Terminology

Understanding vaginal health terminology is key to seeking proper diagnosis and treatment. 

VAGINITIS: defined as vaginal inflammation that can result in discharge, itching and pain.

Common types of vaginitis are:

• CFTR – Cystic Fibrosis transmembrane conductance regulator; it maintains the salt and water balance in the body, which ensures the mucus produced in the body is thin.4 
• Bacterial Vaginosis: see Causes, Symptoms and Treatment chart 
• Yeast Infections: see Causes, Symptoms and Treatment chart 
• Trichomoniasis: Caused by a parasite commonly transmitted by sexual intercourse.
• Viral Vaginosis: Viruses of the vagina mostly spread through sexual contact.

VAGINISMUS: A condition in which involuntary muscle spasms prevent vaginal penetration.

VAGINAL ATROPHY:  A condition in which the lining of the vagina becomes thinner and drier. Vaginal dryness can be caused by a drop in estrogen levels as a result of menopause, childbirth, or ovary removal. Cervical mucus can be affected by vaginal dryness, antibiotics and transplant medication. Further research is underway to understand the impact of CF modulators on cervical mucus.

• Vaginal Flora – the bacteria that live inside the vagina. Bacteria help maintain a healthy vaginal microbiome.

VULVODYNIA: Chronic pain or discomfort around the opening of the vagina.

Researchers are trying to find the causes of vulvodynia [7]. They may include:

VAGINAL CANCER: A disease in which malignant (cancer) cells form in the vagina. Signs and symptoms include pain and abnormal vaginal bleeding. Pap smear tests can detect cancer. The HPV vaccine is an effective prevention.

VULVAR CANCER: A type of cancer that occurs on the outer surface area of the female genitalia. It commonly occurs as a lump or sore on the vulva that often causes itching. HPV vaccination also prevents vulvar cancer.

SEXUALLY TRANSMITTED INFECTIONS (STI): These infections spread through sexual contact, including intercourse, and require testing by a gynecologist or primary care provider. STIs, such as herpes, can negatively impact vaginal health, potential pregnancy, and sexual partners. Self monitoring can help a woman with CF notice and address potential symptoms with their women’s health provider. Symptoms to report include:

A CFReSHC snap poll revealed that 22 of 61 respondents use an antifungal (oral or vaginal cream) routinely or as needed to treat their VYI. Twenty-nine respondents preferred an OTC treatment; 10 used other options such as modifying their diet or using homeopathic treatments [14].

Research on Vaginal Health

Cervical mucus changes according to the fluctuating hormones during the menstrual cycle. As one reaches ovulation, cervical mucus becomes slippery and the color of egg whites, but during the luteal phase (when progesterone rises and estrogen drops), cervical mucus becomes dry until menstruation.[5] 

The CFTR protein is present in the endocervical epithelium and fallopian tubes, and this can affect cervical mucus, making it sticky and thick, and increase the risk of cervical dysplasia for women with CF.[3,6] Modulators seem to be changing this profile. Still, poor lung function, frequent exacerbations, hydration levels, diet, medications, pregnancy, and one’s CF genotype can also affect cervical mucus quality.[7] Monitoring cervical mucus quality in females with CF is essential because individuals with CF are prone to develop yeast infections, which makes the cervical mucus appear curdy, accompanied by pain, redness, and swelling.[8]  

Kazmerski’s survey on sexual and reproductive health among 188 adolescent girls and young adult women with CF found in 2018 that 18% experienced yeast infections and that only 26% had ever undergone a Pap smear or pelvic exam (unlike 57% of women without CF) [1].

Rousset-Jablonski et al. (2018) report that there is a high percentage of females with CF (FwCF) who have abnormal pap smears and cervical dysplasia. This occurs in both transplanted and non-transplanted females.3,4 Despite this heightened risk, the level of cervical screening in females with CF is inadequate, with only 55% of females having reported a previous pap smear.[4, 5] 

Females with CF who are transplanted and taking immunosuppression drugs can also have an even greater risk of cervical dysplasia.[4,6] Because of the positive impact modulator therapy has had on longevity, it has only increased the need for regular Pap smears for females with CF. More broadly, modulator therapy has increased the need to address sexual and reproductive health (SRH) care and decision-making. Indeed, the modulator era has driven a need to consider including SRH issues in delivering CF adolescent and adult care. West et al. and CFReSHC patient-partners both point out that most women with CF want to discuss their SRH care in the CF setting, yet only a minority receive or have such discussions.[4]

1. Ovarian dysfunction and decreased ovarian reserve have been documented…cervical mucus and uterine fluid pH (see questions below); changes after HEMT.[5]
2. Genital Candidiasis and modulator therapy require adjustments in azole therapy.[4]

A CFReSHC Snap poll (n=80) on the topic of vaginal health indicated that 48.1% of respondents experienced vaginal yeast infections. Yet, sexual and fertility issues are rarely talked about during routine CF clinic visits. Since women with CF are now identifying sexual and reproductive concerns as important to them, it is important to address such symptoms and conditions. 

Cervical Mucus

Researchers have found that with the use of modulator therapy, there is a decrease in cervical mucus as compared to previous non-use, and that there is an increase in the pH of mucus viscosity as a result of modulator therapy.  Whereas before modulators, women with CF were considered sub-fertile, researchers are now finding that they are fertile.4,7-8 (See Infertility chapter in this guide)

Conditions that Affect Vaginal Health in CF

Numerous conditions can impair one’s vaginal health. 

1. Vaginal infections. Since people with CF are immunosuppressed, fighting and clearing vaginal infections, like vaginal candidiasis (yeast infection) and bacterial vaginosis, can be more difficult. 
2. Cervical dysplasia. Pap smears of females with CF have shown a high prevalence of abnormal cells in the cervix. Lee et al. found that among females ≥ 25 years of age, the rate of abnormal cytology among non-transplanted females with CF is almost four times that in the general population.9  This may be caused by an inadequate cervical mucus structure in the body.9 
3. Menstrual cycles. Regular menstrual cycles have been seen to be associated with better lung function, better BMI, and better vaginal health overall.6  Often, weight fluctuations can cause CF patients to have missed periods (aka amenorrhea). Medications taken can also cause changes to the estrogen levels, which affect the blood volume during menstruation, thus leading to less lubrication and elasticity in the vaginal area.10 
4. Sexually transmitted infections. Deciding who, when, and how often you have sex is a personal decision, but one must be careful to avoid contracting STIs. STIs include, but are not limited to:

Patients should be transparent with their CF care team about their current sexual activity because the team can provide pertinent information to stay healthy. Patients are highly recommended to get the HPV (human papillomavirus) vaccine, Gardasil. HPV has been shown to be harder to clear for people with CF due to their weakened immune system. STIs like HPV can also cause cancers of the skin and genital area if a person eventually gets transplanted (or is already).[11] Pap smears can help detect early abnormalities in the cervix and precancerous or cancerous cells in the vaginal area that are most often caused by HPV.[11]

Modulators’ Effect on Vaginal Health

How Does Mental Health Affect Vaginal Health?

Mental health includes one’s emotional, psychological, and social well-being. It affects how a person treats, thinks, and interacts with themselves.[13] Having self-awareness of one’s body can assist with maintaining one’s vaginal health.[14]

Depression and anxiety are common in CF. They stem from various concerns, like deteriorating health, having to worry about being exposed to bacteria that can cause a person to be hospitalized, keeping track of medications, staying on top of treatments, and going to doctor visits.  

Experiencing conditions such as vaginal candidiasis, thick cervical mucus, dyspareunia, and vaginal dryness can lead to depression, feeling less satisfied with life, poorer self-esteem, and increased stress levels, which can then interfere with sexual and emotional relationships [1,15].

Depression can affect vaginal health because the brain produces less dopamine and oxytocin in this state.15 Selective Serotonin Reuptake Inhibitors (SSRIs), used to treat depression and anxiety, can cause vaginal dryness, issues with sex drive, and can cause discomfort during penetration.[15] These problems can keep women from seeking additional help or deter them from taking their mental health medications so they can have a more stable vaginal health/sexual drive.

Depression can also impair one’s memory, energy, and executive function and can jeopardize treatment adherence.16 As a result, a person can get a pulmonary exacerbation, which may require antibiotics. A side effect of using antibiotics is fungal vaginitis, which can cause discomfort in the vaginal area and requires treatment.[3] 

In their study on intimacy and CF, Simonnet-Bisson et al. found that the primary cause of sexual issues in females with CF is the lack of vaginal lubrication (78.8%).  Pain (74.1%) and discomfort are the secondary reasons.[3]  

They conducted interviews that revealed that women’s sexual lives were often uncomfortable or painful, unsatisfying, or sex was avoided altogether. Many women either felt sexually different from their peers or betrayed by their bodies.[3]

Image from https://www.bemewoman.com/blogs/be-me/the-mind-vagina-connection-how-mental-health-impacts-vaginal-health

Aging and Vaginal Health

In October 2016, Healthline writer Annette McDermott described how the vagina is a complex organ that plays a role in sexual pleasure and childbirth. She writes  that as you age, your vagina will too [13].

There is a variety of information available about vaginal health and aging, including: 

• Estrogen is responsible for keeping the vagina lubricated, elastic, and acidic. 
• The vagina is designed to be self-cleaning but women may have a normal white or clear discharge. 
• Hormonal changes during a woman’s menstrual cycle affect the amount and type of discharge her/their vagina produces. 
• Unless a woman is having symptoms– such as pain during sex, itching, a foul-smelling discharge, or burning— her vagina needs little maintenance other than daily washing with mild soap and water. 
• After a vaginal birth, the vagina may lose some of its elasticity and stretch more than usual but over time, most vaginas will return to almost prebirth size. 
• Kegel exercises can help strengthen pelvic floor muscles and restore vaginal tone. 
• The inner labia may darken due to hormonal changes after age 29. As estrogen levels in the body decrease, the vaginal walls become thinner and drier. 
• Moisturizers or creams may help combat vaginal dryness and lubricants may help with intercourse [16].
• During menopause, the vulva may appear shrunken; vaginal atrophy is a common problem for many women. 
• Atrophy may occur in the urethra and can lead to urine leakage, overactive bladder, and urinary incontinence [17].
• Low estrogen may change the acidity in the vagina and may increase the risk of infection due to bacterial overgrowth. It can also impact the urinary tract [16].

Although CFReSHC patient-partners stated they experienced vaginal dryness, it is unclear if this occurs more frequently amongst the CF female population or why [18].