Every April 17, the world commemorate the Day of Hemophilia to improve understanding and awareness of the threat of this hereditary disease. Some people may be familiar to hear the term hemophilia, but many of us may not know the initial development of this disease. Towards Haemophilia Day worldwide, it is important people know better the history of this disease.
Hemophilia is a bleeding disorder due to a lack of production of one of the factors of blood clotting in the body. Haemophilia derived from Ancient Greek, which consists of two words namely Haima, which means blood and philia, which means love or affection.
Hemophilia have been found long ago. Hemophilia word first appeared in an article written by Hopff at the University of Zurich, in 1828. According to the encyclopedia Britanica, the term haemophilia (haemophilia) was first introduced by a German doctor, Johann Lukas Schonlein (1793-1864), in 1928.
Schonlein who is a professor of medicine at three major universities in Germany – Wurzburg (1824 – 1833), Zurich (1833 – 1830) and Berlin (1840 – 1859), was the first physician who used a microscope to do chemical analysis of urine and blood for diagnosis of the illnesses suffered by a patient.
Haemophilia is often referred to as “The Royal Diseases” or royal disease. This caused the Queen of England, Queen Victoria (1837 – 1901) is a bearer of properties / carrier of hemophilia. Her eighth, Leopold was a hemophiliac and frequent bleeding. This situation is in the news in the British Medical Journal in 1868.
Leopold died of a brain hemorrhage when he was 31 years. One daughter, Alice, turned out to be the carrier of hemophilia and son of Alice, Viscount Trematon, also died of a brain hemorrhage in 1928.
The most important thing in the history of hemophilia is the existence of the disease in the Russian royal family. Two of the daughters of Queen Victoria, Alice and Beatrice, were carriers. They spread the disease of hemophilia into Spanish, German and Russian Royal Family.
The history of haemophilia in the 20th century
In the 20th century, the experts continue to search for the cause of hemophilia. Until they believe that the blood vessels of patients with hemophilia easily broken. Then in 1937, two doctors from Harvard, Patek and Taylor, to find solutions on blood coagulation, namely by adding a substance derived from plasma in blood. Substance called anti-hemophilic globulin.
In 1944, Pavlosky, a doctor from Buenos Aires, Argentina, makes a lab test and the results showed that the blood of a patient with hemophilia can overcome the problem of blood clotting in hemophiliacs other and vice versa.
He by chance had found two types of hemophiliacs with each – each a different protein deficiency – Factor VIII and Factor IX. And this in 1952, making hemophilia A and hemophilia B as two different types of disease
Then in the 1960s, fresh plasma or cryoprecipitate was found by Dr. Judith Pool. He found that the sediment above the melting plasma contains a lot of Factor VIII. For the first time, Factor VIII can be inserted in patients in need, to cope with serious bleeding. Even allowing for operation in patients with hemophilia.
In the late 1960s and the early 1970s, the essence of which contains Factor VIII and Factor IX are packaged in a dry powder form and has been found frozen. So that can be stored at home and used at any time – if needed.
And now the people with hemophilia do not always depend on the hospital. They can travel, work and live a normal life. Tragically, some treatments resulting from the blood has been contaminated with several types of viruses, like hepatitis C and HIV. Many people with haemophilia affected.
Haemophilia in Indonesia
Although hemophilia has long been known in the medical literature, but in Jakarta in 1965 a new laboratory diagnosis introduced by Kho Lien Keng with thromboplastin Generation Time (TGT) in addition to the procedures for bleeding and clotting time. Treatment is available at the hospital only fresh blood, while the production of cryoprecipitate which is used as the primary therapy of hemophilia in Jakarta, was introduced by Masri Rustam in 1975.
What are the common symptoms of hemophilia?
1. For people with hemophilia, bleeding may occur in every organ of the body. Sometimes the bleeding can be easily seen, but can also not
2. Bleeding in the can after the collision and after surgery. However, there is the possibility of bleeding also occur without any known cause. Bleeding like this is called spontaneous bleeding.
3. In general, infants who suffer from hemophilia rarely bleed.
4. Bleeding often occurs when the baby began to start learning to walk. Bleeding that occurs because of injury usually lasts much longer, especially in the mouth and tongue.
5. When the patient was growing up, the more frequent spontaneous bleeding. Most occur in the joints and muscles.
6. In Indonesia, when the patient has reached puberty baliq period, bleeding can be seen clearly long after he was circumcised.
How does the handling of blood clotting bleeding using a factor?
1. Bleeding in haemophilia is usually treated by giving blood clotting factors directly into the vein (injected). Blood clotting factors can not be given by mouth.
2. Blood clotting factors can be found in various forms such as cryoprecipitate, Fresh Frozen Plasma (FFP), or blood clotting factor concentrates.
3. Desmopressin or DDAVP can be given to people with hemophilia A minor. This product can be given by way menyuntikannya directly into the vein, under the skin, or sprayed through the nose.
4. Giving blood clotting factors usually must be done repeatedly.
What should be done to stay healthy patients?
Medical treatment such as transfusion / injection of blood clotting factor is only one way for patients to have a healthy body. However, there are other ways you can do to stay healthy hemophiliacs.
1. Sports and keep in shape
2. Using the right protective sports moment
3. Perform regular medical examination including checking the situation joints and muscles
4. Receiving vaccinations recommended various infectious diseases physicians, including hepatitis A and B.
5. Memepertahankan balanced weight. Patients with hemophilia who had never exercised a great chance of experiencing excessive weight gain. Patients with hemophilia should also pay attention to his weight, so it does not increase the load on the joints in the lower body, especially for those who are stricken with arthritis.
In addition to hemophilia, there are other blood disorders who also have the same almost like, von Willebrand factor. Von Willebrand Disease (VWD) is a bleeding disorder that affects most people.
In fact, he was not a single disease but a family illness. This type of disease is caused by problems with von Willebrand Factor (vWF). It is a protein in the blood necessary for blood clotting. VWF gene that makes working on two types of cells ie endothelial cells (lining the blood vessels) and platelets.
If there is not enough vWF in the blood, or not working properly, then the blood clotting process takes longer.
How often are found in von Willebrand disease? Doctors now believe that Visual Studio can be about 1 in 100 people. Because many people – these people only experience mild bleeding, then only a small number who knew that he brought this pernyakit.
Von Willebrand disease can be about men and women. However, because many women with VWD have a lot of menstrual bleeding and bleeding shortly after childbirth, more women who have symptoms than men.
Children also can suffer from VWD. They are born with this disease. This is because Visual Studio is diturunkan.VWD abnormalities that can be derived from parent to child if one of the parents have VWD, they can reduce the disease to the child – his son.
Von Willebrand factor itself is taken from the name of a Finnish doctor, Erik von Willebrand, who first describes this condition in 1925. He realized that the disease is not the same as hemophilia, which is in a state of weight falls on the male – male.
What’s more, some researchers found that “… mild VWD can be beneficial to health.” Researchers said this is because Visual Studio makes platelets more difficult to cling to each other. So a person with VWD would be less experienced blockage of blood vessels, as a cause of heart attack or stroke.