Exercise variables and pain threshold reporting for strength training protocols in people with haemophilia: A systematic review of clinical trials.

INTRODUCTION: Although strength exercise is often prescribed for people with haemophilia (PWH), it remains unknown how exercise variables and pain thresholds are used to prescribe strength training in PWH. AIM: To analyse how strength exercise variables and pain thresholds have been used to prescribe strength training in PWH. METHODS: A systematic search was conducted in PubMed, Embase, Web of Science, CENTRAL and CINAHL databases from inception to 7 September 2022. Studies whose intervention included strengthening training in adults with haemophilia were included. Two independent reviewers were involved in study selection, data extraction and risk of bias assessment. RESULTS: Eighteen studies were included. The least reported variables among the studies were: prophylactic factor coverage (11.1%), pain threshold/tolerability (5.6%), intensity (50%), total or partial range of motion (27.8%), time under tension (27.8%), attentional focus modality (0%), therapist experience in haemophilia (33.3%) and adherence assessment (50%). In contrast, weekly frequency (94.4%), duration (weeks) (100%), number of sets/repetitions (88.9%), repetitions to failure/not to failure (77.8%), types of contraction (77.8%), rest duration (55.6%), progression (55.6%), supervision (77.8%), exercise equipment (72.2%) and adverse event record (77.8%) had a higher percentage of reported (>50% of studies). CONCLUSION: Future research on strength training for PWH should improve information on pain threshold and other important variables such as prophylactic factor coverage, intensity, range of motion, time under tension, attentional focus modality, therapist experience in haemophilia and adherence assessment. This could improve clinical practice and comparison of different protocols.

Validation of the patients-specific functional scale in patients with haemophilia.

INTRODUCTION: A complete functional assessment is essential to measure health status and treatment effects in patients with haemophilia. The Patient-Specific Functional Scale (PSFS) is a reliable, valid, simple and quick scale that measures physical function in patients with musculoskeletal disorders. However, the reliability and validity of the PSFS have not been evaluated in patients with haemophilia. AIM: The aim of this study was to validate the Patient-Specific Functional Scale in patients with haemophilia. METHODS: Twenty-eight patients with haemophilia participated in the study. They completed the PSFS and the Haemophilia Activity List (HAL) scales by telephone during an initial session, and then repeated the assessment in a follow-up session 1 week apart. Reliability was analysed by the internal correlation coefficient (ICC), the standard error of measurement (SEM) and the smallest detectable change (SDC). The concurrent validity of the PSFS was determined by correlating the initial score of the PSFS scale to the initial score of the HAL scale. Correlations were calculated by means of scatter plots and Pearson product-moment r correlation coefficient. RESULTS: ICC and SEM values showed excellent reliability for the PSFS scale, with a SDC of 1. A significant moderate correlation was found between the results of the PSFS and the HAL (r = .57, P < .001). CONCLUSION: The PSFS is a reliable and valid scale to measure the functionality of people with haemophilia.

Effectiveness of physical exercise on postural balance in patients with haemophilia: A systematic review.

INTRODUCTION: Balance training is important to improve balance and to decrease the risk of falls in patients with haemophilia (PWH). AIM: To analyse the current knowledge about the effectiveness of physical exercise on postural balance in PWH. METHODS: A systematic search for clinical trials, published before November 2021, was conducted using the following electronic databases: PubMed/MEDLINE, Web of Science, Embase, Wiley Online Library and the Cochrane Central Register of Controlled Trials (CENTRAL). Two independent reviewers extracted the data and assessed the risk of bias. The certainty of the evidence was analyzed using GRADE. RESULTS: A total of ten studies involving 304 patients were included. The studies performed strength and balance exercises, and some included flexibility, mobilization, and/or aerobic exercises in their training programs. Postural balance was evaluated using a force platform and the one leg stand test was the most frequently used. Only five exercise programs achieved a significant increase in balance (pre-post) with a strength, postural balance, flexibility, and aerobic intervention. In general, the quality rate of the risk of bias was fair but the overall quality of the evidence across the studies was very low. CONCLUSION: Five studies showed that there is a positive change in balance after the intervention. However, there is currently an unclear demonstration of evidence for the use of physical exercises to improve postural balance in people with haemophilia. Further studies with a higher methodological quality are needed.

Effects of performing dual tasks on postural sway and postural control complexity in people with haemophilic arthropathy.

INTRODUCTION: People with haemophilic arthropathy (PWHA) have impairments in postural control. However, little is known about the effects of demanding conditions, including the unipedal stance and dual tasks, on postural control in PWHA. AIM: Determine the effects of performing dual tasks while in the one-leg stance on postural sway and postural control complexity in PWHA vs. healthy active (HAG) and non-active (HNAG) groups of individuals. METHODS: Fifteen PWHA and 34 healthy subjects (18 active and 16 non-active) were recruited. Vertical (V), mediolateral (ML) and anteroposterior (AP) centre of mass signals were acquired using a 3-axis accelerometer placed at the L3/L4 vertebrae of subjects as they performed the one-leg stance under single and dual-task conditions. Sway balance and the complexity of postural control were studied via root mean square (RMS) acceleration and sample entropy, respectively. Increased complexity of postural sway was attributed to increased automatism of postural control. RESULTS: RMS values for PWHA were higher than HAG under both conditions for the V and ML axes, and higher than HNAG under the dual-task condition for the ML axis. Sample entropy was lower in PWHA than healthy individuals under the dual-task condition for V and ML axes, and the single-task condition for the ML axis (P < .05). CONCLUSION: PWHA had poorer postural sway and decreased postural control complexity when performing a one-leg stance than healthy people, especially when the dual-task condition was applied. These results may help to design new approaches to assess and improve postural control in PWHA.