Guidelines for the physiotherapy management of MND

✓Supports this

“Get a proper assessment and training before relying on any transfer method or equipment.”

The MND physiotherapy guideline makes this a professional's job rather than something a family works out alone. It asks for a manual handling risk assessment plus handling education for the carers, so that transfers are carried out at a level of risk that is acceptable.

The physiotherapist should complete a manual handling risk assessment and provide education on handling to carer(s) so that assisted exercises, transfers or mobility are performed at an acceptable level of risk.

Recommendation 3.4b.1R

✓Supports this

“Manually lifting someone who cannot support their own weight risks a fall for them and an injury for the caregiver.”

Treats lifting and handling as a two-sided safety problem as weakness progresses, and adds that providing hoists or handling belts early reduces the load on carers and prevents musculoskeletal injuries to them.

As the disability progresses a patient's care needs may require the application of proper lifting and handling techniques, implemented in ways that ensure the safety of the patient and the carer.

Section 3.4 Treatment Management

✓Supports this

“Safe manual handling protects both the person with ALS/MND and the person helping them.”

Makes a manual handling risk assessment and carer education a key recommendation, so that assisted exercise, transfers and mobility happen at an acceptable level of risk. It adds that handling should be done in ways that keep both the person and the carer safe, and that early provision of hoists or handling belts prevents secondary musculoskeletal injuries.

The physiotherapist should complete a manual handling risk assessment and provide education on handling to carer(s) so that assisted exercises, transfers or mobility are performed at an acceptable level of risk.

Key recommendations, 3.4b.1R

✓Supports this

“Training a caregiver in handling and shoulder care early can reduce shoulder pain, which is a common problem in MND.”

The physiotherapy MND guideline gives this as a key recommendation for pain, and elsewhere notes that shoulder pain is often attributable to shoulder weakness, spasticity and joint damage, and that poor manual handling technique can also cause it.

Early training on correct manual handling, shoulder care and range of movement exercises can minimise shoulder pain, a common problem in MND.

Key recommendations, 3.4d.1AA

✓Supports this

“An ankle-foot orthosis is often used where foot drop makes walking unsteady or causes tripping.”

The physiotherapy MND guideline says many people with MND will need an ankle-foot orthosis and that an off-the-shelf one is often enough to support walking. It adds that where a custom device is needed, referral should be early, because deterioration can be rapid and orthotic waiting times long.

Many patients will require an ankle foot orthosis (AFO).

Section 3.4b.1 Recommendations, item M

✓Supports this

“Positioning someone in good alignment can reduce pain, help prevent pressure sores and lower the effort of breathing.”

The physiotherapy MND guideline makes this a key recommendation, and names the same three effects the answer's list does: less pain, fewer pressure sores and less effort needed to breathe. It grades the recommendation as expert consensus rather than trial evidence.

Positioning of the patient in a good alignment while sitting or supine can decrease abnormal muscle tone; prevent or reduce spasticity, contractures, and pain; prevent the development of pressure sores; and minimise the effort required for respiration.

Key recommendations, 3.4b.1O

✓Supports this

“There is no single physiotherapy review schedule that fits everyone with ALS/MND.”

The physiotherapy MND guideline makes this a key recommendation: how often someone is seen is decided person by person, not by a fixed interval.

Frequency of input should be determined on an individual basis.

Key recommendations, 3.4a.1A

✓Supports this

“It is reasonable to ask for an urgent physiotherapy review when something suddenly becomes unsafe.”

The same key recommendation that leaves the frequency of routine input open also says people with MND should be able to reach services urgently when a need arises, and that they should be seen as a priority for assessment.

However, patients should be able to access services urgently when the need arises.

Key recommendations, 3.4a.1A

✓Supports this

“Physiotherapy goals in ALS/MND shift with the stage of the illness, from maintaining mobility early on to comfort and quality of life later.”

The physiotherapy MND guideline sets goals by stage. Early on the aim is to maintain and optimise mobility and function; in the middle stage it is to keep functional mobility going, manage pain and watch closely for respiratory problems; and late-phase goals are designed to maximise quality of life.

The goal in the early stages of MND is to maintain, and optimise, mobility and function.

Section 3.3.1 Recommendations, item B

✓Supports this

“Medicines for spasticity are used judiciously, where spasticity is limiting function and muscle strength is still relatively unimpaired.”

Cites a systematic review which concluded that antispasticity medicines should be used judiciously, where spasticity limits function but muscle strength is still relatively unimpaired. The guideline's own recommendation K says the same, and adds that the physiotherapist's role is to liaise with the medical team about limitations associated with increased tone.

A systematic review of spasticity in MND concluded that judicious use of antispasmodic agents should be considered where spasticity is limiting function, but where muscle strength remains relatively unimpaired.

Section 3.4b.2 Evidence, item K

!Qualifies this

“Whether antispasticity medicines such as baclofen genuinely reduce spasticity, or whether they can cause harm by increasing muscle weakness, is an open research question rather than a settled effect.”

States the weakness concern as something research still has to resolve, not as an established effect, and names further limitation of mobility and function as the harm at issue.

✓Supports this

“Stiffness, cramps and spasticity are common problems in ALS/MND.”

The physiotherapy MND guideline covers the part the survey does not. It lists cramps and spasticity among the skeletal symptoms of the condition and describes spasticity as a common feature.

Spasticity is another common feature of MND, and its effects can be severe, both in general disability and in pain, significantly impacting on mobility and function.

Section 3.4b Maintain Mobility and Function

Used across the whole answer

✓Supports this

“Physiotherapy in ALS/MND aims to keep someone safe, comfortable, mobile and independent for as long as possible, rather than to rebuild lost muscle.”

The physiotherapy-specific MND guideline gives the same aim. Because the condition is progressive, treatment is directed at maximising function through aids, mobility strategies and respiratory management rather than at improving strength.

Often the goal of physiotherapy in the care of people with MND is not to improve impairment in strength or mobility due to the progressive nature of the condition.

Section 3.4 Treatment Management

✓Supports this

“Light to moderate strengthening exercise is most likely to help earlier in ALS/MND.”

Recommends individualised strengthening exercise in the early stage of MND, on the basis of two small randomised studies. It describes the evidence as emerging and the trials as small, so this is a recommendation rather than a settled finding.

Positive evidence is emerging to indicate that individualised strengthening exercise programmes during the early stages of MND are probably effective in improving the function of patients and should be recommended.

Section 3.4b.2 Evidence, item F

Used across the whole answer

✓Supports this

“A soft collar can be enough for occasional use such as transfers or travelling, with a more supportive one as neck muscles weaken.”

The physiotherapy MND guideline draws the same distinction. An off-the-shelf foam collar can meet someone's needs where it is wanted for occasions such as transfers, toileting or travelling in a vehicle, while more rigid and supportive collars may be recommended as the disease progresses, to maintain head and neck alignment.

Persons with MND often need a collar as their neck muscles become weaker.

Section 3.4b.1 Recommendations, item N

✓Supports this

“Equipment and adaptations in ALS/MND are usually better planned earlier than later.”

The physiotherapy MND guideline makes timeliness a key recommendation in its own right, and elsewhere advises early orthotic referral where a custom device may be needed, because deterioration can be rapid and orthotic waiting times long.

Timely provision and regular review of aids and appliances is essential in maximising the patient's function.

Key recommendations, 3.4b.1L

!Qualifies this

“Stretching and range-of-motion exercises help maintain comfort, flexibility, positioning and everyday care routines.”

The physiotherapy MND guideline records that no randomised trial of stretching has been done in MND. It still recommends stretching and passive movements, on the basis that they are widely believed to help maintain joint mobility and manage spasticity and related pain, carrying that belief across from spinal cord injury and intensive care.

No randomised controlled trial of stretching has been looked at in patients with MND.

Section 3.4b.2 Evidence, item C

✓Supports this

“A suitable cushion and being able to change position help protect the skin from pressure damage.”

Names preventing pressure sores as one of the things good sitting alignment achieves in MND, alongside less pain and less effort spent on breathing, and treats getting that position right as physiotherapy and occupational therapy work.

Positioning of the patient in a good alignment while sitting or supine can decrease abnormal muscle tone; prevent or reduce spasticity, contractures, and pain; prevent the development of pressure sores; and minimise the effort required for respiration.

Key recommendations, 3.4b.1O

✓Supports this

“Being supported in a good sitting position helps with comfort, pressure relief, breathing and repositioning.”

Sets out what good sitting alignment achieves in MND: less pain, prevention of pressure sores, and less effort needed for breathing. It names high back wheelchairs and riser-recliner chairs among the equipment used to support and correct that position as the condition progresses.

Positioning of the patient in a good alignment while sitting or supine can decrease abnormal muscle tone; prevent or reduce spasticity, contractures, and pain; prevent the development of pressure sores; and minimise the effort required for respiration.

Key recommendations, 3.4b.1O

✓Supports this

“Equipment and adaptive aids are a standard part of ALS/MND care for everyday activities such as eating, dressing, washing and jobs around the home.”

The MND physiotherapy guideline says the same thing independently, and adds that aids need reviewing over time as well as providing promptly, because what helps changes as the condition does.

Timely provision and regular review of aids and appliances is essential in maximising the patient's function.

Key recommendations, 3.4b.1L

!Qualifies this

“Planning and pacing techniques may help people pace themselves and reduce fatigue.”

The MND physiotherapy guideline does recommend teaching energy conservation, but it puts it more tentatively than this sentence does. It says the techniques may encourage pacing and may reduce fatigue, and the (4) marks it as the guideline's lowest evidence level, expert opinion and formal consensus rather than trial evidence.

Teaching energy conservation techniques may encourage patients to pace themselves and reduce levels of fatigue (4).

Section 3.4e Fatigue Management, recommendation GG

✓Supports this

“Poor sleep contributes to daytime fatigue in ALS/MND, and treating what is disrupting sleep can improve it.”

The MND physiotherapy guideline lists impaired sleep among the things that contribute to fatigue, and its fatigue section then works through the treatable case: night-time under-breathing is common in MND, it is thought to be a major reason sleep is poor, and non-invasive ventilation improves poor quality sleep and quality of life.

Nocturnal hypoventilation is a common symptom of MND and is thought to contribute significantly to impaired sleep.

Section 3.4e.2 Evidence, FF

✓Supports this

“Fatigue is one of the most commonly reported symptoms in ALS/MND, and several different things can contribute to it.”

The MND physiotherapy guideline's fatigue section covers both halves of this. It calls fatigue commonly experienced and worse than in matched people without MND, and it then lists what feeds into it: excessive activity, stress, depression, pain, poor cardiopulmonary function, medication and disturbed sleep.

Fatigue is a commonly experienced debilitating symptom, and can impact on quality of life for patients with MND (Lou et al., 2003).

Section 3.4e Fatigue Management

✓Supports this

“Some of the things that cause fatigue in ALS/MND can be treated, night-time breathing among them.”

The guideline treats night-time breathing as a contributing cause of fatigue that has a treatment. It names poor cardiopulmonary function and impaired sleep as contributing factors, says nocturnal hypoventilation is common in MND and contributes to poor sleep, and recommends that physiotherapists refer for non-invasive ventilation where it applies.

Assistive devices such as NIV have been shown to improve hypercapnic symptoms such as headaches, restlessness, nightmares and poor quality sleep, and therefore improve QOL in patients with MND (Lou et al. 2010).

Section 3.4e.2 Evidence, FF

✓Supports this

“Both occupational therapists and physiotherapists work on energy conservation in ALS/MND.”

Covers the physiotherapy half. This is an MND guideline written for physiotherapists, and teaching energy conservation techniques sits inside its own fatigue management recommendations, as something the physiotherapist does. Advice on energy conservation also appears in its early, middle and late stage physiotherapy plans.

Teaching energy conservation techniques may encourage patients to pace themselves and reduce levels of fatigue (4).

Section 3.4e Fatigue Management, recommendation GG

✓Supports this

“A clinical team can look for causes of fatigue that can be treated, including night-time breathing changes and poor sleep.”

Sets out both halves. It names the contributing factors worth looking for, including poor cardiopulmonary function, impaired sleep, pain, depression and medication, tells clinicians to work on those factors and to bring in other professionals where needed, and gives night-time under-breathing as the worked example, with referral for non-invasive ventilation.

Management strategies should be directed at minimising factors known to impact on these contributing factors, referring to other health professionals as necessary.

Section 3.4e Fatigue Management

✓Supports this

“Weakened breathing in ALS/MND usually shows up during sleep before it shows up in the day.”

Says respiratory muscle weakness impairs gas exchange particularly at night, and that the symptoms of the carbon dioxide retention this causes are night-time and early-morning ones: disturbed sleep, morning headaches and fatigue.

!Qualifies this

“Sniff and mouth pressure tests pick up change in the breathing muscles sensitively, and in some people earlier than other tests do.”

Describes sniff nasal inspiratory pressure as correlating well with diaphragm strength and as sensitive to changes in respiratory muscle strength. It does not compare how early it detects change against lung function testing, so it backs the sensitivity but not the comparison.

✓Supports this

“NIV is normally started for night-time use, because weakened breathing tends to affect gas exchange during sleep first.”

Says respiratory muscle weakness in MND impairs gas exchange particularly at night, and that overnight non-invasive ventilation has become the standard treatment once breathing is failing.

Nocturnal non-invasive positive-pressure ventilation (NIPPV) has become the standard treatment for MND patients with respiratory insufficiency.

Section 2.3 Medical Management

✓Supports this

“Once people have settled into NIV, better sleep, waking more rested, more daytime energy and fewer morning headaches are what is commonly reported.”

Names disturbed sleep, morning headaches and fatigue as the common symptoms of the carbon dioxide retention that weakened breathing causes, and says overnight ventilation improves gas exchange and sleep quality. So the four things people report improving are the four the guideline links to the problem being treated.

✓Supports this

“Bilevel support takes over part of the work of breathing, which can make breathing feel easier and improve sleep.”

Describes a bilevel ventilator as triggered by the person's own effort to breathe in, and says it reduces the work of breathing and improves gas exchange and sleep quality.

✓Supports this

“NIV is usually started for night-time use, because weakened breathing tends to affect gas exchange at night first.”

Says respiratory muscle weakness in MND impairs gas exchange particularly at night, and that overnight non-invasive ventilation has become the standard treatment once breathing is failing.

Nocturnal non-invasive positive-pressure ventilation (NIPPV) has become the standard treatment for MND patients with respiratory insufficiency.

Section 2.3 Medical Management

✓Supports this

“Oxygen on its own is generally not prescribed for respiratory muscle weakness in ALS/MND, because it risks suppressing the drive to breathe when carbon dioxide is already raised.”

States that oxygen is generally not prescribed in MND, and gives the reason: it risks suppressing the drive to breathe when carbon dioxide in the blood is already raised.

In general, oxygen is not prescribed so as not to risk inhibition of respiratory drive in the setting of elevated serum carbon dioxide levels.

Section 2.3 Medical Management

✓Supports this

“Weak breathing muscles affect gas exchange during sleep before the problem is obvious in the daytime.”

Its section on medical management says weak breathing muscles impair gas exchange particularly at night, and that the carbon dioxide building up then shows itself as disturbed sleep, morning headaches and fatigue. Its fatigue section calls night-time under-breathing a common symptom of MND.

Nocturnal hypoventilation is a common symptom of MND and is thought to contribute significantly to impaired sleep.

Section 3.4e.2 Evidence (Fatigue Management)

✓Supports this

“Weak breathing muscles affect gas exchange at night before they show up in the day.”

Its section on medical management says weak breathing muscles impair gas exchange particularly at night, and that the carbon dioxide building up then shows itself as disturbed sleep, morning headaches and fatigue. Its fatigue section calls night-time under-breathing a common symptom of MND.

Nocturnal hypoventilation is a common symptom of MND and is thought to contribute significantly to impaired sleep.

Section 3.4e.2 Evidence (Fatigue Management)

Supports a claim in this answer

In MND, oxygen is generally not prescribed, because of the risk of inhibiting the drive to breathe when carbon dioxide is already raised

✓Supports this

“In ALS/MND the breathing problem is often a build-up of carbon dioxide from weak breathing muscles, not simply a shortage of oxygen.”

Describes the problem as impaired gas exchange from weak breathing muscles, with carbon dioxide retention named as what produces the symptoms. It does not set carbon dioxide against oxygen: its own NIV criteria at Table 2.3.2 accept significant nocturnal desaturation on overnight oximetry as an alternative to a morning PCO2 above 6.5 kPa.

Respiratory muscle dysfunction results in impaired gas exchange, particularly at night, with symptoms of carbon dioxide retention including sleep disturbance, morning headaches and fatigue common

Section 2.3 Medical Management

✓Supports this

“Giving extra oxygen in ALS/MND without specialist assessment can do harm, because it can suppress the drive to breathe when carbon dioxide is already raised.”

Gives exactly this reason, and puts it more strongly than the answer does: oxygen is generally not prescribed in MND at all, because of the risk of suppressing the drive to breathe when carbon dioxide is already high.

In general, oxygen is not prescribed so as not to risk inhibition of respiratory drive in the setting of elevated serum carbon dioxide levels.

Section 2.3 Medical Management

✓Supports this

“The UK guideline recommends considering a cough assist device during a chest infection. Randomised trials up to 2020 had not measured whether it changes how infections turn out, though other studies report fewer hospital admissions and longer survival for cough support with non-invasive ventilation.”

The source behind the second half of this sentence, from outside the randomised trials. The Irish physiotherapy guideline reports fewer hospital admissions and longer survival, but for maximum insufflation and assisted coughing techniques taken together, particularly alongside non-invasive ventilation at times of respiratory insufficiency, not for a cough assist device on its own.

Maximum insufflation or in/exsufflation strategies and assisted coughing techniques have been shown to decrease hospitalisation, and prolong survival, particularly when used in conjunction with non-invasive ventilation

Section 3.4c.2 Evidence, item W

✓Supports this

“A small suction device can clear secretions from the mouth and upper airway when coughing is not enough on its own.”

The Irish physiotherapy guideline for MND recommends oral suction for upper airway secretions that are hard to clear, and says people commonly use it at home. It adds that suctioning further down the throat is not usually done in MND, because assisted cough techniques are preferred and more comfortable.

Oral suctioning using a yanker is useful in patients with upper airway secretions which are difficult to clear.

Section 3.4c.1 Recommendations, item Z

✓Supports this

“A breathing test can measure how forceful a cough is, and show when it has become too weak to clear the airway.”

The Irish physiotherapy guideline says peak cough flow should be measured regularly in MND, and gives the reading below which a cough counts as too weak to clear the airway.

A peak cough flow of less than 270 l/min indicates that the cough is ineffective for airway clearance, with a value of greater than 160 l/min critical in preventing the onset of respiratory failure during a respiratory tract infection (Bott et al., 2009; Senent et al., 2011).

Section 3.4c.2 Evidence, item T

✓Supports this

“An effective cough depends on several muscle groups working together, and ALS/MND can weaken each of them.”

The Irish physiotherapy guideline for MND sets out the same steps, breathing in, building pressure and opening the throat, and says all of the muscles behind them can be impaired in MND.

Effective coughing depends on inspiratory muscles to increase lung volumes; expiratory muscles to produce high thoracoabdominal pressures; and on upper airway muscles to co-ordinate glottis closure and opening.

Section 3.4c Respiratory Symptom Management

✓Supports this

“A cough too weak to clear the airway makes chest infections more likely.”

States it directly: an ineffective cough leads to lung collapse and pneumonia, and peak cough flows too low to clear the airway can speed up respiratory decline. Its wording is blunter than this answer's.

Further reading

A physiotherapy-specific MND guideline on the aims of physiotherapy, exercise and supportive equipment (more detailed, clinician-oriented).