The female reproductive system is a complex, beautiful thing. Most female sex organs are located inside the body and have many parts working together for you to menstruate and be able to carry and give birth to a baby if you choose. With so many systems working together, things sometimes don’t develop properly, as is the case with MRKH.

How does MRKH work?

Mayer-Rokitansky-Kuster-Hauser syndrome, or MRKH, is a rare disorder affecting about 1 in every 4,500 people assigned female at birth. MRKH causes the vagina and uterus to be underdeveloped, while external sex characteristics are present. MRKH varies from person to person, but commonly the vaginal canal won’t fully develop, resulting in a particularly shallow vagina, and the uterus and cervix will not fully develop or be present at all. Healthy ovaries are often still present in people with MRKH, however, as well as fully developed secondary sex characteristics such as pubic hair and breast tissue. 

How does MRKH affect someone?

There are two types of MRKH. Type 1 includes an underdeveloped uterus and vagina, keeping other organs around these areas healthy and functional. Type 2 can affect the kidneys, causing complications or kidney failure altogether.

Since someone with MRKH doesn’t have a uterus or cervix, they won’t ever have a menstrual cycle. MRKH is often diagnosed during puberty because of a lack of a menstrual cycle during someone’s teenage years or when other markers of puberty are present. Pain or difficulty during vaginal sex is also a way to help diagnose MRKH. Since the vagina is underdeveloped, it is often very painful for something to be inserted. MRKH will be officially diagnosed by a doctor after doing several tests, of course, but these are common indicators. 

What is the cause of MRKH?

The cause of MRKH isn’t completely known, although it is clear it’s a result of an underdeveloped Mullerian duct. The Mullerian duct helps develop the uterus, fallopian tubes, cervix, and upper portion of the vagina. The cause of problems with this duct is unknown, and MRKH does not appear to be inherited. The rarity of this condition also makes it hard to pin down a cause. 

Can someone with MRKH have a child?

Since a uterus does not develop if you have MRKH, carrying a child without medical intervention is not an option. If someone with MRKH does want to have a baby, though, there are alternatives. Since the ovaries can still be healthy, using a surrogate with your fertilized egg is an option. Some successful uterus transplants have also occurred, although this is a newer and less common procedure. This procedure takes the uterus of a deceased donor and transplants it into the patient needing a healthy uterus. As of 2019, only two babies in the nation have been born from successful uterus transplants. Although this is a newer option, it is very promising for treatment. 

How does someone with MRKH have sex?

If someone with MRKH doesn’t want to have a baby but wants to treat pain with penetration, they can use dilators to slowly stretch their existing vaginal tissue over time. Dilators are wand-like tools that you slowly put into the vagina, increasing the size of the dilator over time to slowly stretch the vaginal tissue. Dilators are also a great tool for treating pain with penetration for people that don’t have MRKH. Some people will also undergo vaginoplasty surgery, where the vaginal tissue is reconstructed by a doctor. Medical treatment isn’t necessary though if you are able to find other ways to enjoy pleasure and sexuality without vaginal penetration. MRKH does not need to be surgically treated, it is of course, up to the patient.

Although people with MRKH don’t menstruate, sometimes they might still feel menstruation symptoms. They can seek treatment for this as well. It is totally possible to live a healthy, fulfilled life and even reproduce safely with MRKH. 

I remember hearing about “That First Trip” to the gynecologist when I was younger. I wasn’t exactly sure what happened at the gynecologist, I just knew you had to take your pants off in front of some doctor, and this doctor had to stick things inside you to make sure you were healthy. I thought going to the gynecologist was guaranteed to be painful and uncomfortable, and my first trip to the gynecologist was something I was afraid of. When the time for my first visit came I was really nervous, but I was pleased to find all the mystique and scary rumors surrounding my visit were nothing like my actual positive experience.

What is a gynecologist?

A gynecologist is “a physician who specializes in treating diseases of the female reproductive organs and providing well-woman health care that focuses primarily on the reproductive organs.” Gynecologists provide patients with birth control, information about sexual health, menstruation, fertility, as well as treat conditions or vaginal health concerns, and provide Pap smears.

If someone hasn’t already made a visit to the gynecologist well into puberty, it is recommended that you start going for Pap smears either once you become sexually active or once you turn 21. A Pap smear is a test that gynecologists do to test for cervical cancer and overall cervical health. 

What to expect at the gynecologist

After checking in, you’ll be led into an examination room and the nurse will ask you if you have any questions for the doctor. This is a good time to voice any questions or concerns about your sexual health. Asking about contraception, STD tests, pain during sex, or any other sexual health concerns are all things you should feel comfortable asking your gynecologist. For example, I’ve asked my gynecologist how and why some antibiotics make birth control pills less effective.

People often feel uncomfortable talking about sex, but giving you information on your sexual health is what the gynecologist is there for. Even if it seems scary, it is totally normal and important to voice any questions or concerns you have while you are at the office and the doctor can take a look at anything they need to. You won’t be judged – it is literally the gynecologist’s job to help you! 

Asking lots of questions

You can also ask your gynecologist general questions about sexual health and birth control, even if they don’t apply to you. You can inquire about birth control methods you don’t use but have read about, or you could ask about managing emotional health along with sexual health in a relationship. I know that at Planned Parenthood, for example, they always ask questions about your emotional well-being within your sexual relationships, and I just love that.  

Next, you’ll be asked to take your clothes off and wear a robe of some sort and wait until the doctor comes in. You’ll be left alone for a while, then the doctor will arrive. The doctor will likely ask if you have any questions or concerns you want to address, then they’ll start the exam. You’ll scoot your booty to the bottom of the examination table and place your feet in stirrups so your legs are spread nice and wide. The doctor will then place a speculum or a metal instrument into your vagina to hold the vaginal walls open so they can see your cervix. The gynecologist should have various sizes of speculums and will likely use the smallest one when it is your first visit. The doctor will then look at your cervix and take a small little brush to take a sample of your cells. The doctor may also put a finger or two into your vagina and feel around to make sure you have no unnecessary pain or pressure.

It’s not as scary as it seems

I know this all sounds really intense and vulnerable, but it truly is not as scary as it sounds. The most important thing is to try and keep your body relaxed. If you tense up your muscles while the doctor is doing their business, it might be more painful than if you stay relaxed. The actual Pap smear takes less than a minute, so you will only be uncomfortable for a short amount of time.

You can also ask your doctor to explain what they are doing for the exam before they do it. During my first visit, my gynecologist showed me the speculum before inserting it, told me he was using the smallest one and described how he was going to insert it before he did. This helped me feel prepared, relaxed, and overall more comfortable. This visit is about you and your health, so you should feel empowered to ask for what you need to be most comfortable.

How often to go to the gynecologist

While you’re at the visit, the gynecologist will likely also feel your breast tissue to make sure there are no abnormal lumps or anything like that. After that, you’ll have a final chance to ask any questions, then you’ll get dressed, schedule your next appointment, and be on your way.

Talk with your doctor about how often they think you need to get a Pap test, but for most people between the ages of 21 and 29, a test every three years is appropriate. For patients aged 30 to 64 a Pap test with an HPV test every 5 years is appropriate, and patients over 65 might not need Pap tests anymore. It is best to ask your doctor what they think is best for you and your body.

Although visiting the gynecologist isn’t always the most comfortable experience, I always feel good leaving my appointment knowing I’m in control of my sexual health. Knowing that my body is healthy and working correctly makes me feel good. 

If you’re anticipating your first visit to the gynecologist, take a deep breath, relax, and know that people get Pap smears done all the time. It’s important for your health, and after the first visit, you definitely won’t be as nervous.