Cystic fibrosis in adults: a changing scene.
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.


OBJECTIVE
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 insufficient patients. The latter 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.
Blau
Hannah
H
Kathy and Lee Graub Cystic Fibrosis Center and Pulmonary Unit, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel. hblau@post.tau.ac.il
Livne
Moshe
M
Mussaffi
Huda
H
D000223:Adaptation, Psychological; D000328:Adult; D000367:Age Factors; D003550:Cystic Fibrosis / Q000235:genetics / Q000523:psychology* / Q000628:therapy*; D003695:Delivery of Health Care / Q000639:trends*; D005260:Female; D006304:Health Status; D006801:Humans; D007557:Israel; D008297:Male; D008875:Middle Aged; D010818:Practice Patterns, Physicians' / Q000639:trends; D011379:Prognosis; D011788:Quality of Life / Q000523:psychology*; D012917:Social Adjustment