Functional and joint evaluation in a prospective cohort of patients with severe haemophilia.

Abstract

Arthropathy is the main morbidity of haemophilia. Periodic joint health assessment enables a better understanding of the limitations of these patients. To evaluate the functional and joint performance in patients with haemophilia at one-year follow-up, as well as its association with prophylactic treatment and attendance at scheduled consultations at a haemophilia treatment centre. This prospective cohort study included patients with severe haemophilia aged 7years or more and treated at Fundação Hemocentro de Brasília, Brazil, from January 2014 to December 2018. The Hemophilia Joint Health Score and Functional Independence Score in Hemophilia were assessed at the first consultation and after a one-year follow-up. The study included 69 patients. The mean age at study recruitment was 22.5±4.5years, 62.3% of patients aged 18years or older, and 29 patients were receiving primary prophylaxis (38.0%). There was a positive correlation between HJHS and age and a negative correlation between FISH and age. The worsening HJHS was associated with non-primary prophylaxis and non-attendance at scheduled multidisciplinary consultations. The worsening FISH was associated with non-primary prophylaxis. The correlation between FISH and treatment adherence was significant for the delta. The older the patient with haemophilia, the higher the probability of a worsening of the HJHS. In the presence of more arthropathies, the older the patient, the worse the FISH. Patients receiving primary prophylaxis show better results in the HJHS and FISH when compared to patients receiving secondary prophylaxis and/or on-demand treatment.