Brooklyn was 3 months old when a sweat test confirmed cystic fibrosis (CF), an inherited disease that affects the glands that makes mucus, digestive fluids and sweat. With the support of the Cystic Fibrosis Center, her parents, Christa and Ray, adjusted to managing Brooklyn's CF care at home. Now 9 months old, Brooklyn is active and strong and her family is settled into a new routine.
CF results in thickened mucus throughout the body, which impacts the lungs, pancreas and other organs. In the lungs, thick, sticky mucus clogs the airways and traps bacteria, which can lead to infections and extensive lung damage. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.
The news was a shock. Christa knew she carried the gene mutation that causes CF, but their two older daughters were healthy and they didn't know Ray was a carrier. Both parents must pass down the gene mutation for a baby to be born with the disease.
"It was pretty hard to hear," Christa remembers. She booked an appointment at the Cystic Fibrosis Center at Children's Hospital of Philadelphia (CHOP), then turned to the internet to learn about the disease. Looking back, Christa realizes that was a mistake. Scared and upset by what she read online, Christa called the CF Center at CHOP and talked with a nurse.
"She must have sat on the phone with me for two hours going over everything with me because I was a nervous wreck," says Christa. She learned that CF affects kids differently, so the symptoms, treatments and outcomes that she read about online may not necessarily be relevant to her daughter's case. While CF is a chronic, progressive disease, treatments have improved significantly over the years.
Learning to care for Brooklyn at home
When Christa brought Brooklyn in for her first appointment at the CF Center at CHOP, she felt calmer and more prepared. Still, she and Ray were overwhelmed when they learned about the home care their new baby would need.
They met with a team of medical professionals, including a physician, a nurse, social worker and a nutritionist. They learned how to clear their baby's airways with breathing treatments and chest physical therapy to help manage chronic respiratory problems and avoid lung infections that are common in individuals with CF.
Managing CF at Home
The most stressful routine for Christa and Ray was feeding Brooklyn enzymes to help with digestion. They would break open the capsules and mix the tiny beads inside with apple sauce.
"Trying to spoon feed a 3-month-old isn't easy," says Christa. At first, Brooklyn didn't gain weight fast enough, so her daily enzymes were doubled. But soon it became routine, and Brooklyn began to put on weight.
A new normal
With each appointment at the CF Center, Christa and Ray became more comfortable managing their daughter's CF. As Brooklyn grew, the family learned to make adjustments to her care routine.
Now 9 months old, Brooklyn is active and strong. She doesn't like to stay in one spot for her twice daily 20-minute chest PT treatments. Christa solved the issue by enticing her daughter with music, which she loves. She made a special "treatment playlist" featuring some of her daughter's favorite songs. Brooklyn watches the music videos during treatment, often dancing along.
Brooklyn will be old enough for a therapy vest when she turns 1, but Christa says she'll probably continue doing it by hand. She loves their time together.
Brooklyn recently started full-time day care at the same center as her sisters, ages 3 and 5. The care team at CHOP stressed to Christa and Ray that while they had to take precautions to help their daughter stay healthy, they couldn't keep her in a bubble. Giving Brooklyn the same experiences as their two older daughters feels right to the couple.
"Once you figure everything out and you get into the routine, you're going to realize things are OK," says Christa. "You find a way to do it your way and fit it into your everyday routine. Being that I have three kids under 5, that's just hard. But if I can do it, I feel like anybody could."
Originally published March 2018