Woman born without womb speaks about being diagnosed with Mayer-Rokitansky-Küster-Hauser syndrome

Malaysian writer and performer Wani Ardy was diagnosed with a rare condition named Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) at the age of 17 when she realised that she was not menstruating. The diagnosis baffled her as she could not relate to her peers when doctors told her that she did not have a uterus. In the initial years, she kept her condition a secret but after joining a US-based online support group for MRKH women, Wani felt like sharing her story which could increase awareness about MRKH and help others who are suffering from it. “I thought if I could feel this way with a person who was basically across the globe, just imagine how I would feel if I could find an MRKH person in my own country, who would be more relatable in terms of upbringing, background and culture,” she said. Wani founded a Malaysian support group that has grown to over 200 members, including from neighbouring Indonesia and Singapore soon after she went public with her condition. “Because of her … many more girls have the courage to come up and talk and get diagnosed,” said gynaecologist Dr Harizah Hatim. Wani has also acted in the Television series “Rahimah Tanpa Rahim” (“Rahimah Without A Womb”), whose lead character has MRKH. Wani, who is Muslim, said cultural barriers and taboos surrounding sexual health in Malaysia often leave women with MRKH feeling ashamed or unwilling to seek support or treatment.

What is Mayer-Rokitansky-Küster-Hauser syndrome and what are its causes?

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder that occurs in females and mainly affects the reproductive system. It affects approximately 1 in 4,500 newborn girls. This condition causes the vagina and uterus to be underdeveloped or absent, although external genitalia are normal. Affected women usually do not have menstrual periods due to the absent uterus. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 16 (primary amenorrhea). Women with MRKH syndrome have a female chromosome pattern (46,XX) and normally functioning ovaries. They also have normal breast and pubic hair development. Although women with this condition are usually unable to carry a pregnancy, they may be able to have children through assisted reproduction.

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When only reproductive organs are affected, the condition is classified as MRKH syndrome type 1. Some women with MRKH syndrome also have abnormalities in other parts of the body; in these cases, the condition is classified as MRKH syndrome type 2. In this form of the condition, the kidneys may be abnormally formed or positioned, or one kidney may fail to develop (unilateral renal agenesis). Affected individuals commonly develop skeletal abnormalities, particularly of the spinal bones (vertebrae). Females with MRKH syndrome type 2 may also have hearing loss or heart defects.

The cause of MRKH syndrome is unknown. Changes in several genes that are involved in development before birth have been identified in females with MRKH syndrome. However, each has been found in only a few affected individuals, and it is unclear whether these changes cause MRKH syndrome. Researchers are working to determine how genetic changes might lead to problems with reproductive system development in females.

The reproductive abnormalities of MRKH syndrome are due to the incomplete development of the Müllerian duct. This structure in the embryo develops into the uterus, fallopian tubes, cervix, and the upper part of the vagina. The cause of the abnormal development of the Müllerian duct in affected individuals is unknown. Originally, researchers suspected that MRKH syndrome was caused by environmental factors during pregnancy, such as medication or maternal illness. However, subsequent studies have not identified an association with any specific maternal drug use, illness, or other factor. Researchers now suggest that in combination, genetic and environmental factors contribute to the development of MRKH syndrome, although the specific factors are often unknown.

It is also unclear why some affected individuals have abnormalities in parts of the body other than the reproductive system. Certain tissues and organs, such as the kidneys, develop from the same embryonic tissue as the Müllerian duct, and researchers suspect that problems during development could affect these organs as well.

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