Newly diagnosed cystic fibrosis in middle and later life

Abstract

Four patients with cystic fibrosis diagnosed in middle and later life are presented. All had chronic bronchopulmonary infection with a high sweat sodium concentration, and chest radiographic evidence of upper zone bronchiectasis. Two patients had pancreatic dysfunction. Sputum culture grew mucoid Pseudomonas aeruginosa in three patients and Haemophilus influenzae in one. Ages at diagnosis were 63, 42, 40, and 35 years. These patients confirm the possibility of occasional longevity in cystic fibrosis and emphasise the need to consider the diagnosis at all ages. They also provide encouragement for younger patients.