Health: Neurological Conditions

Lord Macdonald of Tradeston Excerpts

Thursday 8th December 2011 Lords Chamber

[Lord Macdonald of Tradeston]

My Lords, I declare an interest as patron of the Dystonia Society. Dystonia is a neurological condition that causes involuntary and sometimes very painful muscle spasms as a result of incorrect signals from the brain. These muscle spasms can force affected parts of the body into abnormal movements or postures. There are thought to be over 70,000 people in the UK who have some form of dystonia, of whom 8,000 are children. It is a cruel condition in that the earlier it starts, the more it spreads. Dystonia that appears in childhood often starts in an arm or a leg. The limb twists and contorts, and the condition then spreads to other areas, perhaps affecting the other arm or leg, the torso, and sometimes the whole body. It can cause severe difficulties with standing, walking and even sitting. Dystonias that appear in adulthood usually affect only one or two parts of the body. The most common types affect the neck or the eye. Neck dystonia usually forces the head and neck to one side, which can be extremely painful, while eye dystonia forces the eyelids shut, sometimes causing functional blindness. Other parts of the body that can be affected include the hands, voice, mouth and tongue. Dystonia can have a devastating impact. The pain and disability caused by muscle spasms and the unpredictability of the symptoms take a heavy toll on an individual’s quality of life.

Dystonia is unusual in that it is not degenerative, but it is also not curable, so people with dystonia often have to live with its disabling symptoms for 40 to 50 years or more. It is therefore essential that they receive treatment that effectively mitigates their symptoms. This can make the difference between a lifetime of disability, relying on others for care and benefits, or a life of economic independence, actively contributing to society. The most common treatment for dystonia is injections of botulinum toxin, which temporarily paralyse the spasming muscle. Where botulinum toxin and other treatments do not work, the treatment of last resort is deep brain stimulation. An electrode is implanted in the brain and connected by an internal cable to a battery implanted in the chest. The electrode sends a pulse that blocks the incorrect signals in the brain, stopping the involuntary spasms.

Support for people with dystonia is provided by the Dystonia Society, a UK charity which aims to ensure that everyone living with dystonia has access to the support and treatment they need in order to enjoy the best possible quality of life. The society also provides information to those who need it, and advocacy where patients are not receiving the treatment they require. It also provides networks and events to enable isolated patients to share experiences and be given encouragement. The Dystonia Society also works with clinicians and commissioners to improve practice in treating dystonia and to raise awareness of this too little known condition. I draw the attention of the Minister to the sad fact that currently there are a number of shortcomings in the provision of treatment for dystonia.

First, because of a worrying lack of awareness among medical professionals, diagnosis takes far too long, at an average of two and a half years for those who do get diagnosed. The Lancet has estimated that at least a third of cases are undiagnosed. Of course, a GP may see only a few cases of dystonia over a career and cannot be expected to recognise every condition; but far too often, symptoms that clearly indicate a likely neurological problem are dismissed as psychological. There is therefore an urgent need to ensure that GPs are given more effective guidance on investigating symptoms where they are not sure of the diagnosis.

Secondly, there are problems with the provision of funding for the treatment of last resort, deep brain stimulation, in some parts of the country. For a small minority of patients in the UK, around 50 a year, other treatments are ineffective, so they require deep brain stimulation as the final option; otherwise they have no alternative to a life of severe disability, which also results in extra costs for the NHS and society in paying for hospital stays, carers and welfare. Some regional specialised commissioning groups have recognised the importance of deep brain stimulation for this small group of patients who are most desperately in need of treatment. In 2010, the East of England and the South West Specialised Commissioning Groups conducted reviews and, on the basis of the evidence, approved funding. Conversely, in the east Midlands, a blanket decision has been taken not to fund deep brain stimulation for dystonia, a dismaying decision which I hope can be reconsidered, since it ignores the overwhelming clinical support for this treatment and fails to recognise its considerable value in reducing costs to the NHS and society. The Dystonia Society is concerned that deep brain stimulation could be under threat as specialised commissioning moves under the auspices of the NHS Commissioning Board. The danger is that access will level down rather than up—for instance, extending the policy of the east Midlands rather than adopting that of the south-west or east of England, where treatment is routinely funded.

There is also a problem with the funding of botulinum toxin in some areas for the rarer types of dystonia such as voice and hand dystonia, despite this treatment being recommended as good practice by the European Federation of Neurological Societies. As these conditions are rare, it is not judged economically viable to obtain Medicines and Health Care products Regulatory Agency licensing despite the treatments being shown to be effective. It seems most unjust that people are being denied treatment simply because their condition is not widespread—the red tape here could surely be cut. In addition, the provision of important additional support, such as pain management and speech therapy, is very variable across the UK.

The main fear is that the number of cases where effective treatment is not provided will rise. As cost pressures increase, hidden cost savings may be made through an extension of the period between botulinum toxin injections. As the benefits of botulinum toxin wear off in around 12 weeks, it is essential that treatment is repeated promptly at clinically appropriate intervals. Dystonia is a lifetime condition, and if those affected are to remain active contributors to society, it is essential that their symptoms are continuously managed. If injections are de1ayed, they may experience a cycle where they have two or three months of being effective and then one or two months of disability as they await the next injection, which is surely an unacceptable compromise. Clearly it would be very difficult for someone to hold down a job or look after children in such a scenario.

I thank my noble friend Lord Dubs for initiating this important debate and for giving me this opportunity to draw to the attention of the Minister and his department the under-recognised needs of the 70,000 people who suffer with the disabling and painful neurological condition of dystonia.