Sonata Saulyte-Trakymiene1, Agne Juodyte1, Viktorija Jusinskaite2, Ruta Kulikauskaite2
1Children‘s Hospital, Affiliate of Vilnius University Hospital Santaros Klinikos
ABSTRACT
Introduction: in patients with hemophilia, recurrent hemarthroses often lead to irreversible joint destruction. Thus, the condition of joints represents the adequacy of hemophilia management.
Aim: to evaluate the condition of joints with several standardized noninvasive methods in patients with hemophilia A and B in Lithuania.
Methods: structural joint damage was evaluated applying the World Federation of Hemophilia (Gilbert) scale and Hemophilia Joint Health Score. Functional joint status was assessed using performance-based tools (Functional Independence Score in Hemophilia, Keitel Functional Test and 6-minute Walk Test) and self-assessment questionnaire (Hemophilia Activities List). Two assessments were carried out with the interval of one year, and the outcomes were compared.
Results: twenty-one pediatric and 21 adult patients were enrolled into the study. 81.0% of children received prophylaxis, while the adults were mostly treated on-demand (61.9%). At baseline, mean HJHS score for children was as low as 6.6. In adults, structural joint damage was more advanced: mean total HJHS score was 30.1, and mean WHF-PES was 23.2. The results of performance-based and self-assessed functional evaluation in adults were lower than those in children. Over a year, the condition of joints worsened more markedly in adults than in children. In adults, strong correlation existed between FISH and HAL, as well as between both tools of physical examination.
Conclusion: the condition of joints in Lithuanian pediatric patients with hemophilia is satisfactory. The joints of adults are more affected, and the damage progresses more quickly. The role of physical activity is beneficial, but needs to be investigated further.
Keywords: hemophilia, bleeding, joint damage, hemarthrosis, prophylaxis, on-demand treatment