Current management of cystic fibrosis.

Although forty years have passed since the discovery of the clinical entity 
called (for lack of a better name) cystic fibrosis, its treatment 
remains both symptomatic and controversial. Moreover, the efficacy of therapy has 
been difficult to evaluate for several reasons: the lack of a 
suitable animal model for therapeutic experimentation, the protean manifestations of the 
disease, the natural variations in its severity, the prolonged periods of 
observation that are required by its very chronicity and frequently the 
lack of satisfactory clinical parameters by which to score response. Stated 
in its simplest form, the treatment of cystic fibrosis is directed 
towards the long-term control of salt loss, malabsorption and bronchial obstruction, 
the management of several complications of the disease and the establishment 
of a positive therapeutic environment that minimizes anxiety and depression. The 
purpose of this review are to help the pediatric practitioner gain 
security in his day-to-day management of children with the disease and 
to advocate an effective working relationship between the practitioner and a 
regional cystic fibrosis or pediatric pulmonary center for long-term patient management. 
Problems in diagnosis, treatment of salt loss, the prevention and treatment 
of malabsorption, the prevention and treatment of bronchial obstruction, the prevention 
and treatment of certain complications, and the psychosocial support of the 
patient and family are discussed. 