AAN Practice parameter guidance

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Supplementary table 1 – Comparison of respiratory and nutritional management guidelines for ALS
AAN Practice parameter guidance
20091,2
EFNS/EALSC Working Group Good
practice points3
MND Association evidence-based
recommendations4,5
Enteral nutrition
Enteral nutrition via PEG
for maintenance of
nutrition
Timing of gastrostomy
Enteral nutrition via PEG should be
considered to stabilize body weight
PEG is the standard procedure for enteral
nutrition in ALS
Insufficient data exists to support or refute
specific timing of PEG insertion in patients
with ALS
An individual approach should be utilized
taking into account bulbar symptoms,
malnutrition (weight loss >10%), respiratory
function and the patient's general condition.
Early operation is highly recommended
Alternatives to PEG in
patients with poor
respiratory function
Palliative NGT is recommended for feeding of
high-risk patients (FVC <30%) and patients
who have declined PEG

PEG and prolonging
survival
PEG and QOL
PEG should be considered for prolonging
survival in patients with ALS
Insufficient data exists to support or refute
PEG for improving QOL in patients with ALS
There is no convincing evidence that PEG
prolongs survival
There is no convincing evidence that PEG
improves QOL



RIG is a suitable alternative when PEG is
deemed hazardous
NGT can be used for short-term feeding
and when PEG or RIG is not suitable
PEG helps to stabilize weight, combat
malnutrition and is a suitable means for longterm nutritional maintenance
An individualized approach to the timing of PEG
is advocated. The patient should be informed
that PEG might not be applicable later on in the
disease progression

RIG should be used when patients have
poor respiratory function

NGT can be considered for short-term
feeding and when PEG or RIG is not
suitable
Randomized controlled trials are recommended
Randomized controlled trials are recommended
Respiratory management
Optimal pulmonary tests
to detect respiratory
insufficiency


Nocturnal oximetry should be considered
for monitoring hypoventilation (regardless
of the FVC)
Supine FVC and MIP could be useful in
routine respiratory monitoring, in addition
to the erect FVC
SNP should be considered to detect
hypercapnia and nocturnal hypoxemia


VC should be considered for respiratory
monitoring on a regular basis (if possible
measured both standing/sitting and lying)
SNP should be considered for monitoring
of inspiratory muscle strength, particularly
in bulbar patients who cannot perform VC
accurately
Nocturnal oximetry is recommended for
patients with symptoms suggestive of
nocturnal hypoventilation




Check for symptoms and signs suggestive
of respiratory insufficiency
Spirometry (especially SNIP) is useful in
detecting early respiratory deficiency and
predicting the course of respiratory failure
For patients with severe bulbar disease who
cannot perform respiratory function tests,
use signs of respiratory insufficiency and
measure arterial blood gases (including
bicarbonate)
Measure arterial blood gases and perform
Criteria for initiating NIV
treatment
Orthopnea or SNP <40cm H20 or MIP <-60
cm H2O or abnormal nocturnal oximetry or
FVC <50%
NIV, respiratory function
and survival
NIV should be considered to treat respiratory
insufficiency in ALS, both to prolong survival
and to slow the rate of FVC decline
At least one of the following: (a) dyspnoea, (b)
orthopnea, (c) disturbed sleep not due to pain,
(d) morning headache, (e) poor concentration,
(f) loss of appetite
(g) excessive daytime sleepiness (ESS>9)
and signs of respiratory muscle weakness
(FVC <80% or SNP <40cm H2O) and
evidence of either: significant nocturnal
desaturation on overnight oximetry, or
morning blood-gas PCO2 >6.5 Kpa

NIV and TIV can prolong survival for
many months and can improve patients'
QOL

Assisted ventilation and
QOL


Improving compliance
with NIV
Targeted respiratory
interventions for clearing
secretions
NIV can be considered to enhance QOL
in patients with ALS who have respiratory
insufficiency
TIV should be considered to preserve
QOL in patients with ALS who want longterm ventilatory support
NIV should be considered at the earliest sign
of nocturnal hypoventilation or respiratory
insufficiency

MIE should be considered to clear
secretions in patients with ALS who have
reduced peak cough flow (<270L/min)

Insufficient data exists to support or
refute HFCWO for clearing airway
secretions in patients with ALS
NIV should be considered before TIV in
patients with symptoms of respiratory
insufficiency
NIV and TIV can prolong survival for many
months and can improve patients' QOL
overnight oximetry in any patient with
symptoms suggestive of nocturnal
hypoventilation
FVC <50% (or even <70%) or serum chloride
<98 mEq/1 or signs of diaphragmatic weakness
on CXR. For patients with moderate or severe
bulbar symptoms, invasive rather than
noninvasive ventilation should be considered
Use noninvasive ventilation or invasive
ventilation for alleviating breathlessness
Use NIPPV or bi-level positive airway pressure
ventilators to provide noninvasive ventilation,
depending upon local availability and expertise

Not addressed
NIPPV and bi-level positive airway pressure
ventilators have been shown to improve
QOL and survival in MND/ALS patients

Invasive ventilation improves QOL and
survival for patients and should be
considered for all appropriate patients, on
the basis of a fully informed decision by the
patient and carer
Not addressed

Not addressed



Teach the patient and carers manual
assisted cough
Provide a portable home suction device
and a room humidifier
Consider using a mucolytic, for example
N-acetylcysteine, 200–400mg three times
daily
If these measures are insufficient, try a
nebulizer with saline and a β-receptor
Use of invasive
ventilation via
tracheostomy in patients
with ALS
Consider invasive ventilation of patients on
NIV who are unable to maintain pO2>90%, or
pCO2 <50mmHg or who are unable to
manage bronchial secretions
Withdrawal of NIV or
invasive ventilation in
ALS


Insufficient data exists to support or
refute specific strategies for withdrawal
of ventilation in ALS
Protocols based on consensus for
withdrawal of mechanical ventilation in
intensive care units include counseling
and symptom control with opioids,
benzodiazepines and anticholinergic
medications
antagonist and/or an anticholinergic
bronchodilator and/or a mucolytic and/or
furosemide in combination

The use of a MIE might be helpful,
particularly in the setting of an acute
respiratory infection

Cricopharyngeal myotomy might be
helpful in rare cases when frequent
episodes of cricopharyngeal spasm occur
with severe bronchial secretions
Consider using invasive ventilation via
tracheostomy in patients with severe bulbar
dysfunction who are not tolerant of NIV and
whose respiratory symptoms decline on NIV
(pCO2<50mmHg; pO2 = 65mmHg and FiO2
<30mmHg for at least 10 hours)
 Parenteral diamorphine, a
benzodiazepine and an antiemetic should
be used when the patient decides that
ventilatory support should be withdrawn
 For symptomatic treatment of dyspnea
with opioids and/or oxygen, no controlled
studies in ALS exist
Consider invasive ventilation when noninvasive
ventilation is not appropriate due to bulbar
symptoms and when noninvasive ventilation is
no longer effective due to progression of disease



Advance directives are essential and should
be reviewed periodically
Since a mentally competent and informed
patient has the right to refuse or discontinue
any treatment including life support and
mechanical ventilation, those responsible
for care must not prolong ventilation against
the patient's will
If withdrawal is requested, appropriate
treatment should be continued to ensure
that symptoms such as anxiety, distress
and discomfort are managed effectively
Abbreviations: AAN, American Academy of Neurology; ALS, amyotrophic lateral sclerosis; CXR, chest X-ray; EALSC, European ALS Consortium; EFNS,
European Federation of Neurological Societies; ESS, Epworth sleepiness scale; FiO2, Fraction of inspired Oxygen; FVC, forced vital capacity; HFCWO, high
frequency chest wall oscillation; MIE, mechanical insufflation–exsufflation; MIP, maximal inspiratory pressure; MND, motor neuron disease; NGT, nasogastric
tube; NIPPV, noninvasive positive pressure ventilation; NIV, non-invasive ventilation; PEG, percutaneous endoscopic gastrostomy; QOL, quality of life; RIG,
radiologically inserted gastrostomy; SNIP, sniff nasal inspiratory pressure; SNP, sniff nasal pressure; TIV, tracheostomy invasive ventilation.VC, vital capacity
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respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy
of Neurology. Neurology 73, 1218–1226 (2009).
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care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality
Standards Subcommittee of the American Academy of Neurology. Neurology 73, 1227–1233 (2009).
3. Andersen, P. M. et al. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and
clinical care of patients and relatives. Eur. J. Neurol. 12, 921–938 (2005).
4. Heffernan, C. et al. Nutritional management in MND/ALS patients: an evidence based review. Amyotroph. Lateral Scler.
Other Motor Neuron Disord. 5, 72–83 (2004).
5. Heffernan, C. et al. Management of respiration in MND/ALS patients: An evidence based review. Amyotrophic Lateral
Sclerosis 7, 5–15 (2006).
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