Pain and pain management in haemophilia

Günter Auerswald1, Gerry Dolan, Anne Duffy

  • 1aKlinikum Bremen-Mitte, Professor Hess Children's Hospital, Bremen, Germany bDepartment of Haematology, Queens Medical Centre, Nottingham, UK cIrish Haemophilia Society, Dublin, Ireland dDivision of Haematology, Cliniques Saint-Luc, Brussels, Belgium eHospital Universitario La Paz, Unidad de Coagulopatías, Servicio de Hematología, Autonoma University, Madrid, Spain fLund University, Department of Paediatrics and Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden gAgency for Hemophilia and Reference Center for Inherited Bleeding Disorders of Tuscany, Department of Emergency and Reception, Azienda Ospedaliero Universitaria Careggi, Florence, Italy hHemophilia Care Center, Bicêtre AP-HP Hospital and Faculté de Médecine Paris Sud, Paris, France iNational Haemophilia Centre, University Hospital Centre Zagreb, Rebro, and School of Medicine, University of Osijek, Zagreb, Croatia.

Joint pain is common in haemophilia and may be acute or chronic. Effective pain management in haemophilia is essential to reduce the burden that pain imposes on patients. However, the choice of appropriate pain-relieving measures is challenging, as there is a complex interplay of factors affecting pain perception. This can manifest as differences in patients' experiences and response to pain, which require an individualized approach to pain management. Prophylaxis with factor replacement reduces the likelihood of bleeds and bleed-related pain, whereas on-demand therapy ensures rapid bleed resolution and pain relief. Although use of replacement or bypassing therapy is often the first intervention for pain, additional pain relief strategies may be required. There is an array of analgesic options, but consideration should be paid to the adverse effects of each class. Nevertheless, a combination of medications that act at different points in the pain pathway may be beneficial. Nonpharmacological measures may also help patients and include active coping strategies; rest, ice, compression, and elevation; complementary therapies; and physiotherapy. Joint aspiration may also reduce acute joint pain, and joint steroid injections may alleviate chronic pain. In the longer term, increasing use of prophylaxis or performing surgery may be necessary to reduce the burden of pain caused by the degenerative effects of repeated bleeds. Whichever treatment option is chosen, it is important to monitor pain and adjust patient management accordingly. Beyond specific pain management approaches, ongoing collaboration between multidisciplinary teams, which should include physiotherapists and pain specialists, may improve outcomes for patients.

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