Sociomedical conditions of swedish patients with cystic fibrosis: a review of the past twelve years

Abstract

Cystic fibrosis--a hereditary disease with chronic pulmonary infections and malnutrition--demands on intensive, troublesome, costly and time-consuming therapy. Only a few years ago nearly all CF-patients died in childhood, but today most of them are expected to reach adulthood. All Swedish CF-patients are registered centrally. This registry has been used for demographic studies, and all the CF-families in the registry have got enquiries about sociomedical conditions. In the years 1968-1977 the number of CF-patients has increased from 116 to 200 and median survival age from 7.5 to 16.0 years. The incidence is estimated to 1:2,200 - 1:4,500. The medical care has been intensified and follows the guidelines of the "Swedish CF-board". The clinical condition of the patients has improved in the period. The most striking social aspects are the long time spent on treatment (mean: 18, maximum: greater than 50 hours/week) and the little help that is given by physiotherapists or community paid helps. The parents have to do all the work themselves! Even if the families receive several specific benefits the economical burden is heavy and strikes very unevenly. School results of CF-children are good. The main wishes from CF-parents are: intensive research to find the basic defect, uniform support to CF-families throughout the country, much information to be given especially to physicians.