Hemophilia is called 'Royal Disease'.
Human beings may be born in one way, but death can happen for a thousand reasons. It may be a normal old age death. There may be an accident. Again this death can come from any pathological cause. Thousands of people are dying of various diseases in the world. Some of these diseases are very well known. Again, the names of some diseases are unknown to most people. There are certain unnamed diseases, which are not commonly seen. One such disease is hemophilia.
World Hemophilia Day is celebrated on April 17 every year;
Usually the diagnosis of a disease increases based on the number of infected patients. The higher the number of patients, the more the disease is known among the common people. Again, the harmful effects of the disease may be one of the reasons for the increase in its awareness.
Hemophilia originates from the descendants of Queen Victoria of Great Britain. The disease gradually spread from the British dynasty to the Russian, Spanish and German dynasties. That is why it is called 'royal disease'.
Queen Victoria;
What is hemophilia?
The word hemophilia comes from the two Greek words haima and filia. Haima means blood and Philia means attraction. When bleeding starts in any part of the body, blood clots usually form there. In medical parlance this process is called clotting. The clotting process causes the blood to clot and gradually stop bleeding.
Truth be told, clotting is when a wound forms on our body and dries up over time. The substance that binds to bleeding is called clot. But if for some reason this clot is not formed at the wound site, then there is bleeding at once.
The process of creating this clot in the body of a person with hemophilia is not normal. This is not to say that the patient's body will continue to bleed profusely and very quickly. Basically a person with hemophilia (those with hemophilia) starts bleeding from the body for a long time.
Different protein factors in the blood help blood to clot;
Another issue needs to be clarified. Many people now think that if they have this disease, they will continue to bleed whenever they cut their hands, feet, knees, etc. That's not the point. Any minor injury to the outside of the body is not a major concern here.
The real concern is internal bleeding or bleeding in any part of the body. This type of bleeding is called hemorrhage. It is mainly found in any joint inside the body such as the knees and ankles. Bleeding can also occur at the junction of various internal tissues and muscles. Such bleeding inside the body is very painful and the affected part starts to swell up a lot.
There is prolonged bleeding inside the body;
Why is hemophilia?
Hemophilia is a hereditary disease. The disease originated through genetic division or mutation. Haemophilia can be divided into three types. These are hemophilia-A, hemophilia-B and hemophilia-C. Hemophilia-B is also called Christmas disease. Eighty percent of patients with hemophilia are diagnosed with hemophilia. One in five to ten thousand newborns develops hemophilia.
This disease is caused by the absence of Clotting Factor VIII in the blood. Hemophilia-B is less common than hemophilia. One in every twenty thousand to thirty-four thousand newborns is infected with hemophilia-B. Hemophilia-B occurs due to the absence of clotting factor-9 in the blood. Hemophilia-C is more rare than the previous two. One in a million people are diagnosed with the disease. Both men and women can get hemophilia-C. This disease occurs in the absence of coagulation factor-9 in the blood.
General bleeding and the type of bleeding in a patient with hemophilia;
Hemophilia is a sex-linked recessive disorder. Men and women each have a pair of sex chromosomes. This pair of sex chromosomes in women is XX and in men it is XY. Among these, mutations in the X chromosome cause hemophilia. Since women have a large number of X chromosomes, they are the main carriers of the disease in most cases.
Fifty percent of cases of hemophilia are transmitted by females to boys and fifty percent to females. From a father with hemophilia, his daughter must carry the gene for the disease. Again, even if the father carries this gene, if the mother is not a carrier, the disease will not occur in the son. If you think these things are a little complicated, you can watch the video below.
The way it became a 'royal disease'
Haemophilia spreads widely among the descendants of Queen Victoria of Great Britain. Several of Queen Victoria's daughters were carriers of the disease. When they get married in the royal family of another country, the disease spreads among them. That is why this disease is called royal disease.
The first hemophilia outbreak in the British royal family was caused by Prince Leopold, the son of Queen Victoria. No such disease has been seen in this royal family before. Prince Leopold was born on April 7, 1853. He was the eighth child of Queen Victoria and Prince Albert.
The disease has not been seen in any of the Queen's predecessors. Moreover, the disease was not seen among the other brothers and sisters of the queen and their children. Even Prince Albert had no symptoms of the disease. It is thought that the disease was caused by a mutation from Queen Victoria or her mother.
Prince Leopold (sitting in the chair) was the first hemophilia patient among the Queen's descendants;
Queen Victoria was always concerned about her youngest son, Prince Leopold. According to history, Leopold also showed signs of epilepsy. He was married to the German princess Helena of Waldeck-Piermont. Prince Leopold died in an accident at the age of thirty-one.
One day, while descending the stairs, he fell and injured his knees and head severely. He died the next morning of cerebral hemorrhage.
Queen Victoria and her descendants;
Among Queen Victoria's children, two daughters, Alice and Beatrice, were carriers of hemophilia. One of Beatrice's daughters was married to a Spanish royal family. Similarly, Alice's daughter Alice was married to a Russian royal family. In 1904, Alice's son Alexis inherited the disease. Thus hemophilia seeds began to be sown in other dynasties of Europe.
At a glance the spread of hemophilia in the royal families of Europe;
Last word
Outbreaks appear to be exacerbated during antiquity. People at that time had very little idea about blood. When the first symptoms of the disease were found in the British royal family, the medical system was not so advanced. The first blood plasma was discovered in 1904. Plasma proteins are then discovered, the absence of which can lead to hemophilia.
Being a hereditary disease, there is no way to prevent this disease completely. However, with the gradual improvement of the medical system, modern treatment of this disease has now begun. It is now possible to quickly diagnose hemophilia through blood tests and take necessary action. Had there been such a medical facility several more years ago, the disease might not have earned the title of royal disease in this way.
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