What Would You Do?

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

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

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!

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

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

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

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

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

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!

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

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

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

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

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.

c1987 by Emily Perl Kingsley. All rights reserved

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

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

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

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

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

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

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

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!