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© ALS Independence 2003-11
23-Jan-08
Contributed by Dr David Oliver of the Wisdom Hospice,
Rochester, honorary senior lecturer at Kent Institute of Medicine and Health
Sciences, University of Kent.
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).
Incurable
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
Symptom control
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
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
Gastrostomy
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
Cognitive deterioration
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.
Speech therapy
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
Ventilation
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
Respiratory distress
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
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
Spiritual evaluation
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.
Living will
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
Resources
Useful websites
- 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
Further reading
- 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.
References
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
"Be not afraid of life. Believe that life is
worth living, and your belief will help create the fact."
William James
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© ALS Independence 2003-11