Patients with cystic fibrosis rarely live to middle age. However, improvements in therapy have produced a fall in the yearly mortality from 20% to between 2% and 6% of affected persons; patients with cystic fibrosis now survive significantly longer than they did one or two decades ago. Shwachman et al1 illustrate this fact, describing 65 patients surviving over the age of 17 years. Not collected from the literature, this series comprises patients from a single institution.
Doershuk et al2 present an equally interesting report, indicating the value of an intensive program of prophylaxis in patients considered not to have irreversible pulmonary damage. These investigators studied 98 patients. Half were assessed, on radiologic evidence, to have irreversible lung damage; the other 49 patients' lung fields did not show evidence of irreversibility. Both groups received intensive therapy, not only during exacerbations but on a regular basis.
Each child came into
CYSTIC FIBROSIS. JAMA. 1966;195(7):579. doi:10.1001/jama.1966.03100070123041