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Women die after childbirth because no one has been diagnosed with haemophilia. Meanwhile, a simple blood test can prevent a young mother from bleeding out.
Only men suffer from hemophilia, women are only carriers. However, this only applies to congenital haemophilia. Acquired also affects ladies, especially pregnant. Both congenital and acquired haemophilia is a lack of blood clotting which makes even a minor trauma to life dangerous. Internal bleeding is also dangerous.
Royal disease
The nickname of “royal disease” gained hemophilia thanks to the queen of Great Britain and Ireland – Victoria, who was its carrier. She passed it on to her children, who, in turn, marrying representatives of royal families made the disease spread among European rulers and called it “royal”. Haemophilia influenced the fate of the entire continent, because the son of the last tsar and at the same time the great-grandson of Queen Victoria – Tsarevich Alexei – was unable to take power from his father due to his illness, which accelerated the outbreak of the revolution in 1917. It is worth adding that he ruled for over 63 years. years Queen Victoria is the ancestor of the reigning monarchs of Great Britain, Denmark, Spain, Sweden and Norway.
Coagulation disorders
Blood clotting is a complicated process. Platelets flow to the place of the cut and stick together to form a clot that inhibits bleeding for several dozen minutes. The role of platelets is very important and that is why before the surgery, the number of patients is reduced and, if necessary, they are rolled over. After several dozen minutes from the formation of the first clot, the clotting factors begin to work, because the reinforcement of the dam is needed. There are a dozen of them, one activating the other, as in falling dominoes. If one is missing, the cascade will not work. Depending on whether the patient lacks platelets or clotting factors, the coagulation disorders may have a different course. – In the first case, the patient will bleed during the procedure, for example on the dentist’s chair, while in the second case, the haemorrhage will occur about two hours after returning from the dentist – explains the hematologist, Dr. Ewa Stefańska-Windyga.
Inheritance of hemophilia
Clotting factors are responsible for the durability of the bleeding-inhibiting clot. Absence of VIII and XI means hemophilia. Although only men suffer from it, women pass it on to their offspring. The ancient Jews already knew that if two sons were bled during circumcision, the mother should not give birth to another. Women who are carriers may have a mild form of haemophilia without any symptoms. It will not appear until the first surgical procedure. – Often the patient does not know that she is a carrier of haemophilia. It is only, for example, after a knee injury while skiing, when he performs a minor surgery, that bleeding occurs on the first or second day, says hematologist Dr. Ewa Stefańska-Windyga.
Acquired haemophilia
There will be times, however, that in a healthy person who does not suffer from hemophilia, or who does not carry it for unknown reasons, the body begins to produce antibodies against one of the clotting factors that destroy it. Then we are talking about acquired haemophilia. It happens very rarely. In Poland, only 40 cases a year. It attacks elderly people and pregnant women. Antibodies may also develop several months after birth. 20% of pregnant women with hemophilia may die in puerperium as a result of bleeding. If it bleeds during childbirth, the patient has a blood transfusion. However, hemorrhages appear even 4 months after delivery.
Symptoms of hemophilia
Blood coagulation disorders are often manifested by menstruation lasting too long. Often, patients are treated by a gynecologist for many years before they go to a hematologist. – A patient came to me, her menstruation lasted 10 days. The gynecologist spooned the uterus several times and finally decided to remove it. Nobody came up with the idea to test the coagulation factors in the patient – says Dr. Stefańska-Windyga.
The first symptoms of acquired haemophilia are bruises on the body. However, these symptoms are neglected. Meanwhile, a simple APTT blood clotting time test may be a clue whether we are dealing with acquired haemophilia, says Dr. Stefańska-Windyga. – I had a patient whose bruises started appearing three weeks after giving birth. Since she had given birth to twins, her doctor decided she was exhausted. She was taking iron, but the bruises grew more and more. Fortunately, she went to a hematologist who decided to rule out acquired haemophilia and it turned out that the patient suffers from it – she says. Starting treatment avoids serious consequences. Especially since, unlike inborn haemophilia, acquired hemophilia can be cured. Dr. Stefańska-Windyga emphasizes that thanks to the early diagnosis and treatment of the disease, there is a chance that the expectant mother will not bleed out during or after childbirth.
Text: Halina Pilonis