Helping patients with cystic fibrosis live longer

The Cystic Fibrosis Foundation (CFF) has been a driving force behind the increased life expectancy CF patients have seen over the last 3 decades. Its contributions include the development of medication through the CFF Therapeutics Development Network (TDN) and disease management through a network of CF Care Centers throughout the United States. The CFF recommends a minimum of quarterly visits to a CF Care Center, and the primary care physician can play a critical role alongside the multidisciplinary CF team.²⁰

At every CF Care Center encounter, the entire team (nurse, physician, dietician, social worker, psychologist) interacts with each patient and their families to maximize overall medical care. Respiratory cultures are generally obtained at each visit. Dual-energy x-ray absorptiometry is performed biannually. Lab work (complete blood count, comprehensive metabolic panel, glycated hemoglobin, vitamins A, D, E, and K, 2-hour glucose tolerance test), and chest x-ray are obtained at least annually (TABLE 2).⁴

Since CF generally involves both restrictive and obstructive lung components, complete spirometry evaluation is performed annually in the pulmonary function lab, with static lung volumes in addition to airflow measurement. Office spirometry to measure airflow alone is performed at each visit. FEV₁ is tracked both as an indicator of disease progression and as a measure of current pulmonary status.

The CFF recommends that each patient receive full genetic testing and encourages patient participation in the CFF Registry, where mutation data are documented among other disease parameters to ensure that patients receive mutation specific therapies as they become available.⁴ The vaccine schedule recommended for CF patients is the same as for the general population.