CYSTIC FIBROSIS – A GUIDE FOR CLINICIANS IN REPRODUCTIVE AND OBSTETRIC MEDICINE

Abstract

Cystic Fibrosis (CF) is the most common life-shortening recessive genetic disease in the UK. Far from being a condition managed exclusively by paediatricians with sufferers dying in early childhood, CF is now a condition in which adults outnumber children with the condition, and the death of a child in a paediatric centre is rare. As increasing numbers of young people grow up relatively healthy it is unsurprising they begin to ask about relationships, sex, fertility and the possibility of having a child. This paper briefly describes the genetics, pathophysiology and clinical features of CF with an emphasis on how it pertains to female reproduction. The management of women with CF considering having a baby including contraception, preparation for pregnancy, the pregnancy itself, delivery and the post natal period will be discussed. Contraindications to pregnancy, method of termination, and pregnancy after transplantation will also be considered. Much of the detailed management is given in the European Guidelines for the Management of Pregnancy in Women with CF on which this paper is based.