Recurrent joint bleedings in people with hemophilia (PWH) often progress into the full clinical picture of hemophilic arthropathy, accompanied by chronic pain. Although chronic pain is commonly present in PWH, investigations assessing pain thresholds have not been performed yet. Thus, the aim of this study was to obtain objective and subjective measures of joint pain in PWH and to relate these to the severity of joint pathology. Thirty-six patients (aged 43±11 years) with hemophilia A and B (31 severe A, 1 B; 3 moderate A, 1 B) and 40 healthy control subjects (aged 42±14 years) participated in this study. Mechanical pain thresholds were obtained as objective parameters using an algometer, while subjective pain intensity and quality were assessed using numeric analogue scales. Quality of life was estimated using the Short-Form Health Survey (SF-36) questionnaire. Overall, we found reduced mechanical pain thresholds as obtained from the knee (PWH--left 38.1 [28.7/57.7], right 29.5 [20.9/49.3]; control--left 67.4 [56.8/112.6], right 60.9 [42.6/97.2]), and elbow (PWH--left 23.4 [15.3/33.4], right 23.5 [20.1/35.1]; control--left 56.7 [32.6/86.6], right 53.0 [30.7/87.7] in N; median [25th/75th percentile]) joints in PWH. Interestingly, this increased pain sensitivity was related to the severity of clinical joint pathology. In addition, PWH reported their pain in a more descriptive and not affective manner and scored similar to controls in the mental domain of the SF-36, thereby indicating good coping strategies despite the chronic nature of their complaints. In conclusion, pain sensitivity at the site of the affected joints is increased and closely related to joint pathology in people with hemophilia.

Introduction: Haemophilia is a congenital bleeding disorder with severe musculoskeletal complications. Resistance exercise is important to increase joint stability and to improve physical performance.Aim: This review aimed to investigate the safety and efficacy of resistance exercise interventions on people with haemophilia (PwH) and evaluate whether the American College of Sports Medicine resistance exercise criteria for healthy adults are valid for this population.Methods: A systematic search in literature was conducted, using the databases PubMed, MEDLiNE, CINAHL, SCOPUS, PEDro and Cochrane Library. Out of 2.440 studies published between 1960 and November 2019, 14 studies (9 randomized controlled trials, 1 controlled trial, 4 single‐group prospective studies) applying resistance exercise in juvenile and adult PwH corresponded to the inclusion criteria.Results: Studies performed dynamic, isokinetic or a combination of isometric and dynamic resistance training. Most interventions were carried out in the context of a multimodal training. Resistance was provided using fixed and free weights, body weight, resistance bands and water resistance. Study protocols included clinical and home‐based settings. Several studies suggest that training intensities lower than those known to increase the strength of healthy people are effective in increasing the strength of PwH. Resistance exercise seems to be a safe intervention if it is adequately monitored, individually adapted and applied with sufficient factor therapy. Due to the heterogeneity of study designs, training interventions and outcome measures a meta‐analysis could not be performed.Conclusions: Further studies of higher methodological quality are needed to determine the optimal types of exercise, optimal dosage and timing.

Background: In patients with haemophilia (PwH), most frequently affected joints are the ankle, knee and elbow. Due to improved factor therapy in the last decades, these previous findings have to be verified in Germany.Aim: The aim of this study is to detect the most affected joint, evaluate the significance of the source of pain and determine the point prevalence of back pain in Germany today.Patients and methods: In a retrospective study, data of n = 300 patients with severe moderate and mild haemophilia were evaluated regarding the most affected joint, the most common source of pain, and the point prevalence of back pain. An anamnesis questionnaire and the German Pain Questionnaire were used for this assessment.Results: The most affected joint in German PwH is still the ankle (41%), followed by the knee (27%) and the elbow (11%). The most common source of pain is also the ankle joint (32%). Back pain was also identified as one of the most common sources of pain, which is comparable to the elbow (elbow:15%; back:13%). The point prevalence in PwH for back pain was significantly higher compared to the general German population (P = .031).Conclusion: Our data showed that the ankle is still the most affected joint and the most common source of pain in Germany. These results also showed the relevance of back pain as a pain source. The evaluations also demonstrated the high point prevalence of back pain in PwH. Future therapies should also focus on the spine because joint changes affect posture.

Background: Haemophilia is a congenital bleeding disorder going along with disease‐specific joint complications and general health implications attributed to the lack of daily movement. Recent guidelines recommend physical activity for people with haemophilia (PwH). Yet, aerobic exercise in PwH is little studied and seldomly prescribed by clinicans.Aim: The aim of this systematic review is to investigate the safety, feasibility and health‐related efficiency of AE in PwH.Methods: A systematic literature search according to the PRISMA guidelines was conducted (PubMed, Web of Science). Inclusion criteria were defined using PICOS. Methodological quality was assessed via TESTEX.Results: Out of 789 studies identified, seven studies (three randomized controlled, two controlled, two single‐group prospective trails) were included. The TESTEX mean score was 8.1 (±3.8). AE was realized as aquatic exercise, nordic walking, treadmill running, bicycle riding and swimming. Neither bleeding rates nor the factor amount increased and AE led to diverse health‐related improvements.Conclusions: Little research has been conducted evaluating AE in PwH. Yet, AE can be considered as safe and feasible when being supervised by experts. However, disease‐specific recommendations for AE are difficult to provide. Therefore, experts can currently only back AE recommendations on experience and nonhaemophilia‐specific general guidelines.