The management of young adults with cystic fibrosis: 'genes, jeans and genies'

Disabil Rehabil. 1998 Jun-Jul;20(6-7):217-25. doi: 10.3109/09638289809166732.


Cystic fibrosis is now an adult disease. The understanding of the basic pathophysiology of this disease has increased considerably over the past decade. This has resulted in effective management protocols and the development of innovative approaches to therapy. Management of young adults is complex and requires expert medical care delivered in a sensitive and appropriate way. The daily treatment schedule for many adults involves physiotherapy; nebulized, oral and i.v. drugs; exercise and nutritional supplements. Adherence to such treatment while living a busy school, work and social life is extremely difficult for patients. The psychological implications of having a disease with very significant morbidity and mortality also has an enormous impact on individuals with CF. Many of these issues have evolved as survival has increased and the next few decades may produce more unexpected 'genies'. These may be from the disease itself or from some of the exciting new treatments currently being developed. It is important that individuals with CF are fully involved in decisions relating to their treatment and that CF teams provide optimal and appropriate care to enable these young adults to enjoy their lives to the fullest extent.

Publication types

  • Review

MeSH terms

  • Activities of Daily Living*
  • Adaptation, Psychological*
  • Adult
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis / psychology*
  • Cystic Fibrosis / therapy*
  • Female
  • Humans
  • Male
  • Patient Care Team / organization & administration
  • Survival Analysis