ORIGINAL ARTICLE
Low dose prophylaxis in Tunisian children with
haemophilia
E. GOUIDER,* † L. JOUINI, † M. ACHOUR,* † H. ELMAHMOUDI, † K. ZAHRA,* † W. SAIED* ‡
and B. MEDDEB* †
*Hemophilia Center Aziza Othmana Hospital; †Tunis Medical University of Tunis, UR14ES11; and ‡Orthopedy Department,
Children Hospital Bechir Hamza, Tunis, Tunisia
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
Introduction
Prophylaxis is the gold standard treatment for haemo-
philia [1]. It prevents bleeding and consequently joint
disease [2,3]. It was initiated since the sixties [4–6].
Different protocols are used, with different doses and
numbers of injections, and the optimal regimen
remains to be defined. In countries with limited
resources, prophylaxis regimen used should be expen-
sive, whereas initiating prophylaxis with low dose
could be recommended. We report our single centre
experience with low dose secondary or tertiary pro-
phylaxis in children.
Patients and methods
Data were extracted from patient diary and medical
files of children with haemophilia.
We include all children (<15 years) receiving low
dose prophylaxis with a regular follow up and out-
come measurement available. The age at initiation
low dose prophylaxis should be less than 15 years.
Children with inhibitor were not included in the
study.
The protocol of low dose prophylaxis used is illus-
trated in Fig. 1.
Outcome measurement was number of bleeding
before and after initiating prophylaxis, in order to
evaluate the efficiency.
We also performed once a year, evaluation of the
joint status with the haemophilia joint health score
(HJHS) version 2.2 [7] and functional independence
score in haemophilia (FISH) [8]. Quality of life with
the Haemo-QoL was performed one year after initiat-
ing prophylaxis [9].
SPSS 16 was used for statistical analysis, using the
comparison means in paired samples with P < 0.05.
Results
Fifty-one children met the inclusion criteria. Forty-two
were haemophilia A. Median age was 9 years (4–17)
and median age at initiating the low dose prophylaxis
is 5.32 years (0.64–11.44) (Fig. 2). Median follow up
is 5 years (1–9).
Number of injections per week, was once, twice and
three times for, respectively, 31%, 51% and 18% of
children with a weekly median injection dose of
31 UI kg
1
. Median dose per injection is 20 UI kg
1
(8–50). Details of median doses used according to the
Correspondence: Emna Gouider, Hemophilia Center, Aziza Oth-
mana Hospital, Place du gouvernement, 1008, Tunis, Tunisia.
Tel.: +21698341985; fax: +21671568228;
e-mail: emna.gouider@gmail.com
Accepted after revision 25 June 2016
© 2016 John Wiley & Sons Ltd 1
Haemophilia (2016), 1–5 DOI: 10.1111/hae.13048