Cystic fibrosis: a clinician's tool for management of care advancing into the adult population

Abstract

Purpose: To discuss the role of the nurse practitioner in medical management of adult patients with cystic fibrosis (CF).

Data sources: A systematic literature review was done by searching online databases: CINAHYL, OVID, and Medline. The Cystic Fibrosis Foundation (CFF) guidelines were reviewed. Tables were compiled for recommended screening and treatment for adult CF patients.

Conclusions: Comorbidities occur among adult CF patients and include: CF-related diabetes (CFRD), fertility issues, CF liver disease (CFLD), and CF bone disease. Diabetes management is reviewed. Current fertility surgical treatments for males and recommendations for females are discussed. Ursodiol is the main treatment regimen for those affected. Bone disease and CF has multiple etiologies and the focus of treatment is based on appropriate vitamin D replacement and bisphosphonates. New standards for vitamin D replacement are discussed.

Implications for practice: Clinicians must be familiar with the current treatments and regimens of adults with CF including comorbidities. The CF adult population is expanding and care is extending beyond the CF clinic. More healthcare providers must be educated on the best practices of adult CF care. The importance of early identification and treatment of CF comorbidities cannot be overestimated.