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Hemophilia is a rare inherited blood disorder that causes prolonged bleeding from even minor injuries. It is caused by the absence of a protein that helps in clotting. Hemophilia A is more common than Hemophilia B. It is usually diagnosed in the first year of life and can be treated with synthetic proteins. The disease is almost exclusively found in males, but female carriers may experience mild symptoms. The disease afflicted Alexei, the son of Nicholas II, the last Tsar of Russia, and his tragic case inspired the common description of the condition as “The Royal Disease.”
Hemophilia is an inherited blood disorder that causes blood to clot normally. Those suffering from this condition may bleed for a long time from even the smallest cut or wound. Unexplained bruising is also common. Although bleeding from external wounds is a problem for people with the disease and can be serious, the danger of hemophilia lies in the tendency to bleed internally. Bleeding into muscles and joints is a common pain symptom, but major internal organs such as the brain, spinal cord, liver, and other critical systems can start bleeding unexpectedly, putting a person’s life at risk.
This disease is caused by the absence of an important protein found in normal blood that helps in clotting. Healthcare professionals recognize two different types of hemophilia: type A and type B. These two types differ in the type of protein that is deficient or absent in the blood. The protein that causes type A is called factor VIII protein, and hemophilia B is caused by the protein called factor IX.
Both types are extremely rare; however, type A is slightly more common than type B. Hemophilia is found almost exclusively in males, although female carriers may experience mild symptoms. Individual cases are considered mild, moderate, or severe depending on the deficiency of the proteins that normally help blood clot. Those with mild hemophilia generally have only 30% of the average clotting protein level. Moderate disease is the result of only 5% of the normal protein level, and severe results of less than 1% of the normal protein presence.
Hemophilia is usually diagnosed within the first year of life and modern treatments are effective. Factor replacement therapy using synthetic proteins is the most common and effective treatment. Other medications are available to increase the body’s production of protein or to aid clotting by other means.
In history, one of the most famous and tragic cases befell the designated heir to the throne of Russia. The son of Nicholas II, the last Tsar of Russia, Alexei suffered from severe haemophilia. Ignorance of the disease at the time made Alexei’s distraught parents trust the mystic Rasputin, who managed to bring relief to the child from his illness. The power and authority Rasputin gained through his healing abilities eventually led to the downfall of the Empire and the demise of the Romanov family. Alexei’s disorder inspired the common description of this condition as “The Royal Disease.”
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