Loss

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?

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?