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Description of the MRKH Syndrome
The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome affects between 1 out 4000 women or perhaps 1 out 5000. This big gap for these figures shows that it is still difficult to give a precise statistic.
MRKH is also known as “vaginal agenesis” or “mullerian agenesis” but often the teenagers who have it ignore the name of the syndrome. Many doctors and gynaecologists never studied this medical case during their studies and never met a patient having this pathology. Consequently young girls anxious about not having periods begin to feel or worry they are being treated like freaks or guinea pigs. Given all of this, they need to be listened, to have supportive relatives and above all they need to be well advised in order to be reassured. They need to be well guided and orientated to the right surgeon or specialist.
This young girl has a normal life until some tests reveal that she has the syndrome. Often she ignores the reason of all theses tests and parents face the feeling of guilt and ignorance. Information is almost inexistent given that it is a rare health problem but, little by little and thanks to the Internet, it is beginning to change and the information becomes available on the Internet.
All theses tests reveal a total absence of the uterus and the vagina but they reveal the presence of functioning ovaries and fallopian tubes. These are often normal and sometimes some young girls are “lucky” to have 1 inch (2 à 3 cm) vagina or a uterine remnants or horns.
The Mayer-Rokitansky-Kuster-Hauser syndrome is due to a congenital anomaly in the development of the embryonic structures affecting the internal reproductive female tract. Some researches have begun the last 5 years to discover the origin of the syndrome which is still uncertain although some suspicions raises …
The psychological trauma caused by the diagnosis is enormous for the patient and her parents and usually very little psychological support is provided. A few young girls stay isolated and refuse to communicate about it, living the syndrome in shame, in secret, and sometimes denying of their feminity. She understands that she is unable to conceive children but is aware that before considering this problem there is one just as important : the affective life. To get love, she feels she needs to resolve the sexual part of the problem : the fact that it is impossible to have physical / sexual intercourse. She will have to choose, with the surgeon, between surgery and manual dilatation.
Given that dilatation is possible only for some cases like having a small vagina (or a pouch), a partner in relationship or not, motivation of the young girl, a decision has to be made.
Treatment and Consequences :
When this young lady is encouraged to do dilatation she must have a follow-up with a specialist to monitor and check the progress. All that involves sometimes expensive travelling (specialists for MRKH syndromes are only a few and far away from home), many appointments, a psychological support from the medical team or from family and relatives. It happens that the young girl feels discouraged and consequently drops the project. Dilatation is often lived as a nuisance and a chore because it has to be done over a few months. Other problems happen when the young girl has to proceed to dilatation in a boarding school sharing a room with others, or when she is at home sharing a bedroom with her brother (s) or sister (s). Knowing that dilatation have to be done lying down on a bed twice a day for 20 or 30 minutes a day it is easy to understand sometimes the failure of this technique.
Although some of the strong advantages for this technique are that this vagina will be permanent, with no scars, in a natural way…
When the surgeon thinks that the operation is the only alternative, the young lady worries about the final result and only wish to be “normal”. She often ignores that sexual intercourse will have to be frequent in order to avoid the closing of the vagina and its shrinkage. If she happens to have in her life a quiet sexual life period it compromises the condition of her vagina (length and width). To keep a functional vagina she will have to do dilatation with a dilatator. It happens that in her thirties or her forties she decides to stop and free her body from this “chore” and this dependence to man or to the object. Therefore her sexual life will be either inexistent or “incomplete” … or non conformed… As a wife and a husband the relationship might change and break up. But vaginal agenesis is not considered as serious because there is no pain and no risk of death.
Just as well for some women the operation is a very good success.
In other situations, the surgery is a failure and a second one has to be done using a different technique.
Double problem : a rare disease and shame / shyness (pudeur)
The fact that it is a “sexual” problem it is difficult to talk about it around like any other digestive problem for example. Consequently isolation, resignation, depression waves can occur.
Personally, I only discovered the name of my “problem” when I was 45 years old, which is 1 year ago, and thanks to the Internet. Doctors used to say that it was “a congenital malformation/abnormality” but I never heard the name of “Rokitansky” or “MRKH”. I was very surprised to see in my medical files dated 1978 (which I asked to receive recently) the word “Rokitanski” written on them.
Not knowing the name of the disease that one can carry leads to a lack of information and the young girl feels that her body has been taken from her. She is dispossessed from her own body which becomes a “very interesting case” of study for doctors.
I am not the only one in this situation and this is what makes me act.
In the name of all these women in my age and the youngest ones who are not aware of the information on the Internet and who suffer in silence, we have decided others and myself, to create a support and information group specifically for our syndrome.
Double impact linked to the syndrome : lack of morbidity and no mortality
Another problem with our syndrome is that from one side we cannot see it, we cannot hear it but it is inside and not visible. On the other side there is no pain or risk of death.
New discoveries have been made where MRKH can be associated with other bone, ear, kidney problems. A new name has emerged which combined MRKH with these other pathologies, it is called MURCS. There is a real pain behind all these terms and especially for the MURCS but there is no real recognition.
Since our suffering does not bring death : we can live without vagina, without uterus, without sexuality, we can live without love, we can live without children, our suffering is denied …
However many of MRKH women can suffer from belly pain without knowing if it is linked or not to the syndrome (ovarian menstrual cycle, active part of the uterus …) or if it is just a digestive problem … Consequently, for every pain we can feel we always wonder if the origin is the syndrome or not.
The Rokitansky syndrome is insidious, it makes its nest in our body without manifesting its presence until we reach the puberty. It annoys a lot our life without putting it in danger. It creates a psychological fragility. It was probably hidden in our parents or grand-parents body without being detected and the uncertainty of the genetic research still refrain us to ask for FIV.
But this is another problem …
Together we, MRKH women, want to create this association and to make it living to break this wall of silence. Parents, in distress and worried, are willing to get involved to our project.
Our main purposes :
• to inform : medical corporation, general public, young girls and women affected by the syndrome as well as their relatives
• to support, orientate, advise these people
• to contribute to medical researches in order to know the origin, the causes of MRKH
• to break isolation of people touched by this syndrome and create or maintain contact with the ones who don’t have the Internet
• to gather every one who is concerned
• to put everyone in a network of parents, relatives, medical corporation and people having the syndrome
• encourage psychological support inside the medical system for people concerned directly or indirectly by the syndrome.
But why did we want to create an association specifically for MRKH ?
• to have an official recognition for the syndrome in France
• to give the chance for MRKH women to have a feeling of identity and to give these women the opportunity to learn more about the syndrome
• to offer to the group a name that speaks for them and of them directly
• to get the freedom and the power of being informed, supported and gathered
• and above all because this association has the right to exist
Our main targets realized :
• a chat room (a forum) to get support and information on the Internet for the young girls, the women and the parents concerned by the syndrome please click here to join us (It is in French)
• this web-site both a data base about the syndrome for medical teams and public
• tracts sent to gynaecologists being aware or ignoring the syndrome and wishing to learn more about it
• to create an exchange network with other countries
• having a recognition of the syndrome at a European scale.
We are reguraly in contact with the PRAM network in Rennes and its team. They all try to determine the exact origin of the syndrome. We have their support and we encourage all MRKH women and young girls to participate to the researches. We are in contact with some specialists for the MRKH to ask them as well to be our technical advisors. We are as well in relation with a few gynaecologists who are particularly aware of our cases and to ask them to be in a support committee.
You can find some interesting information on this link : Please click here. It is a French document about the last researches.
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