April 17, 2025 – World Hemophilia Day

World Hemophilia Day is celebrated annually on April 17. This date was established at the initiative of the World Health Organization (WHO) and the World Federation of Hemophilia (WFH) to raise awareness about this rare and dangerous disease.

Hemophilia is a rare hereditary disorder associated with impaired coagulation (the process of blood clotting). In this condition, hemorrhages occur in joints, muscles, and internal organs, both spontaneously and as a result of trauma or surgery. With hemophilia, the risk of death from bleeding in the brain and other vital organs increases sharply, even with minor injuries. Patients with severe hemophilia become disabled due to frequent bleeding into joints (hemarthroses) and muscle tissues (hematomas).

Hemophilia appears due to a change in one gene on the X chromosome. There are three types of hemophilia (A, B, C).

The leading symptoms of hemophilia A and B are increased bleeding from the first months of life; subcutaneous, intramuscular, subfascial, retroperitoneal hematomas caused by bruises, cuts, various surgical interventions; hematuria; profuse post-traumatic bleeding; hemarthroses of large joints with secondary inflammatory changes.

The disease usually affects men (sex-linked inheritance); women typically act as carriers of hemophilia and can give birth to affected sons or carrier daughters. A total of about 60 cases of hemophilia (type A or B) have been documented worldwide in girls. Due to the fact that modern medicine significantly extends the average life expectancy of people with hemophilia, it can be definitively stated that cases of hemophilia in girls will become more frequent. Furthermore, in approximately 15-25% of cases, examination of the mothers of boys suffering from hemophilia does not reveal the aforementioned gene mutations, which indicates the mutation occurring at the time of parental germ cell formation. Thus, hemophilia can also appear in the absence of gene carriers among parents.

The most common misconception about hemophilia is that a person with hemophilia can bleed to death from the slightest scratch.

Major injuries and surgical operations, tooth extractions, as well as spontaneous internal bleeding into muscles and joints, apparently due to the vulnerability of blood vessel walls in people with hemophilia, pose a problem.

The quality of life for a person with hemophilia depends on the severity of the disease. For example, people with mild and moderate hemophilia (5-40% and 1-5% clotting factor content in the blood, respectively) lead a completely normal life and will only require specialized care for serious injuries, dental treatment, surgical interventions, etc. In contrast, people with severe hemophilia (less than 1% clotting factor in the blood) need constant access to treatment and specialized centers, as they may develop spontaneous bleeding into joint cavities, internal organs, the brain, etc., which can be fatal. Therefore, such patients require prophylactic administration of replacement therapy medication several times a week.

Hemophilia Prevention

Preventive measures include a pregnant woman visiting a medical genetics center to determine the hemophilia gene on the X chromosome (with the aim of preventing the birth of a child with hemophilia).

How to behave if the diagnosis of "hemophilia" has already been made:

• It is mandatory to be registered for dispensary observation. Registration for dispensary observation begins in childhood. A child with this diagnosis is exempt from vaccinations and physical education classes due to the risk of injury. However, physical activity should not be absent from the patient's life. It is necessary for the normal functioning of the body. There are no special dietary requirements for a child suffering from hemophilia.
• Relatives of the patient should also know what hemophilia is and undergo training on providing medical assistance in case of bleeding. They should know which medications can be used (for example, Aspirin should not be given as it thins the blood and can cause bleeding; cupping should also not be applied as it can cause bleeding in the lungs).
• Maintain a healthy lifestyle, avoid stress, physical exertion, and injuries.

State Institution "Rossonsky RCG&E", April 2025