​Haemophilia is a genetic disorder which is characterized by recurrent joint bleedings. Consequently, the majority of adult PwH suffer from very painful and invalidating hemophilic arthropathy. Although the complexity of joint pain has been studied in several chronic joint pain conditions, until present only very limited research has been done on joint pain within PwH. As a result, developing effective treatments for chronic pain in PwH has been clearly identified as a priority for research in bleeding disorders.

Pain experienced by PwH is usually attributed to bleeding or arthropathy. Nowadays, recommendations on pain management are predominantly focused on pharmacological interventions and orthopaedic surgery, typically consisting of coagulation factor replacement, anti-inflammatory medication, moving on to injections and surgical options. But the pain flares are not always associated with acute bleedings and the degree of pain does not always correlate to the stage of joint damage seen by medical imaging. Therefore, haemophilia in adults seems clinically more related to a musculoskeletal disorder than a bleeding disorder, with their experience of pain even so influenced by social factors, expectations and beliefs, emotional factors, etc.

Indeed, most adults with severe haemophilia have experienced multiple painful bleeds during childhood into adulthood. As such, they are at higher risk of post-traumatic stress disorder (PTSD), resulting in intrusive re-experiencing of the event, persistent avoidance, increased vigilance, etc. This is probably similar to the situation of patients with sickle cell disease, another bleeding disorder, evidenced to have a higher degree of traumatic exposure than the general population, in part due to repeated acute pain flare-ups and frequency medical consultations.

Indeed, when memories are encoded under conditions of extreme stress, patients with PTSD may experience pain through a mechanism that probably involves memory rather than nociceptive pathways; pain memories can elicit somatic pain in the absence of an organic cause.

It is thus clear that we need to move forwards from thinking that pain is solely from arthropathy. To guarantee adequate treatment it is necessary to fully understand all these underlying processes, emotions, thoughts and perceptions of joint pain in PwH, taking into account their age category (children, adults, elderlies). A collaboration between haemophilia treatment centers, people with haemophilia, and pain scientists is necessary to gain more insight into underlying pain processes. 

Mira Meeus

2022 Pain in Motion

References and further reading:  

Wells, et al. Letter to the Editor: Pain memories: A new concept to consider in the management of chronic pain in people with  haemophilia. Haemophilia: 2021;1–3. DOI: 10.1111/hae.14480. https://pubmed.ncbi.nlm.nih.gov/34951513/