Scientific Background

Sex characteristics can differ widely even in people of the same sex identity. Sometimes male and female clusters overlap, giving a person intersex characteristics (Carpenter 2018). Some intersex people identify only as intersex; some also identify as female or male (Davis 2015). Even among people without any intersex traits (those who are considered endosex), variability still exists between individuals. 

There is also variability within individuals. For example, people’s anatomy at birth may not match with how their bodies look as adults. When these changes stem from intentional actions to make one’s body reflect a different sex than the one a person was assigned at birth, that is called being transsex (Sumerau & Mathers 2019). A person can also be cissex, meaning their sex was assigned correctly at birth. Many other factors can also influence changes in sexual anatomy over time, including: nonconsensual surgery performed on intersex children, consensual surgery performed on adults of all sex characteristics and identities, accidental injuries, sexual abuse, complications from other chronic conditions, and/or the process of aging itself (see Kuh & Hardy 2002).

Sexual anatomy is a distinct component of sex identity, but is not the entirety of it. An organ inventory helps identify the direction of treatment. It helps for the provider to ask about both organs a person has at present and organs they may have had in the past. Any bacteria that can colonize in the lungs can also cause issues with other organ systems, including sexual and reproductive anatomies. Patients can also have functional differences—bodies may work differently despite having the same basic anatomy. Further, organ transplants may be relevant for SRH care because they dramatically impact multiple aspects of daily life. 

In the future, some people with CF may have uterine transplants in addition to transplants of other organs like the lungs, heart, liver, and kidneys. People with CF might have uterine transplants for a variety of reasons. Some people might get a uterine transplant because they were born without a uterus and want to carry a child. For example, transsex and/or intersex patients might seek this option in the future. Others might be interested in uterine transplants because their original uteri got damaged by persistent infections or other complications of CF.

There is a lack of clinical data on the aging CF population as a whole, and this dearth of information affects our knowledge of sex and sexuality in CF patients. Treatments prescribed today can impact future outcomes of aging patients and potentially cause future complications. A general lack of thorough and person-centered research with feminine people within and beyond the CF community presents additional barriers. Even studies that do address these populations specifically often misses opportunities to critically explore the experiences trans and intersex patients, as well as patients of racial and ethnic minority backgrounds. For example, we do not currently have data about how puberty blockers taken by adolescent CF patients may impact SRH health as those individuals continue to mature. 

Feminine people may experience breast exams differently than those without feminine identities–and the cause of discomfort related to breast exams may be invisible. For example, pleuritic inflammation in the lungs can cause pain on contact. Transplant scarring may also be a factor in doing physical exams comfortably. Some people with CF have breast implants which can impact the process. Others may have had breast reduction surgeries and/or full mastectomies for a variety of reasons, including cancer and sex transition as well as comfort. Finally, some CF patients who have vaginas do not have enlarged breasts but should still get an exam. This can be true for trans and cis patients alike. 

Not every patient with a vagina started out with one. Tissue created from vaginoplasty may not stretch or lubricate as easily as other vaginal membranes. Some people who have had vaginoplasty may also feel sensitive about having their vaginas touched because there is a fear of being perceived as inauthentic. In addition, some people who have had vaginoplasty have much vaginal depth. As a result, so-called “zero depth” or “vulvoplasty” procedures are becoming more common (Jiang et al. 2018). These types of vaginoplasty may even become favored among trans people with CF because of the comparatively lower health risks involved (see Ferrando 2020).

Conversely, intersex patients may have started out with vaginas but share some of the above concerns or have unique ones of their own. Being intersex and having CF may be especially tough because there is still such limited understanding of what intersex characteristics mean or how they intersect with other elements of CF bodies and patients’ health. Most intersex people do have some hormonal differences from their endosex peers; we are learning a lot about how hormones impact CF and vice versa (Holtrop et al. 2021). Feminine people of all sexes also often experience a lot of social pressure about whether their bodies look “right” to others. These concerns can be exacerbated by both being intersex and having CF and their intersection. 

Scientific Background

Gender identity does not necessarily predict sexual and reproductive goals. Similarly,  CF often impacts how we feel about our bodies and social roles. As such, the  intersection between a patient’s health challenges and our gender identity may impact our SRH choices and needs in a variety of ways. 

Stereotypes of disabled people, including those with CF, include assumptions about not being in agentic or leading roles in our lives and in our communities. This has often been an especially challenging experience for AFAB and otherwise feminine patients, given the overall patterns of sexism and infantilization that impact people with chronic conditions, including those with CF.

Paternalism in health care is a general problem that may have particular impacts for SRH choices and the goals of CF patients. Centering the unique wants and needs of AFAB and otherwise feminine people living with CF is an evidence-based move. As more people with CF survive to older ages, the importance of taking a goal directed approach to SRH care increases further. Quality adult CF care in SRH and other areas alike requires providers to consciously nurture patient agency and autonomy.

As highly effective CFTR modulators and other innovations in treatment facilitate changes in CF-sexual and reproductive functioning, many patients have begun to think differently about the range of choices realistically available for our futures. These drugs often thin cervical mucus substantially and induce other changes that can make it easier to conceive children (Heltshe et al. 2017). For patients who want to conceive, the impact of modulators upon cervical mucus is a welcome change. Some people who never thought about becoming parents via sexual reproduction before starting on modulators have realized that having biological children can be an attainable option for them. Conversely, many patients have cemented an understanding that they do not wish to have children by any means as they have advanced to older ages. But for people with CF who have experienced pressure to bear children or unwanted pregnancies or abortion stigma, this pathway is more fraught. In these situations, patients have gained the ability to make informed choices about their reproductive futures rather than defaulting to a sense of fatalism about what their adult lives could include. These choices can both influence and be shaped by how people’s concepts of their own gender develop and change over time.

To understand the SRH needs of gender diverse, CF patients, clinicians need opportunities to meet these types of patients. 

CF impacts our bodies and can reinforce gender stereotypes and cause gender dysphoria or the difficulties related to a strong desire to be another gender. Being able to access health care services that help reduce gender dysphoria regarding how CF affects embodiment is critical. We often think of gender dysphoria as something that only trans people experience. Certainly this is a common—although by no means universal—experience among people with and without CF in the trans community. However, this narrow view of dysphoria misses many important opportunities to provide quality SRH care for people with CF of all sexes and genders.

Some experiences of dysphoria in SRH care for people with CF may be due to the disease’s progression and complications. For example, females who are unable to conceive may struggle with feelings of inadequacy as women and/or females. Likewise, some females both with and without CF may experience dysphoria from excessive questioning about reproductive goals, especially if those goals have been clearly indicated. Some females with CF regardless of their gender identity do not wish to bear children. Within the CF community specifically, dysphoria seems to have become more of a concern in the age of CFTR protein modulators.

The paramount concern in gendered care is to enable the patient to make an informed decision without any pressure from providers.There is no one authentic way to be feminine, only what feels right for a given person. At the same time, it is helpful for providers to remind patients that it is okay for their gendered, embodied experiences to shape and be shaped by CF. How CF affects patients’ lives may also change as new treatments become available and as research that informs CF care becomes more transparent and inclusive (Holman et al. 2020). As such, a provider should explicitly ask how a patient wants to be perceived and how the provider can help them achieve their goals, whether it be gender expression or reproductive wishes. This is the most direct way to provide SRH affirming care for sex and gender diverse people with CF. It is vital to have these care spaces for AFAB and otherwise feminine people with CF who experience forms of marginalization.

Scientific Background

The scientific literature on SRH affirming care in CF is scarce. Some research on stigma and CF has revealed that broad cultural attitudes about chronic disease and sexuality impacts care for people with CF in SRH settings (see Fonte et al. 2018 below). Issues of stigma, sexuality, and CF are situated within larger patterns for people with chronic illness which are described fully in the scientific literature on experiences of chronic illness. 

Desexualization of people with chronic illnesses, for instance, is widely documented in the literature on chronic illness and disability. (Nowakowski 2016). Because people with CF and other disabilities are often infantilized by society, they are also often desexualized, including in health care settings (Fonte et al. 2018). Exocrine pancreatic insufficiency for patients with CF may exacerbate infantilization since it makes CF patients’ bodies very thin, and thus seem childlike; this potential link warrants further study. Finally, people with CF often experience ableism–discrimination against disabled people–relating to their sexual behavior from clinicians and non-clinicians alike (Werner et al. 2019).

Discussions between a provider and a patient related to sexuality requires both to be comfortable raising the issue in clinical settings. Research is just starting to touch on the nuances of different sexualities and how they intersect with CF health care (Schrock et al. 2014). The existing literature makes clear that patients cannot be expected to talk openly about their own experiences without having providers sending clear signals that the patient is safe to do so. The more healthcare providers welcome talking with their patients about the experiences of people of different sexualities with CF, the easier it becomes to talk through these issues. 

Further, discussions around patients’ sexual practices, and how CF impacts them openly, can help maximize quality care. Whatever their specific sexualities may be, all patients can benefit from the ability to talk about these aspects of their lives and how their health intersects with sexuality while receiving SRH care. Understanding that each person with CF is unique in their sexual identities, desires, and experiences can help SRH providers create affirming spaces for these discussions. 

When SRH or CF providers have discussions with patients about these issues, they can try to avoid making assumptions about what kinds of sexual activities a particular person with CF may be doing, or what needs they may have related to these activities. To know what kinds of sexual activities and experiences are relevant for the care of a particular person with CF, a provider must ask them.

Overall, open discussions about sexual practices and CF communicate caring and affirmation for people of all different sexualities. These types of conversations help patients feel more at ease sharing their lived experiences. Having preliminary conversations about these issues can communicate to the patient that their choices matter. This message can help patients explore their preferences on their own and can facilitate effective dialogue with their partners.  

Some of the same practices recommended for helping people with CF of different sexes and genders feel safe and welcome in their communities can also apply to clinic discussions. Providers and patients can share their pronouns; a clinic can have images posted of people in different types of intimate relationships; a clinic can provide a thorough list of options relating to how people can describe their different identities on paperwork; and a clinic can hire staff whose lived experiences reflect the diversity of our community. 

Types of Sexualities

There are no real fixed patterns of number or intensity of sexual relationships by types of attractions, except for some asexual and demisexual patients. “Asexual” describes people who have limited interest in sex; “demisexual” describes people who are only interested in sex when they feel emotional closeness with people (Chasin 2011). These are distinct sexual identities like being gay or lesbian (experiencing attraction mostly to people of one’s own sex) or being bisexual or pansexual (experiencing attraction to people of one’s own sex as well as other sexes) (Pinto 2014). People who are asexual and demisexual often consider themselves to be part of the broader queer community, as do people of other non-hetero sexualities.

People who are asexual or demisexual often have more limited sexual histories and fewer lifetime partners, although this is not always the case. Some people who are very sexual may have low numbers of past partners and/or gaps in their sexual histories. These patterns may or may not have any direct relation to CF disease, though they can affect forms of CF embodiment. For example, someone might be less sexually active during a prolonged period of hospitalization or intensive illness management at home. Another person with CF might be celibate for a couple of years for personal reasons not directly related to the disease, such as a history of abuse or recovery from addiction. These scenarios apply to both people who tend to be “monogamous” or “mono” and to those who tend to have more than one partner at a time, that is, “polyamorous” or “poly” (Simula et al. 2019). 

SRH providers can remind patients with CF that different forms of sexual activity may be more suitable at different times. Pleasure can come from a wide array of different activities, whether with partners or by oneself. It does not have to include contact with the gentical mucosa. Sexual activities that do not involve any genital contact may be especially attractive for people with CF during times when the pelvic mucosa are actively infected or sore or when the patient does not feel as well or energetic.

Patients with multiple partners may need extra support and reminders about certain safe sex practices. For example, using barrier methods is always recommended with any partners where a “fluid bond” does not previously exist (Sumerau & Nowakowski 2019). Fluid bonding for people with CF often involves additional considerations and decisions, like agreeing with one’s partner(s) that their other partners will not be intimate with anyone else who has CF. The issues surrounding partners’ behaviors makes STI testing all the more important. Early detection of STIs may be even more vital for people with CF because it is difficult to eradicate infection in mucosal tissue. Further, asexual people who do not have sex can still get STIs by other means. Providers who recommend appropriate infection control and lubrication strategies can support good SRH for CF patients of all sexualities.