National Institute for Health and Clinical Excellence (NICE)
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NICE hopes to help people with motor neurone disease breathe more easily

People with motor neurone disease could live longer and more comfortably if their respiratory problems are identified and managed more effectively. This is what NICE is hoping to achieve from its new clinical guideline on the management of non-invasive mechanical ventilation, out yesterday.

There are around 5,000 people in the UK who have motor neurone disease; a degenerative and incurable condition which causes everyday functions such as breathing, speaking, swallowing, and moving to become increasingly difficult as muscle strength declines.

The majority of people with motor neurone disease eventually have to use mechanical aids, such as nasal masks and mouth pieces, to support their breathing, which are proven to improve quality of life.

However, as there are variations in clinical practice across the NHS, many people who require breathing support may not be receiving the best available care. This may be due to delays in identifying and monitoring their respiratory impairment or inconsistencies in providing information about the available treatments and when non-invasive ventilation should be considered.

Dr Fergus Macbeth, Director of the Centre for Clinical Practice at NICE said: “This is the first time that evidence-based recommendations have been published for the NHS outlining how respiratory impairment should be identified in people who have motor neurone disease, as well as when non-invasive ventilation should be considered as a treatment option.”

“Respiratory impairment is the main cause of death for people with motor neurone disease, and non-invasive ventilation has been proven to increase survival and improve the quality of life if it is used correctly and at the right times. I therefore encourage all healthcare professionals involved in treating these vulnerable patients to consult our clinical guideline so that they can ensure they are providing the highest possible quality of care.”

Among the recommendations, the clinical guideline calls for:

  • Healthcare professionals to offer to discuss non-invasive ventilation with the patient soon after diagnosis or when their respiratory function is being tested or declines, or if the patient asks for information so that they are aware of it as a treatment option.
  • Healthcare professionals to test and monitor their patient’s respiratory function at least every three months, depending on their rate of progression, individual preferences and signs and symptoms of impairment so that any change can be recorded and discussed with the patient.
  • Families and carers to be involved in discussions surrounding treatment (providing patient consent is obtained), so that they know what to expect and can offer their support with the management of the equipment, if required.
  • A multidisciplinary team to be involved in the patient’s respiratory assessment and subsequent treatment plans, including a neurologist, a respiratory physician, respiratory and motor neurone disease specialist nurses, a physiotherapist, a physiologist, a speech and language therapist and a palliative care specialist.
  • Healthcare professionals to offer to discuss arrangements for end-of-life care with the patient at an appropriate time and in a sensitive manner, such as when they are finding that they are becoming increasingly dependent on non-invasive ventilation, or if they decline treatment.

Professor Gary McVeigh, a Consultant Physician and Chair of the Guideline Development Group said: “Treating motor neurone disease can be complicated as it requires the management of a variety of medical problems; a key symptom being increased respiratory weakness.

“There isn’t one test that can determine when a person should consider using non-invasive ventilation to support their breathing - respiratory function has to be monitored regularly and systematically over time and treatment should always take into account the patient’s individual needs and preferences.

“This is why a range of medical and nursing healthcare professionals should be involved at every stage so that decisions about the person’s care can be informed and tailored to their individual needs and circumstances.”

Kenneth Allott, patient member of the Guideline Development Group said: “Having had motor neurone disease for nine years, I now cannot move my arms or legs and have weakness in my neck and breathing muscles. Sixteen months ago I was offered non-invasive ventilation to support my breathing at night; however, as my breathing ability has declined, I now depend on this equipment most of the time. The advantage of this is that it has extended my life for at least a year so far.

“I hope that this guideline from NICE will reassure patients, like me, who have motor neurone disease and require breathing support that they are receiving the best care they can. Particularly I hope it will improve the opportunities for their respiratory function to be tested and monitored, so that non-invasive ventilation can be discussed with the patient and considered as a treatment option at an appropriate time for anyone who is in need.”

Karen Pearce of the Motor Neurone Disease (MND) Association said: "MND is a devastating, fatal disease with no cure and no real treatment. Many people affected by the disease develop problems breathing as their breathing muscles weaken.

"Research has proved non-invasive ventilation (NIV) can improve the quality of life and the length of survival for many people with MND. However we believe that many who would benefit from NIV are not being offered it. We hope that the new clinical guideline will mean more people have access to this intervention if they choose, and if it is the right treatment for them. We welcome the guideline which will improve care during such a difficult time in their life."

Notes to Editors

About motor neurone disease (MND)

  • MND is an incurable neurodegenerative condition, which causes muscular paralysis and wastage as the brain and spine cells (called motor neurones) deteriorate. This causes a progressive loss of mobility and difficulties with speech, swallowing and breathing.
  • MND affects up to 5000 people in the UK.
  • The incidence of MND increases with age, with a mean onset of 63 years.
  • About 5% of people with MND have a close family relative with the condition.
  • Less than 10% of people with motor neurone disease survive beyond 10 years.
  • Some people with MND need to use mechanical ventilation to support their breathing - these systems are known as non-invasive ventilation and they involve air being sucked in, filtered and pumped into the lungs via a nasal mask, oronasal mask or mouthpiece. To begin with it is usually used to relieve symptoms of hypoventilation intermittently at night. As respiratory muscle strength declines, daytime non-invasive ventilation may then need to be considered.

About the clinical guideline

View the NICE clinical guideline on Motor neurone disease: non-invasive ventilation.

About NICE

  1. The National Institute for Health and Clinical Excellence (NICE) is the independent organisation responsible for providing national guidance on the promotion of good health and the prevention and treatment of ill health.
  2. NICE produces guidance in three areas of health:
  • public health - guidance on the promotion of good health and the prevention of ill health for those working in the NHS, local authorities and the wider public and voluntary sector
  • health technologies - guidance on the use of new and existing medicines, treatments, procedures and medical technologies within the NHS
  • clinical practice - guidance on the appropriate treatment and care of people with specific diseases and conditions within the NHS.

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