M AYER- R OKITANSKY- K USTER- H AUSER SYNDROME

….Was named in 1838 (and again in 1910) for the men who discovered physical commonalties in an unusual situation. This is a condition that involves congenital absence of the vagina, fallopian tubes, cervix and uterus. Some women have uterine remnants, or horns. External genitalia are normal. Chromosome karyotype is 46XX (normal female). The incidence rate is approximately one in 5000. Other symptoms involved can include kidney abnormalities, skeletal problems and hearing loss. The cause is somewhat unclear, but the Syndrome occurs sometime during the 4th-6th week of fetal development….

INTRODUCTION:

A questionnaire was offered to an MRKH support group of 250-300 women. Twenty-one women replied. I found it nearly impossible to summarize the data collected. It became even clearer to me that this is such an individual experience for the women involved. I was hoping for similarities. Common answers to questions in my life that this experience has raised for me. I did find similarities, but this report will differ greatly to the reports I have read by 'medical professionals'. MRKH is more than a medical condition. Society and culture play a major role in how MRKH affects us. Even though there are variations in the answers I received, there is the common loneliness, pride and strength voiced by the women. Some answers are listed below the questions.

This report will not be your typical survey report that wraps it all up. This is not a typical situation to report on. I felt the most honest way to express the replies was to share the direct answers. This is purely a collection of thoughts and experiences. This report should not be used for diagnostic information or medical advice. I didn't bother with a 'control group' comparing our needs, reactions and situations to women who were born with vaginas. It has bothered me to read about my body and my life "as compared to normal women".

The only generalization I can make from the women who replied is that they represent a more positive, proactive group of women. First of all they know they have MRKH. They have the support of each other. These women also have access to information through the Internet. For many women; worldwide and class-wise, information is not available.

THE BASIC STUFF:

How women get diagnosed with MRKH:

There is a difference between discovering the symptoms of MRKH and actually being diagnosed with MRKH. The replies differ upon interpretation.

13 Women discovered they didn't have a vagina or uterus when they didn't start their periods.

1 woman learned from an infant hernia operation.

1 woman had stomach pains that led to hymenectomy. The hymenectomy led to the discovery.

The remaining women listed ultra sound, laparoscopy, and pelvic exams for how they were

diagnosed.

Only 4 of the 21 women had Doctors who knew about MRKH from the start. Two of those 4 had only heard about MRKH and not treated other women. Other women learned about it on their own many years later. A 28 year old woman was diagnosed by an ultra sound technician who also has MRKH.

Did our doctors know anything about MRKH at the time of discovery…

♀ Don't know. I found out 20 years after surgery.

♀ Dr was shocked during pelvic exam and brought in another doc to see "what was up". Other doc ran to his office to look it up. Found a paper with RKH scrawled on it.

♀ Not the 1st one-referred for 2nd opinion.

♀ No name for it-but another patient has it.

♀ Maybe-not told until 2nd visit.

♀ No-called in several Drs to discuss my case.

♀ Yes-but only 1 page study in medical school.

♀ Yes-very knowledgeable.

♀ Treated 1 other patient but definitely wasn't well read on subject.

♀ All they told me was "It" had to be "corrected’’.

The countries represented by the participants…

1 woman lives in Canada ; 16 women live in USA ; 1 woman lives in Israel ;

2 woman live in Australia; 1 woman didn’t answer

VAGINAL TREATMENT:

Types of vaginal surgery/treatment

14 women have had treatment or surgery including…

♀ McIndoe Surgery Procedure (6 women)

♀ Dilation. (2 women)

♀ Boyfriend who was patient/kind created vaginal canal.

♀ Chose my own method of selecting gentle patient lovers who enjoy oral sex when vaginal sex is painful.

♀ Laparoscopy to determine lack of uterus and kidney. Didn't know about the slit for self dilation

♀ 4 surgeries.

♀ Many different surgeries.

♀ Just the laparoscopy.

♀ No treatment / has vagina but no uterus.

♀ Tried intercourse method-now dilating.

♀ 2 women are currently dilating.

♀ Labia flap vaginoplasty

Ages that women had surgery/treatment

9 women had treatment at 20 years old or younger.

2 women had treatment/surgery between 21-30.

2 women had treatment/surgery between 31-40

1 woman had treatment/surgery at 40+

♀ My 1st surgery at 22 years old. The 2nd surgery

♀ at 36 .

♀ Imperforate anus at 1yo- partial hysterectomy at 14yo – vaginoplasty at 22 - colostomy at 25. All mrkh related.

♀ Age 18-exploratory laparotomy to "take inventory". Vaginoplasty was 'threatened'.

♀ Required for me to be sexually active- didn't like dilators.

♀ Wants to share a sexual relationship with the man I love.

♀ Knew it was the only way to ever be sexually active.

♀ Sexuality was never discussed.

♀ Need surgery to have heterosexual intercourse.

♀ Not sure if I was given a choice on the slit-before self dilation.

♀ Husband gave me a choice. I wanted it for us.

♀ I wanted to be as normal as possible.

♀ I wanted it to feel normal, not just for sex.

♀ Required for me to be sexually active.

♀ Correction was a given. Nobody asked me what I wanted.

♀ Told to arrange for surgery when I plan to get married.

Are there regrets about having vaginal surgery/treatment …

6 women answered simply no. Other comments include…

♀ Not so far except for the pain.

♀ NO WAY !

♀ No regrets, but wish I didn't have to go through this experience.

♀ Glad not to have surgery, no regrets w/ boyfriend treatment.

♀ Just the 1st one.

♀ Glad I didn't have Williams procedure.

♀ Regret that it was expected.

Do doctors tell us what to expect after surgery/treatment and are they accurate?

2 women answered no.

♀ Yes but not accurately.

♀ Yes, accurately.

♀ He did-hope he was accurate. He gave me other patient’s phone numbers.

♀ Not the 1st one-that’s why it failed.

♀ NO! Didn't tell me how much pain I would be in after surgery. Or that I would have pain during

Intercourse for the rest of my life. Told me I would be 'normal' after surgery.

♀ Fairly accurate in physical information. No one could prepare me for the emotional aspects.

♀ Yes, accurately.

♀ Yes, and yes.

♀ Yes. But didn't prepare me for inserting dilator. Caused setback / shrinkage.

Do doctors explain the various methods of vaginal surgery/treatment?

3 women answered no

4 women answered yes

♀ Dr referred to metal dilators as "like shoe stretches". Then suggested I become a nun. He told

me "If someone marries you - you will need these dilators".

♀ Don't know much but they're trying.

♀ Thought McIndoe and bike seat were the only options.

♀ Encouraged to come back to discuss it.

♀ Neither of them did.

♀ Not completely.

♀ Absolutely not-his focus was on creating vagina to have sex w/men. And finding Y chrom.

♀ Not the 1st time.

♀ Not discussed.

♀ Yes, but he felt non-surgical treatment was best.

♀ Explained Williams and McIndoe. Joked about bicycle seat dilation.

♀ McIndoe and dilation.

Who decides what to do for surgery/treatment….

4 women had a prescribed treatment.

3 women chose on their own-1 with help of counseling.

2 women followed their doctor’s recommendation.

♀ Told the DR to take a hike. Chose my own method of selecting gentle patient lovers who enjoy oral sex when vaginal sex is painful.

♀ He let me decide with limited information.

♀ Will insist on dilation, if possible.

♀ He advised-I agreed

♀ Not given the option of surgery- I was given dilators but never use them. I chose my boyfriend.

♀ He did what he usually did.

♀ He decided the 1st time. The2nd time I took control.

Doctors opinions about whether surgery/treatment is successful…

8 women were told there surgeries were successful by their doctors.

♀ Yes. I was unwilling at 1st. Dr supported me. Later he thanked me for being his greatest victory. I was his 1st successful dilation.

♀ He never followed up.

♀ He said, "That should be sufficient"

♀ He thinks so.

♀ Progress was "satisfactory".

♀ Never went back. Internist told me I was a little small-but not noticeably.

Our opinions about whether surgery/treatment is successful…

9 women answer yes

♀ My own method of selecting gentle patient lovers is successful.

♀ Yes. I recommend McIndoe. The Drs were amazing.

♀ When I was dilating regularly. I took a break and have shrinkage.

♀ The 2nd one.

♀ Dilation is certainly helping.

Did surgery/treatment leave women with other physical problems that they didn't have before?

9 women answered no.

♀ Gyn said labia were normal size-but I had plastic surgery to make them the size I wanted.

♀ High dose of estrogen before and after surgery caused a reaction to an undiagnosed syndrome that caused

tumor development.

♀ Just those darn scars. 3 other women mentioned the scars from skin graft donor sites.

Complications involved with surgery….

5 women of the nine women who had surgery reported no complications with their surgery. The question didn’t get answers from women who had dilation treatments.

♀ I was constipated in the hospital-when I finally did go I pushed the mold out 2-3 inches which was horrifyingly painful. Not sure if that was a complication or just what went w/surgery.

♀ Closed up completely and bled for months.

♀ Addicted to morphine in hospital.

♀ No. But they didn't prepare me for inserting dilator.

Do we ever consider not having vaginal reconstruction …

Five women simply answered no

♀ Sure of wanting it since I found out.

♀ I definitely want a vagina.

♀ No. Wanted to correct it ASAP.

♀ Still considering not having reconstruction. Depends on whether future partner(s)want me to.

♀ Yes. Husband gave me a choice. I chose it for us.

♀ Not really-just which one.

♀ Seemed a given.

♀ Yes.

Are other symptoms of MRKH adequately cared for…

♀ No other symptoms (4 women).

♀ Yes. I have a single pelvic kidney.

♀ Yes-painful infantile uterus removed-facial surgeries, hearing loss.

♀ Yes (5 women).

♀ Only since changing Drs.

♀ After 14 years of hiding in the dark.

♀ Not really.

♀ No answer (3 women).

♀ No attention needed for missing kidney.

♀ Never knew about symptoms until now. Knowledge of MRKH could have prevented other problems.

♀ Single kidney gives me no problem. Very healthy.

SEX, SEX AND SEX

13 women are sexually active.

7 women are not sexually active.

♀ Not w/other person

Sexual preference/orientation…

14 women are heterosexual

4 women are bisexual

1 lesbian

1 no answer

♀ heterosexual –asexual (I had no sexual identity once treatment was over.)