Abstract
Approximately 35%-50% of people with haemophilia (PWH) report living with chronic musculoskeletal pain. Although exercise based rehabilitation is effective for pain in other arthritises, there are no published guidelines for management of chronic pain in PWH. This review aims to evaluate and appraise the current evidence of effectiveness of physiotherapy interventions on (a) pain intensity, (b) quality of life (QoL) and (c) function in PWH. A systematic review of five databases AMED and CINAHL, EMBASE and MEDLINE and PEDro, as well as trial registries, grey literature and hand searching key journals was completed. Included studies were critically appraised and evaluated for risk of bias. The GRADE approach was used to rate the quality of the evidence. Nine trials consisting of 235 participants met the inclusion criteria. All studies had an overall risk of bias with low methodological quality. Meta-analysis was not possible due to heterogeneity across trials. Studies comparing a range of physiotherapy interventions against no intervention showed no clear beneficial effect on pain intensity or QoL. Only one study, investigating hydrotherapy or land-based exercise against control, showed positive effect for pain intensity, but rated very low on GRADE assessment. Studies comparing one physiotherapy intervention against another showed no clear benefit on pain intensity, QoL or function. LASER with exercise and hydrotherapy were shown to have some positive effects on pain intensity, but no clear benefit on function. At present, there is limited evidence for the use of physiotherapy interventions in addressing the issue of pain in PWH. Better designed trials with higher quality and explicit methodology along with user involvement are needed to assess the efficacy of any proposed intervention.
Highlights
Haemophilia is an inherited bleeding disorder characterized by recurrent and spontaneous bleeding into joints and muscles and fatal bleeding in the untreated state.[1,2] People with haemophilia (PWH) experience transient episodes of acute pain from an early age from musculoskeletal bleeding episodes
Ankle There was no clear benefit on pain intensity with home exercises and education mean difference (MD) −0.55 visual analogue scale (VAS), MD −0.3 VAS, or with manual therapy and exercise MD 0.06 VAS
Three studies included a measure of function as an outcome measure of intervention. It is not clear if there is any beneficial effect on function as measured by the 6-minute walk test (6MWT) with LASER and sham LASER, MD 29.33 minutes, or LASER and exercise compared to Pulsed electromagnetic field therapy (PEMF) and exercise, MD 14.47 minutes
Summary
Haemophilia is an inherited bleeding disorder characterized by recurrent and spontaneous bleeding into joints and muscles and fatal bleeding in the untreated state.[1,2] People with haemophilia (PWH) experience transient episodes of acute pain from an early age from musculoskeletal bleeding episodes. Some PWH continue to have bleeding into their joints and muscles, which can lead to debilitating arthritis with chronic and recurrent pain.[3]. People with haemophilia over the age of 65 had no access to regular treatment until they were in adulthood, with those currently aged in their 40's having no access to effective treatment for the majority of their childhood.[4] many PWH have chronically painful, multi-joint haemophilic arthritis, involving elbow, knee and ankle joints.[5,6,7]. PWH living with pain report limitations in mobility and independence, increased anxiety, poor quality of life and frustration due to restrictions in activities of daily living.[7,11,12]
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