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What is it about ?
Our body has 2 mechanisms to stop bleeding. On the one hand, the platelets clump together and form a clot. On the other hand, our body makes clotting proteins (clotting factors), which strengthen a blood clot and speed up the clotting process.
The production of some of these coagulation factors is impaired in some hereditary conditions. The main conditions involved are hemophilia A and B, and von Willebrand disease. Since these diseases can lead to bleeding and serious complications during operations, they should be detected and treated at the slightest suspicion.
Where do they occur?
Fortunately, inherited bleeding disorders are rare.
Hemophilia A and B only affect men. Women can pass on the anomaly, but do not have it. However, they may present a increased tendency to bleeding during surgical procedures.
Von Willebrand disease affects both men and women.
How to recognize them?
In mild to moderate hemophilia, bleeding is usually related to trauma, such as an accident or surgery.
Severe hemophilia is characterized by spontaneous bleeding (without trauma, for example) in joints and soft tissues (such as muscles), internal bleeding, and prolonged external bleeding. Often the problem is detected the first time when a child is easily injured. blues after a fall or his gums are bleeding while brushing his teeth.
Joint bleeding mainly occurs in the knees, elbows, and ankles, which are swollen, painful, and hot to the touch. Mobility is reduced. Subsequently, this can lead to permanent damage to the joints. Bleeding into soft tissues can be very extensive and lead to anemia, characterized by symptoms such as pallor and abnormal fatigue.
In von Willebrand disease, it is bleeding from the skin and mucous membranes that predominates, resulting, for example, in bruises (bruises) and nose bleeds. The heavy periods and abnormal blood loss during procedures such as tooth extraction are also hallmarks of the disease.
How is the diagnosis made?
The diagnosis is made by means of a blood test and confirmed by additional genetic analyzes. Blood clotting tests are done. If there is a lack of clotting factors, the clotting time will be prolonged; this is because it takes longer for the blood to form a solid clot.
X-rays are rarely helpful. Sometimes a ultrasound is performed to be able to assess the extent of bleeding.
The doctor will always ask if other family members have a tendency to bleed or have blues easily. If so, it will offer them various exams as well.
What can you do ?
Always carry a card with you indicating that you have one of these conditions. Notify any doctors or dentists you see. Keep a careful log of all events and treatments.
Always use great caution so as not to injure yourself. The choice of your profession may be important in this regard. Protect children, especially their knees and elbows, when playing. Inform the school of the illness so that the staff can monitor the child and know what to do if there is a concern.
If bleeding occurs, lift the limb slightly. Apply ice and a compression bandage. Never take aspirin and avoid anti-inflammatory drugs unless prescribed by the doctor. These drugs increase the tendency to bleed. On the other hand, paracetamol is authorized.
What can the doctor do?
In hemophilia, treatment involves giving the missing clotting protein. Administration is in the hospital, by infusion, whenever bleeding occurs. Children are given the clotting factor 2-3 times a week as a preventive measure. In adulthood, this preventive treatment is only given in a few special situations, such as after surgery or joint bleeding.
Sometimes the doctor finds that the treatment is no longer working. This loss of efficiency is usually due to the formation of antibodies, which destroy the administered coagulation protein. The doctor must then administer other coagulation factors. To avoid causing bleeding, injections are not made into the muscle, but under the skin (subcutaneously).
Mucosal bleeding in von Willebrand disease is treated with tranexamic acid. The heavy periods may be reduced by taking the pill. Sometimes desmopressin, a hormone that increases clotting, is used to stop further bleeding or as a preventive measure during surgery. In severe cases, plasma is given with the missing clotting factor.
Want to know more?
- Acetylsalycilic acid (aspirin) – CBIP – Belgian Center for Pharmacotherapeutic Information
- Non-steroidal anti-inflammatory drugs – CBIP – Belgian Center for Pharmacotherapeutic Information
- Paracetamol – CBIP – Belgian Center for Pharmacotherapeutic Information
- Tranexamic acid – CBIP – Belgian Center for Pharmacotherapeutic Information
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