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Slovenska pediatrija 2017; 24: 102-109


Review article

CONTEMPORARY MANAGEMENT OF SEVERE HAEMOPHILIA A

B. Faganel Kotnik
Klinični oddelek za otroško hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana

H. Košir
Klinični oddelek za otroško hematologijo in onkologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

L. Slemenšek
Klinični oddelek za otroško onkologijo in hematologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana

Abstract

In Slovenia, patients with haemophilia are treated in the Haemophilia Comprehensive Care Centre. This treatment includes prevention and treatment of bleeding, haematological care in diagnostic and surgical interventions, precise monitoring of the occurrence of bleeding, organising and monitoring of self-treatment at home, conducting investigations to determine inhibitors, assessment of bones and muscles, orthopaedic treatment, implementation of physiotherapy, resolving emotional and psychological problems and providing genetic counselling. This involves the entire medical team, in which the graduate nurse has a very important role. Patients with severe haemophilia A receive preventive replacement therapy with concentrates of FVIII, which prevents bleeding into joints and muscles, which can lead to permanent joint damage and disability, reduces the risk of life-threatening bleeding and improves the quality of life. The early and gradual introduction of preventive therapy may also reduce the risk of the development of inhibitors against the injected FVIII. Today, these are the most serious and most important complications of haemophilia treatment. Thanks to contemporary management, haemophiliacs are treated mainly in the outpatient clinic and live quality and creative lives and reach a comparable age to their peers without a bleeding disorder.

Key words: haemophilia, preventive treatment, quality of life.


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