Health status and health-related quality of life of children with haemophilia from six West European countries.
A multicentre, international, cross-sectional study was carried out in the frame of field testing of the first haemophilia-specific quality-of-life (QoL) questionnaire (Haemo-QoL). The aim of this paper is to describe health status and health care and their impact on QoL in haemophilic children in Western Europe. Children aged 4-16 years with severe haemophilia without inhibitors were enrolled by 20 centres in France, Germany, Italy, the Netherlands, Spain and the United Kingdom. Clinical information was collected by the physicians with a medical documentation form. Health-related QoL (HRQoL) of children was assessed with Haemo-QoL, available for three age groups. Clinical data were available in 318 patients, 85.5% with haemophilia A. The mean age at first bleeding was 11 months, at first joint bleed 25 months. Functional joint impairments were found in 11.3%. Prophylaxis treatment was given to 66.7% of children in whom breakthrough bleeds occurred 0.4 times a month compared to 1.1 bleeds in children receiving on-demand treatment. A significantly higher factor consumption was found only in the two younger age groups of prophylaxis patients compared to on-demand patients. HRQoL was satisfactory in this cohort: young children were impaired mainly in the dimension 'family' and 'treatment', whereas older children had higher impairments in the so-called 'social' dimensions, such as 'perceived support' and 'friends'. Health care of children in Western Europe is progressively improving with a large diffusion of home treatment and prophylaxis. This provides a high level of health status and HRQoL, being better in haemophilic adolescents on prophylaxis.
Gringeri
A
A
Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Department of Internal Medicine, IRCCS Maggiore Hospital and University of Milan, Milan, Italy. alessandro.gringeri@unimi.it
von Mackensen
S
S
Auerswald
G
G
Bullinger
M
M
Perez Garrido
R
R
Kellermann
E
E
Khair
K
K
Lenk
H
H
Vicariot
M
M
Villar
A
A
Wermes
C
C
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D003430:Cross-Sectional Studies; D005060:Europe; D006304:Health Status; D006467:Hemophilia A / Q000517:prevention & control / Q000523:psychology*; D006801:Humans; D007223:Infant; D011788:Quality of Life