getting closer!!!

So many amazing news headlines and stories have been popping up this month in the CF community - and amazing might just be an understatement... more along the lines of life-changing for a small percentage of those with cystic fibrosis!  This is the news that anyone who battles this disease on a daily basis has been waiting to hear.  I decided to break down all the stories I've read over the past week to highlight some of the key information as drug makers get closer and closer to new treatments for CF that could stop the horrible impacts of the disease.

So many of you who know Kaelyn and our family have reached out earlier this year to help us raise funds for cystic fibrosis, and while you know in your mind that you did a wonderful thing, you might not know just how big of an impact your contribution really is.  Sure you could do what I did - read all the news/information about what's happening on your own.  However, it might not all make sense to you - so here I go..... I'm by no means a scientist, but let me try my best to break down what's happening so you can see what a difference your help has made.

First off, no 2 cases of CF are exactly the same.  Cystic fibrosis is progressive and the progression of this disease along with the severity of it's symptoms vary from person to person.  While it's true the average life expectancy is mid-30's and some live well beyond those years, we unfortunately are still losing lives entirely too young.  I'm talking about children who still aren't making it to elementary school, teenagers who don't get the chance to graduate high school, and the list goes on.  How is that fair?  Is living a life to the age of 35 a long enough life for you?  I'm going to safely assume probably not, so why should we settle for it be a long enough life for someone with CF?

I've seen the destruction CF can bring to families, through the eyes of the others in the CF community.  It's devastating what this disease has done to some of these families and to the young lives that fought the fight and unfortunately lost their battle.  These children so weak, so sick but at the same time so very strong & brave and they are now in the hands of God, as an angel watching over the rest of us like Kaelyn.  I know every time I blow out the candles on my birthday cake, I'm wishing for a cure for our little lovebug and I'm certain Chad wishes for the same thing every year too.  We've said prayers over and over again to watch over Kaelyn and keep her healthy through the years.  Well it looks like some of the wishing and prayers are being heard...

Just last week on October 20th an announcement was made by Vertex Pharmaceuticals that the drug Kalydeco (vx-770) is finally under review for approval by the FDA.  This will be the very first drug on the market that will target the underlying cause of cystic fibrosis.  Currently any treatments CF'ers receive to date only help to relieve symptoms.  While these treatments are effective and prolong life, the new drug should be able to add decades of life over a short period of time!  How amazing, right?!?  This phase of the drug however will only benefit those who are 6 years or older and who have at least one copy of the rare G551D mutation.  Only 4% of the CF population have this copy, but that's approximately 1,200 lives that could potentially be changed in a dramatic way! 

HUH?!??  I'm sure some of you are saying that to yourself right now.  As some of you might remember reading or hearing me say before, cystic fibrosis is a genetic disease which has thousands of known CF mutations - G551D is just one of them.  Kaelyn unfortunately doesn't fall into either of the 2 criteria for the drug - she's only 3 and does not have a copy of the G551D mutation.  Her genetic mutations are DeltaF508 and P5L.  DeltaF508 is the most common gene mutation of CF.  The good news is, right now clinical trials are taking place to evaluate Kalydeco in combination with another drug (vx-809) that would benefit those with a copy of DeltaF508 - And Kaelyn has a copy!  It is their hope that with time they will be able to benefit ALL people with CF and this is a major step in that direction!

It's sometimes hard for friends and family to grasp the enormity of cystic fibrosis.  Most of the time with Kaelyn, no one would even know she was affected.  Right now, we couldn't be more thankful for her health and her pretty much symptom-free 3 years of life she's lived so far.  We know in the back of our minds though that cystic fibrosis IS progressive.  What's unknown is the path and rate of that progression.  We need to stop that progression so kids like Kaelyn can live long, healthy lives!

If you want to be a part of curing a disease that will give futures to many little kids, please continue with us in our fight against CF.  As I said in the beginning, life-changing things are happening right now!  Keep your prayers coming, we're getting closer!! 

tis the season for being sick

Cold and flu season are here again unfortunately. 

For most of us, getting a cold is no big deal.  Sure it sucks, but we all deal with it - the runny nose, congestion, cough, and sneezing.  The symptoms come, our bodies fight it off, and before we know it we're feeling 100% again.  For Kaelyn and other CF suffers, colds can turn into something more serious - lung infections.  Because CF suffers have a faulty gene that allows too much salt into cells and not enough water, a build-up of sticky mucus ends up being the result.  This sticky mucus builds up in the body's passageways, and at times when a cold or virus attacks, the body can't always fight it it off on it's own.   Repeated lung infections and inflammation of the lungs create irreversible damage and threaten the health of CF suffers.

Kaelyn's health is usually outstanding and more than we could ask for - however, she has already had her share of sicknesses this cold season and recently had a virus that she just couldn't fight off on her own.  Runny nose, bad cough with mucus, hoarse voice, reoccurring fever... which meant that for the first time in 3 years, it was time for antibiotics.  Penicillin antibiotics were prescribed and for the first week they seemed to be working well.  However this past Saturday, I started noticing small red bumps on Kaelyn's stomach and back.  As the day progressed, they increased in size and number so a call to the doctor was needed.

It turns out Kaelyn has a severe allergy to penicillin antibiotics.  She's doing fine though and all of the spots from the rash should clear up in about a week.  Her doctor said no antibiotics of any type for a while because Kaelyn could develop allergic reactions to other types while her body is recovering, and we definitely don't want that to happen.

Here's a picture of Kaelyn from yesterday:

what did you do last night?

Cystic fibrosis can be a very high maintenance disease to keep yourself or loved ones healthy.  For some with CF, daily routine includes several breathing treatments, CPT (chest percussion therapy), 40-50 pills/day, vitamins, high salt intake, or even lengthy hospitalizations.

Fortunately for our family, Kaelyn was diagnosed with atypical CF very early on in her diagnosis.  At this time, our primary concern is keeping her lungs healthy and functioning properly.  Most individuals with CF will suffer from both lung and pancreatic/digestive problems throughout their lives.  For Kaelyn however, her pancreas is fully functioning on it's own - meaning she doesn't require the high intake of enzyme pills to digest her food properly.  I'll never forget the day we found out that news.  Around 85% of the CF population is effected by digestive problems and poor growth, so it was a blessing the day we heard Kaelyn was pancreatic sufficient!  She's steadily been on track with her growth over the past 3 years and is currently in the 90th percentile for height and 50th for weight. 

Last night, Chad and I opened a new chapter of learning in our book of CF care for Kaelyn.  Last week she finally received her airway clearance vest for CPT rather than us administering CPT manually (by tapping on her chest and back).  Around the age of 3, kids are old enough to be fitted for a vest and at Kaelyn's last CF appointment she was fitted and sized for her little pink vest.

Learning to use the vest and how to care for it is pretty simple.  The vest itself consists of 2 layers, the outer layer which Kaelyn chose pink as her color, and the inflatable layer that fits inside.  This inner layer will rapidly inflate with air once the air pulse generator is turned on.  The vest will gently compress and release the chest wall which will loosen up any extra mucus that might be stuck in the bronchial walls.  As mobilization increases from the shaking of the vest, Kaelyn can easily cough up anything that might be making breathing difficult and keep her airways clear.  Keeping the extra mucus away from her lungs and out of her airways helps reduce the chance of infections, and ultimately less infections result in better overall health and hopefully staying out of the hospital to be treated for sicknesses.

So far, Kaelyn's been sick twice now since she started school/daycare.  Right now she's currently had a strong cough and cold for over a week and even broke out in hives last week.  I knew from the very beginning of her first exposure to daycare this year (as we all know too well to be the home of excessive germs) that she would probably encounter more sicknesses this year than she has her entire life.  We can only hope that with our care and the amazing treatments that are available to those with CF that we can keep her as healthy as possible.  :)

Kaelyn is required to get airway clearance treatments from her vest twice per day for 20 minutes each.  Last night was her first official treatment and she did surprisingly well.  She got through the first 15 minutes smiling, laughing and talking to us as we joked about her funny voice as the vest was shaking and doing it's work.  The last 5 minutes brought tears as the intensity increased, as well as the noise from the generator.  Every 3-4 minutes the intensity will increase until the full 20 minute session has been completed.  She got through it though, and I have a good feeling that once she gets used to her new vest and what it does, that she'll do just fine with treatments!  She's a tough little girl but it's just hard for a 3 year old to understand why she's even subjected to wearing a "shaky vest" (as she calls it!) in the first place....

"BREATHE EASY"