April 17, 2008

The World Federation of Hemophilia (WFH) started World Hemophilia Day to promote a global effort to secure safe and effective treatment for everyone with a bleeding disorder. In tune with this year’s theme ‘Count Me In’ the WFH is aiming at identifying almost all of those with bleeding disorders in the world.

The day was chosen in honor of the birth day of WFH founder Frank Schnabel. It is celebrated in more than 100 countries now. Hemophilia organizations and treatment centers all over the world commemorate the day by organizing activities to raise awareness of the issues affecting people with hemophilia or related bleeding disorders. 

What is Hemophilia?

Hemophilia is a hereditary bleeding disorder that prevents blood from clotting properly. Being a genetic disorder, it lasts a whole lifetime. People with hemophilia lack a protein in blood that controls bleeding.

Common symptom is prolonged bleeding after an injury, tooth removal, surgery, or an accident. Other serious symptoms involve –
• Internal, uncontrolled bleeds mostly into joints,
• Red blotches  on the skin ,
• Sudden bleeding inside the body without a specific reason. 

Hemarthrosis, hemorrhage, gastrointestinal bleeding and menorrhagia are other serious problems arising from hemophilia.

Male Inherited Disorder  

The most prevalent of bleeding disorders are Hemophilia A, caused by deficiency of coagulation factor VIII, and Hemophilia B, a deficiency of coagulation factor IX. Being a chromosome disorder of the X -linked category, the disease manifests in the males, while females are ‘carriers’ only and  never exhibit any symptoms of the disorder (it must be noted that females have 2 X chromosomes  while  males have one X and one Y chromosome).

Till the  first half of the 20^th century, people with Hemophilia had a miserable existence.  They were usually disabled before 20 years and the average life expectancy for hemophiliacs was 27 years, mostly due to early deaths from bleeding into vital organs. Now with medical advancement, treatment for Hemophilia and related, rare bleeding disorders like von Willebrand’s disease,is available. Although expensive, this allows the patient to live a normal life.

Treatment of Hemophilia

  Treatment of severe Hemophilia A or B falls into one of two categories:

• Prophylaxis, or
• On-demand

Prophylaxis treatment involves treating the disorder before the symptoms arise. It is done by infusing the patient with plasma-derived or recombinant clotting factor concentrates on a regular schedule, in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes.

In case of ‘On-Demand’ treatment, bleeding episodes are treated once they arise. In developed countries, where awareness of the disease is high, and factor concentrates are easily available, problems like severely deformed joints, due to repeated bleeding episodes, are a thing of the past.

Hemophilia, Hepatitis and HIV 

Though the plasma-derived concentrates have saved lives and prevented disability, their use in the past has been complicated by infection with viral pathogens particularly the Hepatitis virus and Human Immunodeficiency Virus (HIV).

Several hemophiliacs who received blood products manufactured by American companies in the early to mid-1980s were tragically infected with the AIDS and Hepatitis virus.   The  concentrate of  Factor VIII ("antihemophilic factor" or "AHF") and Factor IX, which were allegedly drawn from high- risk groups, were manufactured and sold in the U.S. and exported worldwide, without proper screening.