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Apr 18, 2021
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Modern approach. How are hemophilia patients being helped today?

Many people are familiar with the name “hemophilia”. So, for example, the Russians from the history course know well that the son of the last Russian Emperor Nicholas II was susceptible to this disease. And because of this, the family was very worried about him. Today, doctors continue to look for ways to help patients with this diagnosis.

“Hemophilia is an inherited disorder associated with blood clotting disorders. The disease is expressed in an increased ability to bleed, which can be caused by even minor injuries, ”notes doctor therapist-cardiologist of the Clinical and Diagnostic Center “Medintsentr” (branch of the GlavUpDK under the Ministry of Foreign Affairs of Russia) Narine Samsonova… Accordingly, almost everything becomes a problem: from banal falls with bruises and subcutaneous hemorrhages to tooth extraction at the dentist.

This disease is not so common. The prevalence ranges from 1 in 10,000-50,000 men. Pathology debuts in most cases in early childhood, therefore hemophilia is traditionally referred to as pediatric problems. “The vast majority of people with hemophilia are men. There are also known isolated cases of hemophilia in women. At the same time, women can be carriers of the disease and pass it on to their sons, ”the therapist notes.

Modern diagnostics

Previously, the presence of such a problem as hemophilia was recognized after the fact, when a person began to have health problems and blood clotting. Today, the approach to diganostics has changed markedly. So, for example, hemophilia can be determined already in utero.

“It is possible to diagnose hemophilia at the stage of pregnancy: there are various types of screenings for this. Signs of hemophilia can manifest in newborns in the form of hematomas on the body or prolonged bleeding from the umbilical cord. In the future, the child may develop soft tissue hematomas, post-traumatic bleeding from the mucous membranes, hemarthrosis, which can be more dangerous than external bleeding, ”notes Narine Samsonova. Internal bleeding is invisible to the eye, which is why precious time is lost and serious consequences for the body develop.

In addition, genetic tests come to the aid of parents, which can show the carriage of a gene. And family stories to help: the disease is hereditary, so it’s worth knowing everything thoroughly before planning a child.

Who heals today

Specialists of various fields are currently engaged in the treatment of hemophilia, says Narine Samsonova. In particular:

  • neonatologists;
  • pediatricians;
  • genetics;
  • hematologists.

“With concomitant pathologies, you may need to consult with a gastroenterologist, otolaryngologist and neurologist, as well as an orthopedic traumatologist,” says Narine Samsonova. For example, such consultations may be required when problems arise in the spleen, liver, etc., as well as if the nasopharynx and respiratory tract is affected. And, of course, you should be very careful in everyday life in order to avoid situations that provoke injury.

One of the common problems faced by hemophiliacs is the development of hemarthrosis, manifested against the background of repeated bleeding in the joint cavity. Usually they talk about the defeat of one knee, but it also happens that both suffer, which affects the motor abilities of a person. Therefore, it is important to regularly undergo examinations with doctors in order to monitor the condition. But you cannot put an end to a person with hemophilia: he may well live a normal life.

Forecasts

“It is completely impossible to recover from hemophilia. It is necessary to be constantly monitored by a specialist, to conduct laboratory tests: coagulological screening, instrumental diagnostics. They allow visualizing bleeding and hemorrhage of various localizations, ”notes Narine Samsonova.

For mild disease, cuts and external bleeding should be properly handled to stop bleeding and prevent massive blood loss. Narine Samsonova notes that such wounds are treated with thrombin and a pressure bandage is applied. “In severe pathology, patients regularly receive blood clotting concentrates, which reduce the risk of internal bleeding. When planning surgical interventions, additional doses of concentrates are introduced. An additional method of maintenance can be regular blood and plasma transfusions, ”the therapist notes. Naturally, a person with such a history must constantly be under the supervision of a doctor, and all medical interventions should be meticulously discussed even at the planning stage.

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