Medexpress: Please give our viewers an idea of what life is like for the average hemophilia patient in Poland. How does this disease affect daily functioning, what difficulties and challenges does it involve?
Wojciech Mościbrodzki: Hemophilia is a genetically determined clotting diathesis. That is, to put it simply, my blood has very big problems with clot formation. The danger is, of course, all external wounds, but also internal bleeding, primarily to the joints. The joint fills with blood, like a balloon, until the blood pressure is not balanced by the joint envelope. This is associated with further complications, because when the body dissolves this clot, it also damages the joint cartilage. Thus, hemophilia is usually characterized by extensive damage to the joints, compounded over time. Treatment of hemophilia has so far relied on supplementation of the missing clotting factor. Supplementation is accomplished through intravenous injections. The factor is administered after the onset of bleeding. At the moment, a new generation of drugs completely changes the situation of hemophiliacs, because it allows prophylaxis, that is, administration of the drug without waiting until bleeding occurs. This de facto prevents all kinds of dangerous bleeding. Classic therapy required us to be able to insert ourselves into a vein, which was sometimes a problem, if only when a joint in the hand whose dexterity is needed to give ourselves an injection was damaged.
Another problem is the half-life of the supplemented clotting factor - it is very short. To act on existing bleeding, it had to be administered as soon as possible and then repeated intravenous infusions, usually on a schedule of every 8 or 12 hours. This raised certain difficulties - for example, how to apply such treatment ...
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