MRKH Syndrome: A Rare Congenital Disorder

Mayer-Rokitansky-Küster-Hauser syndrome or MRKH syndrome is a congenital disorder of the female reproductive system. It causes the vagina and uterus to be underdeveloped or absent, although external genitalia is normal. MRKH syndrome is a rare disorder that affects at least 1 out of 4500 women. While its cause isn’t known, it could largely depend upon genetics.

Symptoms and Diagnosis

People with MRKH syndrome have normally functioning ovaries and normal sex hormones but do not have menstrual periods due to the absence of the uterus. Due to the absence of the uterus, women suffering from the disorder cannot get pregnant.

They, however, have female chromosome pattern and female secondary sexual characteristics. This normal external appearance of MRKH syndrome makes it difficult to diagnose before puberty. The first noticeable symptom of the condition is the lack of menstruation by the age of 16.

In some cases, the kidneys can be abnormally formed or positioned, or one kidney might be absent altogether. It can also be accompanied by skeletal abnormalities, heart and lung defects, and hearing disorders.

MRKH syndrome is a rare disorder that affects at least 1 out of 4500 women.

Treatments Available

Since women with MRKH cannot get pregnant, they have to opt for other options such as in vitro fertilisation (IVF) and surrogacy in case they wish to have children. With scientific research and advancements, uterine transplants are being performed now. Hence, people with MRKH can choose to have children if they so desire.

Additionally, in most cases, the vagina is not large enough to have sexual intercourse. Vaginal dilation can be performed to expand the length of the vagina to make penetrative sex more comfortable.

Also read: “The Sex Which Is Not One”: Vaginismus And The Construction Of Female Sexuality

Lack of Awareness

Known by multiple names in the medical world, the condition is hardly known by any socially. The rarity of the condition, as well as the social stigma associated with the absence of the uterus and the vagina, have contributed to the lack of awareness about MRKH syndrome. Due to the notion that a woman without a uterus is not ‘woman enough’, this condition is not something people are willing to discuss openly on a public platform. Women’s role in society is still viewed by many as limited to childbearing, and this condition then becomes a hindrance in fulfilling that gendered role.

How this congenital condition is viewed in India is also similar to the attitude prevalent about transgender and intersex people. The regressive Transgender (Protection) Rights Bill which was introduced in 2016 and later withdrawn also placed the utmost importance on primary sexual organs while determining the sex of a person. The bill, which failed to understand the difference between the terms “transgender” and “intersex,” stated that a person would only if considered transgender upon certification by a district committee set up for that purpose. A similar idea is prevalent where women with MRKH are concerned.

A 17-year-old, who would like to remain anonymous, told me that she recently discovered that her uterus is underdeveloped and one kidney is missing. After not getting her period even at the age of 17, she decided to visit a gynaecologist. With regards to menstruation, she said,

“My mother was worried when I did not get my period at this age and insisted that I go to the gynaecologist. I have never related to the women in my family whenever they have had discussions about their experiences about menstruation. I do not feel like I am missing out on anything and I tried to avoid that topic for as long as possible. There is this notion that, as a girl, you must necessarily have your period and that it is a part of womanhood. This, in my opinion, is also a bit transphobic.”

“Until recently, I had not even heard of this condition. This just goes on to show how little awareness there is regarding this,” she added.

Living with MRKH

Purbi, a 35-year-old woman from Mumbai, who was diagnosed with MRKH at the age of 15 talked about her experience of living with the condition. With regards to her condition not being accepted socially, she said, “Not being able to have children is considered a curse. Women with MRKH are devalued on that front. While, in Mumbai, not a lot of people question me about my marital status, in smaller towns and cities, the importance placed on marriage is greater and women are judged for not doing that.”

She added, “As far as dating and marriage are concerned, people with MRKH have different experiences. Some go on to have multiple relationships while others seek social seclusion. For a long time, I shut myself off and did not pursue any relationship. If I ever meet people who are family-oriented, they never wish to marry me because of my condition.”

Psychological Distress

A study published in the Indian Journal of Urology states that people with MRKH suffer from severe body image issues, anxiety, depression, interpersonal sensitivity, and face a lot of psychological distress at diagnosis. For adolescents, guidance and reassurance are necessary.

“Young women diagnosed with MRKH syndrome suffer from extreme anxiety and very high psychological distress when they are told they have no uterus and vagina. Thus, it is recommended that the patient and family attend counseling before and throughout treatment,” according to the National Center for Biotechnology Information (NCBI.)

For coping with a rare condition like this one, therapy, as well as support groups where one can find people with similar experiences, are extremely helpful. Purbi, who runs a Facebook support group called MRKH India said that it took her over five years to come to terms with her condition, during which she suffered from depression. A Facebook page for MRKH helped her cope with her condition.

“I found a Facebook group with many people like me. It made a big difference when I started sharing my feelings and pain with others. That is when I decided to increase awareness by MRKH and created a support group especially for Indian people,” she added.

A study published in the Indian Journal of Urology states that people with MRKH suffer from severe body image issues, anxiety, depression, interpersonal sensitivity, and face a lot of psychological distress at diagnosis.

The fixation on menstruation and childbearing is primarily responsible for the disorder being considered as abnormal and a certain feeling of shame is associated with the lack of primary sexual organs and phenomena.

Purbi, while discussing her family’s reaction to her diagnosis, said, “Nobody in my family was willing to discuss my condition with me after I was diagnosed. I was particularly disappointed with my mother, who did not support me emotionally at all. Not being able to discuss my condition has been extremely painful for me. I was also asked not to tell anybody about my condition.”

Also read: How My Gynaecologists Dismissed My Pain | #MyGynaecStory

While awareness about MRKH is gradually increasing in some countries, India still lags behind in that respect. There isn’t enough medical information pertaining specifically to India. Neither is there enough media coverage on the topic. We seem to be a long way from eradicating the importance that is placed on being either male or female, where any deviation from the standard biological characteristics is not socially accepted.

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