HAEMOPHILIA - AN INTERNAL BLEEDING DISORDER

Haemophilia is a genetic disorder that affects one person in a population of 10,000 males across the world.  Usually only males are the active sufferers, the females being the carriers. There are approximately 80,000 Haemophiliacs in India.

Haemophilia is a bleeding disorder caused by the absence or deficiency of any clotting factor in the blood. Usually the absence of Factor VIII or IX causes Haemophilia. The normal level of clotting factor varies between 50% and 150%, whereas in the case of haemophiliacs, it is as low as less than 1%.  The severity of the deficiency gets classified as mild, moderate and severe based on the factor level.

Haemophilia is a genetic disease.  Mothers with apparently normal clotting ability may, pass haemophilia on to their sons.  In most instances, there is a known family history of haemophilia.  Daughters may carry the gene for Haemophilia but rarely have symptoms of the disease.  Hemophilic males cannot pass the disease on to their sons.  However, any of the daughters may be genetic carriers of the disease and they can pass haemophilia on to succeeding generations.

A person with haemophilia does not bleed faster than anyone else but he may bleed for a longer period of time and it is difficult to stop the bleeding. The major problem for persons with haemophilia is uncontrolled internal bleeding which can begin spontaneously without apparent cause.  If internal bleeding is not quickly stopped with appropriate treatment, it will result in pain and swelling.   Over a period of time bleeding into joints and muscles can cause permanent damage and chronic pain. Bleeding occur with different frequency, depending on the level of severity of haemophilia in that person, from twice a week until once or twice per year or less. 

Most common bleeds affect muscles and joints.  The bleed site will be hot and itchy.  After a while the patient cannot move that part of his body, the area gets swollen and its hurts a lot.  When it hurts and there is both swelling and heat, it means the bleeding is serious.Treatment must be given the moment you suspect there is a bleeding.  Untreated bleedings or late treatment might damage joints and muscles.

Haemophilia is treated by infusing a clotting factor, derived from human blood, which remains active in blood for a short period of time.  Each time internal bleeding occurs an additional clotting factor is needed.  It is important to administer the clotting factor as soon as internal bleeding begins in order to reduce the chances of permanent damage.  Although there are effective controls, there is no cure for haemophilia.  A child born with the disease will have it all his life.

The concentrated Factors are presently not manufactured in India due to Government regulations. The imported medicine is very expensive. Though it is available to Indian patients at a subsidised rate, it is still comparatively lower than the international price.   The average cost of treating the occasional bleeds can be in excess of Rs. 1 lakh per annum.  Considering that no cure has so far been found and that only treatment is possible, the medical support during the lifetime becomes unaffordable in most cases.

Due to repeated bleeding episodes the joints get affected and many people become crippled.  The attendance at schools and colleges get affected and anxious parents generally restrict physical activities.  The deformed joints develop hemophilic arthritis before the age of thirty and getting employed becomes very difficult. 

For Details Contact : Haemophilia Federation (India), Old No. 54, Kasi Estate, Jafferkhanpet, Chennai - 600 083.
Phone : 4712773
Email :hfis@md3.vsnl.net.in.

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