Common Complications in Women with Cystic Fibrosis

Cystic fibrosis (CF) can cause a wide range of health complications that vary significantly by the individual and their unique CF mutation, age, and gender. Females with CF may experience slightly different complications of the disease than males.

More severe lung disease

For reasons that doctors do not yet fully understand, females with CF tend to die at younger ages from severe lung disease than males. Research shows that girls acquire more serious lung infections at younger ages than boys, but again, scientists do not understand why. Women also experience more acute exacerbations per year than males.¹

Some doctors speculate that girls have physically smaller lungs and therefore smaller passages deep inside the lungs. Another theory is that the hormone estrogen influences how chloride transports through cell membranes or how the cilia in the lungs work.¹

More cystic fibrosis-related diabetes (CFRD)

Cystic fibrosis-related diabetes (CFRD) is a form of diabetes unique to people with cystic fibrosis. It has some features of both Type-1 and Type-2 diabetes. Similarly to Type 1 and Type 2, the person’s blood glucose (blood sugar) is too high because the pancreas is not making enough insulin. Females with CF develop CFRD at higher rates, and at younger ages, than males with CF.^1,2

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 with weight gain, which can subsequently trigger menstruation.³

Fertility in women with cystic fibrosis

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

Doctors believe this sub-fertility occurs because thick mucus coating the vagina and cervix makes it harder for sperm to fertilize the egg. In other cases, some women with CF may ovulate less often. This may occur if they do not menstruate because of poor lung function, subpar nutrition, or issues of low weight. However, with good nutrition and lung function, fertility rates of women with CF come close to those of the general population.⁴

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.^5,6

Yeast infections

Females with cystic fibrosis are prone to yeast infections. A fungal infection of the mouth and throat is called thrush, while a yeast infection of the vaginal area is called fungal vaginitis. Fungal infections occur when the microorganism monilia or Candida albicans grows out of control. Yeast infections may result in itching, irritation, discomfort, and pain during sex or urination.

In girls and women with CF, these infections are often caused by the antibiotics and corticosteroids that most females with CF take daily.

Vaginal yeast infections are treated with over-the-counter antifungal creams and oral medications. Severe infections may require oral medication. Though it has not been scientifically proven, many people believe that eating yogurt with live bacterial cultures helps restore the body’s balance of beneficial organisms that fight yeast infections. Because vaginal yeast infections are so common, it is important to get annual well-woman examinations.³

Stress incontinence

Twenty-five percent of women with CF experience stress incontinence. Stress incontinence is the unintentional release of urine, usually during sneezing or coughing. It can occur when frequent coughing or childbirth weakens the pelvic floor muscles.

Kegel exercises can help firm up the muscles that support the bladder. Kegel exercises help many women with incontinence. These exercises involve squeezing and relaxing the pelvic floor muscles for a few seconds, in sets of 10, three or more times a day. Wearing panty liners can keep underwear fresh.³

You should mention any incontinence to your CF care team. If they can’t help resolve the issue, they may refer you to a specialist called a urogynecologist. This is a doctor who specializes in pelvic floor disorders, such as recurrent bladder infections, urinary or fecal incontinence, or prolapse (bulging) of the vagina, bladder, and/or the uterus or rectum.