When Kate was born, she weighed in at a whopping 8 lbs 6 oz, bigger than her brothers by a full pound! Unfortunately, it was shortlived. She quickly lost weight and dropped to 7 lbs 2 oz. She was having a very hard time putting the weight back on. I spent those first weeks feeling worried a lot, but not quite knowing why. She would scream, inconsolably for hours each night as though her tummy was hurting. I held her and cried with her as she screamed in pain. I asked the pediatrician a question at her 2 week appointment that led to the discovery that would change our world forever. She said the words, "I think we should test her for Cystic Fibrosis." The newborn screen came back with elevated levels, leading us to have a sweat test done the next morning at Primary Children's Hospital. By 3:00 that afternoon, we got the dreaded call confirming our worst fear. Kate has Cystic Fibrosis. After collapsing into the deepest devastation we have ever felt, we picked ourselves up and jumped head first into our "new normal."
We began trying to piece the puzzle together. Kate immediately started taking pancreatic enzymes before each feeding to help her absorb nutrients. We learned that her pancreatic function is next to nothing, and she was therefore slowly starving to death, even though we were feeding her as much as she would possibly eat. She started reflux meds, and a specialty formula that already has the milk protein boiled down because her poor little stomach couldn't break it down itself. We began to give fat soluable vitamins, and salt supplements to replenish the things that CF took from her body. It was a lot for a tiny baby, but after weeks of letting these changes take affect, her tummy started to feel better. She started to gain weight, and wasn't screaming and scrunching her legs up in severe pain anymore.
After a couple of months of letting us adjust, her doctors had us begin doing airway clearance treatments to loosen the thick mucus in her lungs so that it would be less likely to harden and breed harmful bacteria. We did this Chest Percussion Therapy (CPT) for about 30 minutes, twice a day where we beat on all the lobes of her lungs while she squirmed and screamed and we all cried together. We got used to it. That "new normal" again. A month later we added another log to the fire. She began a nebulized medication to help break up the mucus and make it easier to get it out. With the screaming, fighting and eyes of a caged animal, we held the mask over her face and cried together through this adjustment as well.
We were grateful we had started CPT when we did, because soon after, she got sick, and increased CPT is your first line of defense. We increased to 4 thirty minute treatments. After 3 rounds of oral antibiotics, and hours of breathing treatments each day for over a month, we decided with her pulmonologist to have her admitted for a 'clean out', or 14 day course of IV antibiotics. This was another devastating blow. Seeing her get poked and prodded, coughing and crying uncontrollably, blowing through IVs in hours and having to have them stick her again...it was horrible. She got a PICC line placed, which made things much easier, but seemed surreal that it was necessary. Dave started a new job working 12-15 hour days the day after she was admitted. Having the boys get to school and arranging for their care from afar was a nightmare, but our friends and family were amazing and we made it work. We were in the hospital for a week, and they allowed us to go home and finish the second week of IVs there. Just like that, we were home health care nurses, flushing her picc line, and administering 2 different IV antibiotics. Dave working his tail off, and me managing 8-10 IV doses per day, running from 20-30 minutes each, 4 CPT treatments, nebulizers, feedings, naps, and all her other daily meds. Oh, and 2 other kids. :) It was hard. So hard.
Since then, we are so thankful to report that Kate has done really well. She seems to catch colds pretty easily, and usually needs antibiotics to kick them, but we have been so fortunate that increased treatments and oral antibiotics have done the trick, and kept the infections from moving into her lungs. She has a vest now that pumps air through noses and shakes her to loosen the mucus so we don't have to manually beat her lungs. It is such a blessing. She takes more daily meds and supplements, and we work very hard to keep up a high fat, high calorie diet and are THRILLED to annouce that her BMI just hit the 55th percentile!!! Her BMI now directly affects her lung function as an adolescent, which is generally when we are expected to see a significant decline. So as you can imagine, it weighs heavily on our minds.
We will do anything and everything for Kate. That's where you come in. Cystic Fibrosis affects about 30,000 people in the US and 70,000 people worldwide. It is the most common genetic disease, but still rare, and therefore considered an 'orphan disease'. This means that it does not affect enough people to receive government funding for research. As a result, we rely on the Cystic Fibrosis Foundation to carry out research. They are amazingly efficient and spend an incredible 90 cents per dollar donated on research for new treatments, and eventually a cure. CF families like us feel a responsibility to do our part in raising money because we are SO CLOSE to amazing things!!
Since Kate was diagnosed, not even 2 years ago, the first drug to treat the underlying cause of CF has been approved and become available to patients. While this drug has had incredible results, it only works for about 4% of those with CF. There are more than 1800 identified CF mutations, and each one affects the cells just a little bit differently. It's not a one size fits all cure. There will be another drug soon, taken in combo with the first that targets people with 2 copies of the DF508 mutation, scheduled for expidited FDA review in July. That will help about 50% of the CF population. Kate only has one copy of this mutation. BUT...just behind that one is another combo in trials that targets those with one copy of this mutation. That's our Kate!!
There have been many advancements in treating symptoms, which have increased life expectancy and quilty of life, but in the end, the disease wins. Almost every day, I see someone that the CF community has lost, and of all ages.
These discoveries taking place could potentially correct function in the cells, making it possible to greatly slow, or maybe even halt the progression of the disease. Can you imagine being able to significantly slow the clock so that Kate has the time to wait for these absolutely life changing advancements??? To be able to keep her lungs as healthy as possible so that she can have a long, full life with a family of her own when the cure comes? This is the hope that we have for Kate. This is the hope that we have for all of our kids. Kate just has a few more obstacles placed in her way. I have faith that we are going to see incredible things during her lifetime, but we need your help to drive the research with our passion and support. We thank you from the bottom of our hearts for being there for our family.
Come walk with us! If you can't be there to walk, help us reach our fundraising goals by making a donation to our Great Strides Campaign, and sharing with your friends. Help us make CF stand for CURE FOUND, and add tomorrows for Kate and all the others suffering with this horrible disease.
Bonus: Your gift is 100% tax deductible. :)