I’m old enough to remember textbooks and biology classes that defined being female as a “default option” in human development.
If the ambiguous sex organ precursors in an embryo “failed” to follow the coveted male route, we became female.
If the SRY gene “failed” to turn on, we became she.
I’d always thought this male-centric teaching of human sexual development disturbingly close to Genesis: “The Lord God fashioned into a woman the rib which He had taken from the man. The man said, ‘This is now bone of my bones And flesh of my flesh; She shall be called Woman, because she was taken out of Man.’”
Lost in the discussion was the paradox of the puny Y chromosome and its meager roster of genes compared to the X. So I was happy when discovery of the Wnt gene pathway to femaleness finally came along and validated my existence, beginning the elucidation of the complex gene cascades that sculpt a female reproductive tract.
Yet the predominance of maleness continues.
To illustrate this post, I perused the offerings at Wikimedia Commons.
For vagina? Eight images, all tastefully anatomical.
For penis? 160! And that’s only the human ones. Wikimedia depicts male organs in various stages of alertness reminiscent of Moh’s scale of hardness in geology. The Wiki take on the male organ also offers artist’s renditions, eclectic enhancements, a handful of abnormalities, and a few action videos that I won’t go into.
With this sort of backstory, it’s little wonder that very young women who discover that their vaginas seem very narrow or short are confused, and then devastated when they learn they have a medical condition that affects the reproductive tract. Any variation on normal anatomy can be disturbing, but the situation is even worse when the affected body part is one we could barely even mention until Eve Ensler gave us the hilarious Vagina Monologues a few years ago.
If it was difficult to begin to talk about women having vaginas, it’s even harder to talk about women whose vaginas do not develop fully. But that’s the case for the 1 in 4,500 women with Mayer-Rokitansky-Kuster-Hauser syndrome, aka “congenital absence of uterus and vagina.” Affected individuals much prefer MRKH, because the clinical description is both damaging to self-image and not even accurate. The reproductive tract develops from two parts of the embryo, and only the part that becomes the upper vagina and some of the uterus and cervix are affected.
I’d never heard of MRKH, until a report in the Lancet in April described a tissue-engineered vagina that, being closer to the real thing than anything else so far, can help women with MRKH. And there have been a lot of contenders.
BUILDING A BETTER VAGINA
Tissue engineering is a form of regenerative medicine that crafts a replacement part using a patient’s own cells plus synthetic scaffolds and molds. Over time, the cells grow and coax surrounding tissue to partake, as the synthetic materials are resorbed, leaving a functional facsimile of what is missing or had been injured. Of course the patient’s own cells are not rejected.
The very fact that a vagina can be tissue-engineered means that it isn’t just a space, a hole, a nothingness, an absence of something. It is a tubular organ leading from one place to another, like an esophagus or intestine.
Skin, bladders, even nostrils have been fashioned this way. Like the tissue-engineered trachea, a vagina connects the outside to the inside. The laboratory-grown organ hails from the Wake Forest Baptist Medical Center Institute for Regenerative Medicine and the Tissue Engineering Laboratory, Children’s Hospital Mexico, where the pilot clinical trial reported in The Lancet took place.
It is a leap forward from past attempts to fashion a “neovagina” in women with MRKH.
Often a newly-diagnosed teen would be sent home with dilators that resemble test tubes with instructions on how to use them to widen and lengthen the tiny vaginal canal, called a “dimple.” In the best case scenario, a medical team teaches the technique and follows the patient carefully, but sometimes a young woman is left to grapple with the procedure in an embarrassing situation, such as in a college dormitory. Dilation is about 90% successful, and if not, then surgery becomes an option.
An early procedure was the Vecchietti technique, which threads a small plastic orb called an “olive” through the navel, going in and out and down, stretching out a canal to about two finger widths. The McIndoe procedure uses skin from the thigh or buttocks to create a neovagina. Other variations on the theme create a passageway and then line it with all manner of tissue: skin, amniotic membrane, abdominal lining, intestine, inside of the mouth, even cellulose.
None of these substitutes, being monolayers, accurately mimics the lining of a true vagina, which has layers of epithelium, muscles, and connective tissue. That’s where the new neovagina comes in.
It begins with epithelial and muscle cells from the underdeveloped lower vagina. The procedures were done on four young women, from 2005 to 2008, leaving plenty of time for evaluation.
The cells are expanded and seeded onto hand-made biodegradable scaffolds designed to fit each woman’s anatomy. Six weeks after sampling the cells, the tissue-engineered replacements are stitched into a surgically-fashioned canal in the right place. And over time, the body fills in the connective tissue, nerves, and blood vessels, as the synthetic materials melt away.
The new organs took their places. They had the requisite three tissue layers. They didn’t close up and they work. The patients report normal sexual desire and arousal, lubrication, orgasm, satisfaction, and intercourse without pain.
“This pilot study is the first to demonstrate that vaginal organs can be constructed in the lab and used successfully in humans. This may represent a new option for patients who require vaginal reconstructive surgeries,” said Anthony Atala, MD, director of the program at Wake Forest, in the news release. The researchers didn’t mention sex reassignment surgery, but that’s obviously a possibility.
A NOT-SO-RARE CONDITION
Although MRKH affects the reproductive tract, women have two X chromosomes and normal external genitalia and secondary sexual characteristics, with no known impact on libido or sexual identity. Fallopian tubes are present. For some women MRKH also brings hearing loss, tinnitus, scoliosis, cardiac problems, fused neck vertebrae, and a kidney that’s abnormal or in the wrong place.
Familial cases of MRKH have been reported as far back as 1888, and a few families with more than one case point to autosomal dominant inheritance. But the highly variable phenotype and inconclusive inheritance patterns suggest a multifactorial etiology.
The Lancet paper on the tissue-engineered vagina led me to a wonderful website, www.beautifulyoumrkh.org, started by PhD geneticist Amy Lossie. One woman writes about doing the exploration that most XXs do when we realize there’s another hole down there, and not finding what she was seeking. She imagines running to her mom yelling, “I can’t find my vagina!” Another recalls a boyfriend who called her a freak.
Language on the website is disturbing: “shame,” “hiding,” “terrible, dark secret.” But the language in the Lancet article makes it understandable: “abnormal,” “defects,” “damage” and “malformations” all appear, some more than once, just in the introduction. It is much worse than being called a default option.
It isn’t easy being different, especially in this most private of parts.
Dr. Lossie told her story in the Huffington Post last year. I recently spoke with her for DNA Science. (My questions in italics.)
Some women on the website report going from doctor to doctor, none recognizing the syndrome — a common experience in the rare disease community. What was yours?
Like most people who have MRKH I just didn’t get my period. I was pretty fortunate because we had a new ob/gyn in my town and he knew what it was right away. I had a diagnosis really early. I didn’t go through a lot of time in doctor’s offices.
How did your feelings change after finding this out about yourself?
In the acute phase of trauma, from 1 to 5 to 10 years, I thought, ‘I’m not who I expected.’ You have to retool your life and look at things from a different perspective. Then, after you do dilation, everyone says you’re ‘fixed.” But I wasn’t fixed.
When I was in my late twenties, everyone started having babies and I couldn’t. You have to figure out how you are going to handle that. For me that was the hardest part.
At the beginning, I had no one to talk to. There were no support groups, there was no Internet yet. You think you are the only person who has this. I spent a lot of time thinking about it by myself. I saw a counselor and went through a grieving process. She gave me permission to grieve for my unborn babies, which helped me accept having MRKH.
The stories on the website are from all over – India, Australia, Argentina, Norway, Canada. How did you start it?
Meeting other women changed my life. It happened at a retreat in Canada, and the woman who hosted it was becoming a therapist and she used it for her MSW thesis. There were six of us, all about the same age. We shared our stories.
There was a lot of sadness. Hurt, anger. But for 3 days I knew that no one was going to ask me, ‘When are you having a baby?’ No one would ask me about tampons. It was a safe place. For the first time I felt like a normal person. I didn’t have to worry that someone would ask me a question I had to lie about or figure out an answer to. I realized what meeting other women with MRKH meant to start accepting who you are.
We started out creating artwork. The first pieces were how you felt about yourself when you were younger. It was interesting because I’d been journaling for awhile and it hadn’t helped me. But by the end of that weekend people painted beautiful pictures and you could see a transformation. So I started a foundation. I couldn’t not start it.
Around this time, my co-founder, Christina Ruth, had started MRKH Support and Awareness, a Facebook support group for MRKH. I was stunned with the number of women who shared their experiences and sought support on this website. So, I emailed her and asked her to join me. After months of conversations, we realized that we shared common core values for the Beautiful You MRKH Foundation and were very compatible. We had different and complementary strengths that led to a strong partnership.
Our Facebook support group has 800 members, and they know they’re not alone. You can find somebody like you to talk to.