Subjective Physical Performance and Its Determinants in Patients With Haemophilia

IF 3 2区医学 Q2 HEMATOLOGY

Haemophilia Pub Date : 2025-03-28 DOI:10.1111/hae.70037

Alexander Schmidt, Fabian Tomschi, Pia Möllers, Marius Brühl, Sylvia von Mackensen, Andreas C. Strauss, Heinrich Richter, Johannes Oldenburg, Thomas Hilberg

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引用次数: 0

Abstract

Introduction

Physical functioning is compromised in patients with haemophilia (PwH). However, factors negatively influencing subjective physical performance (SPP) remain underexplored. Hence, this study aimed to compare the SPP of PwH with healthy controls (CON), to differentiate them based on disease-specific, person-related, and arthropathy-related parameters, and to identify overarching determinants influencing SPP.

Methods

SPP was assessed in 301 PwH and 263 CON via the HEP-Test-Q, which divides SPP into a total score and four distinct dimensions (e.g., mobility). Additionally, disease-specific (i.e., type, severity, treatment regime, HIV, hepatitis), person-related (i.e., age, BMI), and arthropathy-related parameters (i.e., current pain intensity (NRS-now) and average pain intensity across 4 weeks (NRS-4w) and the HJHS) were examined, and associations with SPP were calculated.

Results

All PwH and PwH subgroups demonstrated significantly greater impairment across all SPP dimensions compared to CON. Apart from the type of haemophilia (A vs. B, p = 0.894), significant differences in total SPP were observed among PwH subgroups for severity (severe vs. non-severe, p = 0.012), treatment (prophylaxis vs. on demand, p = 0.002), HIV (no vs. yes), and hepatitis (no vs. yes, both p < 0.001). A multiple linear regression model further revealed significant predictive effects for HJHS (p < 0.001) and NRS-4w (p < 0.001) on the total SPP score.

Conclusion

PwH perceived their physical performance as significantly worse across all dimensions compared to CON. The decreased SPP in PwH can be attributed primarily to arthropathy-related factors, that is, an impaired joint status and persistent pain. To oppose the decline in SPP, tailored sports-therapeutic programs should be integrated into the multimodal treatment concept.

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血友病患者的主观体能表现及其决定因素。

血友病（PwH）患者的身体功能受损。然而，影响主观体能表现（SPP）的负面因素仍未得到充分研究。因此，本研究旨在比较PwH与健康对照（CON）的SPP，根据疾病特异性、个人相关和关节病相关参数对其进行区分，并确定影响SPP的主要因素。方法：通过HEP-Test-Q评估301名PwH和263名CON患者的SPP，该测试将SPP分为总分和四个不同的维度（如活动能力）。此外，还检查了疾病特异性（即类型、严重程度、治疗方案、HIV、肝炎）、个人相关（即年龄、BMI）和关节病相关参数（即当前疼痛强度（NRS-now）和4周平均疼痛强度（NRS-4w）和HJHS），并计算了与SPP的关联。结果：与con相比，所有PwH和PwH亚组在所有SPP维度上都表现出明显更大的损害。除了血友病类型（A对B， p = 0.894）外，PwH亚组在严重程度（严重vs非严重，p = 0.012）、治疗（预防vs.按需治疗，p = 0.002）、HIV（否vs.有）和肝炎（否vs.有，p < 0.001）方面观察到总SPP的显著差异。多元线性回归模型进一步显示HJHS （p < 0.001）和NRS-4w （p < 0.001）对SPP总分有显著的预测作用。结论：与对照组相比，PwH认为他们的身体表现在所有方面都明显更差。PwH中SPP的下降主要归因于关节病相关因素，即关节状态受损和持续疼痛。为了对抗SPP的下降，量身定制的运动治疗方案应该整合到多模式治疗概念中。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Haemophilia 医学-血液学

CiteScore

6.50

自引率

28.20%

发文量

226

审稿时长

3-6 weeks

期刊介绍： Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.