Pregnancy not harmful in cystic fibrosis

After considering other contributing factors, women with cystic fibrosis (CF) who become pregnant do not have an increased risk of death. Cystic fibrosis is a chronic, progressive, and frequently fatal genetic disease of the body's mucus glands. It primarily affects the respiratory and digestive systems in children and young adults. The sweat glands and the reproductive system are also usually involved. Researchers from the University of Washington Medical Centre, USA, examined the impact of pregnancy on 8,136 women with CF enrolled in the U.S. Cystic Fibrosis Foundation National Patient Registry. Six hundred and eighty of the women who became pregnant were matched up with 3,327 women with CF who were not pregnant. Compared with non-pregnant women, those who became pregnant tended to be older, to weigh more, to have better lung function and to have fewer CF-related complications. The pregnant women had an estimated 10-year survival rate of 77 percent compared with 58 percent for the non-pregnant women. The investigators conducted a separate analysis to take into account disease severity. They looked at age, lung function, pancreatic function, and infection with Pseudomonas aeruginosa. A total of 455 pregnant patients were matched with 2,275 controls. Survival was better among pregnant patients than among the matched control patients. The estimated 10-year survival rate of the pregnant patients was 90 percent compared to 86 percent. Also, pregnancy was not found to be harmful in patients with poor lung function, pancreatic insufficiency and insulin-dependent diabetes. While these findings are reassuring, the researchers point out that mothers with CF still face an early death from the disease. They say 20 to 40 percent will be dead before their child is 10 years old. Discussions regarding these possibilities optimally should occur before conception. However, it is imperative for health-care providers to recognise that they should not impose their own views on an individual patient's reproductive decision making.