TREATMENT MOUNTAIN STATE CYSTIC FIBROSIS CENTER
Serving as WV’ s partner with the CF Foundation, the Mountain State CF Center, associated with West Virginia University( WVU) Medicine, was first established in the 1980s. 15 This center is a premier provider of care to CF patients. Originally its care was focused on pediatrics patients, so adult patients were first cared for by pediatric specialists. Advancements in research have significantly improved the average life expectancy and quality of life for those with CF. 16 The adult CF program at WVU is the only accredited adult program by the CF Foundation in WV, and adult and pediatric specialists work collaboratively to ensure a successful transition from pediatric to adult care, described more below. A multidisciplinary treatment approach after diagnosis, including input from specialists in nursing, respiratory therapy, dietary, social work, physical therapy, psychology, and pulmonology is employed. Lung function is closely followed with regular spirometry and monitoring microbiology of the respiratory tract as well as offering nutritional guidance, assistance with medication insurance authorization, chest physical therapy, mental healthcare, and access to clinical trials. 17 The Mountain State CF Center reported no mortalities in 2023. 18
As of 2023, a total of 66 adult patients were enrolled in the Mountain State CF Center, while 93.9 % of enrolled individuals were eligible for CFTR modulator therapy. 18 The center outperformed national averages regarding delivery and coordination of care( Figure 1). 18 WV had a greater number of patients evaluated by other specialties in all categories, with the exception of pharmacy, compared to the national average, while also ranking above the national average in rates of influenza vaccination in CF patients( Figure 1). 18 A major concern for CF patients is malnutrition due to pancreatic insufficiency; however, the center had a greater number of individuals with a body mass index( BMI) greater than or equal to 30( 22.6 % vs. 13 %) and fewer number of individuals with a BMI less than 18.5( 3.2 % vs. 4.5 %) compared to the national average. 7, 18 Fewer patients were reported with one or more CF exacerbation( 4.5 % vs. 15.1 %), a shorter duration of intravenous antibiotics for hospitalized patients( 10 days vs. 13 days) and median number of nights of hospitalization for treatment of a CF exacerbation( 3 nights vs. 8.3 nights) compared to national averages. 18
In the pediatric cohort patients at the Mountain State CF Center, 41.9 % are homozygous for the Phe508del mutation. No deaths among the pediatric cohort occurred in 2023, with 4.2 clinical encounters per patient among the 86 individuals enrolled in the center. Like the adult cohort, the pediatric cohort outperformed national averages among patients evaluated by a respiratory therapist( 100 % vs. 86.4 %), dietitian( 100 % vs. 90.5 %), social worker( 100 % vs. 87.8 %), physical therapist( 67.4 % vs. 21.5 %), pharmacist( 80.2 % vs. 58 %), and mental health coordinator( 80.2 % vs. 50.8 %). Patients aged 18 years and younger receiving care at the pediatric center were hospitalized for fewer nights for treatment of a CF exacerbation( seven nights vs. 10 nights), while those aged 18 years and older had fewer patient reports of one or more exacerbations of CF( 6.7 % vs. 15.1 %) and were hospitalized for fewer nights for treatment of a CF exacerbation( three nights vs. 8.3 nights) compared to national averages.
100 % 90 % 80 % 70 % 60 % 50 % 40 % 30 % 20 % 10 % 0 %
95.5 %
86.4 %
MULTIDISCIPLINARY APPROACH
As a multi-organ disease, CF warrants management from various healthcare disciplines as employed at the Mountain State CF Center and described above. Kerem et al referred to CF as a“ complex disease” requiring a“ multidisciplinary approach” to care, including specialty physicians, nurses, pharmacists, dieticians, and mental health therapists. Adequate funding and institutional support are essential, along with access to specialized consultants. This multidisciplinary approach has improved mortality and quality of life in those with CF. 19 Further, research has found treatment success influenced by financial funding, which varies by country. 19 In the United Kingdom, disease severity determines funding availability, whereas in the US, funding is obtained through individual insurances and the CF Foundation. 19
TRANSITIONING TO ADULT CARE
There is a need to dedicate resources to rural environments for the transition from child to adult care. Studies have found many families face anxieties and difficulties when transitioning care to the adult world. 20 The Mountain State CF Center has specific elements in place to ensure a smooth transition from the pediatric to the adult side of care,
FIGURE 1: Adult Cystic Fibrosis Care in West Virginia
97 % 90.5 %
Evaluated Evaluated by Respiratory by Dietician
Therapist
93.9 %
87.8 %
Evaluated by Social Worker
West Virginia
71.2 %
Evaluated by Physical Therapist
Evaluated by Pharmacist
National Average
Evaluated by Mental Health Coordinator
Influenza Vaccination
Comparison of delivery of CF care to those in WV to national averages. In each category, our center outperformed national averages for referral of CF patients to specialty care. The greatest difference was observed in patients evaluated by physical therapists( 71.2 % vs. 25.1 %), followed by evaluation by a mental health coordinator( 69.7 % vs. 50.8 %).
25.1 %
45.5 % 58 %
69.7 % 50.8 %
89.7 % 72.8 %
West Virginia Medical Journal • December 2025 • 13