Haemophilia A

Haemophilia A Latest News

A simple and affordable point-of-care test kit has been indigenously developed by the National Institute of Immunohaematology for early diagnosis of genetic bleeding disorders haemophilia A and Von Willebrand Disease.

About Haemophilia A

• It is a hereditary bleeding disorder that happens when your blood doesn’t clot as well as it should.

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Haemophilia A Causes

• When you bleed, a series of reactions take place in the body that help blood clots form. This process is called the coagulation cascade. 
• It involves as many as 20 different special proteins called coagulation, or clotting, factors. 
• You may have a higher chance of excess bleeding if one or more of these factors are missing or are not functioning like they should.
• Factor VIII (eight) is one such coagulation factor. Hemophilia A is the result of the body not making enough factor VIII.

How is Haemophilia A Passed On?

• It is usually caused by an altered gene being passed on to a child by their parents (inherited).
• Men with the altered gene will have symptoms.
• Women with the altered gene (carriers) can have symptoms, but this is less common, and the symptoms are less severe than in men.

Haemophilia A Symptoms

• The severity of symptoms varies. Prolonged bleeding is the main symptom. It is often first seen when an infant is circumcised. 
• Other bleeding problems usually show up when the infant starts crawling and walking.
• Mild cases may go unnoticed until later in life. 
• Symptoms may first occur after surgery or injury. Internal bleeding may occur anywhere.
• Symptoms may include:
  • Bleeding into joints with associated pain and swelling
  • Blood in the urine or stool
  • Bruising
  • Gastrointestinal tract and urinary tract bleeding
  • Nosebleeds
  • Prolonged bleeding from cuts, tooth extraction, and surgery
  • Bleeding that starts without cause

Haemophilia A Treatment

• Healthcare providers typically treat hemophilia A with factor replacement therapy. 
• Healthcare providers will inject concentrated factor VIII into your bloodstream.
• Factor VIII replaces the missing blood protein, or factor, that helps blood to clot

Key Facts about Von Willebrand Disease (VWD)

• VWD is a blood disorder in which the blood does not clot properly. 
• Cause: People with the disease have low levels of von Willebrand factor, a protein that helps blood clot, or the protein doesn’t perform as it should.
• Most people with the disease are born with it, having inherited it from one or both parents. 

Von Willebrand Disease Symptoms

• VWD does not always cause symptoms unless you have an injury. 
• Sometimes it’s found when you have more bleeding than usual after surgery or giving birth.
• Symptoms of VWD can include:
  • bruising easily
  • frequent nosebleeds
  • bleeding gums
  • bleeding more than usual after an injury or cut
  • heavy periods
• Some people also have more severe symptoms, such as:
  • heavy bleeding that’s difficult to stop, for example, after an injury or procedure
  • blood in your poo
  • blood in your pee (urine)
  • pain, swelling and stiffness in a joint

Von Willebrand Disease Treatment

• VWD can’t be cured. 
• But with treatment and self-care, most people with this disease can lead active lives.

Source: ET