Neuromuscular Care (North-West)

Lord Evans of Rainow Excerpts

Wednesday 9th February 2011 Westminster Hall

[Graham Evans (Weaver Vale) (Con)]

I thank Mr Speaker for allocating time for this important subject. I pay tribute to the excellent Muscular Dystrophy Campaign, which does such brilliant work in this field and has assisted me in preparing for the debate. This morning, I will bring to the attention of the Minister the significant gaps in specialist neuromuscular care services in the north-west region that many people living with muscular dystrophy and related neuromuscular conditions currently experience. Vital review work undertaken by the NHS North West specialised commissioning group in the past six months is encouraging, but families need to see it translated into real improvements to services. It also provides an opportunity to improve patient outcomes and to reduce the amount of money spent on unplanned emergency admissions to hospital for people in the north-west with neuromuscular conditions.

Let me briefly outline my personal connection to muscular dystrophy. I am 47 years old, and I had a cousin, Stephen Payne, who, if he was still alive today, would be of the same age. He was diagnosed with Duchenne muscular dystrophy at the age of six, and I remember the devastating effects that it had on our family. This was in the late 1960s, when not a lot was known about the condition. I remember my uncle, Mr Barrie Payne, taking his son to Harley street for a diagnosis. I am afraid to say that it was not good. None the less, Mr Barrie Payne is a fighter and a campaigner and he threw all his energies into fundraising to see if a cure could be found for Duchenne.

My very earliest memories, therefore, are of a family fighting and campaigning for a cure for that dreadful disease. I also have very early memories of politicians getting involved in this vital subject. I pay tribute to Lord Alf Morris of Manchester, who, at the time, was the MP for Wythenshawe. Coincidentally, Lord Morris’s sister lived on my council estate, and I remember him driving to our estate in his Wolseley car. At the time, those cars were for very special people. He came to see my family when he visited his sister and I remember thinking at the time what a special man he was. In those days, in the ’60s and ’70s, it was thought that politicians could help ordinary people from working-class backgrounds such as mine. I remember so well the work that my family and Mr Morris did to get a change in legislation. I am pleased to say that Alf Morris was behind the Chronically Sick and Disabled Persons Act 1970, which was the first Act in the world to give rights to people with disabilities.

I did my best to raise money for the muscular dystrophy charity. I used to sell pens, pencils, rulers and pencil cases in my school yard at primary school. Imagine a head teacher allowing a child to go into school with a bag full of goods to sell for cash in these politically correct times. Coincidentally, my head teacher lived next door to my cousin, Stephen Payne, and at school fairs he used every opportunity to raise money for muscular dystrophy.

My uncle continued to campaign to raise money and awareness. People in wheelchairs were often not allowed access to places such as airports and museums, which led to considerable arguments. My uncle rightly felt that people with disabilities should be allowed in. I am glad to say that in the 21st century, things are a lot different.

Stephen was a bright and articulate individual, and he was always forthright in his opinions. He was a Manchester City fan, while I was a Manchester United fan. When Manchester United got relegated in 1973, he certainly let it be known how pleased he was about that. Stephen died in California when he was 21. His peers died when they were in their very early teens, which just goes to show that palliative care and hydrotherapy can extend the lives of people with Duchenne.

The Muscular Dystrophy Campaign is the leading UK charity and focuses on all neuromuscular conditions. It is dedicated to improving the lives of all people with such conditions. Founded in 1959, the Muscular Dystrophy Campaign funds vital research, provides and supports care services and gives information, advice and direct help to individuals living with neuromuscular conditions. I am honoured to be a member of the all-party parliamentary group for muscular dystrophy, which has highlighted shocking gaps in service across the UK and continues to call for improvements in access to specialist neuromuscular care to follow up recommendations in the Walton report.

There are more than 60 types of muscular dystrophy and related neuromuscular conditions. It is estimated that more than 1,000 children and adults in every million of the population are affected by muscle-wasting neuromuscular diseases—in the north-west around 8,000 people in total. Such disorders can be genetic or acquired. A number of conditions, such as Duchenne muscular dystrophy, are particularly aggressive. They cause progressive muscle wasting, weakness, orthopaedic deformity and cardiac and respiratory compromise, and result in premature death. Many young boys in the UK with Duchenne muscular dystrophy are dying before they get beyond their teens; that is unacceptable.

Specialist multidisciplinary care has been developed by leading clinicians as the best model for delivering effective care for such complex multi-system diseases. The provision of expert orthopaedics and early cardiac monitoring and intervention has been shown to improve muscle function and maintain independent mobility. People with neuromuscular conditions, therefore, need specialist multidisciplinary care, support and intervention from a range of professionals and specialists. That was recognised as part of the specialised services national definitions set. Leading neuromuscular clinicians fought hard to get those services recognised as specialist by the Department of Health.

Specialised services are defined in law as services with a planning population of more than 1 million people, which means that a specialised service is not provided by every hospital in England. The SSNDS describes the services in more detail. The definitions provide a helpful basis for service reviews and strategic planning, which enables commissioners to make comparisons of activity levels and spend. They help with the identification of activity that should be regarded as specialised and therefore subject to collaborative commissioning arrangements. The 10 specialised commissioning groups, acting on behalf of the members of primary care trusts, are responsible for the commissioning arrangements for specialised services.

Neuromuscular conditions come under the auspices of a number of specialities; they are genetic conditions, so geneticists sometimes deal with patients. The conditions sometimes affect children, so paediatricians are involved. A number of the adult forms come under the heading of neurology. Some of the conditions require respiratory care, which is provided by a respiratory practitioner, and some are metabolic conditions such as Pompe, and patients are treated by specialists for metabolic disorders. Therefore, health care for people is quite fragmented, and that clouds professional responsibility. “Neuromuscular” must be recognised as a speciality along with neurology.

Dr Ros Quinlivan, a leading consultant in paediatrics and neuromuscular disorders, has outlined the effects of neuromuscular conditions and how they need to be managed:

“Neuromuscular conditions are rare and include: Muscular dystrophies, metabolic myopathies, congenital myopathies, inflammatory myopathies, Spinal Muscular Atrophies. Many of these conditions affect only skeletal muscle and thus cannot be considered to be neurological disorders, in fact skeletal muscle can be considered to be the largest organ in the body. Most neuromuscular disorders are genetic in origin and affect families, but the inflammatory myopathies are acquired and require specific treatment. Affected patients range from newborn infants to elderly people. The effect of many of these conditions is on the skeleton causing skeletal deformities due to muscle contractures and on the heart and lungs causing respiratory or cardiac failure which can significantly limit life expectancy.

The physical management of these disorders is quite distinct compared with conditions affecting either the nervous system or the musculo-skeletal system (bone and joints). Proximal and axial muscle weakness caused specific functional difficulties not seen in patients attending clinics in other specialist areas. The progressive nature of these conditions means that a multi-disciplinary approach to care, with experienced clinicians specialising in neuromuscular disorders, is essential to achieve the best outcomes.”

It is now felt by the clinical community that it is time to make a neuromuscular curriculum part of the medical career, to establish the neuromuscular field in its own right. The conditions have a lot in common, so it makes sense that they are treated by the same person. For example, some forms of limb-girdle muscular dystrophy exhibit similar symptoms to some forms of spinal muscular atrophy, but one is a muscle disease and the other more a neurological disease. They are long-term rare conditions, which makes them quite different from neurological diseases such as Alzheimer’s and Parkinson’s.

Neuromuscular services need to be commissioned on a regional basis. Currently they are commissioned by the 10 regional NHS specialised commissioning groups, with top-slicing of PCTs. That method of commissioning services for these rare and very rare conditions has been endorsed by a new body of experts, the British Myology Society. It makes no sense for one PCT to commission specialised services. Unlike conditions such as Parkinson’s or multiple sclerosis, there are no guidelines from the National Institute for Health and Clinical Excellence for these neuromuscular conditions, which presents real difficulties when trying to set standards of care across the country.

That situation has been a contributing factor to the postcode lottery that has arisen, and which was highlighted in the all-party group on muscular dystrophy’s Walton report, published in August 2009. That followed reports by the Muscular Dystrophy Campaign, as part of the charity’s “Building on the Foundations” campaign, which revealed the shocking lack of specialist care in many parts of the country. The lack of knowledge, training and skills in the sector is a significant concern. For example, there is no specific training or curriculum for neuromuscular services in the neurological field.

I am sure that hon. Members will share my concerns and the frustrations of people living with neuromuscular conditions in the north-west, as expressed in the Muscular Dystrophy Campaign’s patient survey in 2010. Deborah Hurst from Liverpool is affected by facioscapulohumeral—FSH—muscular dystrophy. However, she was not diagnosed with it until she was in her late thirties. She is now 47. Her two daughters were born before she was correctly diagnosed and one of them, who is now aged 25, has inherited the condition. Deborah says:

“I have two daughters and I knew one was affected, but the doctor told me I was fussing and silly. When I finally got them tested, he congratulated me on my actions, which I was very mad about as my daughter took her diagnosis very badly at the time and ‘congratulations’ was not what we wanted to hear.”

Mrs H from Lancashire has a son affected by a neuromuscular condition. She says that in her experience, GPs do not understand such conditions:

“My GP is very good but says, ‘We have about two hours of tutorial on muscular dystrophy in the whole medical training.’ So therefore they have no in-depth knowledge.”

Elaine Sands from Stockport is also affected by FSH muscular dystrophy. She does not receive any specialist care, but she would value the support of a neuromuscular care adviser. She says:

“As I am housebound, it would be nice to have someone give me physiotherapy and also some kind of visitor who knows about my condition, as I live alone and I would appreciate being able to talk to someone who understands the disease.”

However, the situation is different for Joanne Ashton. She has a five-year-old son, Liam, who has Duchenne muscular dystrophy. Through the specialist neuromuscular service at Alder Hey hospital, Joanne and her family have access to a specialist consultant, Dr Stefan Spinty, and to a full multidisciplinary service, including a neuromuscular care adviser. Joanne says:

“Shirley, our Care advisor, is fantastic. We only have to ask and she’s there. Liam had his wish granted, through the “Make A Wish Foundation”, because he was nominated by Shirley. So we are all impressed with the care advisors.”

The Muscular Dystrophy Campaign’s clinician-led report, “Building on the Foundations in the North West”, which was published in June 2009, made a number of findings about neuromuscular services in the north-west.

[Mr Gregory Campbell (East Londonderry) (DUP)]

I thank the hon. Gentleman for giving way and I congratulate him on securing this debate. He has rightly referred to the issue of care advisers. Obviously I understand that the north-west of England is his particular concern, but does he agree that if more emphasis were put on having excellent care advisers right across the UK, like the adviser who he has just referred to, that would help those who suffer from this condition immensely in trying to come to terms with it?

[Graham Evans]

I thank the hon. Gentleman for that intervention. I wholeheartedly agree. When someone is suffering from a condition, there is nothing quite like having people trying to help who understand what they are going through, both physically and mentally. So I wholeheartedly agree.

As I was saying, the “Building on the Foundations in the North West” report found that three out of four neuromuscular patients and their families have no access to a key worker or a care co-ordinator. About 6.5 regional care advisers are needed to serve the estimated 8,000 people —which is up from an earlier estimate of 6,500 people—in the north-west area who have a neuromuscular condition. Many of those people are simply not known to providers of health services.

The report also found that neuromuscular patients have very limited access to treatment, in particular to ongoing physiotherapy. Specialist physiotherapists are required to support outreach clinics and to provide training and professional development for community physiotherapists. In the north-west, two fifths of neuromuscular patients said that they do not receive enough physiotherapy.

Another finding of the report was that there is no dedicated physiological service for neuromuscular patients, despite the importance of such a service as part of multidisciplinary care for that patient group, who have rare and very progressive conditions. Those conditions are often genetic, there are no known cures and there are only limited treatments available. Greater support at the transition from paediatric services to adult services is needed, given the evidence that services are removed or greatly reduced when patients leave paediatric services, even though their needs may well increase given the progressive nature of many of these conditions.

[John Pugh (Southport) (LD)]

I thank the hon. Gentleman for giving way and I congratulate him on raising an important subject. In fact, he highlights a very significant problem. If I understand him correctly, he is saying that we need an adequate clinical network for the rare disease that he is speaking about and a range of providers need to be joined seamlessly, as it were, in some way for the good of the patient. Normally, we expect the strategic health authority to identify the failures to provide such a seamless service and to somehow levy the PCTs to deliver it, which the patients expect and deserve. Is he saying that the central problem is how such a service will progress under the new arrangements, when PCTs, SHAs and other such organisations, which are mandated to resolve these problems, no longer exist?

[Graham Evans]

I thank my hon. Friend for that intervention. He makes a very good point. The new arrangements are a threat, but they are also an opportunity, because services are currently provided by the PCTs but not all of them understand these neuromuscular conditions. The new arrangements are a real opportunity for the Muscular Dystrophy Campaign to get its point across, so that we get off on the right foot when the changes come in. However the GP-led consortia really need to understand and appreciate what is required. It is about having a holistic approach. Shortly I will discuss an excellent neuromuscular centre in Cheshire, which makes a huge difference to patients. It encompasses physiotherapy and the other aspects of care that make life so much more comfortable for those people who have muscular dystrophy.

[John Pugh]

I apologise to the hon. Gentleman and to you, Mr Davies, because I will not be able to stay for the duration of the debate. Consequently, I will not find the answer to the problem that I have just referred to.

[Graham Evans]

Okay. I thank the hon. Gentleman.

[George Hollingbery (Meon Valley) (Con)]

I apologise to my hon. Friend for intervening immediately after another intervention. He has just raised an interesting point about the transition from paediatric services to adult services. In my own area of the south-central region, which is clearly outside the geographical area that is the focus of this debate, we have recently managed to secure at least an advertisement for a new care adviser in the area. However, I understand that that care adviser will not be able to advise on paediatric conditions but only on transition and adult services. Is it not the case that we need a full range of services for all sufferers of this disease and a proper range of advice across all ages?

[Graham Evans]

My hon. Friend makes a very good point and I totally agree. A holistic approach is needed and there should be a seamless transition from being a young person and receiving paediatric services to receiving adult services. However, this point does not just apply to muscular dystrophy. For example, lots of mental health care services suffer from similar problems. Nevertheless, my hon. Friend is quite right. There should be a seamless transition from one service to another.

Greater support at transition from paediatric to adult services is necessary to provide evidence of services being removed or greatly reduced when patients leave paediatric services, even though, as I have said, their needs may well increase given the progressive nature of many of these neuromuscular conditions. There is a three-monthly transitional clinic in Manchester and a monthly transitional clinic that alternates between Alder Hey children’s hospital and the Walton centre, which are both in Liverpool. Both those services—in Manchester and Liverpool—are extremely vulnerable and entirely dependent on the availability of their respective consultants. A transitional clinic at Preston is evolving, but it is not formally funded and is based on the good will of the clinicians. The transitional clinic at Alder Hey children’s hospital is the only one that is attended by the required multidisciplinary team. None of the transitional services in the region is funded or appropriately staffed.

The Walton report, published in August 2009, expressed the concerns that the all-party group on muscular dystrophy developed as a result of its inquiry into access to specialist neuromuscular care. Martyn Blenkharn, chair of the Muscular Dystrophy Campaign’s North West Muscle Group, expressed in written evidence to the all-party group his frustrations about attempts to access NHS physiotherapy:

“No hydrotherapy available in the area - private arrangements can be made, but no assistance from NHS physiotherapists can be obtained. Compared with the previous PCT area (North Cumbria) which covered the area where I used to live, the service in my current PCT area (North Lancashire) is totally unsatisfactory - the quality of care from those who treat me directly is fantastic, but their hands are tied to provide what is really needed.”

The all-party group concluded in its regional summary about the NHS North West region:

“We heard from clinicians in the North West region about the problems facing the multi-disciplinary North West Neuromuscular Network - founded and chaired by Dr Stefan Spinty, Consultant Paediatric Neurologist and lead neuromuscular clinician at Alder Hey Children’s Hospital, Liverpool. Dr Spinty is currently managing this vital network single-handedly without any funding. We believe that the Network should at least be supported by a Network Coordinator. Greater support at transition from paediatric to adult services is also needed, given the evidence of services being removed or greatly reduced when people leave paediatric services. Existing transition services are extremely vulnerable and we were concerned to learn from clinicians that none of the transitional services in the region are funded nor appropriately staffed. Having met with commissioners from the North West Specialised Commissioning Group to discuss these concerns, local clinicians and representatives from the Muscular Dystrophy Campaign were disappointed to be told that there are competing priorities in the region, and that PCT budgets were under strain. We urge the North West SCG and the local PCTs to undertake a service review to address the weaknesses highlighted by clinicians, patients and the Muscular Dystrophy Campaign.”

The NHS North West specialised commissioning group has taken significant steps forward since those findings, and I will outline them shortly.

The North West Muscle Group raises awareness of neuromuscular conditions and provides a forum for people living with the condition to share experiences and advice about access to local and regional services. Since its launch in June 2009, the group has been actively campaigning to improve access to neuromuscular services in the region, engaging with MPs and with the NHS North West specialised commissioning group. Pressure from the North West Muscle Group, alongside the Muscular Dystrophy Campaign and the all-party group, was instrumental in ensuring that the NHS North West specialised commissioning group conducted a thorough review of neuromuscular services.

Joanne Ashton, a member of the North West Muscle Group, commented:

“I’m glad I joined the Muscle Group. It means I’ve got all this information early on and I won’t come up against a brick wall later - I feel I’ve been pre-armed. It’s nice to know I’m not on my own and it’s good to meet other people at different stages. Every day I imagine how bad things could get, but at the Muscle Group I met young lads with Duchenne muscular dystrophy who are in wheelchairs and they’re fantastic, they’re doing so well. Everything had a real positive spin on it too, and I realised that it’s not all doom and gloom.”

The campaign in the north-west was further spurred on by a comment made by Mike Farrar, NHS North West’s chief executive, to Lord Walton of Detchant at an event in Parliament in November 2009. Having referred to the review of neuromuscular services in the south-west, which Sir Ian Carruthers, NHS South West’s chief executive pushed through, Mike Farrar commented:

“Anything Sir Ian Carruthers can do, we can do better”.

As a result of lobbying by the Muscular Dystrophy Campaign, a review of services was set up in the NHS South West specialised commissioning group in February 2008. In February 2009, local primary care trusts approved a new £1 million neuromuscular strategy to reduce the £6.9 million spent in the region on unplanned emergency admissions to hospital for people with neuromuscular conditions.

I am fully aware, as are several hon. Friends, of the excellent work by the NeuroMuscular Centre in Winsford, Cheshire, which is in the constituency of my hon. Friend the Member for Eddisbury (Mr O'Brien). That centre provides ongoing specialist physiotherapy, which reduces the number of falls among people with neuromuscular conditions, and it also offers social enterprise opportunities. However, although the physiotherapy provision is regarded as excellent by those who go to the centre, difficulties are encountered with PCTs and funding, as reported in the Muscular Dystrophy Campaign’s patient survey 2010. Mr A from Cheshire has Charcot-Marie-Tooth disease and travels to the centre for specialist physiotherapy and hydrotherapy, but his local PCT is unwilling to pay, despite not offering him any alternative physiotherapy or hydrotherapy provision. Following concerted pressure by the Muscular Dystrophy Campaign, the North West Muscle Group and the all-party group, Jon Develing, chief officer of the NHS North West specialised commissioning team, made a personal commitment to the all-party group in January to recommend that the NHS North West specialised commissioning group undertake a review of neuromuscular services.

The review was set up by the commissioning group in May 2010, and the steering group set up an effective model for the NHS working with the Muscular Dystrophy Campaign, patients and expert clinicians from the region. To further involve people living with neuromuscular conditions, a stakeholder day was held in September 2010, which represented a vital opportunity for people to have their say about the services that are crucial for an acceptable standard of specialist neuromuscular care in the region. That led to a gap analysis and a report with recommendations for service improvements— the first time ever that neuromuscular services have been looked at in this way, across the whole region.

The recommendations are currently being put to local primary care trusts to approve new investment for a neuromuscular strategy. The proposed minimal required investment to reduce the £13.6 million spent on unplanned emergency admissions to hospital for neuromuscular conditions and to sustain neuromuscular services across the north-west includes 2.5 new fully NHS-funded care advisers, 2.5 specialist consultants, two to three specialist physiotherapists and one specialist neuromuscular nurse.

Will the Minister agree to write to the specialised commissioning group and to PCTs, endorsing the recommendations as necessary steps to save money and reduce unplanned emergency admissions? Will he ensure that a named individual in the NHS in the north-west will take the work forward once the specialised commissioning groups disappear? The neuromuscular network approach is hugely important to ensure the co-ordination of neuromuscular services in specialist multidisciplinary teams, as demonstrated in the south-west of England and by the Scottish Muscle Network, and to ensure links to the primary and secondary care that plays a crucial part in the management of neuromuscular conditions.

Dr Stefan Spinty, consultant paediatric neurologist at Alder Hey children’s hospital, who played a leading role in the north-west neuromuscular service review, said:

“In order to improve patient outcomes and help to reduce unnecessary and costly hospital admissions, it is important that the recommendations from the North West Specialised Commissioning Group report, which is due to be published soon, are implemented. All neuromuscular clinicians who have been involved in the North West neuromuscular service review are committed to support the implementation process to further improve and secure service provision for individuals affected by neuromuscular conditions in the future.”

Christine Ogden, a campaigner and fundraiser from Bolton, whose grandson has Duchenne muscular dystrophy, said:

“It is vital, that essential increase in care, support and advice for families outlined in the North West Specialised Commissioning Group’s report recommendations, which will make a real difference for families living with muscle disease. It is also so important that information for health professionals about neuromuscular conditions is significantly developed.”

Beryl Swords from Liverpool, whose son John has FSH muscular dystrophy, has commented on the provision of physiotherapy for adults with neuromuscular conditions:

“I feel strongly that physiotherapy for adults with muscular dystrophy is totally inadequate. If improvements were made to North West neuromuscular services, then perhaps this area of care could be more freely available, keeping people with the condition mobile for longer.”

The urgency of improving neuromuscular services in the north-west is encapsulated in the following comments by Nicci Geraghty, a north-west campaigner and fundraiser:

“I have two nephews with Duchenne muscular dystrophy. This is not only a cruel condition for my nephews to suffer from, but also very harrowing at times for us as a family to watch each stage of progression knowing that the postcode lottery already exists throughout the country. This has a huge impact on survival rates and I don’t wish to see my nephews robbed of any chance they have to lead the best quality of life possible.”

The evidence provided makes a compelling case for the urgent necessity of developing and improving neuromuscular services in the north-west in order to save money. Will the Minister write to NICE stressing the importance of a NICE quality standard for Duchenne muscular dystrophy, as well as one on home ventilation and respiratory support? Will he attend the next meeting of the all-party group on muscular dystrophy on Wednesday 9 March to update the group on progress, and will he agree to meet clinicians who have been developing a neuromuscular curriculum with the Association of British Neurologists? Thank you for calling me, Mr Davies.