Girls with cystic fibrosis (CF) reach puberty six to eight months later than healthy girls, about 16 percent of young females with CF encounter problems during sex, and 78 percent of these women would like to have children someday.
These are among the key findings of an unprecedented survey conducted in 2015-16, and made public for the first time by its author, Traci M. Kazmerski, MD, an assistant professor of pediatrics at UPMC Children’s Hospital of Pittsburgh.
The report — “Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis” — is based on a survey of 188 females with CF ages 15 to 24 years old. The study took place at five sites: UPMC, Children’s Hospital of Philadelphia, University of Alabama at Birmingham (UAB), Boston Children’s Hospital, and Children’s Hospital Colorado in Denver.
“With over half of all CF patients now over the age of 18, I feel that the CF team must adapt to the developing needs of the adolescent and young adult population,” Kazmerski told Cystic Fibrosis News Today.
“These women with CF view sexual and reproductive health as a key aspect of their emerging adulthood and comprehensive CF care. This is not surprising, as sexual health decisions are often the first medical care many teens and young adults seek independently from their parents,” she added.
Kazmerski presented her findings recently at the 32nd Annual North American Cystic Fibrosis Conference (NACFC) in Denver. A pulmonologist by training — not a sexual and reproductive health specialist — she did her training and residency in pediatric pulmonology at UPMC Pittsburgh, spent two years at Boston Children’s, and had a research fellowship at Harvard before returning to Pittsburgh a few months ago.
Of the 188 women who took part in the survey, 91% were single, 91% were white, and 6% were Hispanic. In addition, 62% of respondents were attending school, while 24% were working full or part time.
Sexual problems often neglected
The survey revealed that 54% of young women with CF reported having had vaginal sex with a male partner, compared with 66% of women surveyed in the general U.S. National Survey of Family Growth — a national representative sample of young women ages 15-24.
Furthermore, 13% of the surveyed CF women had a gastronomy tube, 31% had CF-related diabetes, and 59% had been hospitalized in the past year.
Among other things, the survey — funded by the Cystic Fibrosis Foundation (CFF) — found that girls with CF reach puberty at 11 or 12. That’s six to eight months later than the onset of puberty among girls without the disease.
“Puberty is slightly delayed, but urinary incontinence is something women with CF often experience but rarely talk about,” Kazmerski said. “We had a 15-year-old girl who — if you probed — you’d find she was sick because she wasn’t using her vest, because every time she used her vest, she had urinary incontinence. But no one had ever elicited that.”
Such specialized vests help CF patients clear mucus out of their airways.
Of the 188 survey respondents, 29% perceived a pubertal delay. Also, 49% reported yeast infections, and 16% complained of urinary incontinence.
“Yeast infections are very common with antibiotic use. So is pain during sex. The theory is that their vaginal mucus is very thick and dry,” Kazmerski said. Less common problems include coughing during sex, excessive flatulence, and coughing up blood during or after sex.
Kazmerski’s study also found that 45% of young women with CF expressed a desire to speak about fertility with their CF team, and 31% wanted to discuss pregnancy. In addition, 19% wanted to discuss contraception with a CF provider, 18% yeast infections, and 15% menstruation.
Next survey to include 500 adults
Yet, sexual and fertility issues are rarely talked about during routine clinical visits. Only 26% of women with CF had ever undergone a Pap smear or pelvic exam (compared with 57% of healthy women), while only 37% of young women with CF had ever sought a birth control method, and only 9% reported ever receiving counseling about contraception.
“Young women with CF do have sex, but they are much less likely to have been tested for sexually transmitted infections,” Kazmerski said. “This is important because the face of CF has changed so drastically. More and more of our patients are becoming adults and facing a lot of SRH [sexual and reproductive health] concerns.”
With that in mind, Kazmerski’s next project is a 10-site survey of about 500 women with CF ages 25 and over.
“We’re looking at the whole reproductive life course from periods through menopause because we don’t have good data,” she said. “We’re trying to recruit their partners to complete the survey as well, to understand how they’ve been engaged in discussions with the CF team around parenthood, pregnancy and other SRH concerns.”
The new study, funded by a $300,000 CFF grant, will launch in early 2019. The 10 sites are UMPC, Boston Children’s, UPenn, National Jewish Health (Denver), UAB, University of Washington-Seattle, Northwestern University (Chicago), Baylor University (Waco, Texas), the University of Minnesota (St. Paul), and the University of Texas-Southwestern (Dallas).
“We’ve listened to our adult patients, who have told us we need to work on understanding what’s going on,” Kazmerski said. “Our other main focus is creating tools for them to define and pursue their own reproductive goals.”