Hemophilia - Treatment
Hemophilia can be treated with injections of missing clotting factors. Patients with hemophilia A receive injections of factor VIII, and those with hemophilia B get factor IX.
The frequency of treatment depends on the severity of the disease. People with mild hemophilia may require treatment only when they have been injured. They may also need treatment before surgery or dental work. Patients with more severe forms of the disorder may require regular injections of the missing factor.
In 1837, Queen Victoria ascended to the throne of England. Although she was a much-loved ruler, she left a terrible legacy to her country. She was a carrier for the gene for hemophilia. Although she did not suffer from the disease herself, she passed it on to one of her sons. In addition two of her daughters and three of her granddaughters were also carriers of the gene for hemophilia. Five of Victoria's great-grandsons had the disease.
Queen Victoria's son Leopold, according to the Queen's own accounts, had "been four or five times at death's door" because of the disease. At the age of thirty-one, he fell and hit his head. Doctors could not stop the bleeding, and he died a few days later.
Victoria's descendents eventually passed the genes for hemophilia on to the Spanish and Russian Royal houses. Victoria's daughters Beatrice and Alice both married German princes. They transmitted the gene for hemophilia to their own daughters who married into the Spanish and Russian royal families. It is said that two of Victoria's great-grandsons who grew up in Spain played in a park where all the trees were wrapped with cloth to prevent their being injured.
One of Alice's daughters, Alix, married Nicholas II, son of Alexander III, Czar of Russia. Alix's son, Alexis, also inherited the gene for hemophilia. At the age of three, he was injured and nearly bled to death. Alexis' painful and difficult childhood was a great source of distress to Alix. She was comforted when a man named Grigory Rasputin was able to stop the boy's bleeding. Rasputin was honored by Nicholas and Alix. Rasputin's closeness to the royal family and rumored influence was a contributing factor in the decline of the czarist Russian empire.
The use of injections to treat hemophilia is accompanied by some possible complications. For example, in some cases, the body's immune system begins to make antibodies against factors contained in the injections. Antibodies are chemicals produced by the immune system to protect the body against infection. The immune system may become confused and react as if the injected factors are bacteria, viruses, or other harmful materials.
Complications may result from the way clotting factors are obtained for injections. In some cases, the clotting factors are obtained from people who have donated blood. The donated blood is usually prepared very carefully in order to obtain pure clotting factors. But sometimes mistakes happen. Harmful substances in donated blood may become part of the preparation given to hemophiliacs.
The worst example of this situation involved blood contaminated with the human immunodeficiency virus (HIV). HIV is the virus that causes AIDS (see AIDS entry). In the early years of the HIV epidemic, people who worked with blood did not know about the virus. They were not aware that blood donated by people with HIV also contained the virus. When that blood was used to produce clotting factors, the virus was part of the preparation given to hemophiliacs. Many hemophiliacs developed AIDS in this way. As of 1999, AIDS was still the leading cause of death among hemophiliacs because of this tragedy. Precautions have been put in place to ensure that contaminated blood is not used. The chance of HIV or other harmful agents being present along with clotting factors is very small.
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