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Expert United Kingdom consensus on the preservation of joint health in people with moderate and severe haemophilia A: A modified Delphi panel.
Laffan, Mike; McLaughlin, Paul; Motwani, Jayashree; Alamelu, Jayanthi; Austin, Steve; Classey, Stephen; Dolan, Gerard; Eales, Marie; Gooding, Richard; Grainger, John; Harrison, Catherine; Jones, April; Kelly, Anne M; Oyesiku, Lara; Rodgers, Ryan; Stephensen, David; Talks, Kate; Sonecha, Shaneil; Danquah, Andrew.
Afiliação
  • Laffan M; Centre for Haematology, Imperial College London, London, UK.
  • McLaughlin P; Katharine Dormandy Haemophilia Centre and Thrombosis Unit, Royal Free London NHS Foundation Trust, London, UK.
  • Motwani J; Department of Paediatric Haematology, Birmingham Children's Hospital, Birmingham, UK.
  • Alamelu J; Paediatric Haematology, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Austin S; Department of Haematology, St George's University Hospitals NHS Foundation Trust, London, UK.
  • Classey S; Centre for Haemostasis and Thrombosis, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Dolan G; Centre for Haemostasis and Thrombosis, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Eales M; The Children's Team at Oxford Haemophilia and Thrombosis Comprehensive Care Centre, Oxford University Hospitals NHS Trust, Oxford, UK.
  • Gooding R; Haematology Department, Belfast Health and Social Care Trust, Belfast, UK.
  • Grainger J; Paediatric Haematology, Royal Manchester Children's Hospital, Manchester, UK.
  • Harrison C; Sheffield Haemophilia & Thrombosis Centre, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.
  • Jones A; Newcastle Haemophilia Centre, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
  • Kelly AM; Haemophilia Comprehensive Care Centre, Great Ormond Street Hospital, London, UK.
  • Oyesiku L; Southern Haemophilia Network, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Basingstoke, Hampshire, UK.
  • Rodgers R; Department of Haematology, Glasgow Royal Infirmary, Glasgow, UK.
  • Stephensen D; Kent Haemophilia Centre, Kent and Canterbury Hospital, Canterbury, Kent, UK.
  • Talks K; Newcastle Haemophilia Centre, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
  • Sonecha S; Swedish Orphan Biovitrum Ltd, Great Abington, UK.
  • Danquah A; Swedish Orphan Biovitrum Ltd, Great Abington, UK.
Haemophilia ; 30(2): 306-319, 2024 Mar.
Article em En | MEDLINE | ID: mdl-38239180
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Hemofilia A Tipo de estudo: Guideline Limite: Humans País/Região como assunto: Europa Idioma: En Revista: Haemophilia Assunto da revista: HEMATOLOGIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Reino Unido

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Hemofilia A Tipo de estudo: Guideline Limite: Humans País/Região como assunto: Europa Idioma: En Revista: Haemophilia Assunto da revista: HEMATOLOGIA Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Reino Unido