Facts About Hemophilia
Posted In: Diseases.
Hemophilia is an inherited blood disorder and it is a very rare disease. Its severest form only occurs in 1 in 100,000. It is the disease that stops coagulation of the blood and so the sufferer, when cut, will bleed and bleed. It is carried and passed on by a mother but its symptoms usually only manifest themselves in male offspring. The male himself does not transmit hemophilia.
Disease of European royalty
The British Queen Victoria was a carrier of Hemophilia and passed it on to her son Leopold and to some of her daughters. Because European royalty was a more or less closed inter-breeding system, these daughters were married off to European royal houses and Hemophilia was manifest in the royal families of Spain, Germany and Russia.
The Spanish royal house
Queen Victoria’s granddaughter, Victoria Eugenie of Battenberg, became Queen of Spain and two of her sons suffered from hemophilia and died from internal bleeding after relatively minor car accidents. In both cases, they never sought treatment because no injury was apparent.
The son of the Tsar of all Russia, Alexei Nikolaevich suffered hemophilia. He got it from Empress Alexandra, descendant of Queen Victoria. It is said that the “mad monk” Rasputin successfully treated him. Apparently, treatment was using aspirin, which was in fact thinning the blood and making the hemophilia worse. Rasputin’s success was apparently little more than stopping the aspirin which allowed the condition to improve significantly.
Cure and treatment
There is no known cure. It is an inherited and recessive condition, which is permanent. It is not infectious and can be managed. Usually this involves infusions of the blood clotting factor. A relatively new, recently approved drug by Wyeth involves little human plasma but the genes from the ovaries of Chinese hamsters. It is however expensive. On demand treatment is more common and in moderate cases, quite successful.
There are no peer-reviewed studies to support the use of the various herbs such as Bilberry, Scotch Broom, Stinging Nettle, Grape Seed, Witch Hazel and Yarrow.
There is some evidence to suggest that hypnosis can be effective in reducing bleeds and thus reduce the frequency of need for prophylaxis.
Lifespan for a person with Hemophilia
Before 1938, the average lifespan was 11 years. Before 1968, that number went up to 20 years old and the option for treatment was plasma or whole blood transfusions. After 1968, the average age went up further to less than 40 years and the treatment available was Cryoprecipitate. By 1983, the average was 64 years and treatment option was freeze dried clotting factors. By 1988, the average age went down to around 40 years due to the impact of AIDS in patients with hemophilia. By 1999, the average lifespan for patients with hemophilia, with proper care and treatment, was about the same as the average population. A study done in the UK reported that for severe cases of hemophilia, the average lifespan was 63 years and for moderate or mild cases of hemophilia, the average lifespan was 75 years old.
More on hemophilia: Who discovered hemophilia?