Treatments of von Willebrand disease for women with gynecological complications

/Treatments of von Willebrand disease for women with gynecological complications
Treatments of von Willebrand disease for women with gynecological complications2018-04-04T02:07:45+00:00

HORMONE THERAPY

Oral contraceptives (the Pill) raise the level of von Willebrand factor in the blood for women with Type 1 VWD. For many women with VWD who suffer from menorrhagia, this hormone therapy alone is effective in reducing bleeding to normal.

This hormone therapy will not improve factor levels for women with:

  • Type 2 VWD, because this is a problem in the way the VWF works, rather than the quantity
  • Type 3 VWD, because there is very little VWF in the body to start with.

However, oral contraceptives can be helpful even for these women. They regulate the menstrual periods and reduce the flow of blood.

Other hormone therapies may be prescribed when oral contraceptives do not work well. These include a GnRH analogue to shut down the hormones of the ovary at the level of the brain. High dose progesterone derivatives can also be used in certain situations but their effectiveness in controlling uterine blood loss is somewhat unpredictable.

The use of the Pill for adolescent girls raises some difficult issues.

  • Some parents may not want their daughter to take the Pill, fearing it will lead to early sexual activity. However, many studies have shown that therapeutic use of the Pill is not linked to early onset of sexual activity. This is a very effective means of controlling bleeding from VWD and should not be dismissed for such unfounded reasons.
  • The adolescent girl may hesitate to go against her parents’ wishes, even when the Pill may be the only effective way to control her menorrhagia.
  • Parents and adolescents also fear use of the Pill may lead to cancer, infertility, blood clots or stroke. These concerns can be lessened with accurate information.

The personnel at the bleeding disorder treatment centre can help families work through these issues.

 

ANTIFIBRINOLYTIC AGENTS

(Cyklokapron and Amicar)

For women with VWD and menorrhagia, Cyklokapron and Amicar can be started on the first day of menstrual bleeding and taken for the entire period. They can even be combined with the use of oral contraceptives for women who do not respond to desmopressin.

For some women, medical treatments alone will not work. Heavy, prolonged bleeding during the menstrual cycle will continue. For these women, gynaecological procedures, including surgery, may be considered. It is important that women have all the information before making their decisions.

These are some of the options.

 

MIRENA IUD (levonorgestrel-releasing intrauterine system)

The Mirena IUD is a small, flexible device that is inserted into the uterus through the vagina. It releases low amounts of hormone locally and in many cases this reduces the amount of blood lost during a menstrual period. It must be inserted by a gynaecologist or other specially trained health care provider and can last up to 5 years. It is important that the placement is checked a few months after insertion to be sure it is in the right place.

Advantages

  • Insertion can be done in a clinic and does not require an operation.
  • The Mirena is an effective method of birth control, however, women can still get pregnant after it is removed.
  • Patients don’t have to remember to take pills on a regular basis.

Disadvantages

  • Some patients experience discomfort and cramping during insertion.
  • Menstrual periods can be irregular for the first 3-6 months after insertion

 

ENDOMETRIAL ABLATION (uterine ablation)

The purpose of this operation is to destroy the lining of the uterus. This is the endometrial tissue that bleeds so much during menstruation. The uterine lining is burned away. Hormone therapy can be given for two months before the operation to reduce endometrial growth.

Advantages

  • The operation is done through the vagina so no surgical cutting is needed. There is much less chance of bleeding than with a hysterectomy.
  • The operation can be done in a doctor’s clinic. Therefore, the woman does not have to stay in hospital.
  • The recovery time is much shorter than with a hysterectomy.
  • The success rates are high.

Disadvantages

  • Unlike with medical treatment or the Mirena IUD, the woman can no longer have children.
  • The operation may have to be repeated.
  • In a small percentage of women, this operation does not reduce bleeding

 

HYSTERECTOMY (removal of the uterus)

The purpose of this operation is to remove the uterus so that menstrual bleeding stops once and for all. Sometimes, the ovaries and the fallopian tubes are removed as well.

Unfortunately, this operation is sometimes recommended to women with menorrhagia even before testing for von Willebrand disease or other bleeding disorders. This means that some women lose the ability to have children, when their bleeding could be successfully treated.

Advantages

  • Hysterectomy stops menstrual bleeding once and for all.
  • It may be the only option for women who do not respond to medical treatment or the Mirena IUD, and for whom endometrial ablation is not effective.

Disadvantages

  • Unlike with medical treatment or the Mirena IUD, the woman can no longer have children.
  • A hysterectomy is a major operation. In women with bleeding disorders, there is increased risk of bleeding both during and after the operation. This can be managed with FVIII/VWF concentrates.
  • The operation requires a stay in hospital.
  • The recovery time is much longer than with an endometrial ablation.
  • When a woman undergoes a full hysterectomy (removal of the uterus and the ovaries), longterm hormone therapy is required.

 

LAPAROSCOPIC SURGERY (for endometriosis, or endometrial tissue outside the uterus)

The purpose of this operation is to remove endometrial tissue that has formed outside the uterus. This tissue bleeds during menstruation. The bleeding can cause pain in the pelvis and abdomen. Two small incisions are cut in the abdomen. Two tubes are inserted – one for a tiny camera, the other for the instruments to cut out the endometrial tissue.

Advantage

  • This operation can reduce pain and bleeding in the woman who does not respond to hormone therapy or other medical treatment.

Disadvantage

  • While not a major operation, a woman with a bleeding disorder will need appropriate preparation with FVIII/VWF concentrates.

 

OOPHORECTOMY (Removal of the ovaries)

The purpose of this operation is to stop bleeding from the ovaries. This bleeding may happen even though:

  • a woman is having hormone therapy to reduce menorrhagia
  • an endometrial ablation has been done or
  • a partial hysterectomy has been done.

Advantage

  • It can reduce bleeding and pain.

Disadvantages

  • An oophorectomy is a major operation.
  • Women can no longer have children.
  • Women need to take hormones.

Not recommended treatment

 

DILATION AND CURETTAGE (D&C)

The purpose of this operation is to scrape and clean the lining of the uterus. This may need to be done to diagnose another problem; however, for women with menorrhagia, it will not be effective in reducing bleeding. In fact, the opposite is probably true. The D&C will remove any existing platelet plugs and fibrin clots and make the bleeding worse.

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