Expert United Kingdom consensus on the preservation of joint health in people with moderate and severe haemophilia A: A modified Delphi panel
- PMID: 38239180
- DOI: 10.1111/hae.14934
Expert United Kingdom consensus on the preservation of joint health in people with moderate and severe haemophilia A: A modified Delphi panel
Abstract
Aim: For people with haemophilia A (PwHA), bleeding in the joints leads to joint damage and haemophilia-related arthropathy, impacting range of motion and life expectancy. Existing guidelines for managing haemophilia A support healthcare professionals (HCPs) and PwHA in their efforts to preserve joint health. However, such guidance should be reviewed, considering emerging evidence and consensus as presented in this manuscript.
Methods: Fifteen HCPs experienced in the management of PwHA in the UK participated in a three-round Delphi panel. Consensus was defined at ≥70% of panellists agreeing or disagreeing for Likert-scale questions, and ≥70% selecting the same option for multiple- or single-choice questions. Questions not reaching consensus were revised for the next round.
Results: 26.8% (11/41), 44.8% (13/29) and 93.3% (14/15) of statements reached consensus in Rounds 1, 2 and 3, respectively. HCPs agreed that prophylaxis should be offered to patients with a baseline factor VIII (FVIII) level of ≤5 IU/dL and that, where there is no treatment burden, the aim of prophylaxis should be to achieve a trough FVIII level ≥15 IU/dL and maintain a longer period with FVIII levels of ≥20-30 IU/dL to provide better bleed protection. The aspirational goal for PwHA is to prevent all joint bleeds, which may be achieved by maintaining normalised (50-150 IU/dL) FVIII levels.
Conclusion: The panel of experts were largely aligned on approaches to preserving joint health in PwHA, and this consensus may help guide HCPs.
Keywords: Delphi method; United Kingdom; haemophilia A; joint pain; joints.
© 2024 The Authors. Haemophilia published by John Wiley & Sons Ltd.
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References
REFERENCES
-
- Benson G, Auerswald G, Dolan G, et al. Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management. Blood Transfus. 2018;16(6):535-544.
-
- Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia. Haemophilia. 2020;26:1-158.
-
- Simpson ML, Valentino LA. Management of joint bleeding in hemophilia. Expert Rev Hematol. 2012;5(4):459-468.
-
- World Federation of Hemophilia. Chronic hemophilic synovitis: the role of radiosynovectomy 2004. https://www1.wfh.org/publication/files/pdf-1176.pdf
-
- Mancuso ME, Holstein K, O‘Donnell JS, et al. Synovitis and joint health in patients with haemophilia: statements from a European e-Delphi consensus study. Haemophilia. 2023;29(2):619-628.
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