The Impact of UI on the Lives of Women with CF  

Incontinence affects both women’s clinic behaviors and their daily lives in numerous ways. For example, patients may not exhale fully during pulmonary function testing to avoid the pressure placed on the pelvic floor muscles in an effort to avoid incontinence. This strategy to avoid urinary incontinence can compromise the “assessment and management of their lung disease” [2]. Likewise, patients may limit airway clearance to avoid leakage. They might also abstain from or reduce exercise, intimacy with a partner, or other potential triggers to avoid leakage, especially if those activities occur in public. Women may also urinate prior to need, limit fluid intake, or change their level of activity so they can stay close to a bathroom [5].

For women with CF, chronic contraction of the pelvic floor during coughing can lead some patients to tighten their vagina, leading to painful sex and other vaginal health conditions. Yet, like other women, the barriers that prevent self-reporting of UI, including stigma, shame, and embarrassment, as well as “perceived bother,” may also prevent patients from seeking treatment for incontinence [5]. In 2001, Orr suggested providers need to develop a “practical and sensitive approach” to address incontinence as part of their routine CF management, but this approach has not yet been widely implemented [3]. With provider and patient education, pelvic incontinence screening for CF patients can increase [3,7,15]. Such efforts are needed because leakage can become progressive and chronic, worsening quality of life.

Gaps in knowledge and treatmentThere is no data yet about how reduced cough by patients on CFTR modulators will impact incontinence incidence and prevalence rates. Despite a potential decrease in the incidence rate of urinary incontinence (UI) in patients with CF, these patients may still have weakened pelvic floor muscles. UI could therefore still be present and may require treatment through postural changes, medications, and physical therapy. In addition, there is no data that assesses the prevalence of fecal incontinence (FI); particularly whether it is more common when patients are on antibiotics and experience loose stools. 

Several years ago, the National Institute of Diabetes and Digestive and Kidney Diseases convened a workshop spanning two sessions where experts proposed a UI treatment pathway. The pathway (Figure 1) consists of 5 steps women must navigate to successfully address their symptoms. 

Operationalizing this pathway through routine discussion and assessment of UI in CF clinical care, provision of psychosocial support, and development of a robust referral system could improve access to UI care for female patients. However, a lack of incontinence management in CF clinical care persists.    

Mental Health

Urinary Incontinence (UI) may be a normal part of aging (i.e., it often occurs with perimenopause for women), but it is often embarrassing to those who experience it. For many people, UI can decrease their quality of life and impact their finances and medical status. [4] 

Females’ body image and self-esteem can suffer from UI. Nygaard et al. state that an association between depression and incontinence exists amongst middle-aged women [9]. In their exploratory study of middle aged, partnered women (n=57), Saiki and Meize-Grochowski found that psychological health, relationship satisfaction, and relationship dynamics were correlated, no matter the severity of their symptoms [10]. More specifically, UI can cause women to experience a loss of desire, less lubrication, and less sexual satisfaction. Fear of coital urinary incontinence can lead to less frequent sexual activity, more avoidance behaviors, dyspareunia, and abstinence in women with UI [11]. [see the Sexual Function chapter]. UI can also cause women to avoid achieving orgasm for fear of leaking [6]. These behaviors can affect partners and strain intimate relationships, although women who experience them tend to express larger worries than their partners [10, 12-14].

Around the world, UI has been shown to negatively impact people’s mental health.8 Studies on UI in patients with CF conducted in Australia, the UK, Belgium, and Poland have found that UI hurts their social lives, intimacy, family life, holidays, school/work, and relationships.[9] Frayman’s systematic review found that despite the high prevalence of UI among people with CF, few patients share their UI concerns with their CF providers, mostly due to feelings of embarrassment.[9] Men with CF who experience UI have significantly higher scores for anxiety and depression than men with CF but without UI.[10] For men without CF, moderate UI is associated with the highest rates of depression compared to other forms of UI.[1,4,8,10] 

Though a significant portion of the female CF population experiences urinary incontinence, UI is under-reported and therefore under-assessed. Despite a hesitancy to discuss UI with their CF team, women report that stress UI negatively impacts their ability to exercise, their social lives, and their sexual relationships.[1,11]  

Modulators and Urinary Incontinence

Very little research has been conducted on UI in CF since the emergence of modulator therapy. A study by Van Citters et al. reported that PwCF on modulators showed improvements in lung function, saw fewer hospitalizations, and reduced the number of medications and therapies they took.[12] A majority of the participants reported that,  since starting modulators, they had an improved sense of well-being (69 %), an improvement in their overall health (85%), and improved quality of life (79 %).[12] Their physical and well-being were significantly better than those who were not taking modulators or had discontinued the therapy.[12] Nearly half (47 %) of their respondents on modulators also reported a better experience with care.[12] However, people with CF who are not eligible for CFTRm because of their genotype, or those who cannot tolerate it, may feel that the gains in UI-related symptoms for those on modulators are out of reach for them.

Vargas’ qualitative study addressed UI in women with CF over 18 on modulators.[3] She and her team asked participants to reflect on their experiences with CF and UI, including symptoms, care-seeking behavior, treatment history and preferences, and the role of their CF health care providers in their UI care.[3] 

Five key themes emerged from the Vargas study: 

1. 1. Most women report that their UI symptoms do not interfere significantly with their lives because they prioritize other medical issues. Still, CFTR modulator therapy (CFTRm) has greatly improved UI symptoms because their coughing has decreased.[3] 
   2. Though most women with CF and UI had discussed their UI symptoms with peers or family, they did not seek care because either it was a low priority or because of the stigma.[3]  
   3. Women’s knowledge of UI as a condition that could be treated is very low. They also did not have sufficient knowledge about treatment options. Women with CF were also unaware that UI is comorbid with CF.[3] 
   4. Many women reported that they were hesitant to initiate conversations with their CF providers about UI. Their apprehension limited access to care. Yet most participants said they desired broad screening for UI from their reproductive healthcare team and improved multidisciplinary care relating to UI from their CF clinic.[3]
   5. Women want access to highly effective UI treatment options with low cost and minimal invasiveness. They desire easily accessible resources as well.[3]

Despite showing improvement in their UI symptoms since starting modulator therapy, Vargas reported that participants may still experience incontinence because of weak pelvic floor muscles that result from a history of chronic cough.[3] Researchers need to conduct further research that investigates the relationship between chronic cough, UI, and modulator use in men and women.[3]