my weaknesses, I will do the best I can to "stay strong" in the real world. I have GOT to protect these children...these gifts that I have been blessed with...

I just worry about my Emma. My wish is for her to open up to us. My wish is for her to not be frightened or lonely. With everything that will be going on with mommy, my wish is for her to feel safe. My wish is for me to have enough energy to be able to cater to my children's' needs. My wish is for no belly aches. If you're the praying kind, please pray for no belly aches...

il bel far niente

2010-09-02T09:00:00.001-04:00

Thankful Thursday!

il bel far niente is "the art of doing nothing" in Italian.

This Thankful Thursday I am thankful for my employers awesome disability benefits! I have had a job without a hiatus since the age of 16. I took two maternity leaves, but I had a needy infant to care for during each of those leaves. That was really hard work in and of itself. I have worked part-time when going to school and full-time whenever not in school...my entire life. I feel very fortunate to have had the health and energy to have been able to work. This is not the case for all CFers. With a body that is continually fighting infection, many are just too fatigued to be able to work and take care of their health. In the past couple years, since my health started declining, I have been exhausted. Honestly, I think it's a combination of my disease process, working full-time, raising 2 1/2 kids, being a girl scout leader, trying to work-out 4-6 times a week and managing a household. It makes my head spin just thinking about it.

Next Wednesday I will be starting a 3-4 month medical leave from work. I am going to focus entirely on my health and family. I am excited beyond belief! I can hardly contain myself! I feel like I'm preparing for vacation. Jealous? You should be! Unfortunately, I'll probably be puking my guts out the entire 4 months from the harsh medications I will be taking, but at least I won't be puking and working at the same time! I can focus entirely on puking.

So during my leave I am going to focus on "il bel far niente". I will be infusing, nebbing or vesting for about 5 hours a day, but when I'm not... I am going to try to meditate to clear my mind and put my soul at ease. I am going to rent a movie each week. I am going to read, read, and read some more. I'm going to play with my kids. I am going to rest. I am going to heal. I am going to wear my favorite Cleveland Brown fuzzy slippers most of every day. I am going to even practice doing nothing at all!

I need your help. Please suggest some must read books and must see movies. I plan to keep everyone posted on what I'm reading and watching.

By the way, I love my job! I really, really do! BUT...Yay for awesome disability benefits that will afford me this time to get healthy and heal!

Seriously!!!

2010-08-30T16:55:00.006-04:00

OK, so we have a plan. Funny thing is that it is none of the plans I had discussed with Dr. Dazzle last week.

I spent part of the day on the phone with pharmaceutical company's this morning. The Amikacin that I need is still out of stock. I was trying to find out what I needed to do to get this drug that I NEED! I was BEGGING for my drug. What I found out is that there are 3 pharma companies that manufacture Amikacin in the US. Two of them had to shut down production at the FDA's insistence, due to violations in the manufacturing plant. The third company was shut down for several months, built a new facility and is now just getting up and running. They are the ONLY company of the three that is able to manufacture Amikacin in the US right now. They order the raw materials they need 18 months in advance. That many months ago they did not know they would be the only one's producing the drug. Basically, they are needing to produce 70% more product with very limited resources. Right now they have NONE. They project that they will have it back in stock in early October. Then they have to fill all of the back-orders, so that initial supply will go quickly. Another reason for the shortage is that Amikacin is a generic drug. It is not expensive and not reimbursed at a large rate. Therefore, it really is not a financial priority for them to develop it. It SUCKS!!! I have never been through this before and neither have my doctors. Getting the medication from outside the US is not even an option, because it is an IV drug and my US docs can't administer it unless they get it in the US. If I lived anywhere else I could get my treatment...but I live in the US and this medication is too cheap to be a priority. Can you tell I'm a little peeved? Was I really calling around trying to find my drug, just to be told it's on back-order? Look people, this isn't a sweater that I really want from a catalogue...it's a medication to save my life!

The Infectious Disease specialist at NIH will not be able to see me for a couple months, but Dr. Dazzle consulted with him on my case over the weekend. Have I mentioned how much I appreciate Dr. Dazzle? He has been amazing! We decided to move forward with a different treatment plan. This is really my only option for right now, so no more decisions to make on my part. Oh, happy day! That was probably the best news I've gotten in weeks! Maybe God does work in mysterious ways? My decision was made for me after all. No Amikacin = no other options.

Next Thursday I will be admitted, have my PICC placed, then start on an 8 week course of Tigecycline, Azythromycin and Lenasolid. The Tigecycline is IV...the other two are orals. We are biding our time. Hopefully, when the 8 weeks of IV's are completed the Amikacin will be in stock. I will then take that nebulized for several months. Hopefully, it will be as effective as it would have been in IV form. I feel like we're putting a band-aid on the situation until we can have access to the big guns. Have I mentioned that I'm a tad bit upset?

On a positive note, I will get to see my son off to pre-school next Wednesday. They allowed me to put off my admission until I could make sure my kids were in school and settled. Yay for flexibility!

Kerry's Wish

2010-08-28T21:17:00.020-04:00

This morning Kerry Hutchins, a 33 year old CF patient from Michigan, died from CF while awaiting a lung transplant. She was on the list for three years, but the right set of lungs did not arrive in time. Kerry was a mother of two boys. She had too much to live for...too much living left to do. This was the first article written about her in an attempt to raise awareness about the importance of organ donation.

http://www.mlive.com/news/grand-rapids/index.ssf/2010/08/lung_transplant_patient_organ.html

Few people are aware that in many states expressing your wishes when getting your drivers license renewed is not enough. You have to actually sign up to ensure that your wishes are known. This is what Kerry wanted. She wished that people would make the decision to give of themselves, even after death, after hearing her personal pleas.

This is another article that was written this past Wednesday, when the doctors took Kerry off of the transplant list. She was no longer strong enough to survive a transplant.

http://www.mlive.com/news/grand-rapids/index.ssf/2010/08/sparta_mom_awaiting_double_lun.html

This is an article that was written today. Announcing Kerry's death, but also sharing her final selfless act. Even though Kerry's body was broken, she chose to donate the tissue and organs that were still viable. Through her death, Kerry gave life. What an amazing example!

http://www.mlive.com/news/grand-rapids/index.ssf/2010/08/sparta_mom_awaiting_lung_trans.html

A few weeks back I mentioned to someone that another one of my Facebook friends died of CF. I have 6 friends who have died in the past 6 months from the disease we share. This person, not in an insensitive way, told me that I should stop making friends with people who are dying. That made me stop and reflect. It is hard when someone dies from CF. However, I know that the support that I receive from the CF community is so valuable that I am willing to create relationships with people with uncertain futures, even if that means I will have to cope with many losses as a result. They are the only one's who really understand what it's like to be in my skin...to breathe with my lungs. When I have a question, they give me answers and advice. When I am down, they lift me up. When I am up, they cheer me on. It is 100% worth it! I just hope, hope, hope that I am able to be as supportive to them at times as they are to me...

I never had the pleasure to meet Kerry in person. However, I feel the need to spread her message.

PLEASE, I beg of you, become an organ donor! Please go to http://www.organdonor.gov/. Save a Life!

Out of Stock!

2010-08-27T17:14:00.009-04:00

I had a great, 2 1/2 hour long appointment with Dr. Dazzle today. We have a plan...sort of. You see, Dr. Dazzle, would love to start treating my m. abssessus. However, the Amikacin, one of the most important drugs that I need to supress this infection, is OUT OF STOCK! In preparation for my appointment the team attempted to get their hands on some Amikacin and they got the same story from every supplier...none in stock. What the hell!?!?! The hospitals don't even have a back-up supply. So if you are from outside of the US and you think you can get your hands on some Amikacin for me, I am willing to pay top dollar! I feel like it's Christmas time and I'm on the hunt for zhu zhu pets... Grrr!

Dr. Dazzle assured me that we have some time. No major damage will be done in the time we take to figure this thing out. My second culture came back positive for mycobacterium, but they are going to wait the few weeks to ensure that this second is also the abssessus and not avium strain. Dr. Dazzle also wants me to go see the CF/mycobacterium guru. He's supposed to be the best in the US. Dr. O practices Infectious Disease at the National Institute of Health in Bethesda, Maryland. Hence, I'll be packing my bags to head to Maryland soon. I love NIH! So many important medical discoveries have come out of that place. I participated in a clinical trial there about 12 or 13 years ago. I feel very confident going there for another opinion. I do not, however, feel comfortable seeing an Infectious Disease doctor. Couldn't they call it something else? I can just imagine how rampant my OCD is going to be while sitting in the Infectious Disease waiting room. I think I'm going to have to double mask and double glove. No, Dr. O, I will not be shaking your hand! Crap!

Dr. Dazzle also told me some good news about treating the m. abssessus. First, he consulted with some doctors at other centers about the possibility of doing these antibiotics as a combination of orals and nebulizer treatments. There have been a handful of people very recently who have had some success with this treatment method. However, it is a brand new approach and is not proven. My other option is the IV antibiotics. Since I know a few people who have had m. abssessus and have been on IV's anywhere from 7 months to 2 years, I assumed that would also be my plan. However, Dr. Dazzle said that our goal would be for me to tolerate the IV's for 4-8 weeks! That's it! What a relief! The goal will be 8 weeks, but if they get too toxic we will pull the plug. This is the more standard method of treating m. abssessus...a bit more proven.

The direction we go is completely my choice. Honestly, I think I am going to go with the IV's. I don't want to spin my wheels doing something that may or may not help. I would rather do the IV's. If they get too toxic we can pull the plug, knowing that we still have the orals/nebulized option available. It will be so helpful to hear what Infectious Disease guy has to say!

So, Dr. Dazzle and I talked for 2 1/2 hours and not only about mycobacterium. Many other things were addressed. I am vitamin D deficient. I've known this, but have been too lazy to take it seriously. Honestly, I doubt that anyone who lives in Cleveland is not Vitamin D deficient. The sun does not visit us all too often here in C-Town. What I didn't know was that my level is 10, where normal starts at 50. Some very recent studies have shown that vitamin D deficiency may cause increased lung inflammation. Hence, I am starting 2000 IU's of vitamin D daily.

I have also not been on ANY nebs for many years. I always wondered why. It turns out that my previous doctor didn't think they were necessary. Dr. Dazzle thinks I should be on something. We discussed the differences between Hypertonic Saline and Pulmozyme. Since I have a past history with Asthma we decided that we would start out trying Pulmozyme. I get the first month free, so if I don't like it we can always switch to the Hypertonic Saline. So I will now be doing Pulmozyme every night during my Vest treatment. I am also going to continue on Doxy until we get my treatment plan all figured out. It keeps my cough at bay a bit, so why not?

Anyway, I have nothing but positive thoughts about the future. I really feel like Dr. Dazzle has my best interest in mind. It was interesting to find out that he graduated high school that same year as me! I love that we communicate the same way. We both start every sentence with the word "so". Here's how it went...

Me: So what are my chances of becoming diabetic in the future?

Dr. Dazzle: So there really isn't a percentage I can quote you. It's not certain that you will become diabetic and the fact that you are pancreatic sufficient works in your favor.

Me: So what are the odds that I will become pancreatic insufficient in the future?

Dr. Dazzle: So, again, I don't have any statistics to quote you, but as a CF patient you already have a compromised pancreas. It's just that your level hasn't reached to point yet where it indicates that your pancreas is not functioning. However, we typically start enzymes based on symptoms, not strictly on levels. So just keep me updated with your symptoms and if you see any changes in your digestion.

Me: So then I would start enzymes?

Dr. Dazzle: So, yes, but probably just a couple pills prior to each meal.

This went on and on for 2 1/2 hours. It was the greatest! Today's appointment was the greatest! I truly felt heard, and that meant the world to me. I'll be starting some more treatments and packing my bags for Maryland soon! It's slowly coming together :-)

By the way, I'm serious about the Amikacin. For all of my Canadian, British, Irish and Australian friends out there...if we can find a supply Dr. Dazzle is willing to do what it takes to get it here... Let me know!

20 Questions???

2010-08-23T21:51:00.012-04:00

Seriously...I've already got 20 questions for my new CF doc! For many years I have given my cancer patients the advice to keep a notebook with them in the days and weeks prior to an important appointment, so they can write down every question that comes to mind to ask their oncologist. Research shows that people on average have 5 questions that they would like to ask their physician, but they typically only get to ask 2 or 3 of them. They either forget, become afraid to ask or the appointment time just runs out. I am going to ensure that I don't forget, so I've been carrying my little pink notebook around with me everywhere. Here's what I've got so far. I know that there are no answers for some of them, but I'm going to ask anyway. Let me know if you think of any more good questions!

This is just a little peek into what's been circling around in my brain for the past few weeks...

1) Can I get a new Flutter? My old one is no longer of use, since my children thought the metal ball inside was a toy. That most important little ball is missing for good...grr.

2) Do you think my symptoms I've been having over the past 2 years are from the mycobacterium abssessus or did the staph I've cultured my entire life just start acting up?

3) What are the dangers of waiting to treat?

4) If I do these long term antibiotics, should I get a PICC or a port?

5) Do I need to start this treatment right away or can I choose when I want to start?

6) Are there any stats specifically for the Delta F508 & R117H gene mutation?

7) Do those with R117H have the same life expectancy as the general CF population?

8) What is the probability that I will get CF related diabetes eventually?

9) What is the probability that I will one day become pancreatic insufficient?

10) I read that people with the R117H mutation are more susceptible than other CFers to getting Pancreatitis. What is my probability of getting Pancreatitis?

11) What are the symptoms of Pancreatitis?

12) I know that one of the side effects of one of the antibiotics I will be using is neutropenia (low white blood count). I already have a low white count. It falls between 2-4, when normal is 4-11. Can I get Neupogen injections to increase my white count and prevent complete immune system failure?

13) I am also concerned about hearing and vision loss with these antibiotics. What would we do to monitor this and how often?

14) How often will I have to infuse (how many times a day) if we do IV's?

15) How long do you anticipate I will need the IV's?

16) How long will I have to be in the hospital initially, knowing that I have to be desensitized from the Clarithromycin?

17) I went to the ER last time I was on IV's for chest pain, which ended up being pleurisy. I have had pleurisy a few times prior to this. I had a 4 day episode again a little over a week ago, but in a different area of my chest. Do you think this is pleurisy?

18) I read somewhere that there is not cure for m. abssessus, except for a lobectomy or lung transplant. Is this true, and if so is the goal of treatment remission and not cure?

19) I also read that only 56% of patients respond to this long-term antibiotic treatment. Are there indicators that might predict success?

20) Last time I was admitted my thyroid level was low. Can we check that again?

OK, ok...I've got a few more than 20! I'm glad I have an hour long appointment!

21) If I likely got this from my own household water, is there a way to check if this is in my water-heater or anything I can do to prevent getting this again?

22) Should I be on Hypertonic Saline? It seems like the cool thing to do nowadays!

23) Right now I do one Vest treatment a day, which can be nixed if I do my aerobic work-out. Should we continue with this regimen, or should we increase the frequency?

OK, now that's all I've got for now. I still have 3 days left, so we'll see how many I have by Friday???

Joy & Confusion

2010-08-21T12:37:00.024-04:00

We are just back from an amazing vacation! We went to Hilton Head Island, South Carolina and had a blissful time! These are just a couple snapshots from the trip.

I was so happy that I was feeling well physically, even after going off of the Doxy. I was also able to practically forget about the huge decision I must make in the coming weeks about what to do with my health...up until 5am the morning we left to come home. I woke up in a panic. I think knowing that I was returning to the real world and will have to face this burden again, put my mind in a tailspin. I was so certain that I wanted to be aggressive with this m. abssessus infection last week. Today, I just don't know??? I'm so confused. It may just be the fear setting in...

Just prior to going away on vacation I had another long conversation with Dr. Dazzle. I learned that I need to avoid enclosed showers and hot tubs. I likely got the m. abssessus from breathing in steam from hot water. Dr. Dazzle wanted to warn me about these things to avoid, so that if we treat I do not immediately reinfect myself. I thought I had gotten this from my past surgery, but Dr. Dazzle said that I would have had to get it from the tube that they used to intubate me. If that were the case, they probably would have had an outbreak of mycobacterium in the hospital at that time. It's more reasonable to think that I got this infection from water. I did still go in the hot tub on vacation... Hey, I'm already infected. I may as well enjoy one last dip!

I learned that the 3 antibiotics I will be taking if we treat are Clarithromycin, Amikacin and Cefoxitin. They are IV and I will need them for 3 - 12 months...possibly longer. I can't remember which drug, but one of them is extremely toxic. The possible side effects include hearing loss, kidney failure and neutropenia (low white blood count = weak immune system). I've talked to several people who have had these side effects. They can be brutal. Also, since I'm allergic to Penicillin, I will have to be desensitized to the Clarithromycin. I will be inpatient and they will administer very small doses initially, until they can give me the full dose without side effects. These are the potential risks of taking the aggressive approach. Maybe I will experience none of them...maybe?

My other option is to do nothing. To wait it out. To wait until I have more symptoms. To wait until we see lesions.

This reminds me of the story of Damocles Sword. The term means "an ever-present threat; an impending disaster". Damocles was a poor man in ancient Greece who was jealous of the ruler Dionysius, tyrant of Syracuse, for his wealth. The praise annoyed the king, so he decided to teach him a lesson. He held a party in honor of Damocles. To prove a point, Dionysius hung a sword by a single horse-hair over Damocles' head. Damocles was no longer jealous, as he realized that even those who appear to enjoy great fortune face fears and worries." This is how I have always felt about my CF. I have always been fearful of the sword dropping. I know it will, it's just a question of when. I think this is how I will feel every day if I choose to hold off on treatment for now. Then, what happens if it is beyond the point of being able to be treated effectively when it does progress? Oh boy, I have so, so many questions for Dr. Dazzle.

I feel like I'm teetering between two extremes. This is one of the toughest decisions I have ever had to make. What's weighing heaviest on my shoulders is thoughts about how my family will be taken care of if I'm out of commission for such a long time. I have complete faith in the fact that my husband and family will keep things afloat, but I'm the mommy. I'm supposed to take care of everything.

What if I choose the wrong path? I had a 12 hour conversation with God on the ride home from vacation. I asked...no begged for a sign. I really wish someone else could make this choice for me. I wish someone would tell me what to do.

I turned 35 yesterday. I spent the day with my family and the evening with my dear friend, Elizabeth. It was a fabulous day! Elizabeth was my shoulder to cry on during my last round of IV's. The advice she gave me last night was that I need to make this decision with my heart, instead of my head. My head is just overflowing with too many "what-ifs". I just wish I knew what my heart was trying to tell me. Can anyone hear what my heart is trying to say? I am hoping beyond hope that I have more clarity after my appointment next Friday.

A Difficult Decision

2010-08-11T12:49:00.004-04:00

Today I had a clinic visit. It was hard. I think it was the most emotionally exhausting appointment I have ever had. I decided to transition from my pediatric pulminologist to an adult pulminologist. There were many factors that went into my decision that I won't discuss here, but I thought long and hard about the transition before deciding to take the plunge. Today was my last appointment with Dr. Bob, who I have seen for over 20 years. There were many tears...mine, not his. Mostly from frustration...a few from sadness. I know I can't be fixed, but I at least want to be heard. I am at a turning point with my health and I am excited and scared about the next chapter all at the same time. That's all I have to say about that...

So we have a plan, sort of. I was wrong about culturing MAC. MAC is Mycobacterium Avium, which derived from birds. My Mycobacterium Abssessus is a whole different strain. It turns out it is the worst of the mycobacteriums. If to treat and how to treat is controversial. My new doctor, we'll call him Dr. Dazzle is very aggressive. To say that I like this approach is an understatement. After he introduced himself he said that together we were going to work on keeping my PFT's baseline for the next 50 years. We will not tolerate drops...not one bit!

That being said, we decided that we will move forward with kicking this mycobacterium's ass. First things first, we need a second positive culture result. I gave my lovely "sample" and will follow up with another appointment the Friday after next. Then we're really going to lay it all out there.

What I do know is that I was wrong about the treatment, in a bad way. It turns out that 2 of my 3 drug sensitivities are IV drugs. Therefore, what I imagined would be 12 months of easy peasy oral antibiotics, may be 12 months of IV antibiotics. It may be more like 3 or 6 months, since IV's are so much more potent than orals, but in any case, it's going to be a more difficult path. I wasn't at all prepared for that news. I wasn't prepared for the news of another hospital stay and PICC line. I wasn't prepared for the fact that I am likely going to have to take short-term disability from work for who knows how long. I wasn't prepared for the emotional transition process. I just feel ill-prepared.

I am, however, prepared to feel well again. I am prepared to give myself the rest and care that I need to heal, if that's possible. I am prepared to fight for every single percentage point of lung function. If for the next year I have to Vest, neb and infuse multiple times a day to win this battle, then that's what I'll do! And Dammit, I'm going to try to do it with a smile on my face! I'm sure that will be the hardest part... Fifty years from now, Dr. Dazzle and I, as a team, are prepared to say that I was able to beat the odds. Then, all of our hard work and effort will be rewarded with more hours, more days, more breaths...

A Letter to Patients

2010-08-06T21:35:00.014-04:00

My friend Patti posted this in her FB notes today and I thought is was too good not to share! I will be taking some of this advice to heart...

A Letter to Patients With Chronic Disease

Dear Patients:

You have it very hard, much harder than most people understand. Having sat for 16 years listening to the stories, seeing the tiredness in your eyes, hearing you try to describe the indescribable, I have come to understand that I too can’t understand what your lives are like. How do you answer the question, “how do you feel?” when you’ve forgotten what “normal” feels like? How do you deal with all of the people who think you are exaggerating your pain, your emotions, your fatigue? How do you decide when to believe them or when to trust your own body? How do you cope with living a life that won’t let you forget about your frailty, your limits, your mortality?

I can’t imagine.

But I do bring something to the table that you may not know. I do have information that you can’t really understand because of your unique perspective, your battered world. There is something that you need to understand that, while it won’t undo your pain, make your fatigue go away, or lift your emotions, it will help you. It’s information without which you bring yourself more pain than you need suffer; it’s a truth that is a key to getting the help you need much easier than you have in the past. It may not seem important, but trust me, it is.

You scare doctors.

No, I am not talking about the fear of disease, pain, or death. I am not talking about doctors being afraid of the limits of their knowledge. I am talking about your understanding of a fact that everyone else seems to miss, a fact that many doctors hide from: we are normal, fallible people who happen to doctor for a job. We are not special. In fact, many of us are very insecure, wanting to feel the affirmation of people who get better, hearing the praise of those we help. We want to cure disease, to save lives, to be the helping hand, the right person in the right place at the right time.

But chronic unsolvable disease stands square in our way. You don’t get better, and it makes many of us frustrated, and it makes some of us mad at you. We don’t want to face things we can’t fix because it shows our limits. We want the miraculous, and you deny us that chance.

And since this is the perspective you have when you see doctors, your view of them is quite different. You see us getting frustrated. You see us when we feel like giving up. When we take care of you, we have to leave behind the illusion of control, of power over disease. We get angry, feel insecure, and want to move on to a patient who we can fix, save, or impress. You are the rock that proves how easily the ship can be sunk. So your view of doctors is quite different.

Then there is the fact that you also possess something that is usually our domain: knowledge. You know more about your disease than many of us do – most of us do. Your MS, rheumatoid arthritis, end-stage kidney disease, Cushing’s disease, bipolar disorder, chronic pain disorder, brittle diabetes, or disabling psychiatric disorder – your defining pain - is something most of us don’t regularly encounter. It’s something most of us try to avoid. So you possess deep understanding of something that many doctors don’t possess. Even doctors who specialize in your disorder don’t share the kind of knowledge you can only get through living with a disease. It’s like a parent’s knowledge of their child versus that of a pediatrician. They may have breadth of knowledge, but you have depth of knowledge that no doctor can possess.

So when you approach a doctor – especially one you’ve never met before – you come with a knowledge of your disease that they don’t have, and a knowledge of the doctor’s limitations that few other patients have. You see why you scare doctors? It’s not your fault that you do, but ignoring this fact will limit the help you can only get from them. I know this because, just like you know your disease better than any doctor, I know what being a doctor feels like more than any patient could ever understand. You encounter doctors intermittently (more than you wish, perhaps); I live as a doctor continuously.

So let me be so bold as to give you advice on dealing with doctors. There are some things you can do to make things easier, and others that can sabotage any hope of a good relationship:

Don’t come on too strong – yes, you have to advocate for yourself, but remember that doctors are used to being in control. All of the other patients come into the room with immediate respect, but your understanding has torn down the doctor-god illusion. That’s a good thing in the long-run, but few doctors want to be greeted with that reality from the start. Your goal with any doctor is to build a partnership of trust that goes both ways, and coming on too strong at the start can hurt your chances of ever having that.

Show respect – I say this one carefully, because there are certainly some doctors who don’t treat patients with respect – especially ones like you with chronic disease. These doctors should be avoided. But most of us are not like that; we really want to help people and try to treat them well. But we have worked very hard to earn our position; it was not bestowed by fiat or family tree. Just as you want to be listened to, so do we.

Keep your eggs in only a few baskets – find a good primary care doctor and a couple of specialists you trust. Don’t expect a new doctor to figure things out quickly. It takes me years of repeated visits to really understand many of my chronic disease patients. The best care happens when a doctor understands the patient and the patient understands the doctor. This can only happen over time. Heck, I struggle even seeing the chronically sick patients for other doctors in my practice. There is something very powerful in having understanding built over time.

Use the ER only when absolutely needed – Emergency room physicians will always struggle with you. Just expect that. Their job is to decide if you need to be hospitalized, if you need emergency treatment, or if you can go home. They might not fix your pain, and certainly won’t try to fully understand you. That’s not their job. They went into their specialty to fix problems quickly and move on, not manage chronic disease. The same goes for any doctor you see for a short time: they will try to get done with you as quickly as possible.

Don’t avoid doctors – one of the most frustrating things for me is when a complicated patient comes in after a long absence with a huge list of problems they want me to address. I can’t work that way, and I don’t think many doctors can. Each visit should address only a few problems at a time, otherwise things get confused and more mistakes are made.

It’s OK to keep a list of your own problems so things don’t get left out – I actually like getting those lists, as long as people don’t expect me to handle all of the problems. It helps me to prioritize with them.

Don’t put up with the jerks – unless you have no choice (in the ER, for example), you should keep looking until you find the right doctor(s) for you. Some docs are not cut out for chronic disease, while some of us like the long-term relationship. Don’t feel you have to put up with docs who don’t listen or minimize your problems. At the minimum, you should be able to find a doctor who doesn’t totally suck.

Forgive us – Sometimes I forget about important things in my patients’ lives. Sometimes I don’t know you’ve had surgery or that your sister comes to see me as well. Sometimes I avoid people because I don’t want to admit my limitations.

Be patient with me – I usually know when I’ve messed up, and if you know me well I don’t mind being reminded. Well, maybe I mind it a little.

You know better than anyone that we docs are just people – with all the stupidity, inconsistency, and fallibility that goes with that – who happen to doctor for a living. I hope this helps, and I really hope you get the help you need. It does suck that you have your problem; I just hope this perhaps decreases that suckishness a little bit.

Sincerely,Dr. Rob

And the results are in...

2010-08-05T21:58:00.008-04:00

My culture results finally came back from Texas. I have....drum roll please...

Mycobacterium Abscessus AKA MAC

http://www.cdc.gov/ncidod/dhqp/id_Mabscessus_faq.html

Don't be alarmed when you read that this disease is similar to Leprosy and Tuberculosis. It is NOT either of these conditions. It just has a similar cell structure. I knew that I wasn't contagious, but it's nice to read it here too!

Basically, MAC lives in water, soil and dust. Not sure how I could have avoided those things. It sounds like it also likes to grow on surgical equipment too. Ah...now that made a light bulb go off in my head! I was hospitalized for 9 days with acute belly pain in November of 2007, which resulted in the removal of my gall bladder and appendix. My gall bladder had stopped draining, so we decided to just get rid of the thing all together. The appendix was just a bonus organ removal. The surgeon figured "Why not?", so he did. Funny thing is that the pathology showed that the appendix was diseased and I would have probably needed to have it removed eventually anyway. I asked for a bonus tummy tuck, but the surgeon did not oblige :-(

The great thing about my surgeon, Dr. O, is that he was the one who did spinal surgery on Christopher Reeves a few years before his death. MY surgeon operated on Superman himself! That gave me confidence. I certainly don't blame Dr. O or the hospital, but it seems that I may have contracted MAC from this surgical procedure. It all makes sense to me now. My increased CF symptoms and the drop in my PFT's have all happened in the past couple of years, following the surgery. Truth be told, I will never know for sure how I got this nasty bug, but I'll probably stick with the surgery story.

MAC is typically an infection of the skin. My favorite thing that I read is that "It has rarely been known to cause lung infections in persons with various chronic lung diseases". It's that word "rare" that gets me every time. I am ALWAYS the one who gets the "rare" conditions...UGH! I have a rare genotype of a rare genetic disease, that has progressed in a rare fashion. I also have a rare disease called Undifferentiated Connective Tissue Disease. Now I have a rare lung bug to go with everything else. I just LOVE when my medical team says that they really haven't seen many cases like mine. That means I don't fit into any category that they have experience with and statistics on...Grrrr! I've never had any skin issues, so it looks like MAC decided to take the rare route with me.

I have an appointment next Wednesday to discuss treatment options. If we decide to treat at all it will likely require 2 oral and 1 nebulized antibiotics for about a year. I will have to get my kidneys and liver checked often to ensure that they are handling the drugs well. The abx can also make my white blood cell count drop too low, making me even more immuno-compromised. This makes me nervous about the air travel I have to do for work. I'll chat with my doctor about that issue. Basically, it most likely won't be a walk in the park, but not unbearable either. If I'm given the choice to treat or not, I am going to take the aggressive approach. I don't want to regret sitting on it years from now if it decides to rage out of control. I guess we'll see on Wednesday...

Thankful Thursday ~ Counting My Blessings

2010-08-04T22:39:00.010-04:00

I am going to start trying to devote Thursday's to counting my blessings. I think some other CF bloggers have done this in the past. However, I got my inspiration from my friend, Fayla, who seems to always find a positive nugget within each and every day. She often starts her FB posts on Thursdays by sharing what she is thankful for that day. Fayla also lives with CF. I've had some frustrating times lately and I don't want to lose sight of the fact that I have so, so much to be thankful for...

So... Today I am thankful that I found my CF voice through this blog. I'm thankful for the blogosphere!

Blogging has been such an amazing way for me to let it all out. I rarely talk about my CF at home or with people in my "real" world. Yet, that doesn't mean I don't think about it all of the time. It's really hard not to when you are constantly medicating or treating yourself in an attempt to stay as healthy as the disease allows. Support groups for people with CF are non-existent. We can pass deadly bacteria on to one another, so there is actually a rule that people with CF are supposed to stay at least 3 feet away from one another at all times. On the CF floors of the hospitals, patients must wear masks just to walk the halls. We are literally quarantined from one another. That's always been a difficult reality for me to accept. I wear my emotions on my sleeve, but I have had to shut off this very significant part of my life in order to spare the feelings of those around me, knowing that they couldn't possibly know what I was feeling or experiencing. I didn't want to make them feel helpless, so I just avoided the topic of my disease all together. My parents read my blog. They have both commented on how much they have learned since I started writing.

At first I felt vulnerable sharing such personal information with the world at large. I've gotten to the point where I feel a sense of satisfaction when I click the publish button. I am able to release my feelings, share information and sometimes help someone in the process...all without having to "talk" about CF. It's just too hard to "talk" about CF sometimes. I have received some emails from younger cysters who say that my words, thoughts and story give them hope for the future. Those sentiments alone make blogging worth the time and effort for me. It's helping me and it's a huge bonus if it helps someone else in the process.

I watched 65_RedRoses yesterday. It's a documentary about Eva Markvoort, a young woman with CF who received a lung transplant a few years ago. She was a beauty, wasn't she?

She ended up dying this past March, while waiting for a second transplant after rejecting her new lungs. She touched many people and taught amazing lessons about life and love in her short 26 years. 65_RedRoses was her screen name in the CF cyber world. The documentary followed her online relationships and the importance of the support she received through her blog. Some of her most special friends were people she never met in person. I can completely and totally relate. Since connecting with others in the CF world I have developed some amazing relationships, with some of the strongest souls I have ever "met".

On this Thankful Thursday...I am happy and thankful for my many cyber-friends and for the online support I receive from them each and every day!

But you don't look sick...

2010-08-02T17:59:00.009-04:00

This is something I've heard time and time again throughout my life. I'm not at all sure how to react to this statement. I know for me, disclosing my illness to new people has always been something that has made me feel vulnerable. I sometimes found comfort in the fact that I could keep it my little secret. It's a very personal part of my life that I don't feel like many people in my world (before Facebook) could relate to. It's made me strong and resilient, but also makes me scared and angry. When I choose to tell someone about my disease one of a few things usually happens:

1) They back away slowly and pretend they didn't hear me. I guess it's a little too much information for these folks.

2) They start asking questions like crazy. I like this. In this instance I have a captive audience and they care. They really care.

3) They immediately tell me about so and so who they know who HAD cystic fibrosis. This group always has a story to share about someone who died of the disease. It's just like when your pregnant and everyone wants to tell you all of the gory details about what can go wrong.

4) Then there is the puppy dog eyes, cocked head to the side, I feel so sorry for you look. These are the people who knew someone who died of CF, but refrain from sharing the details. For the record, I never disclose in an effort to make someone feel sorry for me. I beg of you, PLEASE, don't feel sorry for me. That makes me feel awkward. I realize that I have a very blessed life.

5) Or I get the statement "...But you don't look sick". This very effectively shuts down the conversation and we move on to more pleasant topics. I am thrilled not to look sick. However, I know that most CFer's don't always look sick. If only you could see me from the inside out.

I know that this statement is made out of a lack of awareness, but it sometimes makes me feel like my entire experience has been devalidated (if that's even a word). Well, I guess your right then. I guess if I don't look sick, then I must not be sick after all. Forget the fact that I do Vest treatments a couple times a day, am on constant antibiotic treatments and take tons of pills a day.

Maybe it's the meaning of the term "sick". If sick means acutely ill, then no, I am not always sick. However, if "sick" means dealing with a chronic illness on a daily basis to stay alive, then yes, I am sick. I don't like to think of myself as disabled, when at the same time I think I've earned my disabled badge. We CFer's don't like to limit ourselves if our disease doesn't make us. We run marathons, sky dive, climb mountains, have families, work and lead lives filled with the same joys and pains as any healthy person. Yet, some awareness and understanding about the challenges we do face, even though we "don't look sick" is often in order.

Two of the most frustrating statements that are often made to CFer's from people who don't "get it" deal with disabled parking and the smokers cough. I have heard story after story of CFer's parking in handicapped parking spots and being cussed at or put in their place for not belonging there. Then there is the CF cough, which sounds an awful lot like a smokers cough. Nope, we didn't create this cough ourselves. I've heard of people judging our coughs and making rude comments such as "Have another one...". This being said to someone within one of the most anti-smoking communities you'll find. Next time, please hold your judgement!

So even though I live with an illness, that is still considered terminal in nature, I don't want to be considered disabled by the average person. I just want to be understood. I do, however, feel a special kinship with others with illnesses or disabilities. That is why I chose to work in oncology. I can relate.

Here are two blogs from two other special CFer's related to this same topic.

This first blog had me rolling on the floor laughing. Just picturing Josh in this situation makes me smile. Please read!!! Yeah, I got the CF too!

http://www.welcometojoshland.com/2009/02/at-almost-30-i-feel-like-i-have-been_18.html

This next blog was written by Lauren, a 19 year of Cyster, who is wise beyond her years! This is so beautifully put :-)

http://ihavecfsowhat.blogspot.com/2010/06/stand-up.html

OCD at its finest...

2010-07-31T11:38:00.013-04:00

I am in control of everything...until I'm not...

I have always known that I'm a bit Obsessive-Compulsive. It's actually one of my finer qualities. I don't often forget things, because they are constantly spinning around in my head. This has never been a major problem. You won't see me washing my hands raw or returning to my house 14 times to ensure that they stove is turned off. However, the annoying thoughts are there. I see them get worse during times when I have little control over my environment.

We went on a mini vacation this week. We drove down to Cincinnati and went to King's Island Amusement Park for a few days. A great time was had by all!!

Yet, ever since I cultured mycobacterium I have been having thoughts (obsessions) about germs. I have no issue whatsoever with germs in my own home. However, spending several days at an amusement park and hotel just made those thoughts overflow... Hotels are the worst. I will never let my feet touch a hotel room or shower floor. Forget about the remote control...who knows where that thing has been. I have to mentally block out the whole pillow/comforter situation, or I would never be able to get any sleep. The shower curtain is right up there with the remote. If that thing brushes my arm while I'm showering that's another 15 minutes of scrubbing that arm. Then we head to the amusement park. The railings, the safety harnesses...everything is infested with little kid bacteria. It's times like these where I wish everything with germs I needed to avoid would glow red, like in the Lysol commercial. I think the worst thing of all that made me almost panic was the pens I had to use to sign receipts. I have been trying to bring my own pens everywhere I go lately, but I only put what I could fit in my pockets to the park. A pen didn't make the cut. Next time I'm thinking of leaving some cash behind to make room for a pen.

So I noticed these things more during this trip, likely because I have been sick and am worried about what I will catch next. I'm frustrated that I caught this nasty mycobacterium and I haven't a clue where it came from. A normal, healthy person can't even catch these bugs that I have and that I'm susceptible to. I've heard that one of the side effects of some of the drugs I may be taking to treat my mycobacterium is that they can make you dangerously immuno-compromised. I'm thinking I may just have to quarantine myself in my house for the next year. OR I have to get a life and stop worrying so much!

I'm in BIG Trouble!

2010-07-25T13:39:00.008-04:00

Note to self...you need to learn to keep your mouth shut! My husband tells me all the time that I need to try not to make people upset who cook our food and know where we live. He's a police officer, so I'm sure he's seen his share of people retaliating. I, on the other hand, always think telling people how it is in a nice way is what's called a "teachable" moment.

So a couple weeks ago a guy came to our door offering to cut our half-dead tree in our front yard down for a very reasonable price. My husband said he would talk to me about it and that he should come back. The next day the guy came back to the door, cigarette in hand, asking if we made up our mind. He had no problem blowing smoke in my face, on MY front porch. He was talking about the tree and waving that nasty smoking stick around in the air. I told him I wasn't interested right now and kept my mouth shut about the cigarette.

The tree guy just came back a few minutes ago. He asked if we had made our decision. I said that we had decided we would look into another company. He said "but I could get it out of there right now and take it with me today". I told him that, actually, we decided on another company after he came to our door smoking. He apologized profusely and said he has been "trying not to do that". I told him that I had a lung disease and breathing clean air is not a choice, but a necessity for me. I kindly told him that it offended me that he thought it was appropriate to smoke on my dwelling. He apologized again and off he went.

As soon as I shut the door I started kicking myself. I have NEVER, ever been able to get through to a smoker by sharing my story. I think I probably look too well on the outside and their addiction is probably too strong to REALLY hear what they already know. Even with some family members, I have never been able to create a "teachable moment" when it comes to smoking. It gets me angry that people born with healthy lungs would make a choice to destroy them. It angers me that those lungs will not be available for future transplants for me or any of my hundreds of friends with CF who could someday use them for a second chance at life. Although there is some controversy about this issue, it frustrates me that sometimes smokers lungs are transplanted, and this could be a factor in a post-transplant patients death. Something similar to this happened a couple months ago in the UK.

http://abcnews.go.com/Health/Wellness/transplant-patient-dies-receiving-smokers-lungs/story?id=10920607

I am very much in support of people having rights. I believe people have the right to smoke. I wish they didn't, but they have the right. However, I have rights too. I have the right to answer my door without a huge plume of smoke entering my breathing space. I have the right to enjoy dinner out with my family without having to breathe in someones polluted exhale. Thank goodness smoking in public buildings was outlawed in Ohio years ago! I truly think that people who smoke should be mindful of the consequences to others and be considerate when finding a place to smoke.

I feel like I need everyone to know that if anything happens to me, tell the cops to look for the tree guy...ha!!! The tree guy with two missing front-teeth did it ;-)

Disappointments...

2010-07-24T20:20:00.013-04:00

I was taught the principles of the "American Dream" early in life, and I believed in the theory whole-heartedly. If you work hard and do the right thing, you will reap rewards. So I worked hard and did the right thing. Over the past couple of years, and especially in the past month, I have learned that this doesn't apply to Cystic Fibrosis. Sure, being compliant and practicing self-care can, at times, have a positive impact on health outcomes. However, you can also follow all of the rules and your disease can still progress.

I finished my IV's a nearly two weeks ago after having an allergic reaction. I thought I felt better, so the choice was made that I could end this course of IV's after 2 weeks. The first disappoint was that my PFT's did not improve, not even by one percentage point. OK, I can deal with this. Frustrating, yes...but I know this isn't uncommon. I was really hoping, though, that all of my hard work would have paid off in a tangible way. I wanted to see improvement, instead of only feeling it. I wanted hard proof. It wasn't to be had during this appointment. So I rested on the fact that I felt good. My cough diminished and I was no longer short of breathe when climbing a flight of stairs. I also learned a lesson, that I will always go the extra mile and do a third week of IV's.

Much to my dismay, I started getting junky again within 6 days. By this last Wednesday I was having a full-on lung exacerbation. I felt worse than I felt before starting the IV's. For about 4 days I tried to convince myself that this was a normal part of the IV process...feeling worse before feeling better. However, each day was worse than the one before. This past Wednesday I finally called my nurse who said that it sounds like some residual infection was left behind and was rearing it's ugly head again. Disappointment is not a strong enough word to describe how I felt when my lungs decided to fail me so soon after working so hard to improve them. I did everything right, I did what I was told, and I didn't reap any reward...

Back on antibiotics I went. This time just oral Doxycycline. No big deal, really. It's only a bummer, because Doxy and I don't agree when sun exposure is involved. We have a little family vacation planned for this upcoming week, along with a bigger family vacation in 3 weeks. All involve lots of fun in the sun. I bought SPF 100 and am going to keep my fingers crossed. I am already improving vastly. Doxy always works wonders on my lungs. I just always decline right after I go off. Maybe this time the combination of IV's and Doxy will do the trick. I'm hopeful...

Mycobacterium is my next battle to fight. My sample was sent to Murray in Texas last week. I guess my local hospital had to watch the sample for the first 3 weeks to ensure that nothing else grew. The good news is that other than the mycobacterium and staph, it was clear. Now Murray is going to watch the growth for the next 3 weeks, he's figure out my strain and come up with a treatment plan. The treatment plan will likely include 3 antibiotics for about a year. I've heard of it taking both shorter and much longer periods of time for people to eradicate this bug, so I guess we'll see.

This mycobacterium has been another huge disappointment. It may be what has been causing this decline over the past couple years. I am obsessing about how I got it. I travel so much for work, maybe I got it on a plane. I have worked for years with cancer patients, maybe I got it from one of them. That leads to obsessing about how to prevent future exposure to deadly bugs. Should I wear gloves and a mask to clinic visits? Should I start wearing a mask on all of my flights? These thoughts have been floating around and around in my head for days.

The mycobacterium also acted as a reality-check. What jumped off of the page at me when I researched this bug is that only people who are immunocompromised contract it. I guess this means I'm immunocompromised. I mean, I knew I was immunocompromised...but I guess I really KNOW I'm immunocompromised now. There's no ignoring it now. It's frustrating that I can do everything right and I won't be able to change the fact that I have a genetic abnormality that will continue to make me sick. Then, when I get sick, I will likely always need strong medications to get to some level of well. Until I don't...

I guess I would say that June has been disappointing. Not unbearable...just frustrating.

Birthing Jake 4 Years Ago Today!

2010-07-21T18:43:00.023-04:00

Guess who's 4 years old today? That's right, my little sweet pea, Jacob! We celebrated with family on Saturday and I've been stepping on Lego's ever since. Those little things hurt...

I had a very easy pregnancy and birth experience with my first child, Emma. The pregnancy with Jake was great, but the birth was a bit more challenging...after 40 hours of labor. You heard right, 40 hours of labor! I was induced 10 days early, as I was with Emma, because my lungs were getting too crowded. I was admitted into the hospital at midnight the morning of July 21st and the induction process started. About an hour after I arrive a bright yellow "Precautions" sign was placed on my door and nurses started gearing up in space suits just to see me. To my surprise they said that I had MRSA. How did that happen? I hadn't gotten a cultured for about 4 months. Well, I guess I cultured MRSA the last time I was seen by my CF doctor, but they never told me. Huh? It was explained that it could have been a false positive, so they recultured me. The problem being that it would take at least 3 days for the culture results to come back and by that time I would be long gone from the hospital. So they had to take all of the MRSA precautions for this birth.

I started contracting very soon after the initial induction process. I was rearing to go, but there was no birthing room available for me. I spent about 24 hours laboring in a regular room, until I was moved to a birthing room. I was RARELY visited, I'm certain because of how much time and effort it took for them to suit up before popping in. Finally, the time was near. I was ready to push. Wouldn't you know, that little bugger just wanted to keep holding on. Two and a half hours of pushing later, I had a PERFECT little baby boy.

It was exhilarating, but I was exhausted. I ran a marathon in 2002 and this labor experience was 10x more challenging. After about an hour of snuggling I was more than ready for them to take my little bundle of joy to the nursery so I could get some much needed zzz's. Whoa, was I surprised to find out that my little guy was now considered a MRSA patient, so he was not allowed to go to the nursery with all of the "normal" babies. Seriously! I wanted to cry...but I did it with the help of my hubby and family.

Discharge time was nearing and my son hadn't been circumcised. I had been asking about it every 10 minutes since Jake was born. Nobody had to guts to tell me that since my baby couldn't leave the room, they couldn't circumcise him. What?!?!?! They wanted me to bring him back at a later date to do it. That just was not happening. So I pulled out all the stops...I turned on the water works. I cried and cried... *whimper* "I can't possibly..." *sob* "...bring my son back when he's even older..." *sniff* ...to get circumcised...and I've been treated like a freak...and..." *whimper* "...this has just been so hard!" *sob*. It worked! The doctor whisked my son away, did the deed, and brought him back within 15 minutes. She did a great job too, I must say ;-)

All of this just to find out that my latest culture showed no evidence of MRSA. I either got rid of it on my own or is was a false positive. I've not cultured it since.

After having a step-daughter who thinks her father walks on water and having a daughter who has always been a "daddy's girl", I finally have a little boy who loves nothing more than to kiss, hug, hold, snuggle and love me!

Here are 4 things I've learned from Jake in the past 4 years:

1) I learned who Jar Jar Binx is and that we tend to find him annoying...

2) I learned that boys of ALL ages are intrigued by their privates!

3) Jake has Asthma and severe food allergies. Seeing him struggle to breathe and rushing him to the ER during his worst attack gave me a glimpse of what it must have been like to be my mother, or any mother parenting a child with CF. I learned that it's really hard...

4) I learned that "Mama's Boy's" really do exist and they are WONDERFUL!

Happy Birthday to My Dearest, Jake!

Spirits, Sightings & Signs from Beyond

2010-07-17T19:12:00.043-04:00

Last week I posted on my FB status that I was looking for some blog inspiration. I asked for suggestions on topics to write about. As a result of this status update I got this Formspring anonymous question:

"Have you ever seen a ghost?"

I KNOW one of my close friends who knows me well must have asked this question, wanting me to share some of my experiences in this area. Be prepared! After reading this post you will either think that I had a very special experience, or you will think I'm a huge wacko...

The short answer to the question is "yes". The long answer is that I think that after someone has lost a loved one, they often experience signs, sightings or visions that are unexplained. These signs often provide a lot of comfort to the grieving person. I feel very privileged to share that I have experienced one such sighting, many years ago.

I was 14 years old, a new Freshman in High School, when my great-grandmother from Atlantic City, New Jersey was diagnosed with terminal lung cancer. She had no one to care for her, so she moved in with my mother, brother and I in Cleveland. We cared for her, along with a Hospice team. My great-grandmother was not a spiritual or religious person. I don't believe that she had any expectations about what the after-life was like...most likely she didn't believe in an after-life at all. Several special things happened during my great-grandmothers 6 months with us.

One day after school I went to ask my great-grandmother what she wanted to eat for dinner. As soon as I entered the doorway to her room, she asked me who the two little people were standing next to me. Without the slightest pause, I responded that they were my guardian angels. I have no idea where that response came from? I continued to get my great-grandmothers dinner order and went along on my merry way. Oh, how I wish this many years later that I could have that moment back. Who, after all, has someone get a glimpse of their guardian angels. THAT only happens in the movies, right? If I could go back I would ask more questions. What did they look like? Did they have anything to say? My great-grandmother also talked to her dead husband all of the time. He resided in her TV stand...really. She had long and very interesting conversations with him. After one of these conversations she had us write a date on a piece of paper. After her death we found the piece of paper in her room. The date she had us write down,months in advance, ended up being the date she died...

All of these things were experiences of a woman who was dealing with cancer that had moved to her brain. She was also heavily medicated. Therefore, all of these sightings and signs can be passed off as hallucinations or stress on her brain. Maybe there is a physiological explanation, similar to the medical explanation given when people have near-death experiences who see the "white light". I have heard scientists pass this off as a biological reaction to a lack of oxygen in the brain. So here's what I think. I think my great-grandmother was preparing for death. I like to think that she was given a little bit of access to whatever was on the other side. This explanation gives me comfort, so I'm going to go with it.

Several months after my great-grandmother died we had moved to a new house. I was having a very difficult time adjusting to high school, dealing with my great-grandmothers death and dealing with my own health issues. One night I was sleeping soundly. All of a sudden I started hearing whispers. I opened my eyes and looked over toward my vanity, where I heard the whispers coming from. Sitting on my vanity chair was a black "shadow figure". It was looking in the mirror. When I looked, it's head turned toward me. I still could see no face, nothing identifying. My heart was pounding. I tried to scream, but I was paralyzed. My voice would not work, I could not move. I was really scared and really struggling. The figure very slowly stood up and started walking in place. It then disappeared into the vanity mirror. Just like that, it was gone. I continued to struggle for a few more minutes, until I shot straight up and gasped for air. I felt awake the entire time. I had never experienced a "dream" quite like this... I knew this was my great-grandmother. Too scared to get out of bed, I instead prayed, probably for hours, until I fell back to sleep.

I got up for school the next day with a new little secret. I finally told my friend Lisa a few years later and I was actually surprised by her lack of judgement. In graduate school for Social Work I took a class called "Death & Dying" where I started to hear about these types of sightings and signs being well documented in literature. I read a book called "Final Gifts", written by two hospice nurses, that spoke of instance after instance of similar sightings/visions to my own. This started to normalize my personal experience. From time-to-time I would share of this experience when in private conversation with close friends. If only I had known that these things happen more than I would have ever imagined. Here are some examples that have been shared with me by those close to me...

My mother got a prank phone call in the middle of the night one day. She answered, nobody was there, she looked at the clock, then she went back to sleep. When she woke up she was informed that her father had never woken up that morning...he had died of a massive heart attack during the night. It was very sudden and he died instantly. The autopsy showed that he died very near the time that she received the prank call. Maybe a coincidence? However, we like to interpret it as her father calling to bid farewell.
My friend Josh's sister died when he was 16 years old. He shared with me that he had a very similar "dream/sighting" of his sister. To this day, Josh still feels Angie's presence very often!

My friend Julie experienced the death of a close relative. Over drinks one night recently we began discussing the movie "Paranormal Activity". A little tipsy, I let down my guard and shared a little bit about my experience with the paranormal, only to have Julie say that she had the exact same type of vision! We compared details. It was amazing how similar our experiences were...from the paralyzed feeling to the inability to speak. It was fabulous to know that I was not alone, even within my circle of friends.
I have a friend in my neighborhood who had a high school friend commit suicide, while they were still in high school. This friend was a jokester...always pulling pranks and being silly. Soon after his death she had a vision/sighting where she woke up from her sleep to see and feel him jumping on the end of her bed. Of course, being as silly as he was in life, it was no surprise that he found a humorous way to reconnect after death.
Finally, little Conner Jones, age 7, died almost a month ago from CF. Read this beautiful blog post about the sign Sarah Jones received from little Conner last week: http://notsobrightandshiny.blogspot.com/2010/07/he-is-here.html

Are we all crazy? I don't think so. Were/are we all grieving? Absolutely. Could these experiences be our attempt to cope with the loss? Yes. Could grieving cause a biological change in our brains that made us "hallucinate"? Possibly. However, I tend to think that my sighting and signs, and those of my family and friends, were real. I think that we were connected to these souls in a way that allowed them to "communicate" with us even after death. If you've never had this type of experience, I'm sure you think I need some psychological help right about now. I completely understand! Yet, I feel blessed to have had this "sighting". For years I have prayed for my great-grandmother to come back for another very welcome visit. This time I wouldn't be frightened and I would collect myself enough to try to ask all of the important questions. Apparently, it doesn't work that way...

Mutations, Uncertainty & Aging

2010-07-13T10:40:00.026-04:00

I am a Type A personality. Most of the time it works for me, but I think in my CF world it works very much against me. I see things in black and white. Things are either all good or all bad. The gray area is extremely uncomfortable. I do not sit idly by, or go with the flow. It's not in my personality...it's not who I am. It is often seen as my biggest flaw. I am fully aware.

My gene mutation/combination is Delta F508 and R117H. "Dr. Bob" has told me on numerous occasions that the "R" mutations are very unpredictable. He can't really give me stats, because everyone with this mutation presents completely differently. I like my stats! CF stats have been ever-changing since my diagnosis. I am fully aware that stats don't mean much to each persons individual case, but I like to know my odds. Should I take the over or under? Should I put my money on red or black? The only true stat I have to go on currently is that 50% of people with CF will live to see 37.4 years of age. Does that mean I have a 50% chance of being alive 2 years, 5 months and 7 days from now (I'm not sure my math is correct...I'm just a Social Worker)? What happens after that 37.4 mark? That fact is that NOBODY can answer this question for me individually. Nobody has this answer. Ugh...there's that uncertainty thing again!

The CF world is so interesting. Most of us have a habit of introducing ourselves with our name and age. I'm known as, Stacey~ 34 yo w/cf. Nobody does that in the real world. For those with CF, our age is extremely significant. The honest truth is that I'm an aging CFer. I am completely honored to be an "old" patient...to have reached an age where non-CF people think my story is inspiring for the simple fact that I will be turning 35. When the average person says to me that I'm still so young, I think in my head that they have no idea. I wonder what they would think if they knew that I have made a conscious effort to keep all of my assets liquid and not contribute additional earnings toward my 401K. My financial guy urges me to think of the future, that 30 years from now I'm going to need this money to maintain my lifestyle. Huh, I guess I've gone with the under when it comes to finances. There's that uncertainty thing again...

I completely realize that nobody is guaranteed tomorrow. I am completely appreciative that I have been able to live to (almost) 35. I am hopeful! Anyone who knows me in my "real" life knows that I am positive. I ALWAYS try to look on the bright side...I ALWAYS try to put things into perspective, if not immediately, then eventually. So is it possible to be hopeful, but realistic? Is it OK that I sometimes feel uncertain and frightened, while mostly being positive and thankful? If so, than I am. I think I lived happily in denial for many years...but that's over. This is a process. Some days are better than others. I think it's much easier to be positive and happy when your body is cooperating. I haven't yet mastered how to keep this feeling going every moment that my body is failing me. I have learned that I need the yucky days, to help make the great one's even more meaningful.

I have had a couple of really crappy days over the past few weeks, with some amazing days mixed in. That is what has inspired this post about uncertainty. So now I'm going to complain. Here's where you can tune out it you don't want to listen to me whine... Last night we realized after 2 weeks on IV Nafcillin that I'm allergic. I had a delayed allergic reaction, 2 full weeks after starting the drug. I felt like I had the flu 10x over. I haven't been quite that sick in a long time. My lungs felt great! Everything else hurt... So I had to quit. I am a quitter. I could only make it through 2 weeks with this poison dripping into my veins, until my broken body couldn't handle it anymore. Now I'm allergic to all sulfa drugs, along with Penicillin's and I am becoming resistant to Keflex. Gotta cancel out all of those options... On top of that I now most likely have C. Diff and am on Flagyl. Flagyl in and of itself can be cruel, but C.Diff is worse. I was hospitalized for a week in 2000 for C.Diff and it sucked. I'm susceptible every time I am on IV's or long-term antibiotics, due to my history with it. Thanks goodness we caught it early this time. I am still waiting for my mycobacterium culture results, which may take weeks if not months to process. That's just the nature of that bug. I KNOW it could be much worse. I've been reading the blogs of my transplant friends and the struggles they have been faced with just recently. The crap that I've had to deal with over the past few weeks it absolutely nothing compared to their experiences. For what it's worth, though, I think this CF stuff SUCKS! Whew...that felt good!

So now I'm moving right along... Now, I'm going to flip the switch and bring it back to the moment. I am going to try to forget that I was writhing in pain during this allergic reaction last night and I am going to try to focus less on the gray area, that being the future. Today is today! Today I feel much better!

My (un)Scientific Study

2010-07-10T22:28:00.010-04:00

I have been on my IV's for about 2 weeks now. I love the freedom of being able to administer my IV medications at home. It's not an option for everyone, so I am thankful that I have been able to take this path. I must say, the most fun part of this whole experience for me has been doing my own little experiment in human behavior.

I am fortunate enough to live right between a Target and a Walmart. They are both within walking distance. I have to admit that I am a Target girl all the way! No offense to all of you Walmart peeps out there. Target just feels a little like home. Maybe I've just become hypnotized by the logo...

I have a couple of rules I follow when shopping at Target and Walmart. For Target, I never wear a red shirt. This causes major confusion for everyone involved. I don't know how many times I have asked a random shopper in a red shirt for help. Walmart is a bit different. I can be colorful, but I must remember to wear day time clothes, that are not ill-fitting and that cover all of the appropriate parts. If not, I risk becoming one of the "People of Walmart" from the now famous website.

I have been to Target 3 times this past week and a half (yes, I go that often). I was not infusing my medication during any of these trips, so my IV lines were neatly wrapped up in a coban bandage. You can still see what's going on in there, but it's not quite as obvious as it would be if I had the medication hooked up. During each of the trips I got a few people who stared, a couple who whispered to the person they were with, a few of the cocked head and puppy dog eyes and one very frightened little girl who looked at my arm then backed away slowly until she reached the security of her mothers side. My favorite is when a person is staring and when you look their way they quickly try to look at something else. I really don't mind at all. I would stare and wonder too.

I needed Lego's for my son, who's turning 4 in a couple weeks. I've learned that when you need toys, Walmart is the way to go. WAY cheaper and much better selection. I had a few minutes one evening after dinner, but I was in the middle of infusing my meds. Off to Walmart I went anyway. I had no bandage over my line, my tubes were dangling and I was holding the medication dispenser in my hand. I walked in expecting the type of reaction I got from the Target customers, but instead I got NO reaction. Not one person seemed to notice. I walked in, loaded up on cheap Lego's, walked out and did not get a second glance or scare any children. Maybe everyone was so mesmerized by the roll-back prices? Maybe they were too busy seeking out wardrobe malfunctions?

I found that the differences here were very obvious. I'm kinda concluding that the "People of Walmart" are a bit more accepting or apathetic about differences. Keep in mind that I'm saying this as a Target girl, myself. Hmmm! It just made me think...

Around and Around We Go...

2010-07-06T19:13:00.007-04:00

Last week I was in the hospital for a few days, getting my PICC line placed and starting my 3 weeks of IV antibiotics. This was a planned visit. My PFT's had decreased by 30% last year. After a full year of oral antibiotics that never really made an improvement, my doctor and I decided it was time for a tune-up. Honestly, I should have done it sooner.

When you go into the hospital prepared for what is ahead, surprises are not always welcome. My doctor...we'll call him "Dr. Bob" just for giggles...loves me as if I were his own daughter. I'm convinced of that. This conversation between Dr. Bob and I has been making me chuckle and confused all week, so I have to share.

Dr. Bob: Well, your culture results came back and it looks like you either have Tuberculosis or
Atypical Mycobacterium growing in your lungs.

Me: Huh, that kinda sucks. Wouldn't I know if I had TB?

Dr. Bob: Not necessarily, but I don't really think you have TB. We're going to test for it anyway.

Me: So what is this atypical mycobacterium business all about.

Dr. Bob: It's actually something that nobody really knows anything about. Well, there is one guy who knows about it, but he's in Texas.

Me: That does me no good. I'm gonna google it.

Dr. Bob: OK, google it.

Me: OK, I'm googling it. click..click...click...click... Huh, it says here that only sick people get this mycobacterium stuff. How the hell did I get it then?

Dr. Bob: Huh, I don't know? (this is written in sarcasm font)

Me: So what are we going to do about it?

Dr. Bob: We're either going to treat it, or we're not going to treat it.

Me: Well, which is it?

Dr. Bob: I don't know yet. It's going to take a couple weeks for the culture to grow before we can decide. We have to see what strain of mycobacterium it is first. Whatever it is that you have, you have a lot of it, though.

Me: That's completely unacceptable. I have to make plans and need to know what my treatment plan will look like now.

Dr. Bob: Well, I can't tell you anything yet.

Me: Why would we not treat? How can having a lot of anything like this be OK?

Dr. Bob: The treatment is very toxic. It usually lasts about a year and if we can avoid it we will. If we decide to treat we have to send your samples off to the guy in Texas so he can test it against a bunch of antibiotics so they can come up with your own unique recipe of antibiotics. That will also take a long time.

Me: I really hope nothing happens to that guy in Texas before he has a chance to analyze my samples. So, how bad can this get?

Dr. Bob: I would certainly rather you didn't have it. It's what used to always kill AIDS patients in the past. It can be really nasty.

Me: So we'll treat it?

Dr. Bob: Not necessarily. The treatments can also kill you or make you antibiotic resistant, so I'm not saying we'll treat either.

Me: Huh...sounds like it just sucks all around.

Dr. Bob: Well, it could also just stay dormant and not do anything. We'll have to see.

Me: This is just unacceptable....

So this same conversation went on about four times throughout the day. Back and forth we went. Around and around we went. What's the plan? Treat? Don't Treat? How Long? Who's this guy in Texas? How much do I have? When will I know? Around and around we went...

The good news is that I don't have TB! Good thing, because TB is so 1923... I'm still not sure what type of mycobacterium I have, but I'm hoping for the lazy kind. I'm also really hoping this guy in Texas doesn't retire or kick it any time soon!