Home > Health Guides by Topic > Gynecology & Reproductive Health > Vaginal Agenesis & MRKH > A Guide for Parents & Guardians MRKH (Mayer Rokitansky Kuster Hauser Syndrome)
and Vaginal Agenesis:
A Guide for Parents and Guardians
Learning that your daughter has MRKH may bring up many questions and emotions. It is normal to be worried about how your daughter is coping. At the same time, it is important to be aware of your own reactions to this news. Discussing issues such as puberty, reproduction, and sexuality is often challenging for parents. When your daughter is diagnosed with vaginal agenesis, you may feel overwhelmed with some of your own feelings as well as your lack of knowledge about the condition and how to discuss it with your daughter, your family, and her primary health care provider or pediatrician. This information is intended to help you to understand MRKH syndrome, your reactions, and ways to help you support your daughter. Commonly asked questions and concerns are included at the end of this guide.
Accepting your daughter's diagnosis
What is MRKH? 
MRKH is a congenital condition of the female reproductive system that affects approximately 1 out of every 5000 females. Girls diagnosed with MRKH have vaginal agenesis, which refers to an absent or incomplete vagina. The uterus is also very small or absent. It is important to understand that young women with this syndrome are genetic females. They have normal ovaries and will experience puberty without having periods. MRKH is a syndrome that may or may not be associated with renal (kidney), skeletal, and hearing problems.
Accepting your daughter's diagnosis
Before you can begin to accept your daughter's diagnosis, it is likely that you will experience a range of emotions. It is common to feel confused, guilty, sad or helpless. Some parents/guardians may feel overwhelmed and numb or have changes in their appetite, sleep and/or energy levels. Others may move into "high gear," seeking all the information they can access over the Internet or from other health care providers. You may feel the need to discuss your daughter's new diagnosis frequently with your family and friends or you may feel the need to withdraw from friends and family for a while and take time to adjust to the news. It is also common for parents/guardians to want to "check-in" with their daughters more often than usual about their thoughts or feelings. Typically parents/guardians compare these feelings to riding a "roller coaster" of emotions. Keep in mind that you and your family will be adjusting to your daughter's diagnosis in different ways. As you learn more about your daughter's diagnosis and treatment options, it is unlikely that you will feel the same intense feelings that you initially experienced. Over time you will begin to accept your daughter's diagnosis and be able to support her emotionally and with any treatment she chooses.
Taking care of yourself first
Taking care of yourself is important for you and your daughter. As you become more comfortable with understanding your own reactions about her diagnosis, you will feel stronger, enabling you to support her.
Allow yourself time to experience your own feelings. Since many teens are able to sense when their parents/guardians are stressed, it's fine to let your daughter know that you are also adjusting to the news of her diagnosis. Assure her that your feelings are not a response to her, but rather that you need time to understand this new information about her body.
Parents/guardians need time to separate what they feel from what their daughter might be feeling.
Helping your daughter
It is natural for you as a parent/guardian to want to help your daughter. Knowing when and how to be helpful may be difficult in the beginning because you may not know exactly how she is coping. Your daughter may be quiet and withdrawn, retreating to her room, making it difficult to know if she wants to talk or be left alone. She may feel isolated or embarrassed because she is not menstruating and can't take part in conversations about "periods" with her friends. It is important to remember that most teenagers in general are struggling for independence from their parents/guardians while at the same time seeking their support. The diagnosis of vaginal agenesis, treatment options, sexuality, and future fertility issues, make this struggle more complex than usual!
The following suggestions may help you to support your daughter as you and she become more comfortable with new knowledge about her body.
Remember, it is ALWAYS important to begin communicating by asking your daughter how best you can help her! Your daughter should be as involved as possible in the planning of her care and ultimately deciding her treatment plan. |
Talking with other siblings
It is important to first talk with your daughter about what she wants her brothers or sisters to know, if anything, about her diagnosis. You can help her decide what should be said and by whom. She may not want them to know anything. However, if your daughter is using dilators, is going to need some extra private time. It may be helpful to say something to her siblings such as, "Your sister is healthy and she has some special medical issues she needs to take care of. She will need to have some private time to herself every day." The information that you and your daughter share with her siblings will depend on the age, language development, and the personality of the child. If siblings are adults, your daughter may choose to talk more openly with them.
Common Questions Parents Ask about MRKH
How is the reproductive system affected?
The reproductive system is formed during the first few months of fetal life. It includes all the female organs: uterus, vagina, fallopian tubes, and ovaries. Vaginal agenesis (incomplete or absence of the vagina) occurs when the reproductive system doesn't finish developing. When this happens, the vagina can be shorter than usual or absent. The uterus is also typically absent or smaller than usual. The ovaries (the organs that make hormones) and fallopian tubes are normal.
Why is it that we are only finding out about this now?
The average age that MRKH is diagnosed is between 15-18 years of age. A young woman typically presents to her pediatrician at this time because she has not had her first period yet. If a pediatrician does an internal exam a diagnosis may be made right away. In other cases, a young woman is referred to a specialist such as an adolescent gynecologist. Typically further testing such as a pelvic ultrasound or MRI (magnetic resonance imaging) is done to confirm whether or not a uterus can be visualized and if there are 1 or 2 kidneys. Since a pelvic exam is not done at birth or during childhood check-ups, it is uncommon for this diagnosis to be made before the teen years. Since girls with vaginal agenesis experience puberty normally, they have secondary sex characteristics such as pubic hair and breast development. It is rare for a health care provider to suspect that anything might be wrong before this time.
Are there other tests that are done to confirm this diagnosis?
In addition to an ultrasound or MRI, sometimes a blood test may be done to confirm normal karyotype (female genes) and ovarian function.
Is there any way I could have caused my daughter's diagnosis?
No. MRKH is not heredity or genetic. It is a congenital anomaly or birth defect that occurs during fetal development in about 1 out of 5,000 female births. There is no known cause for this condition and there is nothing an expectant mother can do to prevent this from happening to her unborn daughter.
What are the treatment options for my daughter?
If your daughter chooses treatment, there are basically two treatment options. She can use vaginal dilators to create a vagina or have surgery. Typically, vaginal dilators are recommended first as a non-surgical option that generally has positive results. In most cases a young woman can create a vagina with the use of vaginal dilators, which are made of hard smooth plastic and shaped like a tampon applicator. With the use of a dilator the skin is stretched over time, creating a vaginal pouch. Your daughter's gynecologist will talk to her about how this is accomplished and tell her how to obtain the dilators. In the beginning she will learn how and where to apply pressure using a small dilator. The dilator is held against the skin and pressure is applied for 20 minutes 1-3 times a day. This is not a painful process; however, pressure should be felt.
Generally, your daughter will be asked to return for monthly follow-up appointments so that her progress can be monitored. As the skin stretches, she will be given the next size dilator until she is comfortable with the length and width of her vagina. The length of time it will take to create a vagina will depend on how consistent your daughter is with using the dilator. Generally it takes about 6-12 months but can take as little as 4-6 months if dilation is done, for example, three times a day.
Some young women who are in a steady relationship may choose to create a vagina with gentle penile penetration instead of using dilators. This method typically works best when there is some vaginal length with which to begin. Intercourse can be quite painful if the length of the vagina is initially short.
The most common surgical procedure performed to create a vagina is called a "McIndoe" procedure. This type of surgery involves taking a skin graft, usually from the buttocks, and making a vagina using a vaginal mold. This is major surgery that requires a young woman to remain on bed rest in the hospital for approximately 1 week. Young women who opt for this treatment will be taught how to use a soft vaginal dilator until the new vagina is completely healed. Until your daughter is having vaginal intercourse on a regular basis, she may need to continue to use a dilator about once a week.
Some surgeons promote the use of a section of bowel to create a vagina. Most gynecologists are not advocates of this technique as the woman will most likely need to use a pad on a daily basis due to mucus secretion from the bowel vagina.
When should my daughter start to create a vagina?
The best time for your daughter to either use vaginal dilators or have surgery is when she is ready! Timing is different for everyone. The usual age that girls decide they are ready is around the age of 16 or 17. Most girls choose to begin treatment before they leave for college. Others may not decide to have intervention until they are older or not at all. Some young women prefer to wait until they have a partner before beginning treatment. Please remember that as a parent/guardian you may wish for her to address the issue as soon as possible to help resolve her vaginal agenesis, but this is your daughter's vagina. A vagina should be created when your daughter is ready and wants to create a vagina.
What if the vaginal dilators don't work or my daughter can't use them?
If your daughter is having difficulty or worried that she is using it incorrectly, she should talk with her gynecologist. If she is not pleased with her progress after she has used dilators for about 6 months, she and her doctor should discuss the options of surgery or waiting until later.
What if my daughter is not using her dilator?
There may be one or several reasons why your daughter is not using her dilator. She may feel like she doesn't have the time, is worried that it is painful, or perhaps she is concerned about not having privacy. It is fine to ask your daughter how things are going, if she is feeling comfortable with her treatment plan but it is not okay to constantly remind her to use her dilator. It is important for your daughter to keep regular check-ups with her gynecologist so that the medical team can assess any difficulties your daughter may be experiencing and to give her feedback and support regarding her progress. If your daughter continues to avoid using her dilator, it may be that she is simply not ready to create her vagina. Having a discussion about delaying treatment until she is ready may relieve your daughter's anxiety and allow her to have control over the situation.
Will my daughter be able to experience sexual pleasure?
Most sexual pleasure and orgasm comes from stimulation to the clitoris and intercourse can enhance that pleasure. Typically, women do not experience pain with intercourse after treatment. If your daughter feels discomfort with sexual intercourse it may be that she needs to use a lubricant such as K-Y jelly. Before attempting vaginal intercourse, your daughter should talk with her doctor.
Will my daughter be able to have children?
If your daughter was born without a uterus or if her uterus is tiny, she will not be able to "carry" a pregnancy. Since her ovaries are normal and make eggs, she may be able to fertilize an egg with the assistance of fertility experts. A surrogate mother could then carry the pregnancy to term. Your daughter and baby's father would be the biological parents of the child. Adoption is another choice for some couples. Keep in mind that fertility options are improving every day. By the time your daughter is ready to have children, she may have more options available to her.
In rare cases, if your daughter was born without a vagina but has a normal size and normally-located uterus, it is likely that she will be able to become pregnant with the assistance of fertility interventions (in-vitro fertilization) and carry a baby.
How can I talk with my daughter when I'm uncomfortable discussing anything related to her sexuality?
Teens can usually sense when their parents are stressed. Be honest. It's fine to tell your daughter that you are feeling a little awkward. At the same time tell her that you want to talk to her and offer her support. Most likely she is uncomfortable too. Although you may both be feeling shy about talking, she will know that you are there to help her cope.
My daughter doesn't want to talk with me about her vaginal agenesis. What should I do?
Your daughter may be uncomfortable about discussing her diagnosis because this often raises issues about her sexuality. The diagnosis of vaginal agenesis may prematurely force a discussion about this topic that some daughters and parents may find uncomfortable or embarrassing. Attempting to have a conversation when she is unwilling to do so will be frustrating for both of you so wait until your daughter is ready. You can suggest that she read about vaginal agenesis at: www.youngwomenshealth.org, join a professionally moderated chat room discussion with other girls who have this diagnosis, or talk with another trusted friend, sibling or adult about her feelings. You can also let her know that you are available to listen if she would like to talk. There are also on-line support groups that can offer support and guidance before, during and after treatment.
How can I be respectful of my daughter's right to make decisions about her body?
If your daughter is under 18 years old, your consent for medical and surgical treatment is necessary, however, it is critical that all options be discussed in clear language with your daughter so that she can participate in important decisions about her body. Ultimately her medical team will respect her wishes regarding treatment.
Should I tell anyone about my daughter's diagnosis?
Telling someone about your daughter's diagnosis is a personal decision. It is always best to ask your daughter for her permission first. Most parents of girls with vaginal agenesis tell us that they are selective with whom they share their daughter's personal information with but at the same time they find comfort and support by talking with at least one close relative or friend.
How do I respond to people's questions about my daughter's diagnosis, the treatment and future implications?
It is up to you to decide if you want to share information and with whom. However, the more you are comfortable understanding your daughter's diagnosis, the easier it will be to respond to specific questions. Being able to share information factually will make these discussions easier. Additionally, listen carefully to what the other person is asking. What is it that they really want to know? Is it medical information or reassurance that your daughter will be physically and emotionally healthy? Answer the questions as well as you can and do not hesitate to say "I don't know." It is fine for parents to set limits when people ask questions they feel are inappropriate.
How can I tell if my daughter needs professional help?
It is common for young women with a new diagnosis of MRKH to have temporary changes in their emotions, and behaviors as they adjust to a new understanding of how their body has developed. These concerns should decrease over time as your daughter understands her diagnosis, is able to verbalize her concerns, and becomes involved in her medical care. Typical signs that your daughter is upset may include changes in her sleep or eating patterns, worried comments about her body, concerns about treatments and medical appointments, and changes in behavior, such as becoming quiet and withdrawn, or aggressive and angry.
If the changes in her emotions and behaviors lasts longer than a few weeks or seem very concerning to you, your daughter may be having a more difficult time adjusting to her diagnosis. If you, your daughter, or her medical team has concerns, don't wait too long. You should talk with her gynecologist about having her meet with a counselor, therapist, or social worker. This can usually be arranged by obtaining a referral from her primary health care provider. It is essential that the counselor, therapist or social worker have experience with adolescents who have medical issues. You can direct them to: www.youngwomenshealth.org/vaginalagenesis.html or share written information with them prior to your daughter's first meeting.
Learning about MRKH and vaginal agenesis will empower you to communicate effectively with your daughter. Understanding the different treatment options, including the choice for no treatment, will help guide discussions with your daughter and allow you to offer sound advice. Keep in mind that it is normal for you and her to feel an array of emotions. Over time you will be able to accept your daughter's diagnosis and offer her much needed support. |
Updated 7/17/2006
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