Self‐reported prevalence, description and management of pain in adults with haemophilia: methods, demographics and results from the Pain, Functional Impairment, and Quality of life (P‐FiQ) study

Abstract

Introduction: Haemophilia is characterized by frequent haemarthrosis, leading to acute/chronic joint pain. Aim: To assess self-reported prevalence, description and management of pain in adult males with mild-to-severe haemophilia and history of joint pain/bleeding. Methods: Participants completed a pain survey and five patient-reported outcome instruments assessing pain, functional impairment and health-related quality of life (HRQoL). Results: Of 381 participants enrolled, median age was 34 years; 77% had haemophilia A, 71% had severe disease and 65% were overweight/obese. Many (56%) were not receiving routine infusions; 30% never received routine infusions. During the prior 6 months, 20% experienced acute pain, 34% chronic pain and 32% both acute/chronic pain. Subjects with both acute/chronic pain (vs. none, acute or chronic) were more likely to be depressed (30% vs. 0-15%), obese (35% vs. 20-29%) and have lower HRQoL (mean EQ-5D visual analog scale, 69 vs. 83-86) and function (median overall Hemophilia Activities List, 60 vs. 88-99). Most common analgesics used for acute/chronic pain during the prior 6 months were acetaminophen (62%/55%) and non-steroidal anti-inflammatory drugs (34%/ 49%); most common non-pharmacologic strategies were ice (65%/33%) and rest (51%/33%). Hydrocodoneacetaminophen was the most common opioid for both acute/chronic pain (30%); other long-acting opioids were infrequently used specifically for chronic but not acute pain (morphine, 7%; methadone, 6%; fentanyl patch, 2%). Conclusion: Patients with chronic pain, particularly those with both acute/chronic pain, frequently experience psychological issues, functional disability and reduced HRQoL. Treatment strategies for acute pain (e.g. routine infusions to prevent bleeding) and for chronic pain (e.g. long-acting opioids) may be underused.

Prevalence and impact of obesity in people with haemophilia: Review of literature and expert discussion around implementing weight management guidelines

“…Furthermore, conflicting reports were obtained from a Dutch postal survey that found severe haemophilia to be associated with lower rates of overweight and a Canadian paediatric chart review that found severe haemophilia to be associated with higher rates of obesity . Existing evidence also suggests a minimal effect of joint symptoms on risk of obesity, as participants in the Pain, Functional Impairment and Quality of Life (P‐FiQ) study, who were selected for the presence of joint pain or bleeding, showed no apparent increase in rates of overweight (36%) or obesity (29%), compared with the general population . The Universal Data Collection (UDC) programme, a collection of surveillance data provided by US HTCs in collaboration with the CDC, identified similar prevalences of overweight or obesity among adults (age 20+ years) in the haemophilia and general populations (overweight: 34.5% vs 32.6%; obese: 23.5% vs 22.3%)…”

Gaining insight into the complexity of pain in patients with haemophilia: State‐of‐the‐art review on pain processing

“…Traditionally, a differentiation is made between pain resulting from an acute bleed (haemarthrosis) and chronic pain originating from joint damage (arthropathy). 1,4,7,8 A study comparing perceptions regarding the aetiology of pain in PwH with ultrasound evaluation confirmed that a diagnosis based on the sole perception of pain is unreliable in the majority of the cases: the aetiology of pain (bleeding versus joint inflammation or other regional pain syndrome) was correctly judged in only 30% of the cases, either by the physician or the patient! Firstly, the terms "acute" and "chronic" originally referred to a time frame and not to a specific underlying cause of pain.…”

Neuropsychological function in children with hemophilia: A review of the Hemophilia Growth and Development Study and introduction of the current eTHINK study

“…Incorporation of routine prophylactic factor replacement therapy, starting in early childhood for severe disease, now allows patients with this disorder to participate in many more physical activities and has reduced missed school and work time . However, despite the more widespread use of factor replacement therapy to limit symptoms of physical disability in children with severe hemophilia, little is known about the impact of the disease on their cognitive development.…”