Management problems of the adult with cystic fibrosis

Aggressive palliative therapy which includes antibiotics, physiotherapy, exercise and adequate nutrition remain the basis for treatment and account for improved survival into adulthood. However, although mean actuarial survival into the third decade of life is to be expected in well organised cystic fibrosis centres, a plateau has probably been reached and we need innovative forms of treatment before we can expect further improvements. Indeed, as patients have matured into adulthood new clinical problems have arisen; diabetes is more common, liver disease can progress, and vasculitis may affect joints, skin and brain. Furthermore, social and psychological problems are magnified by patient realization of a deteriorating lethal disease. This article discusses the recommended provision of care for adults with cystic fibrosis, the management of medical and social problems, and finally, how the introduction of heart lung transplantation has affected patient management in the terminal phase of the disease.