In honor of Cystic Fibrosis Awareness Month, we bring you one mother’s story of discovery and learning how to live with the disease in her home.
Kassi Bacquet and son Trax
How would you describe CF to someone who is unfamiliar with the disease? (And what are the common symptoms?)
“Cystic Fibrosis (CF) is a genetic, terminal disease that affects the lungs and digestive system. People with cystic fibrosis have unusually thick, sticky mucus that can clog the lungs, which may lead to life threatening lung infections. The mucus also obstructs the pancreas, which stops the natural enzymes in victims’ bodies from breaking down food and absorbing its nutrients. CFers can have a variety of symptoms like persistent coughing (sometimes with phlegm), shortness of breath and/or wheezing, salty skin, poor growth and weight gain (failure to thrive) due to the malabsorption of food, frequent lung infections, greasy, bulky, stinky stools, and many bowel movements a day.”
How did you discover that your child had it? What did you do and how did you feel?
“Man, what a roller coaster finding out was! While I was pregnant with the twins, Trax had an echogenic bowel show up on his ultrasound at twenty weeks (also the day we found out it was twins) and the tech told us not to Google it when we got home. My husband Daniel, of course, Googled it right away and found that it was a soft marker for either Down Syndrome or Cystic Fibrosis. I pushed it out of my head and tried not to think about—I had enough on my mind after finding out my fourth child was now my fourth AND fifth. At my thirty-six week ultrasound it was still there and I just prayed he would be ok. The tech was actually more concerned he was going to be born without a rectum! My birth was amazing, Tavvi and Trax seemed perfectly healthy and were each over six pounds. I was breast feeding two babies like a champ, but Trax just kept losing weight. We were on a very rigorous feeding schedule due to his lack of weight and finally at two weeks old his pediatrician sent us to the hospital for failure to thrive and she was concerned about the possibility of him having CF, since his newborn screening came back positive for it. We spent a week in Texas Children’s Hospital in the NICU. That first night, Dan stayed with Trax since they wouldn’t let me stay there with Tavvi, too. I was heartbroken. To had just given birth and to leave your child is one of the hardest things I have had to do. The rest of the week I was there every day, with Tavvi , too. Dan and I were able to stay in the Ronald McDonald House and teams of residents were testing Trax for all sorts of things. In our hearts we knew it was CF, so we really pushed for the Sweat Test they give to confirm if our suspicions were correct. A week after leaving the hospital, when the twins were four weeks old, we got the news that Trax did indeed have CF. Hearing that your child has a terminal, genetic disease is a swift and brutal blow. Our hearts were broken.”
What immediate steps did you take?
“With heavy hearts, but a desire to take this head on, we started doing research. We got Trax in for his first CF clinic visit and immediately got him on pancreatic enzyme replacement treatment, which almost immediately caused him to gain ounces. Over the next month, we worked closely with his pediatrician and his doctors at Texas Children’s. The doctors who diagnosed Trax went on to become part of his CF team. They are our lifeline and have been a great support from the beginning. Trax has two doctors, a respiratory therapist, dietician, social worker, CF nurse, and a hepatologist.”
What is it like caring for a child with CF?
“Honestly, it’s the same as caring for any child (aside from all his treatments, of course). On the outside, a child with CF looks no different than you or me. They act no different than any other ‘normal’ child. Trax is not delayed in any way and appears super healthy. He is active, funny, loves to dance to Black Sabbath, is a cuddle bug, and a bruiser—hitting anything and anyone in his path. He is a normal 19-month-old boy, but he has a forever cough, problems with his digestive tract, takes a lot of medicines, and has daily treatments.”
What is his prognosis and day-to-day treatment?
“Trax is doing amazingly well. He has good clear lungs, he is getting close to the 50th percentile for weight, and he is tolerating his breathing treatments (sometimes). Before any meal, Trax has to take his pancreatic enzymes, whether it be a meal, a cup of milk (or Pediasure), or a snack. Enzymes are key in helping him keep his weight on track. He also has issues with stomach acidity, so he’s also taking Prevacid to assist with that. Most days Trax has two, 30-minute airway clearance and nebulozed saline treatments, where he wears a special vest that shakes him to help break up the mucus in his lungs and the nebulized saline to assist. When he has a cold, his treatments are increased to four times a day…being a mom of five kids, that can be a daunting task. Whenever Trax comes down with something, Dan and I are on high alert because if his O2 saturation levels drop, it’s straight to the Children’s Hospital. He’s also dosed with antibiotics from his CF doctor, as bacterial infections happen often and are common with CF.”
Are there any wide misconceptions about CF that you’d like to clear up?
“I can’t think of any wide misconceptions. The primary thing we run into is a general lack of knowledge about CF. Dan and I were clueless about it prior to Trax’s diagnosis—aside from my favorite movie being Alex, The Life of a Child, which aired on ABC in 1986 and was about a little girl who died after battling CF for eight short years. And therein lies one big issue. CF is a rare disease and is often not in the spotlight of mainstream media. One thing we found that I don’t think people realize, is how expensive a disease like this is—the medications, the equipment, the hospital stays, the countless doctor visits—it all adds up quickly and if you don’t have great insurance it can be very hard financially.”
What can people do to help children with CF?
“Being that it is a rare disease, raising awareness about CF is number one. Our dear friend, whom we met through the Instagram community, sells necklaces (the pray4trax necklace) that not only raises awareness for CF but all the proceeds go to Trax’s gofundme account. Also, my sister sells shirts where half the proceeds go to Trax’s gofundme and the other half to The Cystic Fibrosis Foundation. Unfortunately, drug companies are not quick to dump a lot of money into CF drug research so helping to spread awareness and working with organizations like the Cystic Fibrosis Foundation to raise money is how we are going to create a healthy future for those with CF. Since we’re a family of surfers, one organization that Dan and I hold close to our hearts is the Mauli Ola Foundation, which takes kids with CF surfing. Salty air does wonders for breaking up the thick mucus in their lungs. Through prayer, raising awareness, and supporting organizations like cff.org and Mauli Ola Foundation, we are confident that a cure for CF will one day be a reality. The average life expectancy of a CFer is only 38 years old, which is another reason to come alongside us to find a cure and raise awareness for this awful disease.”