Terciární profylaxe u dospělých pacientů s hemofilií

Authors

ROMANOVÁ Gabriela SMEJKAL Petr POLÁK Pavel PENKA Miroslav

Year of publication 2017
Type Article in Periodical
Magazine / Source Remedia
Citation
Keywords hemofilie – artropatie – krvácení – léčba on demand – profylaxe
Description Prophylaxis is defined as rational application of the clotting factor concentrate with the aim of preventing life-threatening haemorrhage as well as bleeding inside joints. The goal is to maintain blood coagulation factor level above 1–3%, but if we want the occurrence of spontaneous bleeding to be prevented completely we have to keep the target level above 12%. Tertiary prophylaxis is defined as a clotting factor substitution in a patient of adult age with pre-existing haemophilia arthropathy, often following previous on demand treatment. The introduction of tertiary prophylaxis can be accompanied by various obstacles, such as financial, and psychosocial issues, age, and comorbidities. Switching an adult patient from the on demand treatment to tertiary prophylaxis must be assessed strictly on an individual basis and such a change must clearly reduce the patient’s frequency of bleeding and improve the quality of life. At the same time, the financial costs incurred for prophylaxis should be ideally compensated by delaying the costly, especially orthopaedic surgical procedures. Evaluation of joint score (HJHS , Pettersson score), joint imaging (X-ray, ultrasound, MRI ), orthopaedic, physiotherapy, and psychological examination should help in assessing the effect of prophylaxis.

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