Prevalence and perceptions of pain in people with haemophilia: A UK study

Abstract

IntroductionJoint bleeds in haemophilia cause destruction of articular structures, impaired function and pain. Up to 70% of people with haemophilia (PWH) report chronic pain. Little is known about the pain experiences in PWH in the UK.AimTo identify prevalence and perceptions of pain among PWH living in the UK.MethodsA cross‐sectional, non‐interventional survey study conducted among PWH (all severities). The survey incorporated elements from validated tools (EQ‐5D; EQ‐VAS) and was distributed via participating treatment centres.ResultsFive hundred and ninety‐nine PWH responded, 91% aged > 18. 81% used factor prophylactically or on demand. More pain was reported by those treated on demand versus prophylaxis particularly in those who reported daily pain. 65% reported ‘problem joints’ based on individual impact rather than medically defined ‘target joints’, 2/3 reported multiple joint issues. The ankle was most commonly affected. 59% reported frequent pain, with 56% aware of pain constantly or most of the time and were more likely to report less favourable EQ‐5D or EQ‐VAS scores (p < .001). Pain frequency/awareness was consistent across all severities. Most discussed pain with care teams, 31% only when asked; 25% did not discuss it. Pain discussions resulted in physiotherapy referral (63%) analgesia prescription (48%), and a minority specialist pain referral (9%). Most felt well supported with regard to their pain, but 70% reported learning to live with it.ConclusionPain affects PWH of all ages and severities even in a well‐resourced country significantly impacting quality of life. Clinicians must be more aware of chronic pain in PWH. Biopsychosocial approaches to pain assessment and management are recommended.

Challenges in ageing persons with haemophilia

“…22 Sites of pain typically correlate with common sites of intra-articular joint bleeding and resultant arthropathy or 'problem joints' , which are the ankles, knees and elbows. 23 The differentiation between joint pain from acute bleeding compared to that of chronic arthropathy can be challenging for patients and clinicians due to overlapping symptoms, 21,24 complicating the assessment, treatment, and management of haemophilia-related pain. Pain is also associated with cardiovascular strain and chronic stress, which are to be avoided to benefit cardiovascular health.…”

“…27 The influence of age on the clinical profile of pain also warrants consideration, as adults who commenced secondary or tertiary prophylaxis may live with more severe arthropathy, longstanding chronic pain and physical dysfunction compared to younger PwH on primary prophylaxis. Multi-site pain and a greater need for analgesic medications are associated with older age amongst PwH 23,28 ; however, it should not be disregarded that health-related quality of life and activities may also be impacted by chronic joint pain amongst children with haemophilia. 29 Infants and children may also experience acute pain early in life due to venepunctures and acute bleeding.…”

“…31 There have been recent developments in understanding the pathophysiological mechanisms of haemophilia-related pain, 24 pain perceptions and experiences amongst patients and healthcare professionals, 22,32,33 as well as the development of haemophilia-specific tools for pain assessment. 34 Many PwH view pain as a part of life 23,32 but this does not diminish a desire to cure or eradicate pain. 33 A number of guidelines and reviews regarding the treatment and management of haemophilia-related pain have been published, recommending a variety of pharmacological and non-pharmacological interventions, and referral to surgical or pain specialists in persistent cases of debilitating pain.…”

“…21,[35][36][37][38][39] Compared to pain management recommendations for other arthritic conditions, such as osteoarthritis, the evidence underpinning haemophilia-related pain management is relatively limited and largely empirical. 23,39 Research and guidance are also lacking in the specific management of pain and potential functional limitations amongst female patients with haemophilia and carriers who may also experience musculoskeletal bleeding and joint pain, 40 and may uniquely experience significant menstrual health-related pain.…”