Low dose prophylaxis in Tunisian children with haemophilia

E Gouider; L Jouini; M Achour; H Elmahmoudi; K Zahra; W Saied; B Meddeb

doi:10.1111/hae.13048

Low dose prophylaxis in Tunisian children with haemophilia

Haemophilia. 2017 Jan;23(1):77-81. doi: 10.1111/hae.13048. Epub 2016 Dec 11.

Authors

E Gouider^{1

2}, L Jouini², M Achour^{1

2}, H Elmahmoudi², K Zahra^{1

2}, W Saied^{1

3}, B Meddeb^{1

2}

Affiliations

¹ Hemophilia Center Aziza Othmana Hospital, Tunis, Tunisia.
² Tunis Medical University of Tunis, UR14ES11, Tunis, Tunisia.
³ Orthopedy Department, Children Hospital Bechir Hamza, Tunis, Tunisia.

PMID: 27943510
DOI: 10.1111/hae.13048

Abstract

Introduction: Low dose prophylaxis could be recommended in countries with limited resources.

Aim: We report our single centre experience in children with haemophilia.

Patients: Fifty-five children were included in our study with a weekly median dose of 30 UI kg^-1 given once, twice or thrice a week. Age of initiation of prophylaxis is 5.32 years (0.64-11.44). Outcome assessment used were number of bleeding before and after initiating prophylaxis, haemophilia joint health score (HJHS), functional independence score in haemophilia (FISH) and quality of life with the Haemo-QoL.

Results: Reduction of number of bleeding was clear in all patients; HJHS, FISH and Haemo-QOL were satisfactory.

Conclusion: Low dose prophylaxis is effective and better than on-demand therapy. It should be the starting point for prophylaxis in countries with limited resources.

Keywords: functional independence score in haemophilia; haemophilia; haemophilia joint health score; low dose prophylaxis; prophylaxis.

MeSH terms

Adolescent
Child
Female
Hemophilia A / drug therapy*
Humans
Male
Tunisia