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

Abstract

Introduction Arthropathy is the main morbidity of haemophilia. Periodic joint health assessment enables a better understanding of the limitations of these patients. Objective 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. Methods This prospective cohort study included patients with severe haemophilia aged 7 years 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. Results The study included 69 patients. The mean age at study recruitment was 22.5 ± 4.5 years, 62.3% of patients aged 18 years 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. Conclusion 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.

Demographics, clinical profile and treatment landscape of patients with haemophilia B in China

“…8 Prophylaxis is of great significance to prevent joint bleeding and deformity to reduce the burden of disease, and to improve the quality of life. 9 The proportion of severe HB patient on prophylaxis was noticeably higher in Europe (58%), Australia (75%), the United States (74.8%)…”

Joint status, pain and quality of life in elderly people with haemophilia: A case‐control study

“…Expectedly, poorer joint outcomes were observed in this subgroup when compared to healthy controls and mild PwH, in both statistical analyses. Such severe joint abnormalities are most probably related to late prophylaxis initiation and haemophilic arthropathy progression 24 …”

“…Such severe joint abnormalities are most probably related to late prophylaxis initiation and haemophilic arthropathy progression. 24 In mild PwH, abnormalities on ultrasound were observed in 50% of knees and 61% of ankles. These percentages seem higher than those of De la Corte-Rodriguez et al 25 who reported 36% and 43%, as well as than those of Alvarez Roman et al 26 reporting 29−31% and 36−43%, both respectively for knee and ankle abnormalities.…”

Altered neural control of gait and its association with pain and joint impairment in adults with haemophilic arthropathy: Clinical and methodological implications

“…In addition, the total score on the HJHS (0–124 points) was included. At the joint level, the HJHS has a good correlation with X‐rays (Pettersson score), and the total HJHS has a moderate correlation with self‐reported functions 27,28 . Furthermore, the knee flexion contracture (i.e.…”

“…At the joint level, the HJHS has a good correlation with X-rays (Pettersson score), and the total HJHS has a moderate correlation with selfreported functions. 27,28 Furthermore, the knee flexion contracture (i.e. loss of joint extension) was assessed to measure knee joint deformity.…”