Senior Hematology Consultant IRCCS Humanitas Research Hospital Milan, Italy
Acute and chronic pain, especially affecting the musculoskeletal system, are frequently reported in persons with hemophilia (PwH), including children and adolescents, despite regular prophylaxis. Up to 50% of adult PwH have chronically painful joints. Pain can greatly limit activities of daily life and participation and result in decreased health-related quality of life. Chronic and acute pain could be experienced concurrently, which poses unique challenges with respect to both assessment and management. Pain management requires a multidisciplinary approach because it might have different and concurrent causes. This might lead to treatment failure if incomplete assessment is done and pain is solely attributed to occurrence of breakthrough bleeds. Indeed bleeding episodes into joints cause pain related to the inflammation triggered by the presence of blood and iron into the joint space and tissues. In this light prophylaxis and intensification of treatment in case of breakthrough bleeds and/or detection of synovitis could be beneficial in controlling acute pain. In some cases, arthrocentesis may be useful. Beside that, the altered functional pattern which is immediately generated by breakthrough bleeds and/or structural changes in the joints, can contribute to maintain chronic pain related to muscle contractures and chronic stimulation of nociceptors. Majority of PwH have nociceptive pain, however a proportion demonstrate signs of neuropathic pain and/or altered central pain mechanisms, which require specific treatment with opioids, gabapentinoids and/or antidepressants. A step-wise use of analgesics is highly recommended including the use of paracetamol, cyclooxygenase-2 (COX-2) inhibitors, non-steroidal anti-inflammatory drugs (NSAIDs), and opioids, suggested. Beside pharmacological approaches, functional training and physiotherapy, including land- and water-based exercises, are mainstays of multidisciplinary integrated care of pain in PwH. All these approaches should be considered not as sequential treatment but often need to be implemented in parallel to address different causes and components of pain in different individuals.
Learning Objectives:
Pain represents one of the most relevant issues in PwH
Combined medical and non-medical strategies could be the most helpful way to manage pain successfully across ages and diseases stages