Pain and functional disability amongst adults with moderate and severe haemophilia from the Irish personalised approach to the treatment of haemophilia (iPATH) study

Eur J Haematol. 2022 Jun;108(6):518-527. doi: 10.1111/ejh.13763. Epub 2022 Mar 30.

Abstract

Objectives: To establish the prevalence of pain and functional disability in Irish adults with moderate and severe haemophilia, and to examine demographic and lifestyle influences.

Methods: Males ≥18 years with moderate or severe haemophilia participated. Pain and function were examined using the PROBE questionnaire.

Results: Of 49 participants [median age 44 (IQR 32, 52) years], most had severe haemophilia (Factor VIII = 30; Factor IX = 13) and were on regular prophylaxis (88%). Those with moderate haemophilia (Factor VIII = 5; Factor IX = 1) treated on demand (12%). Acute (72%) and chronic pain (71%), functional difficulties (58%), and analgesic requirements (92%) were prevalent. Age was significantly associated with more advanced haemophilic arthropathy (p = .002), chronic pain (p = .029) and functional difficulties (p = .036). Adults who reported chronic pain commenced prophylaxis significantly later in life [32 (20, 51) vs. 8 (1, 23) years; p = .004]. Physical activity was significantly lower in those with functional difficulties (p < .05). A disparity between self-perceived 'target joints' and clinically defined target joints was also identified (76% vs. 23%).

Conclusion: Haemophilic arthropathy, pain and functional disability were prevalent amongst Irish adults with moderate and severe haemophilia. Age-dependent lifestyle, analgesic and treatment influences on pain and function warrant further investigation.

Keywords: function; haemophilia; pain; physical activity; prophylaxis.

MeSH terms

  • Adult
  • Chronic Pain* / diagnosis
  • Chronic Pain* / epidemiology
  • Chronic Pain* / etiology
  • Factor IX / therapeutic use
  • Factor VIII / therapeutic use
  • Hemarthrosis / drug therapy
  • Hemophilia A* / complications
  • Hemophilia A* / drug therapy
  • Hemophilia A* / epidemiology
  • Hemophilia B*
  • Humans
  • Joint Diseases*
  • Male

Substances

  • Factor VIII
  • Factor IX