Haemophilia is an inherited bleeding disorder characterised by recurrent and spontaneous bleeding into joints and muscles, and fatal bleeding in the untreated state.
People with haemophilia (PWH) experience acute pain from an early age from musculoskeletal bleeding episodes. As a result of such bleeds, many PWH have chronically painful, multi-joint haemophilic arthritis. 35-50% of PWH report living with chronic musculoskeletal pain, with most reporting their pain was poorly managed by their healthcare providers.
There are no published guidelines for management of chronic arthritic joint pain in PWH. In recent years however, there have been attempts at some trials investigating a wide range of physiotherapeutic interventions for people with haemophilia.
This review aims to evaluate and appraise the current evidence of effectiveness of a range physiotherapy interventions on (1) pain intensity, (2) quality of life (QoL) and (3) function in PWH.
A systematic review of five databases AMED and CINAHL (through EBSCO), EMBASE and MEDLINE (through OVID) and PEDro, as well as trial registries, grey literature and hand searching key journals was completed.
Two authors blindly reviewed abstracts against predetermined inclusion/exclusion criteria, and data extraction was completed using a standardised pro-forma. Included studies were critically appraised and evaluated for risk of bias, with the GRADE approach utilised to rate the quality of the evidence.
Nine trials consisting of 235 participants met the criteria. Due to large variations in study design, participants and interventions, meta-analysis was not possible.
Low or very low quality evidence was found for all studies. In studies comparing physiotherapy interventions (consisting home exercise and education, manual therapy and exercise, fascial therapy) versus no intervention, no clear beneficial effect was apparent on pain intensity or QoL.
Only one study, investigating hydrotherapy or land exercise against control, showed positive effect for pain intensity.
In studies comparing one intervention against another (consisting joint mobilisations, exercise, manual therapy, home exercises) no clear benefit was apparent on pain intensity, QoL or function.
LASER (compared to sham LASER and pulsed electromagnetic field treatment) and hydrotherapy (compared to land exercise) were shown to have some positive effects on pain intensity, but no clear benefit on function.
This review demonstrates low/ very low quality evidence for physiotherapy currently in the management of pain in people with haemophilia. Although hydrotherapy and LASER appear to have some positive effect, these studies had a high risk of bias, and like all included studies had very low numbers of participants. Pain was not a specific inclusion criteria in any of the studies. Many interventions investigated were passive, had a high degree of heterogeneity or were pilot studies. There was no evidence of any participant involvement in trial design.
This review highlights the gaps in the current research agenda regarding the effectiveness of physiotherapy for pain management in haemophilia. It is clear that better designed trials, with user involvement are crucial. Better defined inclusion criteria are needed to assess efficacy of any proposed intervention.
NIHR funded Clinical Doctoral Research Fellowship: Ref no: ICA-CDRF-2017-03-050.
This work was presented at Physiotherapy UK 2019