BACKGROUND: The survival of patients with cystic fibrosis (CF) now occurs into the fourth decade of life. Our aim was to evaluate the fertility and pregnancy outcomes of men and women with cystic fibrosis within a large British cohort. METHODS: A population based cohort study of 4659 males and females registered with the UK Cystic Fibrosis Database in 2001. RESULTS: Poor respiratory health (forced expiratory volume in 1 s FEV1 <50%) and CF-related diabetes increased significantly post-puberty compared with childhood. Few individuals with CF sought fertility treatment (1% men, 0.5% women) or achieved pregnancies (1.3% of partners of men, 5.7% women). However, the majority of pregnancies had a good outcome (67% live term birth among male partners, 74% among women). Women who achieved a pregnancy were less likely to suffer from poor respiratory health (FEV1 <50%), age adjusted Odds Ratio 0.6 (95% CI 0.3-0.9), less likely to be homozygous for the DF508 genotype OR 0.5 (95% CI 0.3-0.9) and had a significantly earlier median age at diagnosis (0 years vs 2 years, P=0.001). CONCLUSIONS: Increasing numbers of males and females with cystic fibrosis are reaching reproductive age but currently very few have a child. Optimal adult health should improve the reproductive prognosis for both men and women.
BACKGROUND: The survival of patients with cystic fibrosis (CF) now occurs into the fourth decade of life. Our aim was to evaluate the fertility and pregnancy outcomes of men and women with cystic fibrosis within a large British cohort. METHODS: A population based cohort study of 4659 males and females registered with the UK Cystic Fibrosis Database in 2001. RESULTS: Poor respiratory health (forced expiratory volume in 1 s FEV1 <50%) and CF-related diabetes increased significantly post-puberty compared with childhood. Few individuals with CF sought fertility treatment (1% men, 0.5% women) or achieved pregnancies (1.3% of partners of men, 5.7% women). However, the majority of pregnancies had a good outcome (67% live term birth among male partners, 74% among women). Women who achieved a pregnancy were less likely to suffer from poor respiratory health (FEV1 <50%), age adjusted Odds Ratio 0.6 (95% CI 0.3-0.9), less likely to be homozygous for the DF508 genotype OR 0.5 (95% CI 0.3-0.9) and had a significantly earlier median age at diagnosis (0 years vs 2 years, P=0.001). CONCLUSIONS: Increasing numbers of males and females with cystic fibrosis are reaching reproductive age but currently very few have a child. Optimal adult health should improve the reproductive prognosis for both men and women.