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Impact of von Willebrand disease on health‐related quality of life in a pediatric population
Author(s) -
DE WEE E. M.,
FIJNVANDRAAT K.,
DE GOEDEBOLDER A.,
MAUSERBUNSCHOTEN E. P.,
EIKENBOOM J. C. J.,
BRONS P. P.,
SMIERS F. J.,
TAMMINGA R.,
OOSTENBRINK R.,
RAAT H.,
VAN DER BOM J. G.,
LEEBEEK F. W. G.
Publication year - 2011
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/j.1538-7836.2010.04175.x
Subject(s) - toddler , medicine , von willebrand disease , quality of life (healthcare) , population , pediatrics , disease , psychology , von willebrand factor , developmental psychology , platelet , nursing , environmental health
Summary. Background: Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder. Whether VWD is associated with health‐related quality of life (HR‐QoL) in children is unknown. Objectives: This nationwide cross‐sectional study measured HR‐QoL in children with moderate or severe VWD. Our primary aim was to compare HR‐QoL of VWD patients with that of reference populations. Additionally, we studied the impact of bleeding phenotype and VWD type on HR‐QoL. Methods: HR‐QoL was assessed with the Infant/Toddler QoL Questionnaire (0–5 years) and Child Health Questionnaire (6–15 years), and compared with reference population scores. Multivariate analysis was used to evaluate the influence of type of VWD and bleeding phenotype on HR‐QoL scores. Results: Preschool children (0–5 years, n = 46) with VWD had lower HR‐QoL scores for general health perceptions and parental time than reference populations. School children (6–15 years, n = 87) with VWD had lower scores for physical functioning, role functioning – emotional/behavioral, general health perceptions, and physical summary. Type of VWD was associated with HR‐QoL in school children for bodily pain, general health perceptions, parental emotion, family activities, and physical summary. Scores of children with type 3 VWD were, on average, 15 points lower than those of the reference population on the above‐mentioned scales. A more severe bleeding phenotype was associated with a lower score on 11/15 physical, emotional and social scales. Conclusion: HR‐QoL is lower in VWD children than in reference populations, in particular in school children. The negative impact of VWD is sensitive to type of VWD and bleeding phenotype; as well as physical scales, emotional and social scales are affected.