Let’s Talk about Sex: Behaviors, Experience and Health Care Utilization in Young Women with CF

In this issue of the journal, Kazmerski et al describe the sexual and reproductive health concerns and related healthcare utilization of young women with CF ages 15–24 years at five CF centers compared to that of the general population^1,2. They found that the sexual activity of young women with CF mimics the behaviors of non-CF counterparts; however women with CF have less access to sexual and reproductive health information and services. This lack of education, and sometimes care, is particularly troubling given the known and potential complications CF can have on sexual and reproductive health³ and vice versa⁴. The contribution of hormonal changes to the substantial decline in lung function and increased acquisition of Pseudomonas aeruginosa that is observed in adolescents and young adults with CF has yet to be completely defined⁵. However, this phenomenon underscores the idea that for young women living with CF, their care providers have a responsibility to help them make informed decisions regarding sexuality and family planning, in addition to their overall health.

The transition from adolescence to young adulthood is fraught with challenges including puberty, development of sexuality, risk taking behavior and planning for the future⁶. For many with CF, transition of care from the pediatric CF center that they have known their entire lives to the adult center can be tumultuous, and perhaps itself is a barrier to addressing anxiety-provoking topics such as sexuality and reproduction. For example, the pediatric CF team may be uncomfortable having conversations about sex with a patient they have known since infancy, and the adult CF team is also challenged to have intimate discussions with a patient they have only just met. Both teams may assume the other has or will address such topics –so neither has the conversation. Transition of care of people with chronic medical conditions from pediatric to adult oriented care needs to be a “purposeful, planned process that attends to the medical, psychosocial, educational and vocational needs” of the patient⁷. It is recommended that the process for transition preparation should begin between the ages of 11–13 years of age⁶. The work by Kazmerski et al^1,2 makes it poignantly clear that discussion of sexual and reproductive health must be added to this process by the pediatric CF team, and later reinforced by the adult CF team on transition of care.

So what are the barriers to CF providers initiating the sexual and reproductive health discussions that 66% of the respondents said they want to have with their CF team? Based on previous work by Kazmerski et al, although most providers felt that sexual and reproductive health was important to discuss, many also felt uncomfortable with such discussions. Much of this discomfort appeared to stem from a lack of knowledge about the topics, lack of age-appropriate educational resources and lack of guidelines and standardization⁸. While there are currently published clinical care guidelines for other important health issues in CF⁹, there is no guidance for sexual and reproductive health care in women with CF. A published sexual and reproductive health guideline written jointly with international obstetrics and gynecology societies might provide the confidence to physicians to initiate these much needed and patient desired discussions.

Fortunately, more than 50% of those living with CF are now over the age of 18 years, and the predicted survival continues to increase (47.7 years for those born in 2016)⁵ as a result of the development of new therapies, including CFTR modulators.^10,11 Kazmerski et. al.¹ opened their survey one month before the CFTR modulator, Orkambi®, was approved for people homozygous for F508del CFTR mutation (affecting approximately 45% of the population). It is important to consider the possible impact of this timing on their findings. Could it be that intrauterine devices (IUDs) were more prevalent in this population because contraception choices are limited with CFTR modulators¹¹? Is enthusiasm for CFTR modulators shifting the perceptions and expectations about family planning among women with CF? There is some evidence that pregnancy rates among women with CF are increasing,¹² and Kazmerski et. al. report that 78% want a child in the future¹, but these aspirations may be complicated by the unknown effect of modulators on contraception efficacy, fertility, pregnancy, fetus, and breastfeeding safety ^{13, 14}. There has been a case report of a safe, successful pregnancy on ivacaftor¹⁵, but there is at least one modulator in development shown to be teratogenic in preclinical studies¹⁶. Furthermore, as Kroon et al highlight in this issue of the Journal, safety data for the use of therapeutics during pregnancy and breastfeeding is still lacking for a wide range of drugs that are commonly used in people with CF.¹⁴

In summary, sexual and reproductive health in women with chronic diseases such as CF has been understudied in research and not sufficiently emphasized in clinical care. The studies by Kazmerski et al in this issue of JCF highlight key areas for improvement. People with CF are living longer, fuller lives. We have an obligation to provide them with informed sexual and reproductive health choices that take into account their specific health challenges and life-long therapies. An integrated approach to better understanding of the needs of older women (over 24 years of age) and also men with CF is crucial for comprehensive care and counseling on pubertal delay, sexual dysfunction, contraception, pregnancy and child rearing. This approach will require engagement of family practitioners, obstetricians, gynecologists, psychologists and CF health care teams including endocrinologists to conduct qualitative and quantitative research via surveys, clinical trials, or registries, and then rapidly disseminate that information to all providers who care for patients with CF.¹⁷