Experience at an adolescent and adult cystic fibrosis clinic. An analysis and overview.

Cystic fibrosis (CF) is the most common serious genetic condition in the White population groups. Thirty-three White patients (mean age 20 years, range 14-32 years) seen at an adult CF clinic at the Johannesburg Hospital between January 1980 and January 1983 are reviewed. All had elevated sweat chloride levels. There was a family history of CF in 45,5% of the cases. Most were of normal height but significantly underweight. Chronic obstructive pulmonary disease was present in 94% of the patients. Pulmonary infections played a major role in both symptomatic exacerbations and progressive lung disease. Most frequently, mucoid Pseudomonas aeruginosa and/or Staphylococcus aureus were cultured from the sputum. The management of infective exacerbations is discussed. Other respiratory complications included cor pulmonale, haemoptysis, recurrent pneumothorax and sinusitis. Chest radiographs and pulmonary function tests are also analysed. Gastro-intestinal complications included pancreatic exocrine insufficiency (94%), intestinal obstruction, cholelithiasis, and liver disease. Insulin-dependent diabetes occurred in 9%. Five pregnancies were recorded in 4 patients. By January 1983 there had been 7 deaths (mean age 19,4 years). Most of the patients are highly motivated, with few psychological problems, and appear to be well-functioning and integrated members of society.