Section 1: Epidemiology and symptomatology
Motor neurone disease (MND) is a group of related diseases characterised by progressive degeneration of motor neurones of the brain and spinal cord. It includes amyotrophic lateral sclerosis (affecting 65 per cent of MND patients) and progressive bulbar palsy, where speech and swallowing are affected (25 per cent). Less common are progressive muscular atrophy (10 per cent) and primary lateral sclerosis (about 2 per cent).
MND has an incidence of about two per 100,000 of the population. At least three people die every day from MND in the UK and it affects more than 5,000 people at any one time.
Despite medical advances, MND remains incurable. Riluzole, the only treatment licensed for MND, appears to reduce its rate of progression and was recommended by NICE for the amyotrophic lateral sclerosis form of MND in 2001.
Other treatments can improve quality of life, but do not arrest progression.1 On average, patients die within three to five years of onset. Difficult practical and ethical questions about quality of life, choice and end-of-life decisions inevitably arise.
The patient's GP, with the help of a co-ordinated multidisciplinary team, plays a pivotal part in managing overall care.2
Palliative care starts at diagnosis, which can be difficult because of the insidious nature of the condition.2 Physical symptoms and the speed and pattern of progression can vary widely.
Progressive muscle weakness can affect the limbs, trunk, neck, speech and swallowing, resulting in wasting or loss of function and total dependence on others. Symptoms can occur at any time from presentation onwards and are varied (see box).2
Pain may be musculoskeletal in origin and may respond to NSAIDs and physiotherapy.
Cramps from spastic muscle can be helped by muscle relaxants, and discomfort from unchanging position and skin pressure may require regular analgesics and often opioids.3
| MND symptoms |
|Symptoms experienced by patients with MND|
Section 2: Nutrition and communication
Up to 20 per cent of MND patients become malnourished and they have been shown to have an energy requirement 10 per cent higher than average.
Patients with MND should be recommended to follow a high-calorie, high-protein diet immediately after diagnosis.
Nutrition should be monitored. A 10 per cent weight loss in six months or less is cause for concern.
There are a number of interventions that can aid nutrition as the disease progresses, such as food fortification, percutaneous endoscopic gastroscopy (PEG) or percutaneous radiological gastroscopy (PRG).4
Decisions about these interventions should involve the patient, dietician, and speech and language therapists. Adequate training for the support team and carers is essential.2
A PEG or PRG can be used to aid nutrition when swallowing becomes difficult. A PEG is suitable early in the disease when the patient has good respiratory function. A PRG can be inserted in more advanced disease, for patients with a forced vital capacity (FVC) <50 per cent.
When swallowing becomes difficult and the patient starts having problems with choking gastrostomy should be considered. Insertion is safer when it is undertaken before swallowing has deteriorated and when respiratory function is reasonable.5
More than 65 per cent of MND patients appear to suffer from frontal lobe dysfunction. This can result in cognitive deterioration making decision-making more difficult.2 Speech impairment can also occur, often resulting in a total inability to communicate. This can be one of the most distressing aspects of MND for both patient and family.
It is important when communicating with MND patients to have patience and allow the patient to finish, rather than interrupt them when it first appears clear what it is they wish to convey.
A speech and language therapist should be involved as soon as any changes in voice or articulation occur.2
Voice amplifiers may be helpful for patients with good articulation but a weak voice.
Alternatives to speech aids include writing devices. One such device employs sensitive switches that respond to the slightest muscle movement of the user.
Other practical solutions include a personal passport or communications book, containing information about the patient and their likes and dislikes. An alphabet board can help with spelling out words.
Equipment should be introduced to the patient well in advance, so that they can become comfortable and competent with it.
Section 3: Respiratory support
As the diaphragm, intercostal and abdominal muscles weaken breathing problems will occur in most MND patients.
Indicators of respiratory problems include daytime somnolence, morning headaches and poor concentration, which all point to nocturnal hyperventilation (see box).
FVC reflects respiratory muscle strength and serial measurements may be useful in predicting the onset of respiratory failure.2 Methods, such as sniff nasal inspiratory pressure measurement, may be more reliable. Referral to a respiratory specialist may be necessary.2
Non-invasive ventilation (NIV) may be considered when the FVC is less than 50 per cent of that predicted and it is helpful to discuss more invasive ventilation with patients and carers at an early stage.
NIV is portable and easy to use at home. It is especially helpful for patients who become breathless lying down and may extend life by several months.6
However, it can be restrictive because it prevents eating and talking.
Careful discussion should occur when NIV is started, so patients and their family are aware of the problems that may be experienced.
Other management of respiratory failure, including invasive ventilation with a tracheostomy or palliative measures, should be discussed and considered at an early stage.
The use of advance statements and advance decisions to refuse treatment may also be considered.2
Episodes of acute respiratory distress can create anxiety in patients and fear of suffocation is not uncommon.7
Distress caused by breathlessness can be treated and much of the anxiety can be removed by discussing the issues with patients and carers.
Leaflets for patients, families and professionals are available from the Motor Neurone Disease Association.
They also provide a box with two compartments, supplied on a named-patient basis to the GP.
One compartment is designed to hold medication that may be administered by the carer (midazolam to be given buccally) and the second holds medication to be used by a GP or nurse (injections of morphine, midazolam and glycopyrronium bromide).
The patient can keep the box at home so medication is readily available in case of emergency and any crisis can be eased at home, without the need to consider admission to hospital.
|Signs of respiratory insufficiency|
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Section 4: Patient and family concerns
MND patients commonly have fears and concerns about their diagnosis, symptoms, and how they will cope with disability and dependence. They also have fears about death and dying.
The patient's carers share many of these anxieties and are often concerned about their ability to communicate with the patient.
Many carers try to take on the burden of understanding the patient's needs and they may also be worried about finances and coping with the process of death and dying.2
For some patients questions of spirituality can arise. It may be helpful for a chaplain or spiritual counsellor to contact the patient a few weeks after diagnosis, or a patient may ask for this contact nearer the end of their life.
Dying at home
Most patients with MND wish to die at home. This requires close co-operation and integrated care from health and social services, and in some cases, specialist care systems, such as hospices.
MND patients require care from a multidisciplinary team including the patient's GP and practice team, clinical nurse specialists, occupational therapy, speech and language therapy, dieticians, social workers and the specialist palliative care team.
Specialist palliative care nurses can provide further advice on symptom management, or for respite or terminal care as required by the patients family.
The patient's desire to control their own care is often overlooked and this can lead to a sense of frustration among patients who have no way of preventing their MND from progressing, but feel that they need to be allowed autonomy.
With the high media profile given to cases of euthanasia and assisted suicide, the patient must be reassured that their death need not be distressing.
Most patients with MND die from pulmonary infections or respiratory failure.
Contrary to popular belief, death by choking is very rare and the final stages of MND are usually peaceful and dignified.8
A living will or advance statement may be considered so that everybody is aware of the patient's wishes. When considering specific treatment options, an 'advanced decision to refuse treatment' may be completed.
The views of the patient and the patients family should be regularly reviewed.9
- This article was originally published in MIMS Oncology and Palliative Care. To register to receive copies go to www.hayreg.co.uk/specials
- MND Association www.mndassociation.org
- Scottish MND Association www.scotmnd.org.co.uk
- Patient factsheet on PEG feeding can be found at www.corecharity.org.uk
- Oliver D. Motor Neurone Disease - a Family Affair (second edition). Sheldon Press, London, 2006.
- Oliver D, Borasio G, Walsh D (eds). Palliative Care in Amyotrophic Lateral Sclerosis - from diagnosis to bereavement (second edition). Oxford University Press, Oxford, 2006.
- Nielson S, Clifford-Rose F. Motor neurone disease - the 'at your fingertips' guide. Class Publishing, London, 2003.
1. Anderson P, Borasio G, Dengler R et al. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol 2005; 12: 921-38.
2. Oliver D, Borasio G, Walsh D (eds). Palliative Care in Amyotrophic Lateral Sclerosis - from diagnosis to bereavement (second edition). Oxford University Press, Oxford, 2006.
3. Oliver D. Opioid medication in the palliative care of motor neurone disease. Palliat Med 1998; 12: 113-5.
4. Heffernan C, Jenkinson C, Holmes T et al. Nutritional management in MND/ALS patients: an evidence based review. Amyotrophic Lateral Scler Other Motor Neuron Disord 2004; 5: 72-83.
5. Miller R, Rosenberg J, Gelinas D et al. The care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology 1999; 52: 1,311-23.
6. Heffernan C, Jenkinson C, Holmes T et al. Management of respiration in MND/ALS patients: an evidence based review. Amyotrophic Lateral Scler Other Motor Neuron Disord 2006; 7: 5-15.
7. Bourke S, Tomlinson M, Williams T et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomized controlled trial. Lancet Neurol 2006; 5: 140-7.
8. Neudert C, Oliver D, Wasner M et al. The course of the terminal phase in patients with amyotrophic lateral sclerosis. J Neurol 2001; 248, 612-16.
9. Oliver D. The quality of care and symptom control - the effects on the terminal phase of MND/ALS. J Neuro Sci 1996; 139 (Suppl), 134-6.