Improvement in school absence after factor replacement in students with haemophilia in Upper Assam, India

Anupam Dutta; Dipjyoti Boruah; Taniya Sarkar Dutta; Angshuman Boruah; Bhabani Shankar Dhal

doi:10.17225/jhp00166

Abstract

Abstract Background

Patients with haemophilia who are not adequately treated experience a lifetime burden of joint complications and loss of functional ability due to repeated bleeding episodes caused by low levels of clotting factor VIII or IX in the blood. These complications can significantly impact day-to-day life, including active participation in school and academic study in children with haemophilia (CwH). Treatment with factor replacement therapy can help to prevent this, but access to factor has been challenging in low-resource settings such as Northeast India. This study shows the impact of factor replacement therapy on bleeding episodes, joint complications and school absence among CwH in this setting.

Methods

A retrospective observational study was undertaken to examine the impact of receiving regular factor replacement therapy (prophylaxis or on demand) on school absences among CwH registered with the haemophilia treatment centre at Assam Medical College and Hospital. Annual bleed rate (ABR), Haemophilia Joint Health Score (HJHS) and Functional Independence Score in Haemophilia (FISH) were also assessed.

Results

Thirty-eight CwH were eligible for the study; 26 (68.4%) were on prophylaxis therapy and 12 (31.5%) received on-demand therapy. In the year before starting regular treatment, the mean ABR was 37.8 (+20.0), HJHS was 31.1 (+18.1) and mean FISH score was 21.1 (+4.2). At the end of the study period the mean (+SD) ABR in prophylactic therapy was significantly lower at 5.8 (+4.6) (p<0.001) and the HJHS was significantly lower at 4.7 (+4.6). FISH score significantly improved to 27.9 (+3.3) (p<0.001). Prophylaxis showed better (but not significant) results in comparison to on-demand therapy.

Conclusions

Treatment with factor replacement significantly reduces school absence in CwH and correlates strongly with joint health and functional improvement, with the effect slightly better with prophylaxis than on-demand therapy. Low dose prophylaxis is a good treatment option in low-resource settings, but improvements are also needed in rates of diagnosis.

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Authors

Anupam Dutta

dranupamdutta80@gmail.com

Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India .
Dipjyoti Boruah

Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India
Taniya Sarkar Dutta

Department of Paediatrics, Assam Medical College and Hospital, Dibrugarh, India
Angshuman Boruah

Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India
Bhabani Shankar Dhal

Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India