History, Development & Current State of Research

Epidemiological studies have repeatedly demonstrated the importance of physical activity for preventing various diseases. Regular exercise can, for example, help prevent cardiovascular disease. Beyond broad prevention, targeted exercise therapy can also alleviate symptoms in the context of rehabilitation.

Until the 1960s, people with haemophilia were effectively excluded from using physical activity as a means of prevention or rehabilitation. As recently as 30 years ago, any activity not strictly necessary for daily living was discouraged—sport was almost forbidden. The prevailing view was that inactivity was the best prophylaxis to avoid joint bleeds in haemophilia. However, the consequences of inactivity can be dramatic: inadequate development of strong, well-coordinated skeletal muscles compromises optimal joint protection. The resulting muscular imbalance, together with musculoskeletal and coordinative dysfunction, leads to faulty movement patterns that promote joint bleeding and thus contribute to haemophilic arthropathy.
Studies on the consequences of physical inactivity in haemophilia have identified the following deficits compared with healthy controls:

• Reduced endurance capacity in children with haemophilia
• Reduced lower-limb strength in children and adults with haemophilia
• Diminished coordinative abilities in adults with haemophilia
• Decreased anaerobic performance and muscle strength in both children and adults with haemophilia

Understanding these relationships led to a shift in perspective over the following years. With the availability of clotting factor replacement products in the early 1960s, cautious attempts began to allow physical activity for people with haemophilia—and ultimately to consider it a necessity. Beyond unsupervised exercise, physiotherapy was often the only preventive or rehabilitative option.

Today there is consensus on the value of selected physical activity for people with haemophilia, and supervised exercise conducted by qualified instructors can be recommended. However, a fully developed, diagnosis-specific training or exercise therapy—such as has long been established for cardiovascular disease—has still not become a standard adjunct treatment for haemophilia. Uncertainties about precise training content, methods, and modalities and their effects in patients with haemophilia continue to pose challenges to designing and implementing targeted exercise therapy. Despite these obstacles, the importance of sports therapy has grown in recent years. Benefits include the sustainable transfer of trained motor skills to activities of daily living and the positive influence of group dynamics on social competence. Active therapeutic exercise can also significantly strengthen patients’ self-confidence and thereby alter pain perception.

We see strong potential to make sports therapy accessible and beneficial to people with haemophilia—particularly to improve joint status. In a study by Hilberg (2001), neuromuscular coordination was shown to be markedly impaired in people with haemophilia compared with non-haemophilic controls. Encouragingly, the study also demonstrated that training can improve these abilities in people with haemophilia. The function of a high-quality neuromuscular interplay—often called coordination, or in a narrower sense proprioception—is essential to ensure optimal joint protection.

Recent Publications

Cost-utility of a six-month Programmed Sports Therapy (PST) in patients with haemophilia.

Köberlein-Neu J, Runkel B, Hilberg T. (2018). Haemophilia.

Programmed Sports Therapy (PST) in people with haemophilia - "sports therapy model for rare diseases".

Hilberg T. (2018). Orphanet Journal of Rare Diseases.