She never got her period, and no one told her why

G* and her family didn’t notice anything unusual about her until her 16th birthday. Ten days later, she was seated alongside her aunt at a clinic in Hunza, her hometown, on their third visit. She hadn’t gotten her period yet.

For the next several years, the duo travelled across Gilgit-Baltistan, visiting one doctor after another. Every time they visited a clinic, G* underwent a battery of tests, x-rays and ultrasounds. There was no issue except that she could not bear children, the doctors told them. It’s genetic; not much could be done, they said, insisting that the rest was all good.

But what they failed to tell G* would shape the rest of her life. By the time she found out, she was 36 and divorced. G* was diagnosed with a rare condition, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, where a woman has no womb or vagina, and can also find herself with just one kidney.

A woman who has MRKH can never get periods or conceive, unless she gets a uterus transplant — an option unavailable in Pakistan at the moment. It is also an option that G* cannot afford.

A rare condition

According to Dr Novera Chughtai, clinical assistant professor at the Aga Khan University Hospital’s Department of Obstetrics and Gynaecology, the genetic disorder manifests in one in 5,000 girls.

“Normally, when the embryo or foetus is six weeks old, it begins to develop genital differentiation, which is basically the distinction between whether the baby would be a boy or a girl,” said the doctor. “So technically, the lower area of the genital has to fuse with the upper part.”

The problem begins when the fusion messes up or there is a lack of it. In case of the latter, both the upper (uterus) and lower (vagina) parts of the genital tract do not develop. In cases where the fusion is delayed, the areas are both partially developed, and an even further delay may mean that areas surrounding the genital tract may be developed, but without any fusion.

For G*, the fusion did not take place, which deprived her of the ability to conceive or even partake in sexual intercourse. Like other girls, at the tender age of puberty, she was not aware of what was happening inside her.

“From birth up until the age of 11 to 13, girls are unaware whether something is wrong with them unless other systems in their bodies are also not working,” explained Dr Novera. “Usually, patients don’t even know if they have one kidney, which is common in 30 per cent of cases.”

In most cases, she continued, the syndrome and its variations only manifest when medical investigation begins. “For example, if a patient has a uterus but no vagina, they would get periods, but blood would remain inside the system in the absence of a vaginal tract.”

This often results in severe infections and severe pain.

Other patients whose uterus is malformed or rudimentary don’t report periods or pain. “In these cases, parents bring the girls with concerns that while they were otherwise healthy, they hadn’t gotten their period despite reaching the age of puberty.”

In both instances, the genital area is both visually and functionally malformed.

Ignorance is not always bliss

G*, who grew up with an aged grandfather after her mother’s demise, did not have the guidance or support to understand this until her aunt moved in with her. She recalled to Dawn how growing up, she always felt different from other girls in her village — as if a big part of life was missing.

G* didn’t try too hard to fit in either, and so she engrossed herself in housework, and music helped her create a world of her own, where she felt she belonged.

But life has its own way, and G’s* world almost toppled when she got married and was unable to consummate because of her vaginal deformities. “The first time I got married, my husband passed away within just a couple of days,” she recalled to Dawn.

Her second marriage brought another heartbreak. Unable to consummate, her husband flew her down to Karachi for scans and X-rays. “After the results came out, he took me to Hunza and then returned, saying that he would come back to take me home,” she said. A few days later, he faxed divorce papers to her house. It had been exactly one month since their marriage.

Word soon spread across her village as well. “They started calling me khusra behind my back … they said I am neither a man nor a woman … they started blaming me for the divorce,” G* shared.

The constant chatter got to her, pushing her into a deep and dark abyss of depression. At first, she did what most do: distraction. “I took up the job of a nanny at a big house in the nearby village, which would keep me busy throughout the day.”

The depression and isolation G* experienced is the story of most other women who suffer from the syndrome. “The stigma surrounding the condition is prevalent in our society because it is linked to the reproductive organs of a woman,” highlighted Dr Novera.

She recalled receiving cases where patients kept trying intercourse and ruptured their rectum in the process because they didn’t know what was happening to them. They only visit a doctor when the problem escalates into “bache nahi ho rahe”.

“This is a part of our infertility workup,” the doctor stressed, “there is so much ignorance.”

Part of this also comes from early marriages in Pakistan, as pointed out by Dr Lubna Razzak, a gynaecologist at the Dow University Hospital. “Women mostly get married in their early years, around 16-18,” she lamented.

“They are then coming to us with their husbands with the complaint of being unable to consummate the marriage, oblivious to the fact that they never got their period. It all comes down to either not being able to have intercourse or infertility.”

The treatment and its kinds

The distraction of work couldn’t stymie G*’s growing questions. In December last year, she travelled to Karachi to seek answers. By January, her treatment had begun; on March 6, she underwent surgery to expand her vaginal opening, allowing for sexual intercourse — though she still cannot bear children.

According to Dr Novera, the treatment of patients with MRKH syndrome is dependent on their condition. “If there is a uterus but no outflow, we operate then and there,” she told Dawn, adding that in such cases, girls are young and usually do not understand what is happening to them.

“The process includes internal vaginal examinations and post-operation care, all of which can be traumatising.”

On the other hand, when it comes to a patient without a uterus, there is an understanding that they won’t get periods. In such cases, surgery is done to create the patient’s vaginal tract, which, as the doctor explained, was basically an injury to the natural body.

“When you get a bruise or cut, it is natural for the wound to be filled over time, just like cuts and incisions heal,” Dr Novera said. “But with the vaginal tract, for it to remain open, it must be constantly used.” Hence, for such patients, this treatment is advised before marriage.

In G*’s case, the procedure, which included all aspects of vaginal construction, involved collaboration with laparoscopic surgeons, adolescent gynaecologists and plastic surgeons. While it was not life-threatening, the surgery could end up becoming complicated because the bladder and rectum — both near the vaginal area — could be at risk of being hurt in the process.

“Sometimes, you could do your best, but the vagina would still start blocking.” This is because the syndrome is rare and manifests differently for everyone. “The success rate, therefore, depends on the post-op care,” Dr Novera emphasised.

And, she continued, there are two sides to the post-op care: one is the anatomical follow-up and the other is psychological counselling. The latter is not just restricted to the patient, but also extends to her partner, if she has one.

One part of the counselling involves explaining the dos and don’ts of sexual intercourse to the partner and making them understand why it is important to be careful. The other side of it, meanwhile, helps the couple deal with the social pressures that come with the condition.

From her experience, Dr Lubna told Dawn, patients from upper socio-economic backgrounds have a sense of understanding that life can be lived without a child. “But it is extremely difficult to explain the same to patients from the middle or lower classes because they equate a woman’s life to her uterus.”

This, she said, added to the patient’s trauma. “So we have to counsel the patients slowly and gradually … not just them but also the partner and the family, all the while using the right words.”

In a society where womanhood is equated with motherhood, support and empathy are often missing — leaving women like G* to fight their battles largely alone.

And so, in many cases, the revelation of the diagnosis leads to the partner opting for a second marriage. Hence, many patients try to hide their condition from their partners — a trend both Dr Lubna and Dr Novera have seen.

Fighting the taboo

Part of the blame for the reaction the condition receives also lies in the lack of awareness among people — not just about the MRKH syndrome, but also women’s reproductive health.

“So far, at the AKUH, we are looking at 10 cases per year because there is a lot of referral system from neighbouring cities, provinces and even cities such as Iran and Afghanistan,” said Dr Novera. “And from what we have seen, patients are not aware of the syndrome themselves.”

To mitigate this, the AKUH holds conferences and training sessions at hospitals such as the Jinnah Postgraduate Medical Centre, along with using social media to raise awareness.

Dr Novera added that there was a lack of training even among doctors, who fail to realise or identify what kind of case they are dealing with. Thirdly, patients with financial constraints usually end up at smaller clinics and hospitals that lack investigative capabilities, even basic MRI and CT scan machines.

This is exactly what happened with G*, who kept running from one clinic to the other for years. She was only able to afford the treatment at the AKUH because of the funds she secured from a donor. The surgery itself costs anywhere between Rs700,000 to Rs800,000.

At public hospitals such as Dow, the same is more affordable, ranging between Rs250,000 to Rs300,000, said Dr Lubna.

Life for G* has changed since her vaginoplasty, for which she keeps travelling between Karachi and Hunza. She has been prescribed a couple of exercises to ensure that her vaginal tract remains open and functional until she gets married.

Ever since her treatment, G’s* family has begun looking for a groom. The 36-year-old, whose previous experience in this regard still gives her tremors, has her own set of fears, but along with them is also a newfound hope and confidence.

“I know none of this [the syndrome] is something I have control over,” she told Dawn. “And I also know that this doesn’t define me … whoever wants to marry me will do so while accepting this.” In the meantime, G* continues her job, which is no longer a distraction.

“I have realised I love children and being around them brings me a lot of joy,” she said. “It finally feels like there is something to look forward to in life.”