看護とケアのジャーナル

Journey to the Center of a Disease: The Cystic Fibrosis Saga

Abstract

Robert L. Vender* and Amanda Florey

Since the first clinical description of the disease cystic fibrosis (CF) in 1938, there have been innumerable milestones in relation to delineation of disease pathogenesis and subsequent treatment which continue to this day. This narrative review: 1) details key discoveries that have significantly impacted overall CF patient health, 2) correlates these important advancements specifically to clinical observations from providers at the Penn State Health Milton S. Hershey Medical Center (PSH-HMC) adult CF program and 3) provides potential future directions since the regulatory approval of cystic fibrosis transmembrane conductance regulator (CFTR) protein therapies. With the availability of highly effective CFTR modulator therapies, providers from the PSH-HMC adult CF center have observed the following major changes in the health and lives of adult CF patients including: 1) improved survival, 2) reduced hospitalizations and 3) an increase in the number of women with CF successfully completing pregnancy, child-birth and subsequently entering into motherhood. This changing clinical landscape of all patients with CF will probably necessitate revisions in current practice patterns and adjustments by multiple care providers and care systems including the addition of providers previously minimally involved in CF patient care.