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No difference in quality of life between persons with severe haemophilia A and B |
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| Authors: | Kristina Kihlberg Fariba Baghaei Maria Bruzelius Eva Funding Pål Andre Holme Riitta Lassila Vuokko Nummi Susanna Ranta Nadine Gretenkort Andersson Erik Berntorp Jan Astermark |
| Affiliation: | 1. Clinical Coagulation Research, Department of Translational Medicine, Lund University, Malmö, Sweden;2. Department of Haematology and Coagulation, Sahlgrenska University Hospital, Gothenburg, Sweden Department of Internal Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden;3. Department of Haematology, Karolinska University Hospital, Stockholm, Sweden Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden;4. Department of Hematology, Rigshospitalet, Copenhagen, Denmark;5. Department of Haematology, Oslo University Hospital, Oslo, Norway Institute of Clinical Medicine, University of Oslo, Oslo, Norway;6. Department of Haematology, Coagulation Disorders Unit, Comprehensive Cancer Centre, Helsinki University Hospital, Helsinki, Finland;7. Paediatric Coagulation, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden;8. Department of Haematology, Oncology and Radiation Physics, Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden |
| Abstract: | IntroductionGood health-related quality of life (HRQoL) is an important goal in the treatment of persons with haemophilia B (PwHB). Studies focusing on this population are limited, however, and data are insufficient.AimTo assess the HRQoL in PwHB and to compare this to data on persons with haemophilia A (PwHA), as well as to evaluate the impact of joint health on HRQoL and to identify areas of insufficient care.MethodsThe B-NORD study enrolled persons with severe haemophilia B and matched controls with haemophilia A. HRQoL was assessed using the EQ-5D-3L questionnaire and joint health using Haemophilia Joint Health Score 2.1 (HJHS).ResultsThe EQ-5D-3L was completed by 63 PwHB and 63 PwHA. Mobility problems were reported by 46% of PwHB and 44% of PwHA, pain/discomfort by 62% and 56%, and anxiety/depression by 33% and 17%, respectively. No significant difference was observed between PwHA and PwHB in EQ-5D profiles, level sum score, EQ-5D index (PwHB mean .80, PwHA mean .83, p = .24), or EQ VAS score (PwHB: mean 70, PwHA: mean 77, p = .061). Linear regression adjusted for age demonstrated that an increase in HJHS score was associated with a significant decrease in both EQ-5D index (B -.003, R2 .22) and EQ VAS score (B -.37, R2 .17).ConclusionDespite the majority of patients being treated with prophylaxis, impaired HRQoL was reported in both PwHB and PwHA. No differences in HRQoL were found between the two groups. Impaired joint health had a significant negative impact on HRQoL. |
| Keywords: | arthropathy coagulation factor IX EQ-5D haemophilia A haemophilia B quality of life |