Sunday 3 July 2016

Other Treatments that are helpful to Treat Bleeding in Hemophilia A and B

Desmopressin


Desmopressin is a synthetic drug which is a copy of a natural hormone. Desmopressin is not made from blood. Desmopressin is useful in treating people with mild or moderate hemophilia A. It is of no value for people with severe hemophilia A or with any type of hemophilia B.

It acts by releasing von Willebrand factor stored in the lining of the blood vessels. Von Willebrand factor is another protein which is important in blood clotting. One of its roles is to transport factor VIII in the bloodstream. Doctors think that by increasing von Willebrand factor levels, more factor VIII is brought to the site of damaged blood vessels.

Desmopressin can be taken in three different ways.


  • It can be injected into a vein. Most often, the brand name for this kind of desmopressin is DDAVP.
  • It can be injected under the skin. The brand name for this kind of desmopressin is often Octostim.
  • It can be taken by nasal spray. The brand name of the nasal spray is often Octostim Spray.
Desmopressin is usually effective for mild and moderate hemophiliacs. However, different people respond to desmopressin in different ways. Therefore, a doctor needs to do tests to find out each individual's response to the drug. Ideally, these tests are done before any urgent need for the drug, such as surgery.

Since desmopressin acts by releasing von Willebrand factor stored in the body, you cannot 'go to the well' too often. A sufficient amount of time, usually 24 hours, must elapse between doses of desmopressin to allow the body to rebuild its stores.

In serious bleeds or major surgery, desmopressin alone may not be enough to control bleeding. In such a case, a person should also receive a concentrate of factor VIII. (See What are the blood products of choice for hemophilia A?)

Desmopressin can sometimes have some mild side effects. These are:
  • facial flushing
  • mild headache
  • nausea and abdominal cramps.

Desmopressin can make the body retain water. Therefore, doctors recommend that after receiving desmopressin people drink only enough fluid to satisfy thirst.

If a person has a very bad headache or has not been able to pass water 24 hours after taking desmopressin, he / she should go to the hemophilia / bleeding disorder clinic or emergency room for help.


Cyklokapron and Amicar


Cyklokapron (tranexamic acid) and Amicar (aminocaproic acid) are useful in treating both hemophilia A and B.

Cyklokapron and Amicar are drugs that help to hold a clot in place once it has formed. They act by stopping the activity of an enzyme, called plasmin, which dissolves blood clots.

They do not help to actually form a clot. This means they can not be used instead of desmopressin or factor VIII or IX concentrate.

They can be used to hold a clot in place in mucous membranes such as:
  • the inside of the mouth
  • the inside of the nose
  • inside the intestines (the gut)
  • inside the uterus (the womb).

Cyklokapron and Amicar have proven very useful for people with hemophilia and for carriers who experience bleeding.
They are used:
  • before dental work
  • when a person has mouth, nose and minor intestinal bleeding
  • for carriers with heavy, prolonged menstrual bleeding.

These drugs come in tablet form.

Cyklokapron and Amicar can sometimes have some mild side effects. These are:
  • feeling sick to the stomach (nausea)
  • feeling tired or sleepy
  • feeling dizzy
  • having loose bowel movements (diarrhea)
  • having pain in the stomach.

These mild side effects go away when:
  • a person stops taking the drugs or
  • the doctor reduces the dosage.

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