How Does Cystic Fibrosis Affect Reproductive Health and Fertility?

Infertility can be a complication for adults with cystic fibrosis. Men and women with cystic fibrosis (CF) usually produce normal levels of sex hormones such as progesterone, estrogen, and testosterone, and can, therefore, enjoy a normal sex life.

However, people with CF often face special challenges when it comes to building a family. That’s because the genetic mutation that causes cystic fibrosis results in infertility in almost all males and sometimes makes getting pregnant more difficult for females.

Men with cystic fibrosis and infertility

Ninety-eight percent of men with CF are infertile.^1,2 Although their body creates sperm, a blocked or missing vas deferens prevents the sperm from mixing with the semen. This blocked or missing vas deferens is one of the problems caused by the CFTR genetic mutation. Sexual desire and performance are generally normal.

Males with CF also are four times more likely to develop fluid accumulation around the testes (hydrocele), which can decrease how well the testes work. There is also a 15-fold increase in having an undescended testicle. Some produce a lower volume of sperm or no mature sperm.³

Men with CF who want to father children may be able to have their sperm extracted and used to fertilize an egg. There are different procedures used to take sperm. There are:

• Microepididymal sperm aspiration (MESA)
• Percutaneous epididymal sperm aspiration (PESA)
• Testicular sperm extraction (TESE)¹

After the sperm are collected, they can be used for in vitro fertilization (IVF).

Women with cystic fibrosis and infertility

Cystic fibrosis does not seem to cause any changes to the physical structure of the female reproductive organs the way it does in males. However, women with CF are thought to generally be less fertile than women without CF.

This is because the mucus that coats the vagina and cervix is thicker than usual, making it more difficult for sperm to travel and fertilize the egg. Some women with CF may ovulate less often if they do not menstruate because of lung, nutrition, or weight problems. However, with good nutrition and lung function, fertility rates of women with CF come close to those of the general population.³

If a woman with CF is having trouble getting pregnant, several techniques are available that may help. These procedures include:

• Insemination
• In vitro fertilization²

The number of women with CF who get pregnant has grown since the 1990s. According to the CF Foundation’s Annual Data Report, 138 females with CF ages 14 to 45 years old reported a pregnancy in 1990. By 2017, that number had roughly doubled to 273 pregnancies.⁴

Family planning

As people with cystic fibrosis live longer, healthier lives, family planning has become a more common consideration. Your CF care center may be able to recommend a fertility specialist who specializes in helping people with CF get pregnant.