Effect of intracranial bleeds on the health and quality of life of boys with hemophilia

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Abstract

Objectives

To compare the health, physical function, and quality of life (QoL) of boys with hemophilia with and without a history of intracranial hemorrhage (ICH).

Study design

Of 172 patients with hemophilia A or B, 18 (10%) had at least one episode of ICH. For outcome assessments, 16 of 18 (89%) boys with ICH and 32 controls, matched (1:2) for age and severity of hemophilia, were available. The outcome measures were neurologic function, physical function, and QoL.

Results

The median age of the boys at the first ICH was 5.9 months (range, 1 day to 2.7 years). Boys with ICH had a higher incidence of inhibitors and lower mean household income. Neurologic examination was abnormal in seven of 16 (44%) boys with ICH and nine of 32 (28%) controls (P = .3). The mean physical function in boys with ICH was lower (82%±25%) compared with controls (93.5%±12%, P = .045). The QoL was decreased in boys with ICH compared with controls (6.8±3.2 vs 8.5±1.4, P = .02), whereas health-related QoL was not significantly different between groups.

Conclusion

The poorer long-term outcomes of boys with hemophilia appropriately treated for ICH, especially in the domain of QoL, suggest that new strategies to prevent ICH and to manage ICH effectively in this population are needed.

Section snippets

Study design

We studied an inception cohort from the Hospital for Sick Children Pediatric Comprehensive Care Hemophilia Center. This study was approved by the Hospital for Sick Children Research Ethics Board, and informed consent was obtained from all study subjects' parents and all study subjects able to consent. Subjects were recalled for on-site testing at the Hospital for Sick Children, and all examiners were blinded with respect to participants' ICH history.

Study population

Eligible subjects were identified by a review

Study population

Of 172 patients with either hemophilia A or B, ranging in severity from mild to severe, 18 (10%) had at least one episode of ICH (Table I). Sixteen boys with hemophilia and a history of at least one ICH (total, 22 ICH episodes), and 32 matched (1:2) controls participated in this outcome study. Two boys with hemophilia and a history of ICH did not consent to participate in the study (Table I, subjects 17 and 18). The reasons for refusal were parental refusal in one boy and transition to another

Discussion

We found that boys with hemophilia and ICH have a worse clinical outcome, especially in the domain of QoL, than other boys with hemophilia. The long-term outcomes in an unselected population of boys with hemophilia and ICH were assessed through the use of multiple standardized tests, and the effect of having hemophilia was controlled by using boys with hemophilia as matched controls. Boys with ICH differed from the control group in three demographic variables: higher percentage of inhibitors,

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    Supported by the Aventis Behring Canada competition for hemophilia/von Willebrand disease bleeding disorders. Dr Revel-Vilk is a recipient of the Baxter Bioscience Pediatric Hemostasis Fellowship at the Hospital for Sick Children, Toronto, Canada (2001-2002). Dr Feldman holds a Canada Research Chair in Childhood Arthritis.

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