Physical activity and factor VIII levels in patients with haemophilia: A real-world prospective observational study.

INTRODUCTION: Regular physical activity (PA) is recommended for patients with haemophilia (PwH). For PwH it is crucial to ensure a sufficient factor level to prevent PA-induced bleedings. However, there is a gap in the literature dealing with specific factor levels, which are needed when performing specific types of PA. AIM: To provide data on factor VIII (FVIII) levels at the start of PA performed by PwH. METHODS: In this prospective 12-month real-world observational study, 23 PwH recorded every PA they performed and the FVIII levels at the start of the PA using a pharmacokinetic application. PA types were clustered according to the collision and injury risk into three categories (Cat I = low, Cat II = medium, Cat III = high risk). Haemophilia Joint Health Scores (HJHS) were performed at baseline, after 6 and 12 months. RESULTS: 795 PA sessions of Cat I, 193 of Cat II, and 23 of Cat III were documented. FVIII levels at the start of PA were different between categories (Cat I: 29.8 ± 32.1%, Cat II: 38.3 ± 33.4%, Cat III: 86.6 ± 29.2%). Out of all PA sessions, 145 (14%) were performed at a factor level of ≤3%. Three PA-induced bleeding occurred. Baseline HJHS was 14.5 ± 13.6 points and did not change throughout the study. CONCLUSION: This study provides real-life data on FVIII levels at the start of 1011 PA sessions. PwH are mainly active in low-risk sports with higher FVIII levels observed in Cat II and III, respectively. Only three PA-induced bleeding occurred, even though several PA were started with low FVIII levels.

Changes in pain profile of patients with haemophilia during 1-year follow-up.

INTRODUCTION: Patients with haemophilia (PwH) may experience increased sensitivity to pain. Based on the assessment of the somatosensory system, a recent study showed a specific pain profile in PwH when compared to controls by using Quantitative Sensory Testing (QST). AIM: This study aimed to evaluate the pain profile of affected joints (knee or ankle joints) and a non-affected site (dominant hand) in adult PwH over a 1-year period. METHODS: Twenty-four PwH (severe haemophilia A = 19, B = 3; moderate haemophilia A = 1, B = 1; age: 52±8 years) and 21 healthy controls (age: 52±12 years) were examined by QST. Both knee or ankle joints and the hand as reference were examined twice with an interval of 1 year in order to assess several detection (DT) and pain thresholds (PT). RESULTS: Statistically significant altered mechanical (P < .001) and pressure (P < .05) PT were found at affected joints and at a non-affected site in PwH when compared to controls. Mechanical DT showed a significant increase at all assessed sites (e.g., at ankle joints PwH vs. controls at baseline/follow up in mN: 13.9±9.8 vs. 12.0±8.2/19.4±12.4 vs. 13.7±11.1; P < .01) in both cohorts. Nevertheless, changes in most parameters within 1 year occurred similarly in both groups. CONCLUSION: The statistically significant different QST profile between PwH and controls does not seem to deteriorate further over the course of the year. Thus, under prophylactic treatment, the existing difference in the pain profile between PwH and controls at baseline does not appear to be a progressive process within 1 year.

A single bout of coordination training does not lead to EIH in young healthy men - a RCT.

OBJECTIVES: Physical activity can lead to hypoalgesic effects and is often recommended as part of multidisciplinary pain management. Based on the idea, that in future specific and more differentiated sports therapeutic interventions could be used for a multidisciplinary pain management, various type of sports and their effects on pain sensitivity should be analysed. Whereas endurance as well as strengthening exercises are associated with a decrease in pain sensitivity in healthy people as well as people with chronic pain states, the effects of a specific coordination training (CT) on pain sensitivity have not yet been sufficiently investigated. Therefore, aim of the present study was to examine if a single bout of CT leads to exercised-induced hypoalgesia in young healthy men. METHODS: Thirty five healthy men (mean age 27 ± 3 years) were examined in a randomised crossover design before and after a single bout of 45-min CT and a 45-min resting session as control condition by means of Quantitative Sensory Testing (QST). The QST is a validated instrument to assess the function of the somatosensory system, by applying thermal and mechanical stimuli. By doing so, various detection and pain thresholds were determined at the dorsum of one foot. Exercises of CT were chosen to generate high proprioceptive input for the ankle joints. RESULTS: Analysis of the QST data in respect of the factors group (CT/control condition), time (pre/post) and stimuli (parameter of QST) revealed no statistically significant main effects of a single bout of CT on somatosensory system, neither for the factors group*time (p=0.51), nor the factors group*time*stimuli (p=0.32). All stimuli remained constant in the course of both conditions (e.g. mean ± sd of heat pain threshold pre/post in °C: coordination: 44.7 ± 3.1/44.8 ± 2.9; rest: 45.5 ± 3.0/44.9 ± 3.0). CONCLUSIONS: In this setting, a single bout of CT had no effect on the somatosensory system in young healthy men. Therefore, this specific CT did not lead to an exercised-induced hypoalgesia in healthy people. Intensity of sensory input during training intervention might be too low to generate analgesic effects in a non-pathological altered somatosensory system of young healthy men. Further research is needed to clarify if a CT can induce exercised-induced hypoalgesia in people with pathological alterations of the somatosensory system. In addition, it has to examined if analgesic effects can be induced by changing the intensity of CT in healthy people. Detailed knowledge regarding the effects of different training interventions on pain modulation is needed to completely understand the mechanism of exercised-induced hypoalgesia.

Establishing an online physical exercise program for people with hemophilia.

BACKGROUND: Hemophilia is a congenital bleeding disorder with an estimated frequency of 1:10,000 births. Repeated joint bleeding is a hallmark of the disorder and leads to painful hemophilic arthropathy. Regular exercise can help improve joint stability and function, reduce the risk of injury and bleeding and improve physical fitness and quality of life. This method paper describes an online training concept aiming to offer access to appropriate exercise instructions for people with hemophilia who are not able to attend regular training at a hemophilia center. METHODS: The online exercise program is accessible through the homepage of the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University Vienna as well as through scanning a QR code printed on information material using a smart phone or tablet. RESULTS: The program contains exercises to improve mobility, coordination, muscular strength and flexibility. A brief introduction is given by a hematologist, a pediatrician and a physiatrist. An introductory video informs about contraindications and essential precautions, such as medical attendance and sufficient factor therapy to consider before starting the training. Another video gives advice on the exercise composition. The demonstrated exercises are explained by a physician and are available for adults and children. To individualize training recommendations and offer further diagnostic tools and physical treatment options as necessary, the Department of Physical Medicine, Rehabilitation and Occupational Medicine of the Medical University of Vienna will establish consultation hours for people with hemophilia. CONCLUSION: As hemophilia is an orphan disease, patients are mainly treated in specialized centers. For patients who live far from these centers or have limited access to a training there for other reasons, the physical medicine consultation hour and the implementation of online exercise instructions offer individually adapted exercise information for a regular home-based training to benefit from increased physical fitness and joint stability.