Cystic fibrosis.

The median age at death from cystic fibrosis has increased from six months in 1959 to 17 years in 1986.1 It is assumed, although proof is lacking, that this improved survival is the result of the liberal use of better antibiotics, closer attention to nutrition and improved pancreatic enzyme supplements, more successful management of meconium ileus, increased ascertainment of milder forms of the disease, and the develop- ment of special centres. In the past, incurability was both an attitude of mind and a self fulfilling prophesy, and the notion of early demise insidiously undermined treatment and sapped therapeutic zeal. The greatest change has been a more positive approach. Regrettably, enthusiasm to treat has not been matched by good quality clinical trials, which are hampered by the highly variable nature of the disease, the complexity of clinical scoring systems, and by a system of medical research that encourages short term single centre projects (often carried out by passing registrars whose real interests are not research) when the major need is for longer term multicentre studies of therapeutic approaches. As a result, the modern management of cystic fibrosis remains largely un- validated. Heated debate about the value of cystic fibrosis centres, the reasons for better survival in certain towns and countries, and the usefulness of neonatal screening are the legacy of poor data. There is a danger that hopes for a cure arising out of research in molecular biology may impede more down to earth objective evaluation of current and future treatment strategies.