Cystic fibrosis: A clinician's tool for management of care advancing into the adult population
Article first published online: 9 JUL 2012
©2012 The Author(s) Journal compilation ©2012 American Academy of Nurse Practitioners
Journal of the American Academy of Nurse Practitioners
Volume 24, Issue 11, pages 625–632, November 2012
How to Cite
Nast, D., Paniagua, C. and Anderson, P. (2012), Cystic fibrosis: A clinician's tool for management of care advancing into the adult population. Journal of the American Academy of Nurse Practitioners, 24: 625–632. doi: 10.1111/j.1745-7599.2012.00763.x
- Issue published online: 22 OCT 2012
- Article first published online: 9 JUL 2012
- Received: September 2011;, accepted: June 2012
- 25-hydroxyvitamin D;
- malabsorption syndrome;
- advanced practice nurse (APN);
- health promotion
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.
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Disclosures The authors report no competing interests.