Today is Cystic Fibrosis awareness day and what an important day it is! Everyone is encouraged to wear purple to show their support in finding a cure for this brutal disease. Meet Paul or baby Paul as he is known as by his 656 fans on his Facebook page! I have known Paul's mama since high school, she and I were on the swim and water polo team together and being the small world that it is, his Daddy works with the Sheriff but in a different department. Below is what Baby Paul's mama has posted on his Facebook page, I have deleted a few things to protect Paul's privacy.
Picture borrowed from his FB page.
Cystic fibrosis (CF) is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF.
In people with CF, a defective gene and its protein product cause the body to produce unusually thick, sticky mucus that: Clogs the lungs and leads to life-threatening lung infections, obstructs the pancreas and stops natural enzymes from helping the body break down food and absorb vital nutrients.
In the 1950s, few children with CF lived to attend elementary school. Since then, tremendous progress in understanding and treating CF has led to dramatic improvements in the length and quality of life for those with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
In order for a person to have CF both parents need to be carriers of the CF gene. Even when both parents are carriers, there is still only a 1 in 4 chance of an offspring having CF. Unbeknownst to them, Paul's parents are both carriers of the CF gene. Paul has the most common mutation of Cystic Fibrosis (CF), DDF508. He was diagnosed through newborn screening and we were informed of his diagnosis when he was 2 weeks old. His parents were unaware of any family history of CF carriers, therefore, didn't do any genetic testing during the pregnancy. It was a normal pregnancy and delivery. He was born at 38weeks 6 days gestation, 8lbs, 7oz, and 22 inches long. The diagnosis came as a huge surprise...the kind of surprise no parent wants to get.
Paul has been a more symptomatic CFer for his age and is considered a serious CF case by his team of doctors.
Paul is severely pancreatic insufficient and takes pancreatic enzymes every time he eats in order for his body to absorb food better. Despite taking enzymes he still struggles with food mal-absorption issues.
Paul also does 3-4 breathing treatments/day to help open his airways, loosen the thick, sticky mucus that sits in his lungs and pancreas, and help break up the mucus. Each breathing treatment is approximately 35 minutes long. He has been doing breathing treatments since he was 4 weeks old. When he was 10 months old he received a vest that he wears during the treatment that shakes his lungs and helps to break up the mucus.
Photo borrowed from his FB page.
He was diagnosed with his first serious lung infection when he was only 8 weeks old. He was 3 months old when he had his first bronchoscopy. At 4.5 months old when had his second bronchoscopy, was hospitalized and received a PICC line for the first time. His parents administered IV antibiotics at home for two weeks through Paul's PICC line. At 11 months old he had a CT scan of his lungs, an Infant Pulmonary Test (to test his lung function), another bronchoscopy, and an upper GI endoscopy. The results were not good. The back of Paul's lungs show significant damage and disease progression and his lung function was only 38%. He also has the start of liver disease. This devastating news resulted in Paul having his second PICC line placed and 3 weeks of IV antibiotics at home.
Paul had a PH Impedance test done at the end of July. The results of this test determined that Paul is experiencing acidic and non-acidic reflux. Paul's pulmonary, GI and infectious disease doctors suspect the reflux is draining to the back of his lungs which is why they are so badly diseased, damaged, and full of mucus, despite Paul being rather non-symptomatic (not sounding congested or coughing up mucus plugs, moderate shortness of breath, clear sounding lungs with a stethoscope).
The doctors are optimistic that with a medication adjustment and medication addition the reflex will be under control. The hope is that once the reflux is under control his lung function will improve.
At the end of October 2014, Paul went under anesthesia for the 5th time and the doctors repeated the same tests they did in July (lung CT scan, Infant PFT test, and bronchoscopy). The CT scan showed about a 60% improvement to the back of his lungs. His lung function went from 38% (at the end of July) to 62%. The bronchoscopy showed some improvement, but there is always more work that needs to be done!
Our goal is to keep Paul healthy through cold/flu season and repeat the CT, Infant PFT and bronchoscopy again in April 2015 to see if Paul's lungs are continuing to improve or if we have a good idea what his baseline lung function is. Our hope is that his lung function will continue to increase and the CT scan will continue to show improvement.
Paul has already proven that CF isn't going to get him down. Paul is a happy boy who resilient beyond his years, loves to swim in his pool at his Nana's house, loves his dogs, loves playing outside, has a laugh and a smile that can turn anyone's frown upside down. He is affectionately referred to as "Baby Paul" and/or "Champ" by his family and friends.
You can follow Paul on Facebook here, if you are not on Facebook no problem you can find him on Instagram and Twitter @paulspatrolmama and if you are wearing purple today, take a selfie and hashtag #paulspatrol and #cdcfstrong to show your support, I know I will be wearing purple today.
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