Center aids West Virginia’s children with cystic fibrosis

Fifteen-year-old Gabby Dumire enjoys reading Harry Potter books, playing the flute in her high school’s band, and riding her bike when the weather is warm. A middle child sandwiched between two brothers, Dumire lists her favorite foods as tacos, asparagus, and egg whites.

When she eats those foods, though, Dumire takes replacement enzymes that help her body digest food and absorb nutrients. She also has a feeding tube and requires regular breathing treatments.

Dumire is one of about 250 West Virginians with cystic fibrosis (CF), an inherited chronic disease that affects the lungs and digestive system. A defective gene and its protein product cause the body to create unusually thick, sticky mucus that clogs the lungs and leads to lung infections. This obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

A Preston High School sophomore, Dumire resides in Bruceton Mills, West Virginia. She sees Kathryn Moffett, MD, every three months, which is the standard schedule for CF patients.

“When I come to an appointment, I see the dietitian first; then I do pulmonary function tests. That’s when I have to blow into a little tube, and it tests my lung function,” Dumire said. “Dr. Moffett comes in, and we talk about how I’m doing, if I’m sick, if I need to go to the hospital, if I need antibiotics, or if I’m doing fine. We talk about my weight and if I’m eating well.”

Most West Virginia children with CF receive their care at the WVU Mountain State Cystic Fibrosis Center, some of them traveling two, three, or four hours for appointments. The center ranks among the top 10 centers of its kind in the country, according to pulmonary and nutrition data from the Cystic Fibrosis Foundation’s Care Center Network.

Dr. Moffett, professor in the WVU Department of Pediatrics, leads the center’s multidisciplinary team of physicians, dietitians, respiratory therapists, and social workers. Linda Baer, RN, is the center’s program coordinator. They have about 100 pediatric patients whose CF ranges from mild to severe.

Since earning accreditation from the CF Foundation in 2000, the WVU Mountain State CF Center has been entering patients’ information into a national registry that tracks important measures like lung function and body mass index (BMI) from 165 centers across the country.

“This allows us to compare our data with other centers,” Moffett said. “I can look and say, ‘Where do we fall in terms of average lung function or average weight?’”

The most recent available statistics are from 2012, and WVU’s numbers put it above the national average.

“Our median BMI percentile for 2- to 19-year-olds is in the top 10 nationally,” Moffett explained. “If you look at lung function for 6- to 17-year-olds, there are five centers above us. And we are number one in the nation for teenagers – the highest in lung function for 13- to 17-year-olds.”

Dumire is part of that group, and Moffett refers to her lung function as “excellent.” Meeting that mark, however, comes with challenges and requires hard work. “She has a feeding tube, is doing chest airway therapy, and is on 10 or 12 medicines,”

Moffett said about Dumire, who was diagnosed with CF at three months of age. “She’s got a really good attitude about it. She’s a delightful young lady.”

Much of the interaction at appointments between Moffett and Dumire – including the questions Moffett asks and the conclusions she draws – is influenced by years of quality improvement (QI) projects. The center has completed many QI projects, examining nutrition, vitamins, and lung function. They ask specific questions, set goals, and implement changes.

“We might look at someone and say, ‘He doesn’t weigh enough,’” Moffett said. “Well, how much do we want him to weigh? Have we talked with the parents? Have we plotted the patient? Let’s look at the BMI.”

As a result, the center uses a stoplight system to help patients understand whether their BMI is in an acceptable range. Green indicates the patient is doing well and should continue gaining weight and growing. Yellow stands for caution because the patient is at risk for nutritional failure, which is signified by red.

“About a third of our pediatric patients were in nutritional failure – in red – when we started the QI in 2006,” Moffett explained. “Now, we have no child in red. None.”

Another change that has occurred because of QI projects is the use of a scoring sheet, an objective way to evaluate patients’ symptoms and determine if they are having a pulmonary exacerbation. Moffett and her team also provide education on vitamins, even quizzing patients to test their knowledge of vitamins and what each one does for the body.

Despite WVU’s progress in improving the health of West Virginia CF patients, Moffett has adopted the CF Foundation’s stance that there is always work to be done.

“Even though our lung function is where it is, there are many other things to work on,” she added. “The foundation is not stopping, and they want us to constantly be working. They provide education and service and are striving for a cure but, in the meantime, not letting anyone forget, ‘While you’re in the lab looking for a cure, you still have patients who need help.’”

While the primary job for Moffett and Baer and the entire center’s team is to provide medical care to CF patients, they also offer support to the children’s families.

“I’ve been working with CF patients for 22 years, and it’s rewarding,” Baer said. “I get to know the families because the patients have been coming here since they were babies. We become a part of their family. They know us and trust us. I can’t cure CF, but one of my roles is to make sure patients have what they need. I know I make a difference.”

Moffett continued, “The relationship with these families is a very special one because we celebrate their milestones; we cry with them when they get green on their nutrition or when they graduate. We help and support them because they need that. As we see things decline, we still work toward improving. What I tell the families is, ‘I am here no matter what.’”