How to support people with MRKH syndrome?

Mayer's Syndrome - Basic Information About the Disease

Mayer Rokitansky Küster-Hauser syndrome is a congenital lack or underdevelopment of the uterus and vagina - this means that these organs did not develop properly in fetal life. People affected by the disease do not have periods, without appropriate treatment they are unable to have vaginal intercourse, and they are also unable to give birth to a child.

The name of the syndrome combines the names of Georges André Hauser, Hermann Küster, August Mayer and Karl von Rokitansky (hence the abbreviation: “MRKH syndrome”), four physicians who, independently of each other, were the first to present the results of their research on the condition.

In some cases, the disease affects only the reproductive organs, but sometimes there may be co-occurrence of abnormalities in the functioning of other organs (e.g. heart or kidney failure) or systems (e.g. skeletal system defects). Hearing loss is also possible. It should be emphasized that in the vast majority of cases, the ovaries maintain normal function, and the external genitalia (such as the labia or clitoris ) are fully developed.

It is estimated that MRKH syndrome occurs in one in 4,500 women – so it may affect approximately 4,200 Polish women.

If the disease is not accompanied by other ailments (no disorders in the functioning of other organs, e.g. kidneys, have been observed), it may be practically asymptomatic. Therefore, it is possible that, for example, congenital absence of the vagina will be diagnosed in the gynecological chair only during the patient's puberty. This is the moment when the lack of the first bleeding should be a cause for concern and lead to an appointment for a gynecological examination and consultation with a doctor. If the first menstruation does not occur by the age of 15, this is called primary amenorrhea.

Mayer's Syndrome - Causes of the Disease

As for the reasons, research is still ongoing.

It is known that MRKH syndrome has a genetic basis. Many medical specialists suspect that the cause may be a mutation of a single gene, but it has not yet been possible to clearly indicate it.

Mayer's Syndrome - Treatment Process

Now that you know what MRKH syndrome is, you are probably wondering what the treatment is.

There are various methods that can be used to lengthen or create a vagina – these are discussed during a medical consultation to select the one that best suits the needs and expectations of the patient. At this point, it is worth emphasizing that none of the options is the only right one – it all depends on the initial length of the vagina and the preferences of the patient.

Some decide to undergo the procedure. You should be aware that there are many methods of surgically creating a vagina, so it is good to familiarize yourself with the procedure of the center you have chosen, and discuss any questions or concerns calmly with the doctor. After the post-operative wounds have healed, the patient should take care of regular vaginal intercourse or use dilators, otherwise the vagina may unfortunately be shortened.

Many patients successfully use non-surgical methods, including using a set of vaginal dilators (dilators). In such a case, a detailed exercise plan is established with the gynecologist (how many times a day the exercises should be performed, for how long the exercises should be performed, etc.). It is worth being patient - such therapy can take up to several months.

Many patients decide to exercise with a partner - it may be slightly more physically demanding, but a loving person can also be an additional support, and the effects are as good as those obtained with other methods.

A few words about sexuality

The claim that a successful sex life is impossible for Seedless is a myth, and a very harmful one at that. An example of sexual intercourse is not only genital intercourse, nor is it the only way to achieve satisfaction. Sex is much, much more. You can also derive pleasure through oral or anal intercourse or masturbation. You should also not underestimate the importance of mutual acceptance with your partner, closeness and tenderness on an emotional level.

You can also read our article on how to talk about sex with your partner .

I'm seedless - can I still become a mother?

Although a person affected by the disease cannot give birth naturally, the MRKH Team does not rule out motherhood . In Poland, this is currently only possible through adoption, but in many other countries, patients can use the help of a surrogate. Although Seedless women do not menstruate, their ovaries can produce eggs that are capable of fertilization. This is important from the perspective of the proper functioning of the hormonal system and the possibility of being a biological mother in the case of using in vitro fertilization and carrying the pregnancy to term by a surrogate.

In some countries, uterine transplantation is also possible.

Who is Zuzanna Piontke, or where to look for support

This is the theory when it comes to the course and treatment. In practice, each case is different, and obtaining reliable, detailed information or appropriate support is not always easy. Many texts describing MRKH syndrome treat it as a sentence or the greatest tragedy in life for women, often suggesting between the lines that the inability to give birth to a child is synonymous with incomplete femininity. Zuzanna Piontke, founder of the Bezpestkowe project, repeatedly emphasizes how harmful such a presentation of the problem is - in conversations she recalls that the most difficult thing for her was not the diagnosis of the gynecologist_g itself, but finding herself in the message, which instead of dispelling doubts and giving comfort, had the opposite effect. So she started writing texts on the subject herself, and over time, a dedicated email box was created for the Pestless: bezpestkowe@gmail.com, a Facebook group , and a website whose mission is to provide reliable information about MRKH syndrome (the content is co-created by specialized doctors, and there is also a list of recommended specialists you can go to) and to be a kind of support group so that others who have been diagnosed do not feel lonely and disoriented. Many of the texts are very personal. They do not ignore such natural feelings as anger, sadness, or pain, and at the same time, there are no attempts to scare or stigmatize. They are more like support from a loved one - we have been in a similar situation, we know that it can be difficult, but we also know that you can get through it.

I have a diagnosis - what now?

Okay, you've noticed something disturbing, you've made an appointment, the gynecologist has confirmed - Mayer's syndrome. Now what?

I'm sure you have a lot of emotions running through your head. I'm also assuming you have a lot of questions.

First of all: have a lot of understanding and patience for yourself. Allow yourself to consciously experience all the phases of acceptance - denial, anger, negotiation, depression and finally acceptance of the existing state of affairs.

Remember to rely only on verified information. Much of it can be given to you during your first visit by sam_a lekarka_z.

Seek support. These two stages can be combined – content from other patients who are undergoing treatment or have already been treated often serves both purposes. Direct contact can also be more helpful – you can join a group of other Seedless, but if that’s not something you’re comfortable with, you can also connect with a specific person.

In addition to the established treatment and basic medical tests, it is also worth considering using the help of a psychologist. A specialist will help you carry out the necessary work with your experiences, emotions, and fears. However difficult it may seem, try to be gentle with yourself - there is no reason to suppress emotions, there is no reason to reproach yourself. Show yourself understanding. If this is difficult for you, psychological consultations can be really helpful.

A close person with Mayer syndrome

Second situation: MRKH syndrome was diagnosed not in you, but in someone close to you. What can you do in such a situation?

First, learn about the topic. You don't have to become an expert overnight, but familiarize yourself with the basics that will give you an idea of ​​what the patient is struggling with.

Secondly, be there for her. Don't scare her, lecture her, or give her "good advice". Avoid general words of comfort such as "don't worry" or "it's not the end of the world", which can have the opposite effect to the intended one. Instead, simply show your support and concern - assure her that the diagnosis has not changed your perception of her, offer to always listen to her, ask if there is anything you could do for her. Maybe you could take on difficult phone calls, arrange a visit, help with household chores if she doesn't feel up to doing them herself? Remember, don't force anything: if you hear a refusal, don't push it. The same goes for asking questions - don't be afraid to do it, but don't demand answers if for some reason she doesn't want to give them to you. Also remember to be discreet and don't inform others about her health without her knowledge. Apart from that, try to treat her_go the same way you have been doing so far – find time for your favourite ways of spending time together, go for a walk together, make something delicious to eat and devour it while watching your favourite series on Netflix. The mere presence of a trusted person can have a calming effect, and peace and a sense of security are especially important in a difficult situation.

Summary

The absence of a uterus is not usually something that is easy to come to terms with. The days, weeks, and months following the diagnosis will likely be incredibly difficult for Seedless.

That is why it is so important to provide adequate support and, in a broader perspective, to educate ourselves and others so that differential sexual development, exemplified by MRKH syndrome, is not perceived as a taboo subject and a life disaster.

So that no one has any doubts that this is not something that determines the value of a person.

So that no Seedless person would feel like they are completely alone with their diagnosis.