Cystic fibrosis in adults: A changing scene

Hannah Blau*, Moshe Livne, Huda Mussaffi

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

Background: Cystic fibrosis is no longer a terminal illness of childhood and mean survival is now over 30 years. Adult patients with atypical CF are increasingly being diagnosed. In Israel, all patients are still followed in pediatric centers. Objectives: To describe our experience with adult CF, stressing the importance of adult-related health and psychosocial issues. Methods: Twenty-five CF patients aged 20-50 years, constituting 44% of the 57 patients followed at our center, were analyzed for pulmonary and extrapulmonary features and management. Results: Nineteen were diagnosed as children and 6 as adults. Nineteen were pancreatic-insufficient and 6 were pancreatic-sufficient, including 5 diagnosed as adults. Pulmonary status was usually stable, with forced expiratory volume in 1 second 66.3 ± 21% (mean ± SD) and no difference between pancreatic-sufficient and insufficent patients. The tatter had more hemoptysis, Pseudomonas infection, intestinal obstruction, liver disease and diabetes. Two patients died of malignancy and two of advanced lung disease. A majority received continuous inhaled and oral antibiotics, bronchodilators, Dnase, physiotherapy and periodic home intravenous antibiotics. Psychosocial functioning was excellent: 60% were employed, 36% were married and 40% had children (none with CF). Patients diagnosed as adults had mild multisystem disease or isolated severe lung disease. Conclusions: CF adults generally have a good quality of life. Advances in understanding the CF defect and a plethora of new treatment modalities bode well for the future. Patients must be maintained in optimal condition to reap the benefits, and there is an urgent necessity for adult physicians to develop expertise in CF.

Original languageEnglish
Pages (from-to)491-495
Number of pages5
JournalIsrael Medical Association Journal
Volume5
Issue number7
StatePublished - 1 Jul 2003

Keywords

  • Adults
  • Cystic fibrosis
  • Multidisciplinary team
  • Prognosis
  • Transitioning

Fingerprint

Dive into the research topics of 'Cystic fibrosis in adults: A changing scene'. Together they form a unique fingerprint.

Cite this