Hemophilia Facts Worth Knowing
Most people have heard of hemophilia at one time or another, or at least heard about someone having this blood disorder. As with any disease or disorder that is somewhat feared, not everything presented as factual is necessarily true, and at times when the truth is being told, it has been exaggerated.
Point The Finger At Queen Victoria - Hemophilia is a genetic disease, carried from one generation to the next. It is often referred to as the royal disease, seeming to strike members of royalty as much as any other population group. There is something to this, since royals tend to wed other royals, and carriers of hemophilia within one royal family can introduce the genetic defect to other royal families. There are hemophilia facts bearing this out. A member of the Russian royal family, Tsarevich Alexei, who coincidently was killed by the Bolsheviks during the Russian revolution, was a hemophiliac, having inherited it from his mother, one of Queen Victoria's daughters. In fact Queen Victoria was a carrier of the gene, and three of her nine children inherited the disease. They of course married into other European royal families, taking their defective gene with them. The Austrian royal family, the Hapsburg's, were known hemophiliacs, or bleeders as they were sometimes called.
One of the hemophilia facts you may have heard, which happens not to be true, is that a hemophiliac can easily bleed to death if cut or scratched. While this may have happened in isolated instances, it is generally not true. Hemophilia is a disorder which prolongs the time it takes blood to clot and bleeding to stop. Being a hemophiliac doesn't mean that your blood will not clot if you have a cut, but simply that the clotting process is slower, which could of course be a dangerous situation if a wound is very large. There are a number of steps involved in the blood clotting process and, when someone is a hemophiliac, one of the steps is missing.
Internal Bleeding Is The Real Danger - The real danger the hemophiliac faces is from internal bleeding, especially around the joints. The bleeding may be slight and infrequent, but over a period of time may cause the joint to become arthritic. The person involved may or may not feel pain, but in some instances pain can be severe enough to have a crippling effect. Young people are more prone to ill effects from hemophilia than are adults, as bleeding in joints and muscles can be more pronounced during years of rapid growth. The digestive tract is also at times affected.
Women Carry It, Men Get It - Several other hemophilia facts worth noting: (1) while women carry the defective gene as often as men do, women seldom come down with the symptoms. (2) If a woman is a carrier, her sons have a 50% chance of coming down with the disease, and her daughters have a 50% chance of being carriers of the disease. (3) If a man has hemophilia, any daughters he may have will be carriers, but unless the man's wife has hemophilia, his sons will not be affected. (4) Approximately 4 people in every 100,000 have hemophilia. In by far the majority of these cases, the symptoms are mild, and only pose a danger in the event of a major injury or major surgery.
Symptoms - Symptoms of hemophilia include bleeding easily from falls, repeat bleeding from a clot that is disturbed, and blood in the urine. A bleeding muscle, yet another symptom, cannot be seen but may be felt as pain. The most severe symptoms, which can be life-threatening, are bleeding in the area of the brain, and around the central nervous system. Fortunately, only a small fraction of hemophiliacs exhibit such symptoms.
Treatment of hemophilia varies from the use of blood clotting techniques or medication in mild cases, to the need for periodic blood transfusions in more severe cases. The most important of the hemophilia facts as far as the average person is concerned is, if you have the disease and prick your finger, it is highly unlikely you will bleed to death. At worst, you may bleed just a bit longer than the other fellow.