EVIDENCE FOR THE EFFECTIVENESS OF CARE FOR NEUROMUSCULAR DISEASES AT THE NEUROMUSCULAR CENTRE, WINSFORD,CHESHIRE.

BACKGROUND

Neuromuscular disorders (NMDs) cover a wide range of conditions including neuropathies (either acquired or inherited), muscular dystrophies, amyotrophic lateral sclerosis, spinal muscular atrophy as well as a range of very rare muscle disorders.Most NMDs are incurable, however, an effective rehabilitation program can help maintain a patient's quality of life (QOL), as well as maximise the patient's physical and psychosocial functions. Specialist multi-disciplinary care has been developed by clinicians and is proposed as the best model for delivering effective care for such complex, multi-system diseases. The provision of physiotherapy, orthotics, early cardiac monitoring and intervention and corticosteroids has been shown to improve muscle function and maintain independent mobility. [1]

The Muscular Dystrophy Campaign (MDC) has presented evidence in their report1 that patients with neuromuscular diseases are faced with a fragmented, sub-standard system of care, with significant variations in survival across the UK. The report states that recognised specialist neuromuscular centres, which are highly dependent on charity subsidy, are currently only located in London, Newcastle, Oxford and Oswestry. The key

findings from the report are that:-

  • Survival for patients with for example, Duchenne muscular dystrophy is an average of 18 years in the South West and 30 years in the North East.
  • Two out of three primary care trusts (PCTs) are failing to support a muscle clinic for either adults or children meaning that many patients do not receive specialist multi-disciplinary care, as found in an FOI request.

Subsequently the MDC presented the evidence contained in their report to the Welsh Affairs Committee’s enquiry into the provision of cross border services for Wales. [2]The report stated that some Welsh patients with NMDs, particularly those in North Wales and Mid Wales failed to receive adequate care and need to access services across the border. A survey of the local health boards (LHBs) in Wales in January 2008 revealed that approximately 70% of the LHBs who responded, did not support a muscle clinic that offers services to adults or children with NMDs.

The LHBs have requested the National Public Health Service to consider the evidence on the effectiveness of the service offered by the Neuromuscular Centre in Cheshire so that the information can be used to aid decisions on commissioning services for their patients with NMDs.

PATIENT GROUP

Over 1,000 children and adults for every 1 million of the population are affected by muscle wasting neuromuscular diseases in England and Wales. It is estimated that 3000 people are affected by a NMD in Wales.

INTERVENTION

The NeuroMuscular Centre, Winsford, Cheshire, [3]a registered charity, was founded in 1990 and provides physiotherapy, hydrotherapy or active exercise for adults with neuromuscular diseases.

ALTERNATIVE TREATMENTS

Standard NHS provision of care.

RESEARCH EVIDENCE

A rapid review of relevant databases was performed using previously validated methods. There was a lack of good quality Level 1 and Level 2 evidence to support the effectiveness of the care provided specifically at the Winsford NeuroMuscular Centre.

The report from the MDC1 did not cite any good quality evidence for specialist care. One cohort study from the Netherlands[4] assessed the clinical and economic impact of the specialist multidisciplinary approach compared with usual care for patients with NMD. Usual care represented the current occupational therapy (OT), physical therapy (PT) and speech therapy (ST) use in the authors' context. Compared with the multidisciplinary advice, there was 40% underuse of OT among patients with neuromuscular disease. For PT, there was 32% overuse and 22% underuse; for ST, there was neither over, nor underuse. The costs of the multidisciplinary advice were estimated at 245 euros per patient. The multidisciplinary approach, if fully implemented, would result in a mean cost savings of 85euros.per patient. The authors concluded that amongst patients with NMD, who do receive some form of allied healthcare, quite a few receive these treatments for too long periods of time.

COST EFFECTIVENESS

There was a lack of data on cost effectiveness of specialist treatment at the neuromuscular centre in Cheshire.

ONGOING RESEARCH

No ongoing trials were found in a search of the controlled clinical trials databases.

CONCLUSIONS

  • There was a lack of good quality high level evidence to support the effectiveness of the care provided by the NeuroMuscular Centre in Winsford, Cheshire for NMD patients.
  • There was some observational evidence to indicate that specialist care should be provided for NMD patients.
  • The Muscular Dystrophy Campaign has presented evidence to the UK Parliament showing inequalities in the provision of care for NMD patients in England and Wales.
  • The commissioning of care by the LHBs for NMD patientshas been discussed by the Welsh Affairs Committee’s enquiry in 2008 into cross-border services for Wales.
  • No data was found on cost effectiveness or future reaearch

1

Dr M Webb, version 1, December 2008

REFERENCES

[1] Hanna MG; Muntoni F; Reilly M et al. Building on the Foundations: Establishing a Specialist Neuromuscular Service across England. Available at:-

[Accessed 7th December 2008].

[2] The United Kingdom Parliament. Memorandum submitted by the Muscular Dystrophy Campaign. Available at: [Accessed 7th December 2008].

[3] The NeuroMuscular Centre. Working with muscular dystrophy: creating an environment where full potential and optimal wellbeing can be achieved. Available at: [Accessed 7th December 2008].

[4] Cup E H; Pieterse A J; Knuijt S et al. Referral of patients with neuromuscular disease to occupational therapy, physical therapy and speech therapy: usual practice versus multidisciplinary advice.Disability and Rehabilitation 2007;29:717-726