How Are Women Affected By Cystic Fibrosis?

While the basic complications of cystic fibrosis (CF) tend to be the same for females and males with cystic fibrosis, girls and women do experience some unique problems with the disease.

For example, females with CF tend to have higher rates of CF-related diabetes and higher body mass index (BMI). They tend to acquire more serious lung infections earlier in life than males (9.5 years in females and 11.2 years in males), which damage lung tissue and make long-term survival more difficult. Girls and women also get more acute exacerbations per year.¹

For adults with CF who were diagnosed as children, lung function declines more rapidly in women after age 40 than in men.¹

Lower survival rates in childhood

Females account for roughly half of all cases of cystic fibrosis (CF) but generally have worse outcomes than males until about age 20. After that, men and women experience roughly equal outcomes for long-term survival. Still, males with CF tend to live an average of 2 years longer than females.^1,2

The differences in mortality are not fully understood. Some researchers theorize that estrogen influences how the cilia in the respiratory system work and in sodium transport across cell membranes. Others think that females have a disadvantage because the lungs and airways are smaller.¹

Delayed puberty and irregular periods

Girls and women with CF who have low body weight, poor nutrition, and poor lung function may also fail to ovulate or menstruate regularly. This may result in delayed puberty in girls. Improving lung function and nutrition usually helps a girl put on weight which can lead to getting their first period.³

Fertility in women with cystic fibrosis

Cystic fibrosis does not cause any changes to the physical structure of the female sex organs the way it does in males who have CF. However, women with CF are thought to generally be less fertile than those who do not have it.

This sub-fertility occurs because the mucus that coats the vagina and cervix makes it harder for sperm to fertilize the egg. Also, some women with CF may ovulate less often if they do not menstruate because of lung, nutrition, or weight issues.⁴

Yeast infections

Females with cystic fibrosis are prone to yeast infections, which is also called fungal vaginitis. Fungal vaginitis occurs when the microorganism Candida albicans grows out of control in the vagina and the area outside the vagina. Yeast infections may result in itching, irritation, discomfort, and pain during sex or urination.³

Stress incontinence

Stress incontinence is the unintentional release of urine and 25% of women with CF experience it. Usually, a small amount of urine gets squeezed out during sneezing or coughing. It is generally the result of pelvic floor muscles weakened by frequent coughing or childbirth.³

Late-life diagnosis of cystic fibrosis

Unlike females diagnosed as children, women diagnosed with cystic fibrosis after age 40 had similar rates of lung function decline as men and overall live 9 to 13 years longer than men.²