Cystic fibrosis. A WHO/ICF(M)A meeting.

Cystic fibrosis represents a major health problem in developed countries. Although its distribution is worldwide, in most developing countries there is little awareness of its prevalence and little opportunity to provide treatment. The improvements in survival of cystic fibrosis patients in developed countries over the past two decades have brought mean life expectancy to 20 years or more, and many adult patients with cystic fibrosis at present are fully integrated in society and have an acceptable lifestyle. Improvements in standard of living, general health, diet, surgical and medical care, including nutritional support, pancreatic enzyme supplementation and antibiotic therapy have all contributed to extended survival of patients and improved quality of life. The role of the community is important in providing support for cystic fibrosis patients and their families. Cystic fibrosis organizations, and mutual-help parent groups play a vital part in educating the public about the disease, disseminating information, and supporting research.The lack of knowledge regarding the fundamental defect of the disease and the lack of appropriate methods for fetal diagnosis and heterozygote detection, limit the possibility of controlling the disease. International collaboration is required to provide internationally useful guidelines for treatment, to promote initiatives in public health, and to assist in the dissemination of educational material. In practice this will be most important in developing countries, where underdiagnosis of cystic fibrosis is widespread. Definition of the gene for cystic fibrosis and its resultant metabolic defect will require intensive specialized and systematic fundamental research, which could also be accelerated by international collaboration.