It Takes a Family
By Lorna Oppedisano | Photography by Alice G. Patterson
“When you’re a mom and you have kids with cystic fibrosis, you wake up in the morning and every day is unpredictable, because your day could change from minute to minute,” Cindy Nappa said.
Cindy’s connection to CF began when she was a nurse at the CF clinic at Upstate Hospital about two decades ago. Then, in 2005, her daughter, Amy Nappa, had her first child, Gracie. After a few weeks, Gracie was diagnosed with CF, “a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time,” according to the Cystic Fibrosis Foundation. The disease is caused by a defective gene that causes buildup of mucus in the lungs, pancreas and other organs.
For the first year of Gracie’s life, the family was in and out of the hospital quite frequently. They celebrated her first birthday in the hospital cafeteria, they remembered.
A couple years later, Amy went for an ultrasound on her second child, Joey. They discovered blockage in his intestines, and sent Amy to a specialist. After another test, she was told Joey had CF, too.
“Even though they have the same [disease], they’re two completely different children when it comes to CF,” Amy said.
Cystic fibrosis is caused by two copies of the defective CF gene containing an abnormality called a mutation. There are more than 1,700 known mutations of the disease, according to the CFF.
While Gracie and Joey have the same mutation, it is expressed differently in each child, resulting in differently predominant symptoms; Gracie’s symptoms are primarily respiratory and Joey’s are digestive.
For Amy’s family, each day is scheduled around a variety of treatments for Gracie and Joey, ranging from airway clearance therapies to inhaled medicines to enzyme supplements. Each child’s treatments take close to three hours, if not longer, each day.
“The hardest part sometimes is managing the treatments. When are you going to do it? What do you have to do? What time do you have to be home?” Amy said, adding that any kind of travel — even an overnight at a friend’s house — adds another level of difficulty.
“There is never a break from CF,” Cindy said. “It doesn’t matter what day of the week, what day of the month, where you’re going or what you’re doing. It’s the same routine with treatments, medications and airway clearance therapies. You can’t miss any treatments ever, regardless of other things happening or plans that day.”
Adding yet another challenge to the Nappa family’s daily routine is the fact that people with CF are encouraged not to be around each other, since “medical studies show that people with CF are at particular risk of spreading certain germs among others with the disease,” according to the CFF.
The foundation warns people with CF to stay at least six feet apart, since that’s the distance germs can spread from a sneeze or cough. Having two children with CF can present a challenge.
“You just do the best that you can,” Amy said, explaining that Gracie and Joey never share anything that touches their mouths.
Since Cindy first encountered CF at Upstate, a great deal of research has been done, resulting in medical advances that have added years to the lives of those diagnosed with CF. According to the CFF, in 1989, the median predicted age of survival was 29. In 2000, it was 32 years old. Now, people with CF are living into their 30s, 40s and beyond.
“It truly is amazing,” said Tracy Nappa Marino, Amy’s sister and the administrative manager at the CNY chapter of the CFF. “We owe it to the science, which is owed to the fundraising, which is owed to the families’ efforts who support us in the community, and the businesses and corporations that support us in the community, as well.”
The entire Nappa family has been involved with the CFF since Gracie’s diagnosis. Amy’s attended the annual Great Strides walk since 2006. Cindy began volunteering with the organization in 2005. Tracy started by joining and chairing fundraising event committees, and became a CFF employee about five years ago.
Working for the foundation has given Tracy a unique perspective. She sees both sides of it, which gives her insight to the emotional, mental and physical struggles families dealing with CF encounter on a daily basis.
In her office at work, she has a quote on her wall — “Remember why you started.”
“Having these two and their smiling faces definitely keeps me focused and [helps me] stay the course, even when some of the days are a little
tough,” Tracy said. “It’s easy to remember why.” SWM
May is Cystic Fibrosis Awareness Month. Join in the annual Great Strides walk on Saturday, May 12, at Long Branch Park. For more information on the Cystic Fibrosis Foundation, and to find out how you can get involved with the Central New York chapter, visit cff.org/CentralNY.