Reproductive health in women with cystic fibrosis

Abstract

The life expectancy of women with cystic fibrosis has doubled in the last 20 years. A major implication of this is the advent of previously unseen reproductive health problems. We review the management problems presented by these women throughout their reproductive lives, including pregnancy.

Pregnant With Cystic Fibrosis: One Case Study Explores the Nursing Interventions of a Twin Gestation CF Patient

“…Cystic fibrosis, the most commonly inherited genetic disorder in Caucasian Americans (Willett & Ellis, 1999), is manifested as exocrine gland dysfunction with altered epithelial cell secretions. The dysfunction leads to the production of thick, sticky secretions that prevent transport of sodium and chloride through cells lining the lungs and pancreas.…”

“…One in 31 Americans (Cystic Fibrosis Foundation, 2001) carries the classic ∆F508 CF autosomal recessive gene (Willett & Ellis, 1999). To inherit cystic fibrosis, an individual must receive a CF gene from each parent.…”

“…The most important predictor of maternal and fetal outcome is pulmonary function (Gillijam et al, 2000). Nutritional status, pancreatic function, severity of disease, colonization of Burkholderia cepacia (bacteria associated with an increase in morbidity and mortality of CF patients) and frequent infectious exacerbations were determined to be potential predictors of long-term survival and pregnancy outcomes (Cohen et al, 1980;Frangolias et al, 1997;Gillijam et al, 2000;Kent & Farquharson, 1993;Walsh, Casano, Manangan, Sinkowitz-Cochran, & Jarvis, 2002;Willett & Ellis, 1999).…”

“…These changes may provide additional physical challenges for CF women who may already have compromised pulmonary, gastrointestinal and pancreatic systems. Women with CF are considered to have a high-risk pregnancy (Willett & Ellis, 1999). Table 1 contains information regarding major system manifestations, implications and recommended management activities for pregnant women with cystic fibrosis.…”

“…These authors further acknowledge use of an epidural for pain management may reduce the increased ventilatory demands associated with labor discomfort. Due to the potential complications of pulmonary function, an epidural is the preferred method of anesthesia during a cesarean section of a CF patient (Willett & Ellis, 1999).…”