HEMOPHILIA – The Royal Disease

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hemo2.bmpHemophilia is a life long genetic bleeding disorder. One of the clotting factors in his blood is almost absent or deficient. This means that whenever there is an injury, the bleeding does not stop. Bleeding can also happen anywhere inside the body even without an injury, and mostly in the joint. It is passed on at birth from an affected to daughter who then becomes the carrier of hemophilia gene and from mother to son who may himself have hemophilia.

Disability due to frequent bleed into joints, HIV/AIDS and Hepatitis B& C infections, expensive treatment causing financial risks, high insecurity leading to mental tension and anxiety are the risks associated with this disease. HEMOPHLIA IS INCURABLE BUT MANAGEABLE; WITH TIMELY AND PROPER FACTOR REPLACEMENT PERSONS WITH HEMOPHILIA CAN LEAD A NORMAL LIFE. PHISIOTHERAPY PLAYS AN IMPORTANT ROLE IN CONTROLLING BLEEDING EPISODES.

Hemophilia has often been called “The Royal Disease”. This is because Queen Victoria, Queen of England from 1837 to 1901, was a carrier. Her eighth child, Leopold, had hemophilia and suffered from frequent hemorrhages.. Even more important to history was the existence of hemophilia in the Russian Royal family. Alexandra, Queen Victoria‘s granddaughter, married Nicholas, the Tsar of Russia. Alexandra, the Tsarina, was a carrier of hemophilia and her first son, the Tsarevich Alexei, a hemophiliac. Nicholas and Alexandra were pre-occupied by the health problems of their son at a time when Russia was in turmoil. The monk Rasputin gained great influence in the Russian court, partly because he was the only one able to help the young Tsarevich. He used hypnosis to relieve Alexei’s pain. The illness of the heir to the Tsar’s throne, the strain it placed on the Royal family, and the power wielded by the mad monk Rasputin were all factors leading to the Russian Revolution of 1917.

In the 20th century doctors started investigating into the cause of hemophilia. Until then, they had believed that the blood vessels of hemophiliacs were simply more fragile. In the 1930’s doctors looked at defective platelets as the likely cause. Then, in 1937, Patek and Taylor, two doctors at Harvard, found they could correct the clotting problem by adding a substance, which came from the plasma in blood. This was called “anti-hemophilic globulin”. In 1944, Pavlosky, a doctor from Buenos Aires, Argentina, did a lab test, which showed that blood from one hemophiliac could correct the clotting problem in a second hemophiliac and vice-versa. The interaction of the different factors in blood clotting was named the “coagulation cascade”. In the 1950’s and early 1960’s, hemophiliacs were treated with whole blood or fresh plasma. Unfortunately, there weren’t enough of the Factor VIII or IX proteins in these blood products to stop serious internal bleeding. Most people with severe hemophilia and some people with mild or moderate hemophilia died in childhood or early adulthood.. The pressure of massive bleeding into joints and muscles made hemophilia one of the most painful diseases known to medicine. Then, in the 1960’s, Dr. Judith Pool discovered cryoprecipitate. Dr. Pool found that the sludge on top of thawing plasma was rich in Factor VIII. Even surgery became possible. Later freeze-dried powdered concentrates containing Factor VIII and IX began to be available. could be kept at home and used as needed. They revolutionized hemophilia care. Hemophiliacs were now independent of hospitals. They could travel, hold steady jobs and hope to lead normal lives. Unfortunately, only 20% of the world’s hemophiliacs enjoy this level of care.

Hemophilia Federation (India) was established in 1983. It is self-help NGO run by persons with Hemophilia themselves, with help from medical fraternity Today persons with Hemophilia can approach any of these centers, in India, for medical as well as psychosocial support. Lately the HFI has began importing Anti Hemophilia Factor (AHF) an extremely expensive life saving drug, for use by the hemophilia community. The government has supported HFI by waiving the import duty. There policy is, “those who can pay, should pay, but no one should be denied treatment”.

Even as the country focuses on headline – grabbing diseases, Hemophilia, a little known blood disorder, is turning out to be a silent killer with over 50,000 people affected. World Hemophilia Day is observed every April 17 to promote a global effort to secure appropriate care and treatment for a disease that is the oldest known inherited bleeding disorder caused by a defect in the genes.

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Aastha Khurana

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