Health-related quality of life and physical activity in Nordic patients with moderate haemophilia A and B (the MoHem study).

arthropathy haemophilia A haemophilia B health-related quality of life physical activity prophylaxis

Journal

Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916

Informations de publication

Date de publication:
20 Nov 2023
Historique:
revised: 12 09 2023
received: 22 06 2023
accepted: 04 11 2023
medline: 20 11 2023
pubmed: 20 11 2023
entrez: 20 11 2023
Statut: aheadofprint

Résumé

The impact of moderate haemophilia on health-related quality of life (HRQoL) and physical activity (PA) is not well known. In previous studies, persons with factor VIII/factor IX activity (FVIII/FIX:C) below 3 IU/dL were associated with a more severe bleeding phenotype than predicted. To explore HRQoL and PA in patients with moderate haemophilia A (MHA) and B (MHB). A cross-sectional, multicentre study covering patients with MHA and MHB in Sweden, Finland, and Norway. HRQoL was assessed with the EuroQoL 5-Dimensions (EQ-5D) form and PA with the International Physical Activity Questionnaire among participants aged ≥15 years. We report on 104 patients aged 15-84 years from the MoHem study. Overall, EQ-5D utility was .85 (median) (Q1-Q3 0.73-1.0) with corresponding visual analogue scale (VAS) 80 (70-90), which were similar regardless of treatment modality, FVIII/FIX:C, and MHA or MHB. Pain and mobility were most frequently affected dimensions. Utility (r = -.54), VAS (r = -.42), and PA (r = -.32) correlated negatively with arthropathy (HJHS). Only patients aged 41-50 years displayed lower utility (p = .02) and VAS (p < .01) than the Norwegian population norm. Patients on prophylaxis aged 35-54 years reported higher PA than those treated on-demand (p = .01). Haemophilic arthropathy had negative impact on HRQoL and PA in Nordic patients with moderate haemophilia. Middle-aged patients captured lower utility and VAS than observed in the general population. Tailored prophylaxis and improved joint health may influence positively on HRQoL and PA also in moderate haemophilia.

Identifiants

pubmed: 37983883
doi: 10.1111/hae.14899
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Bayer Health Care

Informations de copyright

© 2023 The Authors. Haemophilia published by John Wiley & Sons Ltd.

Références

Fischer K, Bom JG, Mauser-Bunschoten EP, Roosendaal G, Berg HM. Effects of haemophilic arthropathy on health-related quality of life and socio-economic parameters. Haemophilia. 2005;11(1):43-48.
Goddard NJ, Mann HA, Lee CA. Total knee replacement in patients with end-stage haemophilic arthropathy: 25-year results. J Bone Joint Surg Br. 2010;92(8):1085-1089.
Fischer K, de Kleijn P, Negrier C, et al. The association of haemophilic arthropathy with health-related quality of life: a post hoc analysis. Haemophilia. 2016;22(6):833-840.
Osooli M, Steen Carlsson K, Baghaei F, et al. The association between health utility and joint status among people with severe haemophilia A: findings from the KAPPA register. Haemophilia. 2017;23(3):e180-e187.
Nilsson IM, Berntorp E, Lofqvist T, Pettersson H. Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B. J Intern Med. 1992;232(1):25-32.
Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med. 2007;357(6):535-544.
Noone D, O'Mahony B, Prihodova L. A survey of the outcome of prophylaxis, on-demand or combined treatment in 20-35 year old men with severe haemophilia in four European countries. Haemophilia. 2011;17(5):e842-843.
Noone D, O'Mahony B, van Dijk JP, Prihodova L. A survey of the outcome of prophylaxis, on-demand treatment or combined treatment in 18-35-year old men with severe haemophilia in six countries. Haemophilia. 2013;19(1):44-50.
Lindvall K, Von Mackensen S, Berntorp E. Quality of life in adult patients with haemophilia-a single centre experience from Sweden. Haemophilia. 2012;18(4):527-531.
Aledort LM, Haschmeyer RH, Pettersson H. A longitudinal study of orthopaedic outcomes for severe factor-VIII-deficient haemophiliacs. The orthopaedic outcome study group. J Intern Med. 1994;236(4):391-399.
Steen Carlsson K, Hojgard S, Glomstein A, et al. On-demand vs. prophylactic treatment for severe haemophilia in Norway and Sweden: differences in treatment characteristics and outcome. Haemophilia. 2003;9(5):555-566.
Fischer K, Steen Carlsson K, Petrini P, et al. Intermediate-dose versus high-dose prophylaxis for severe hemophilia: comparing outcome and costs since the 1970s. Blood. 2013;122(7):1129-1136.
Versloot O, Berntorp E, Petrini P, et al. Sports participation and physical activity in adult Dutch and Swedish patients with severe haemophilia: a comparison between intermediate- and high-dose prophylaxis. Haemophilia. 2019;25(2):244-251.
O'Hara J, Noone D, Jain M, et al. Clinical attributes and treatment characteristics are associated with work productivity and activity impairment in people with severe haemophilia A. Haemophilia. 2021;27(6):938-946.
den Uijl I, Biesma D, Grobbee D, Fischer K. Outcome in moderate haemophilia. Blood Transfus. 2014;12(1):s330-s336.
Måseide RJ, Berntorp E, Astermark J, et al. Joint health and treatment modalities in Nordic patients with moderate haemophilia A and B-The MoHem study. Haemophilia. 2020;26(5):891-897.
Buckner TW, Witkop M, Guelcher C, et al. Impact of hemophilia B on quality of life in affected men, women, and caregivers-assessment of patient-reported outcomes in the B-HERO-S study. Eur J Haematol. 2018;100(6):592-602.
den Uijl I, Biesma D, Grobbee D, Fischer K. Turning severe into moderate haemophilia by prophylaxis: are we reaching our goal? Blood Transfus. 2013;11(3):364-369.
Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of Hemophilia, 3. Haemophilia. 2020;26(6):1-158.
Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost. 2014;12(11):1935-1939.
EuroQoL. EQ 5-Dimensions (EQ-5D). https://euroqol.org/
Buckner TW, Wang M, Cooper DL, Iyer NN, Kempton CL. Known-group validity of patient-reported outcome instruments and hemophilia joint health score v2.1 in US adults with hemophilia: results from the pain, functional impairment, and quality of life (P-FiQ) study. Patient Prefer Adherence. 2017;11:1745-1753.
Kempton CL, Wang M, Recht M, et al. Reliability of patient-reported outcome instruments in US adults with hemophilia: the Pain, Functional Impairment and Quality of life (P-FiQ) study. Patient Prefer Adherence. 2017;11:1603-1612.
Kind P, Hardman G, Macran S. UK Population Norms for EQ-5D. 1999; https://www.york.ac.uk/che/pdf/DP172.pdf
Stavem K, Augestad LA, Kristiansen IS, Rand K. General population norms for the EQ-5D-3 L in Norway: comparison of postal and web surveys. Health Qual Life Outcomes. 2018;16(1):204.
Craig CL, Marshall AL, Sjostrom M, et al. International physical activity questionnaire: 12-country reliability and validity. Med Sci Sports Exerc. 2003;35(8):1381-1395.
Hilliard P, Funk S, Zourikian N, et al. Hemophilia joint health score reliability study. Haemophilia. 2006;12(5):518-525.
Feldman BM, Funk SM, Bergstrom BM, et al. Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: validity of the hemophilia joint health score. Arthritis Care Res (Hoboken). 2011;63(2):223-230.
Martinoli C, Della Casa Alberighi O, Di Minno G, et al. Development and definition of a simplified scanning procedure and scoring method for Haemophilia early arthropathy detection with ultrasound (HEAD-US). Thromb Haemost. 2013;109(6):1170-1179.
Kihlberg K, Baghaei F, Bruzelius M, et al. No difference in quality of life between persons with severe haemophilia A and B. Haemophilia. 2023;29(4):987-996.
Posthouwer D, Plug I, van der Bom JG, Fischer K, Rosendaal FR, Mauser-Bunschoten EP. Hepatitis C and health-related quality of life among patients with hemophilia. Haematologica. 2005;90(6):846-850.

Auteurs

Ragnhild J Måseide (RJ)

Department of Haematology, Oslo University Hospital, Oslo, Norway.
Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

Erik Berntorp (E)

Department of Translational Medicine, Lund University, Malmö, Sweden.

Jan Astermark (J)

Department of Translational Medicine, Lund University, Malmö, Sweden.
Department of Haematology, Skåne University Hospital, Malmö, Sweden.

Anna Olsson (A)

Department of Medicine, Region Västra Götaland, Sahlgrenska University Hospital, Gothenburg, Sweden.

Maria Bruzelius (M)

Molecular Medicine and Surgery, Karolinska Institute, Stockholm, Sweden.
Department of Haematology, Karolinska University Hospital, Stockholm, Sweden.

Tony Frisk (T)

Pediatric Coagulation, Karolinska University Hospital, Stockholm, Sweden.

Vuokko Nummi (V)

Coagulation Disorders Unit, Haematology, Comprehensive Cancer Centre, Helsinki University Hospital and Research Program in Systems Oncology, Faculty of Medicine, Helsinki University, Helsinki, Finland.

Riitta Lassila (R)

Coagulation Disorders Unit, Haematology, Comprehensive Cancer Centre, Helsinki University Hospital and Research Program in Systems Oncology, Faculty of Medicine, Helsinki University, Helsinki, Finland.

Geir E Tjønnfjord (GE)

Department of Haematology, Oslo University Hospital, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

Pål A Holme (PA)

Department of Haematology, Oslo University Hospital, Oslo, Norway.
Research Institute of Internal Medicine, Oslo University Hospital, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

Classifications MeSH