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Impact of prophylaxis on health-related quality of life of boys with hemophilia: An analysis of pooled data from 9 countries.

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机构： [1]Child Health Evaluative Sciences Program, Research Institute, The Hospital for Sick Children (SickKids), Toronto, Ontario, Canada [2]Evaluating Children's Health Outcomes Research Centre, Laurentian University, Sudbury, Ontario, Canada [3]Division of Pediatric Hematology/Oncology, Department of Pediatrics, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada [4]Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children (SickKids), University of Toronto, Toronto, Ontario, Canada [5]Unitat Hemofilia Hospital Vall d'Hebron, Barcelona, Spain [6]Department of Pathology, University of the West Indies (UWI), Mona, Jamaica [7]Centro de Hemofilia e Instituto da Crian?a, Hospital das Clínicas da Faculdade de Medicina da Universidade de S?o Paulo, Sao Paulo, Brazil [8]Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada [9]Institute of Health Policy, Management & Evaluation, the Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada [10]Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands [11]Royal Manchester Children's Hospital, Manchester, UK [12]Department of Pediatric Hematology and Oncology, Charité University Medicine, Berlin, Germany [13]Department of Pediatrics, Laboratory Medicine and Pathology, University of Ottawa, Ottawa, Ontario, Canada [14]Hemostase Clinique, Groupement Hospitalier Universitaire Est, Hospices Civils de Lyon, Bron, France [15]Unit of Hemophilia IHTC, Cláudio L.P. Correa, Hemocentro Unicamp, INCT do Sangue, University of Campinas, Campinas, Brazil [16]Hematology/Oncology Center, Beijing Children's Hospital, Capital Medical University, Beijing, China [17]Department of Hematology, Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, Brazil [18]Service of Hematology, Hospital das Clínicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil [19]Department of Nursing, The Hospital for Sick Children (SickKids), Toronto, Ontario, Canada [20]School of Rural and Northern Health, Laurentian University, Sudbury, Ontario, Canada

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Prophylaxis reduces the frequency of bleeds in boys with severe hemophilia and is the standard care for their management in resource-abundant countries. The effect of prophylaxis on Health-Related Quality of Life (HRQoL) has not been established, because the sample sizes of most studies are too small to explore the relationship of multiple factors that influence HRQoL. The aim of this study was to assess the impact of hemophilia severity and treatment regimen on HRQoL and to establish the minimum important difference (MID) using the international level of score distributions. HRQoL data were pooled from 7 studies across 9 countries. HRQoL was measured using the Canadian Hemophilia Outcomes-Kids' Life Assessment Tool (CHO-KLAT). A mixed-effect linear regression analysis was employed to assess the impact of prophylaxis on the CHO-KLAT score. Data from 401 boys with hemophilia were analyzed (57.6% severe hemophilia and 57.6% receiving prophylaxis). The model revealed that receiving prophylaxis was significantly associated with higher HRQoL (regression coefficient 8.5, 95% confidence interval [CI] 3.9-13.1). Boys with severe hemophilia had a significantly lower HRQoL as compared to boys with moderate and mild hemophilia whose CHO-KLAT scores were 7.0 and 6.6 points higher, respectively. There was a significant interaction between treatment and disease severity (P = 0.023), indicating prophylaxis has the most significant impact in boys with severe hemophilia. Based on these pooled data, the MID of the CHO-KLAT was established at 6.5. This study confirms the positive effect of prophylaxis on HRQoL in boys with hemophilia in a real-world setting and provides initial benchmarks for interpreting HRQoL scores based on use of the CHO-KLAT instrument.

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大类 | 3 区医学

小类 | 3 区血液学 3 区外周血管病

第一作者：

第一作者机构： [1]Child Health Evaluative Sciences Program, Research Institute, The Hospital for Sick Children (SickKids), Toronto, Ontario, Canada [2]Evaluating Children's Health Outcomes Research Centre, Laurentian University, Sudbury, Ontario, Canada

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通讯机构： [3]Division of Pediatric Hematology/Oncology, Department of Pediatrics, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada [*1]Division of Pediatric Hematology/Oncology, Department of Pediatrics, IWK Health Centre, Dalhousie University, Halifax, NS, Canada.

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