keeping Kaelyn healthy

It's been quite a while since I've been able to post on our blog, but now that that craziness of the holidays is a thing of the past, I plan to post much more.

Today I wanted to share a little about Kaelyn's CF care in order to keep the mucus out of her small lungs, and maintain her overall health.  This is a day in the life of Kaelyn:

Everything on this table is part of Kaelyn's daily CF care currently. For those of you who follow Kaelyn's story and know about her CF case, one thing you will notice that's missing from her daily routine are pancreatic enzymes. 85% of those with CF have pancreatic problems as well. Fortunately Kaelyn does not.  Almost everyone with cystic fibrosis needs to take pancreatic enzyme supplements with meals and snacks. Taken by mouth, the enzymes go to work in the intestines to help digest food so it can be absorbed by the body.

1.  Nebulizers – Clearing the Airways
Since inhaled drugs more easily reach the airways, they are common in CF care. Inhaled treatments can be given by aerosol, a mist treatment made from liquid medicines. In this case, the drug goes into a nebulizer (on left in picture) and is attached to a small air compressor (in back of picture). The compressor blows air through the nebulizer and makes a mist. CF patients inhale the small particles in the mist through a mask (on right in picture) or mouthpiece (front in picture) for several minutes to help clear the mucus.

2.  Inhaled Medication #1 - Pulmozyme
CF is a complicated disease that causes your body to produce extra-thick, sticky mucus. Pulmozyme uniquely targets one of the causes of this thick, sticky mucus to help keep mucus thin and loose. Pulmozyme may help improve lung function and reduce the risk of infection in mild to moderate disease.

3.  Inhaled Medication #2 - Albuterol
Albuterol is used to prevent and treat wheezing, difficulty breathing and chest tightness. It works by relaxing and opening the air passages to the lungs to make breathing easier.

 4.  Vitamins and Minerals
Most people with cystic fibrosis do not absorb fats properly, so certain vitamins, or "fat-soluble" vitamins, are not absorbed. These vitamins are Vitamins A, D, E and K. People with CF usually take these vitamins daily.
Kaelyn's main deficiency is Vitamin D.

5.   Avoid The Spread of Germs
Cystic fibrosis puts the airways at risk for lung infections. There are, however, effective ways to lessen the risk. One way is to limit contact with known germ sources. Although germs are everywhere and cannot be avoided, one of the best ways to keep from catching or spreading germs is through effective hand-washing, whether with soap and water or alcohol-based hand gels. Everyone with CF should avoid unnecessary contact with people who have a cold or any other contagious illness.
I keep these mini ones in my purse and we recently got Kaelyn the ones she can attach to her bookbag to have at school.

6.  The Vest - Chest Therapy
The Air Pulse Generator rapidly fills and deflates an inflatable vest, gently compressing and releasing the chest wall up to 25 times per second. This process, called High-Frequency Chest Wall Oscillation (HFCWO), creates mini-coughs that dislodge mucus from the bronchial walls, increase mobilization, and move it along toward central airways.

Here are some photos of Kaelyn getting treatments with her vest:

CF care is so, very important!  It's daily, it's time consuming, but it keeps Kaelyn strong and helps our brave little girl beat CF every day!  It might not be much fun for any of us, but keeping Kaelyn healthy and happy is our #1 priority!

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: