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Introduction: As a result of centralisation of haemophilia care to a limited number of intramural settings, many persons with haemophilia have to travel long distances to attend their haemophilia specialised treatment centre. However, regular physiotherapy treatment can be provided by primary care physiotherapists in the personʼs own region. Due to the rarity of the disease most primary care physiotherapists have limited experience with this population. This study aims to provide a clinical practice guideline for primary care physiotherapists working with persons with bleeding disorders. Method: A list of the most urgent key‐questions was derived from a previous study. Literature was summarised using the grading of recommendations assessment, development, and evaluation (GRADE) evidence‐to‐decision framework. Recommendations were drafted based on four 90 min consensus meetings with expert physiotherapists. Recommendations were finalised after feedback and >80% consensus of all stakeholders (including PWH, physiotherapists, haematologists and the corresponding societies). Results: A list of 82 recommendations was formulated to support primary care physiotherapists when treating a person with a bleeding disorder. These recommendations could be divided into 13 categories: two including recommendations on organisation of care, six on therapy for adult patients with bleeding disorders and five on therapy adaptations for paediatric care. Therapy recommendations included treatment after a joint‐ or muscle bleed, haemophilic arthropathy, chronic synovitis, non‐haemophilia related conditions and orthopaedic surgery. Conclusion: An evidence‐based practice guideline, based on current evidence from literature and clinical expertise, has been developed for primary care physiotherapists treating a person with haemophilia. To improve care, the recommendations should be implemented in daily practice. [ABSTRACT FROM AUTHOR]

We encourage therapists to critically examine their own practice, as well as asking centre directors and therapy managers to see the value of a well-defined physiotherapy service within haemophilia. GLO:EVW/01mar21:hae14150-fig-0001.jpg PHOTO (COLOR): 1 Recommendation for reviewing provision of a local haemophilia physiotherapy service gl It remains unclear if new treatments (EHL's, non-substitutive therapies) will considerably reduce the frequency of bleeding in patients treated. Acute pain significantly impacts quality of life and is greater when present on a backdrop of ongoing persistent pain.6 An increase in age is also associated with a decrease in quality of life predominantly related to haemophilia-specific MSK complications, that is, joint disease. [Extracted from the article]

Purpose: This study aimed to compare assessments of joint status conducted by a physiotherapist using a clinical scoring system, Haemophilia Joint Health Score (HJHS 2.1), and an ultrasound-based scoring system, Haemophilia Early Arthropathy Detection with UltraSound (HEAD-US), for detecting early signs of haemophilic arthropathy. Materials and methods: The study group comprised 32 patients aged 6-17 years with a moderate or severe bleeding disorder, haemophilia A. The elbow, ankle and knee joints were evaluated using HJHS 2.1 and HEAD-US. Results: There was a strong correlation between total scores of the clinical scoring system and the ultrasound scoring system (rS =0.66, p < .01) for elbows and knees (rS = 0.57, p < .01 and rS = 0.76, p < .01, respectively). The correlation for ankles was substantially weaker (rS = 0.36, p = .04). The clinical scores correlated also with annual bleeding rate. Conclusions: The total and individual joint scores, especially for knees and elbows, using “Haemophilia Early Arthropathy Detection with UltraSound” correlated well with “Haemophilia Joint Health Score”. The clinical score, also measuring function, correlated better with bleeding rate. The relevance of HEAD-US as a prognostic tool is still unclear. [ABSTRACT FROM AUTHOR]

Introduction: Detection of early arthropathy is crucial for the management of haemophilia, but data on moderate haemophilia are limited. Therefore, we evaluated joint health and treatment modalities in Nordic patients with moderate haemophilia A (MHA) and B (MHB). Aim: To explore and compare the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) and Haemophilia Joint Health Score (HJHS) to detect early arthropathy in moderate haemophilia. Methods: A cross‐sectional, multicentre study covering Nordic patients with MHA and MHB. Arthropathy was evaluated by HEAD‐US and HJHS 2.1. Results: We assessed 693 joints in 118 patients. HEAD‐US scores (medians [interquartile ranges]) were as follows: elbows 0 points (0–0), knees 0 (0–0) and ankles 0 (0–1). Respectively, by HJHS: elbows 0 (0–1), knees 0 (0–1) and ankles 0 (0–1). Cartilage (14%) and bone (13%) were most commonly affected by HEAD‐US. Frequent HJHS findings were crepitus on motion in knees (39%), and loss of flexion (23%) and extension (13%) in ankles. HEAD‐US correlated strongly with HJHS (elbows r =.70, knees r =.60 and ankles r =.65), but 24% had discordant scores. Joints with HJHS zero points, 5% captured HEAD‐US ≥1 point. Moreover, 26% had HJHS findings without HEAD‐US pathology. Notably, 31% of knees had crepitus on motion and normal HEAD‐US. Conclusion: Overall, the joints attained low scores implying good joint health. HEAD‐US correlated strongly with HJHS. In 5%, HEAD‐US detected subclinical pathology. Crepitus on motion was frequently reported despite normal HEAD‐US, thus not necessarily reflecting arthropathy. HEAD‐US therefore improves the joint assessment in moderate haemophilia. [ABSTRACT FROM AUTHOR]

Introduction: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption. Aim: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real‐life data and expert opinion. Methods: A cross‐sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0‐124, optimum score 0) in persons with haemophilia A/B aged 4‐30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%). Results: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0‐29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0‐17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction. Conclusion: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort, which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment. [ABSTRACT FROM AUTHOR]