Palliative Care in Motor Neurone Disease

Last updated 1 Jun 2026 · Palliative care

📋 Key Information Summary

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Motor neurone disease (MND) is a progressive, fatal neurodegenerative condition with a median survival of 2–3 years from symptom onset; palliative care should be integrated from diagnosis alongside disease-modifying therapy.
Anticipatory advance care planning (ACP) must address communication loss, ventilation preferences, nutrition decisions, and place of death — ideally initiated early while capacity is preserved.
Augmentative and alternative communication (AAC) — including high-tech eye-gaze devices and low-tech communication boards — should be offered proactively by a multidisciplinary team including speech pathologists.
Dysphagia affects >80% of people with MND; regular swallow assessments, texture-modified diets, and timely discussion of percutaneous endoscopic gastrostomy (PEG) or radiologically inserted gastrostomy (RIG) tube placement are essential.
Respiratory failure is the most common cause of death in MND; non-invasive ventilation (NIV) improves quality of life and may extend survival by several months.
Respiratory symptoms including dyspnoea, orthopnoea, excessive daytime somnolence, and morning headaches should prompt urgent respiratory function assessment and arterial blood gas analysis.
Opioids (morphine 2.5–5 mg PO/SC PRN or regular) are first-line for refractory dyspnoea; low-dose benzodiazepines (midazolam 2.5 mg SC) may be added for associated anxiety or breathlessness unresponsive to opioids alone.
Riluzole 50 mg PO BD is the only TGA-approved disease-modifying agent; it modestly extends tracheostomy-free survival (2–3 months) and is PBS Authority Required.
Sialorrhoea (drooling) is managed with glycopyrronium (glycopyrrolate) 200 µg SC/PO TDS, amitriptyline 10–25 mg nocte, or botulinum toxin injections to salivary glands.
Multidisciplinary care (MND nurse coordinator, respiratory physician, speech pathologist, dietitian, physiotherapist, occupational therapist, palliative care physician, psychologist) is strongly associated with improved survival and quality of life.
Aboriginal and Torres Strait Islander Australians in the Tiwi Islands and parts of Arnhem Land have among the highest recorded incidence rates of MND globally; culturally safe, community-embedded palliative care pathways are critical.
Ventilation decisions — including acceptance or refusal of tracheostomy invasive ventilation (TIV) and initiation/withdrawal of NIV — require sensitive, repeated conversations tailored to disease stage and patient values.

Introduction & Australian Epidemiology

Motor neurone disease (MND), also known as amyotrophic lateral sclerosis (ALS) in its most common form, is a relentlessly progressive neurodegenerative disorder characterised by the selective degeneration of upper and lower motor neurones in the motor cortex, brainstem, and spinal cord. This leads to progressive weakness of skeletal muscles, culminating in respiratory failure. Palliative care is not a separate phase but an integral component of holistic management from the time of diagnosis, addressing physical, psychosocial, spiritual, and existential needs throughout the disease trajectory.

In Australia, approximately 2,000–2,500 people are living with MND at any given time, with an incidence of approximately 8–9 per 100,000 person-years and a lifetime risk of approximately 1 in 300. The median age of onset is 58–63 years, with a slight male predominance (M:F ratio ≈ 1.5:1). Median survival from symptom onset is 27–30 months, though this varies considerably by subtype — bulbar-onset disease carries a worse prognosis (median ~18–24 months) than limb-onset disease (median ~36–48 months). Approximately 5–10% of cases have a family history (familial MND), with C9orf72 repeat expansion being the most common genetic cause in Australian cohorts.

MND places a substantial burden on patients, families, and health services. The economic cost to the Australian health system is estimated at

.05 billion annually (direct and indirect costs). The disease demands high equipment needs (power wheelchairs, hoists, hospital beds, NIV devices, communication devices, suction machines), intensive allied health involvement, and significant carer support. The MND Australia network, state MND associations, and the National Disability Insurance Scheme (NDIS) are key support structures for Australians living with MND.

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Diagnostic delay: The average time from symptom onset to confirmed MND diagnosis in Australia is 10–16 months. Early referral to a neurologist with MND expertise is essential, as diagnostic delay impairs timely palliative care planning, equipment provisioning, and access to clinical trials.

Pathophysiology

MND arises from the progressive degeneration of both upper motor neurones (UMN) in the primary motor cortex and lower motor neurones (LMN) in the anterior horn of the spinal cord and brainstem motor nuclei. The resulting clinical picture combines UMN signs (spasticity, hyperreflexia, pathological reflexes) and LMN signs (fasciculation, muscle wasting, weakness, hyporeflexia). Several pathophysiological mechanisms are implicated:

Protein misfolding and aggregation: Cytoplasmic inclusions containing TDP-43 (TAR DNA-binding protein 43) are found in ~97% of MND cases. Superoxide dismutase 1 (SOD1) aggregates occur in ~2% (familial SOD1 mutations).
Glutamate excitotoxicity: Impaired glutamate reuptake leads to excessive stimulation of motor neurones, calcium influx, and cell death — the basis for riluzole's mechanism of action.
Neuroinflammation: Microglial activation, astrocytosis, and peripheral immune cell infiltration contribute to a toxic motor neurone environment.
RNA processing defects: The C9orf72 hexanucleotide repeat expansion (GGGGCC) — the most common genetic cause — leads to RNA foci, dipeptide repeat proteins, and haploinsufficiency.
Mitochondrial dysfunction and oxidative stress: Impaired energy metabolism and excessive free radical generation compound motor neurone vulnerability.
Impaired axonal transport: Disruption of cytoskeletal transport leads to distal denervation and neuromuscular junction failure.

The clinical phenotypes reflect the anatomical pattern of neuronal loss: limb-onset ALS (~65%), bulbar-onset ALS (~25%), primary lateral sclerosis (PLS, UMN-predominant, slower progression), progressive muscular atrophy (PMA, LMN-predominant), and progressive bulbar palsy (PBP, early bulbar involvement). About 15% of patients develop frontotemporal dementia (FTD), reflecting the ALS-FTD spectrum and shared TDP-43/C9orf72 pathology.

Respiratory failure occurs due to progressive weakness of the diaphragm, intercostal muscles, and accessory muscles of respiration. Bulbar dysfunction leads to aspiration, impaired cough, and recurrent lower respiratory tract infections — a common terminal event. Autonomic involvement is increasingly recognised and may contribute to sudden death.

Clinical Presentation & Diagnostic Criteria

The clinical presentation of MND depends on the region of onset and the balance of UMN and LMN involvement. Diagnosis relies on clinical assessment supported by electrophysiology, neuroimaging, and exclusion of mimics.

Common Presentations by Subtype

Subtype	Initial Symptoms	Key Signs	Median Survival
Limb-onset ALS	Distal hand weakness, foot drop, tripping, difficulty opening jars	Fasciculation, wasting, UMN signs in different myotome	36–48 months
Bulbar-onset ALS	Dysarthria, dysphagia, nasal speech, tongue fasciculation	Pseudobulbar affect, jaw jerk ↑, tongue wasting	18–24 months
Respiratory-onset ALS	Orthopnoea, exertional dyspnoea, morning headaches, sleep disturbance	Paradoxical breathing, ↓ FVC, ↓ SNIP	18–24 months
PLS (primary lateral sclerosis)	Spasticity, slurred speech, pseudobulbar affect	Pure UMN signs; no LMN features	>10 years

Revised El Escorial / Awaji Criteria

Diagnosis requires evidence of progressive UMN and LMN dysfunction in ≥1 body region, with exclusion of other causes. The Awaji criteria (2008) improve sensitivity by equating fasciculation with fibrillation as LMN evidence. Electromyography (EMG) is essential for confirming widespread LMN involvement.

Key Differential Diagnoses to Exclude

Cervical myelopathy / radiculopathy (MRI spine)
Multifocal motor neuropathy (anti-GM1 antibodies — treatable with IVIg)
Kennedy disease (X-linked bulbo-spinal muscular atrophy)
Inclusion body myositis
Myasthenia gravis
Monomelic amyotrophy
Structural brainstem or foramen magnum lesions

Communication Support

Progressive dysarthria affects the majority of people with MND and is often the presenting symptom in bulbar-onset disease. Loss of intelligible speech is one of the most distressing aspects of MND, profoundly impacting identity, relationships, autonomy, and the ability to participate in advance care planning. Proactive speech pathology input from diagnosis is essential to preserve communication throughout the disease trajectory.

Trajectory of Communication Loss

Early stage

Mild dysarthria; voice becomes quieter, slurred, or hypernasal. Speech rate slows. Fatigue-related worsening. Intelligibility >90% to unfamiliar listeners.

Mid stage

Significant reduction in intelligibility (50–90%). Lofstedt (low-tech) communication aids introduced: alphabet boards, word boards, picture charts. AAC assessment by speech pathologist with assistive technology expertise.

Late stage

Speech functionally unintelligible (<50%). High-tech AAC devices (eye-gaze systems, e.g., Tobii Dynavox) become primary communication mode. Head-switch or EMG-based access if eye movements preserved.

End of life

Even minimal responses (eyelid movement, eye blinks) can convey yes/no. Pre-agreed personal communication protocols with family and care team. Music, touch, and presence as non-verbal communication.

Augmentative and Alternative Communication (AAC)

AAC Level	Examples	Access Method	Australian Access
No-tech / Low-tech	Alphabet board, word board, picture communication book, eye-pointing frame	Hand pointing, eye pointing	Speech pathology department; MND Association loans
Mid-tech	Voice amplifiers, BIGmack™ single-message buttons, recorded phrase devices	Hand press, switch	MND Association equipment loan; NDIS-funded
High-tech	Speech-generating devices (SGDs): Tobii Dynavox, Grid Pad, Predictable™ app on tablet	Eye-gaze tracking, head mouse, touch, switch scanning	NDIS-funded; state AT (assistive technology) services; specialist AAC clinics

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Key principle: AAC must be introduced before speech becomes unintelligible. The "communication window" — when the person can still participate in device selection, vocabulary programming, and voice banking — is limited. Early engagement maximises autonomy and supports advance care planning.

Voice Banking and Message Banking

Voice banking uses synthetic voice technology to create a personalised digital voice from recordings of the person's own voice while speech is still functional. Australian services include Acapela My-Own-Voice and ModelTalker. Message banking records personally meaningful phrases (e.g., "I love you," favourite jokes, greetings) in the person's natural voice for later use on AAC devices. Both are time-sensitive and should be offered at or soon after diagnosis. The MND Australia speech pathology guidelines recommend initiating discussion within the first specialist consultation.

Communication in Palliative Care Settings

Ensure AAC device is available at bedside in hospital, hospice, and residential aged care — it is as essential as a call bell.
Train all members of the care team (including after-hours and locum staff) in basic AAC access.
Prepare a one-page "communication passport" describing the person's preferred communication method, yes/no signals, key vocabulary location on device, and personal preferences.
For end-of-life conversations, ensure quiet environment, adequate time, and allow the person to direct the pace using their AAC system.
Support family members who may experience anticipatory grief related to communication loss; involve psychology/social work early.

Pseudobulbar Affect

Pseudobulbar affect (PBA) — involuntary, uncontrollable episodes of laughing, crying, or both — affects approximately 30–50% of people with MND and can significantly disrupt communication and social interaction. Management options include:

Dextromethorphan/quinidine (Nuedexta®) 20/10 mg PO BD — TGA-approved for PBA; PBS Authority Required. The only agent with RCT evidence for PBA in MND/ALS. Quinidine component requires ECG monitoring (QTc prolongation risk).
SSRIs (citalopram 10–20 mg PO daily, sertraline 50–100 mg PO daily) — second-line; moderate evidence; PBS General Benefit.
Tricyclic antidepressants (amitriptyline 10–25 mg nocte) — useful if concurrent sialorrhoea or insomnia; PBS General Benefit.

Dysphagia & Nutrition

Dysphagia develops in >80% of people with MND during the disease course and is the presenting feature in bulbar-onset variants. It progresses from mild oral-phase difficulty (chewing, forming a bolus) to severe oropharyngeal dysphagia with aspiration risk. Nutritional decline and aspiration pneumonia are major drivers of morbidity and mortality. A proactive, multidisciplinary approach — involving speech pathology, dietetics, gastroenterology, and palliative care — is essential.

Assessment of Swallow

Clinical bedside assessment by speech pathologist: cranial nerve examination, trial of various textures, observation for coughing, wet voice, and delayed swallow reflex.
Instrumental assessment: Videofluoroscopic swallow study (VFSS) or fibreoptic endoscopic evaluation of swallowing (FEES) to objectively characterise swallow impairment, aspiration risk, and guide texture modification recommendations. Available at major tertiary centres across Australia.
Nutritional assessment: Serial weight, BMI, bioelectrical impedance analysis (BIA), albumin/pre-albumin (limited utility in MND due to acute-phase response). Dietetic review at minimum every 3 months, increasing to monthly or more frequently as disease progresses.

Nutritional Management Strategies

Strategy	Indication	Details
Texture modification	Mild–moderate dysphagia	IDDSI framework (International Dysphagia Diet Standardisation Initiative): Levels 0–7. Minced & moist (IDDSI 5) → smooth pureed (IDDSI 4) → mildly thick liquids (IDDSI 2). Supervised by speech pathologist.
Oral nutritional supplements	Inadequate oral intake; weight loss >5%	Ensure®, Fortisip®, Sustagen® — energy-dense (1.5–2.0 kcal/mL). Dietitian-directed. PBS-subsidised under RPBS for eligible conditions.
PEG / RIG tube	Significant dysphagia with aspiration; weight loss >10%; FVC >50% predicted (for PEG); FVC <50% (consider RIG or PIG)	Percutaneous endoscopic gastrostomy (PEG) preferred if FVC >50%. Radiologically inserted gastrostomy (RIG) if FVC <50% or if endoscopy contraindicated. Procedure-related mortality ~1–2%.
Nasogastric tube (NGT)	Short-term bridge (days to weeks); pending PEG/RIG decision; acute illness	Not suitable long-term in MND due to comfort, aspiration risk, and tube displacement. Should prompt PEG/RIG planning if enteral feeding likely to exceed 4 weeks.

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Timing of PEG/RIG insertion: Gastrostomy placement must be considered early — ideally when FVC is still >50% predicted. Delaying until respiratory function is severely compromised increases procedural risk and may preclude safe placement. The decision to insert a gastrostomy should not be made in crisis but through structured advance care planning discussions.

The Nutrition Conversation in Palliative Care

Decisions about artificial nutrition and hydration (ANH) in MND are deeply personal and must be framed within the person's goals of care. Key principles:

Enteral feeding via gastrostomy tube does not prevent aspiration of oral secretions — aspiration risk persists.
Weight loss in late-stage MND is driven by muscle atrophy and hypermetabolism, not solely by inadequate caloric intake; ANH may not reverse cachexia.
Some patients choose comfort-focused care without gastrostomy — this is a valid and supported choice.
Gastrostomy tube can be used for medication administration, hydration, and symptom management even if the person elects to minimise enteral nutrition.
If gastrostomy is inserted, it should be clearly documented as a palliative/comfort measure if that aligns with goals — not as a "life-prolonging" intervention in all contexts.
Withdrawal of ANH in MND is an ethically complex decision that requires multidisciplinary ethics review, palliative care team involvement, and open communication with the family.

Sialorrhoea (Excessive Drooling)

Sialorrhoea in MND is caused by impaired swallowing (not overproduction of saliva) and affects up to 50% of patients. It causes significant social distress, skin maceration, and risk of aspiration. Management options include:

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Glycopyrronium (Glycopyrrolate)

Glycopyrronium bromide · Antimuscarinic

Adult dose 200 µg SC TDS, or 1–2 mg PO TDS; titrate to effect

Route Subcutaneous (preferred in late-stage) or oral

Key side effects Dry mouth, constipation, urinary retention, blurred vision, ↑HR

Renal adjustment Use with caution; reduce dose if eGFR <30 mL/min

PBS status ✔ PBS General Benefit

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Amitriptyline

Endep® · TCA (anticholinergic for sialorrhoea)

Adult dose 10–25 mg PO nocte; titrate slowly to 50 mg if tolerated

Key benefits Also helps insomnia, neuropathic pain, pseudobulbar affect

Caution Sedation, QTc prolongation, constipation, falls risk

PBS status ✔ PBS General Benefit

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Botulinum toxin A

Botox® · Dysport® · Toxin injection to salivary glands

Dose Ultrasound-guided injection to bilateral parotid ± submandibular glands; dose varies by formulation (e.g., onabotulinumtoxinA 50–100 U total)

Duration 3–6 months per injection cycle

Setting Specialist procedure (ENT or neurology); requires ultrasound guidance

PBS status ⚠ PBS Authority Required (for neurological conditions with sialorrhoea)

Ventilation Decisions

Respiratory muscle weakness is inevitable in MND and is the primary cause of death. Ventilation decisions — whether to initiate, continue, escalate, or decline ventilatory support — are among the most consequential choices a person with MND will make. These decisions must be explored proactively, revisited regularly, and supported by honest, compassionate communication.

Types of Ventilatory Support

Mode	Description	Indication	Survival Benefit	Australian Access
Non-invasive ventilation (NIV)	BiPAP via nasal or oronasal mask; typically nocturnal initially, progressing to daytime use	Symptomatic respiratory insufficiency: FVC <80% predicted, ↑PaCO₂, orthopnoea, morning headaches, excessive daytime somnolence, SNIP <40 cmH₂O	Median survival extension ~7–13 months (best evidence in bulbar-intact patients)	Prescribed via respiratory/sleep medicine; CPAP/BiPAP devices funded through state respiratory equipment programs or NDIS; consumables (masks, tubing) ongoing cost
Tracheostomy invasive ventilation (TIV)	Permanent tracheostomy with mechanical ventilator	Patient choice with full understanding; NIV no longer tolerated or adequate	Can extend survival by years (median ~2+ years with full care); requires 24/7 nursing/carer support	Requires ICU admission for insertion; long-term ventilation at home or facility. Enormous care needs. Rarely chosen in Australia (<5% of MND patients)

When to Initiate the Ventilation Conversation

The ventilation discussion should begin early — ideally at or soon after diagnosis, as part of a broader advance care planning conversation. It is not a single discussion but an ongoing dialogue that evolves with disease progression. Key triggers for active discussion include:

FVC declining toward 80% predicted
New-onset orthopnoea or disrupted sleep
Morning headaches or excessive daytime somnolence
Nocturnal oximetry showing sustained desaturation <90%
Rising daytime PaCO₂ (>45 mmHg / >6.0 kPa)
Any acute respiratory illness (e.g., aspiration pneumonia) that decompensates respiratory function

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Bulbar dysfunction and NIV: In patients with significant bulbar impairment, NIV efficacy is reduced due to poor mask seal, air leak, aspiration around the mask, and difficulty tolerating the interface. These patients should still be offered a trial of NIV with appropriate interface selection (e.g., oronasal mask, total face mask, mouthpiece ventilation), but expectations should be realistic and early palliative care referral recommended.

The Advance Care Planning Framework

Explore values and goals

What gives your life meaning? What are you most afraid of? What level of disability would you find acceptable? These questions guide ventilation decisions more than any clinical parameter.

Provide honest information

Explain what NIV involves (mask, machine, adjustments), what TIV involves (tracheostomy, 24/7 care), and what comfort-focused care without ventilation looks like. Written materials from MND Australia are helpful.

Document decisions

Record in an Advance Care Directive (ACD) or Goals of Care plan. In Australia, state-specific ACD legislation applies (e.g., Medical Treatment Planning and Decisions Act 2016 in Victoria). Include substitute decision-maker appointment.

Revisit regularly

Preferences may change. A person who initially declines NIV may reconsider when symptomatic. A person on NIV may wish to withdraw if quality of life deteriorates. Respect autonomy at every stage.

Withdrawal of Ventilatory Support

Withdrawal of NIV or TIV at the patient's request is ethically and legally supported in Australia, provided the patient has decision-making capacity and is making a voluntary, informed choice. This process should involve:

Confirmation of capacity and voluntariness (ideally documented by two senior clinicians)
Clear explanation that withdrawal will result in death — typically within minutes to hours (NIV) or minutes (TIV)
Pre-emptive symptom management: subcutaneous morphine 2.5–5 mg and midazolam 2.5–5 mg for dyspnoea and distress — titrated to comfort
Emotional and spiritual support for patient and family
Involvement of palliative care and ethics team where available
Documentation in the medical record in accordance with the relevant state health legislation

Respiratory Symptoms

Respiratory symptoms are the most clinically significant and distressing features of MND and directly impact quality of life, sleep, and survival. Effective palliation of respiratory symptoms requires a combination of non-pharmacological interventions (positioning, NIV, mechanical insufflation-exsufflation), pharmacological therapy (opioids, benzodiazepines), and timely anticipatory planning.

Symptom Profile

Early Respiratory

FVC 80–60% predicted

Exertional dyspnoea, mild orthopnoea (needing 1–2 extra pillows), early morning headaches, subtle sleep disturbance. May be asymptomatic at rest.

Setting: Outpatient respiratory monitoring; consider NIV trial

Progressive Respiratory

FVC 60–40% predicted

Dyspnoea on minimal exertion, significant orthopnoea, fragmented sleep, excessive daytime somnolence, poor concentration, recurrent lower respiratory infections, weak cough.

Setting: NIV active use; cough assist device; respiratory physician involved

End-Stage Respiratory

FVC <40% predicted

Dyspnoea at rest, unable to lie flat, continuous NIV dependence, CO₂ retention (headache, confusion, asterixis), secretion management difficulty, respiratory failure.

Setting: Palliative care / hospice; comfort-focused respiratory management

Pharmacological Management of Dyspnoea

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Morphine

MS Contin® · Kapanol® · Opioid analgesic

Dose — dyspnoea 2.5–5 mg PO or SC every 4 hours PRN; or 2.5–5 mg SC continuous infusion over 24h via syringe driver if regular dosing needed

Titration Increase by 30–50% every 24–48 hours if dyspnoea uncontrolled

Paediatric dose Not applicable (MND is adult-onset)

Renal adjustment Reduce dose 50% if eGFR 10–50; avoid active metabolites (M6G) — consider fentanyl or hydromorphone if eGFR <10

Key side effects Constipation (always prescribe stool softener), nausea, sedation, respiratory depression

PBS status ✔ PBS General Benefit (palliative care authority for higher doses)

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Midazolam

Hypnovel® · Benzodiazepine

Dose — refractory dyspnoea / anxiety 2.5 mg SC PRN; or 2.5–5 mg SC continuous infusion over 24h via syringe driver. Add to morphine if dyspnoea persists or anxiety is prominent.

Duration Ongoing; titrate to symptom relief

Key side effects Sedation, respiratory depression (additive with opioids), paradoxical agitation

PBS status ✔ PBS General Benefit

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Fentanyl

Durogesic® · Sublimaze® · Opioid analgesic

Indication Alternative to morphine in renal impairment (no active metabolites); transdermal patch useful if swallowing compromised and no PEG/RIG

Dose Transdermal: 12–25 µg/hr patch (equivalent to ~30–60 mg oral morphine/24h). SC: 25–50 µg PRN; or 25–50 µg/h SC infusion.

PBS status ✔ PBS General Benefit

Non-Pharmacological Respiratory Interventions

Positioning: Elevated head of bed (30–45°), prone or side-lying if tolerated. Adaptive seating with tilt-in-space for wheelchair users.
Mechanical insufflation-exsufflation (MI-E): CoughAssist™ device assists with clearance of secretions in patients with weak cough (peak cough flow <270 L/min). Available through MND Association loans and NDIS funding in Australia. Should be used proactively, not just during acute infections.
Manual assisted cough: Abdominal thrust technique timed with cough effort; taught to carers by physiotherapists.
Suctioning: Portable suction machines for oral secretion management in late-stage disease. Thin catheter oral suctioning preferred over nasopharyngeal (discomfort, mucosal trauma).
Secretion management medications: Hyoscine butylbromide 20 mg SC TDS or glycopyrronium 200 µg SC TDS to reduce upper airway secretions (death rattle prophylaxis in last days). Avoid hyoscine hydrobromide (crosses BBB → delirium).
Fan therapy: A handheld fan directed at the face activates trigeminal nerve afferents and reduces the sensation of breathlessness — simple, effective, and patient-controlled.

Respiratory Infections and Aspiration

Aspiration pneumonia is a common terminal event in MND. Management must balance treatment of reversible infection with the person's goals of care. For patients in the comfort-focused phase, antibiotics may be withheld or used selectively for symptom relief (e.g., amoxicillin 500 mg PO TDS for 5 days to reduce fever and purulent sputum distress) rather than curative intent. In patients wishing active treatment, IV broad-spectrum antibiotics per local hospital guidelines should be initiated promptly.

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Opioid safety in MND: Low-dose opioids for dyspnoea in MND do not hasten death when titrated carefully. Under-treatment of breathlessness causes significant suffering. Start low (morphine 2.5 mg SC/PO), titrate to symptom relief, and monitor closely. The principle of double effect applies — the intention is symptom relief, not respiratory depression.

Investigations

Investigations in MND serve three purposes: confirming diagnosis and excluding mimics, monitoring disease progression and respiratory function, and guiding palliative care planning. In the palliative phase, the focus shifts to symptom-directed monitoring rather than diagnostic workup.

Essential

Forced vital capacity (FVC) — seated and supine

Serial measurement every 3 months. Supine FVC drop >25% from seated suggests diaphragm weakness. FVC <50% predicted — urgent NIV discussion; PEG/RIG timing. Available in respiratory function laboratories across Australia.

Essential

Sniff nasal inspiratory pressure (SNIP)

More sensitive than FVC for early respiratory muscle weakness. SNIP <40 cmH₂O or <70% predicted suggests significant respiratory compromise. Can detect decline before FVC changes.

Essential

Arterial blood gas (ABG)

Baseline and when symptomatic (morning headaches, daytime somnolence). PaCO₂ >45 mmHg (6.0 kPa) with respiratory acidosis indicates ventilatory failure. Send with patient seated if possible.

Essential

Overnight oximetry (SpO₂)

Screening for nocturnal hypoventilation. Sustained SpO₂ <90% for >5% of recording time, or nadir <85%, warrants NIV assessment. Can be performed via community respiratory outreach or sleep service.

Available

Peak cough flow (PCF)

Measured using peak flow meter. PCF <270 L/min indicates ineffective cough and need for cough assist device (MI-E). Measure every 3–6 months.

Available

Nerve conduction studies / EMG

Diagnostic (not routinely repeated). Shows widespread denervation with active and chronic changes. Confirmatory alongside clinical assessment using Awaji criteria. Electrophysiologist referral via neurology.

Available

MRI brain and whole spine

Excludes structural mimics (cervical myelopathy, foramen magnum lesion, brainstem tumour). Expected to show corticospinal tract T2 hyperintensity in some cases but not diagnostic in isolation.

Referral

Genetic testing (C9orf72, SOD1, TARDBP, FUS)

Recommended for all MND patients (Royal Melbourne Hospital MND genetics clinic model). Family planning implications. Available through state genetics services; Medicare-funded if clinical criteria met. Genetic counselling pre- and post-test is mandatory.

Available

Videofluoroscopic swallow study (VFSS) / FEES

Objective swallow assessment to guide texture modification and gastrostomy timing. Available at major hospitals; FEES increasingly available in community settings with portable equipment.

Available

Weight, BMI, bioelectrical impedance analysis

Serial monitoring of nutritional status. BMI <18.5 or >5% unintentional weight loss over 3 months — escalate nutritional support and dietetic review. BIA available in some dietetic departments.

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MBS item numbers relevant to MND monitoring: Respiratory function tests (MBS 11503, 11506), nerve conduction studies (MBS 11000-series), dietitian consultations (MBS 10950 — allied health under chronic disease management plan), speech pathology (MBS 10952). Patients with MND are eligible for chronic disease management (CDM) plans enabling Medicare-rebated allied health visits.

Disease-Modifying & Symptom-Targeted Pharmacotherapy

While there is no cure for MND, disease-modifying therapy (riluzole) modestly extends survival and should be offered to all eligible patients. Symptom-targeted medications address specific complications of the disease. All medications must be reviewed regularly and adjusted as swallowing deteriorates.

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Riluzole

Rilutek® · Glutamate release inhibitor

Adult dose 50 mg PO BD (on empty stomach)

Mechanism Inhibits presynaptic glutamate release; may have additional neuroprotective effects

Efficacy Extends median tracheostomy-free survival by ~2–3 months; NNT ~12 for 12-month survival benefit

Key side effects Nausea, fatigue, elevated ALT (monitor LFTs monthly for 3 months, then every 3 months), diarrhoea

Renal adjustment No adjustment required

Notes Can be administered via PEG/RIG tube (crush tablets). Can be ceased at patient request or if side effects intolerable. Decision to cease should be documented in advance care plan.

PBS status ⚠ PBS Authority Required (must be prescribed by or in consultation with a neurologist)

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Edaravone

Radicava® · Free radical scavenger

Dose 60 mg IV infusion daily for 14 consecutive days, followed by 14 drug-free days, then 10 of 14 days per 28-day cycle

Efficacy Slowed functional decline in a selected subgroup of early-stage ALS (RCT); modest effect; not a cure

Australian status TGA-registered but NOT PBS-listed. Out-of-pocket cost significant (~,000–,000 per cycle). Access via compassionate use / special access scheme in limited cases. Not standard of care in Australian palliative MND practice.

PBS status ✘ Not PBS-listed

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Dextromethorphan/Quinidine

Nuedexta® · PBA treatment

Adult dose Capsule 20/10 mg PO BD (dextromethorphan 20 mg / quinidine 10 mg)

Indication Pseudobulbar affect (PBA) — uncontrollable laughing/crying episodes in MND

Key side effects QTc prolongation (quinidine), dizziness, nausea, diarrhoea. ECG required before initiation and if dose adjustment. Avoid with CYP2D6 inhibitors.

PBS status ⚠ PBS Authority Required

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Baclofen

Lioresal® · Muscle relaxant (spasticity)

Adult dose 5 mg PO TDS, titrate to 20 mg TDS if tolerated

Indication Spasticity, cramps (off-label for cramps in MND)

Caution Sedation, weakness (may worsen MND weakness); abrupt cessation can cause seizures

Renal adjustment Reduce dose; start 5 mg daily if eGFR <30

PBS status ✔ PBS General Benefit

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Mirtazapine

Avanza® · NaSSA antidepressant

Adult dose 15–30 mg PO nocte

Indications in MND Depression, insomnia, appetite stimulation, weight gain — multipurpose in palliative MND care

Key side effects Sedation, increased appetite, weight gain, dry mouth

PBS status ✔ PBS General Benefit

Monitoring & Disease Progression

MND monitoring is a continuous, multidisciplinary process aimed at detecting functional decline, initiating interventions at appropriate disease stages, and facilitating timely advance care planning. The MND multidisciplinary team (MDT) should review patients at minimum every 2–3 months, with more frequent contact as the disease progresses.

Monitoring Schedule

Domain	Assessment	Frequency	Key Thresholds
Respiratory	FVC (seated & supine), SNIP, SpO₂, ABG if symptomatic	Every 2–3 months (monthly if declining rapidly)	FVC <80%: NIV assessment. FVC <50%: PEG/RIG timing, TIV discussion. SpO₂ <90% nocturnal: urgent NIV referral.
Swallow / Nutrition	Weight, BMI, clinical swallow assessment, PCF	Every 2–3 months; speech pathology every MDT visit	Weight loss >5%: intensify nutrition support. PCF <270 L/min: cough assist device.
Function	ALSFRS-R (ALS Functional Rating Scale-Revised) — 12-item scale	Every MDT visit	ALSFRS-R decline >1 point/month = rapid progression (worse prognosis)
Communication	Speech intelligibility, AAC assessment, voice banking status	Every MDT visit	Intelligibility <90%: begin AAC discussion. <50%: AAC as primary communication.
Psychosocial	PHQ-9 (depression), GAD-7 (anxiety), carer burden (Zarit Burden Interview), ACP status	Every MDT visit; social work/psychology every 3 months	PHQ-9 ≥10: depression treatment. Carer burden: respite and support referrals.
Riluzole safety	LFTs (ALT, AST)	Monthly for 3 months, then every 3 months	ALT >5× ULN → cease riluzole

ALSFRS-R Trajectory and Prognosis

The ALSFRS-R is a validated 48-point scale (12 items, each scored 0–4) tracking speech, salivation, swallowing, handwriting, cutting food, dressing, turning in bed, walking, climbing stairs, dyspnoea, orthopnoea, and respiratory insufficiency. A decline of ≥1 point per month indicates rapid progression and a median survival of approximately 12–18 months. The ALSFRS-R rate of decline is the most reliable clinical prognostic marker and should be plotted over time to guide advance care planning conversations.

Multidisciplinary Team Composition

Australian MND best-practice care involves a coordinated MDT, ideally led by an MND nurse coordinator (funded through MND Australia state associations and some health services). Core team members include:

Neurologist (MND specialist) — diagnosis, riluzole prescription, prognostication
MND nurse coordinator — care coordination, patient/family support, liaison
Respiratory / sleep physician — NIV initiation, respiratory monitoring
Palliative care physician / team — symptom management, ACP, end-of-life care
Speech pathologist — dysphagia, communication, AAC, voice banking
Dietitian — nutrition, gastrostomy planning
Physiotherapist — mobility, positioning, respiratory physiotherapy
Occupational therapist — equipment, home modification, upper limb function
Psychologist / social worker — mental health, carer support, NDIS/My Aged Care navigation
General practitioner — chronic disease management, CDM plans, end-of-life care in community

Special Populations

👴

Elderly Patients

MND in patients >75 years

Diagnostic confusion with other age-related conditions (cervical spondylotic myelopathy, stroke). Bulbar-onset is more common in the elderly. Prognosis is generally worse — median survival ~18 months from onset. Riluzole benefit preserved; consider polypharmacy and drug interactions. NIV tolerance may be lower; comfort-focused care may be preferred.

Residential aged care facility (RACF) considerations

Staff education on MND disease progression, AAC device use, and feeding modifications essential. Equipment needs (hoist, hospital bed, NIV, suction) must be resourced. MND Australia provides education packages for RACF staff. Consider NDIS access for under-65s or My Aged Care for over-65s for equipment funding.

👨‍👩‍👧

Families & Carers

Carer burden

MND carers report among the highest burden of any neurological condition. Progressive increase in physical care needs (transfers, feeding, toileting, suctioning, NIV management) leads to burnout, depression, and physical injury. Respite care, carer health assessments (MBS item 701/707), and psychology referrals should be proactively offered.

Bereavement support

Anticipatory grief begins early — from diagnosis, loss of speech, loss of mobility. Post-death bereavement follow-up by palliative care team at 6 weeks, 6 months, and 12 months. MND Australia provides peer support for bereaved families.

Children and young people in the family

When a parent has MND, age-appropriate information and psychological support for children is essential. MND Australia provides resources for families with children. Consider school liaison and referral to paediatric psychology services.

🫘

Renal Impairment

Opioid selection in renal failure

Morphine active metabolites (M6G, M3G) accumulate in renal impairment → neurotoxicity, respiratory depression. If eGFR <30: prefer fentanyl (no active metabolites) or hydromorphone. Reduce doses by 50% and extend intervals. Monitor closely for over-sedation.

Gabapentin for cramps

Often used off-label for MND cramps. Renally cleared — significant dose reduction required if eGFR <60 (300 mg daily if eGFR 30–59; 300 mg every other day if eGFR 15–29). PBS General Benefit.

🛡️

Immunocompromised / Comorbid

Diabetes and MND

Enteral nutrition via gastrostomy tube may require insulin adjustment. High-calorie feeds can cause hyperglycaemia. Endocrinology involvement for glycaemic management if HbA1c >53 mmol/mol. Conversely, appetite loss and reduced intake may cause hypoglycaemia in those on oral hypoglycaemics.

Coexistent dementia (ALS-FTD)

~15% of MND patients have comorbid FTD. Impacts decision-making capacity, adherence to treatments (NIV, gastrostomy), carer burden, and advance care planning. Capacity assessment by geriatrician or neuropsychologist should be sought. Substitute decision-makers must be appointed early. Behavioural disturbance may require low-dose risperidone or olanzapine with caution.

End-of-Life Care in MND

End-of-life care in MND is shaped by the choices made throughout the disease — regarding ventilation, nutrition, and place of death. With good advance care planning, most people with MND can die peacefully in their preferred location (home, hospice, or hospital). Palliative care involvement should be offered from diagnosis but becomes intensive in the final weeks to months of life.

Recognising the Dying Phase

Rapid functional decline (ALSFRS-R near zero in all domains)
Severe respiratory failure not responsive to NIV (or NIV declined/withdrawn)
Increasing somnolence, reduced responsiveness
Unable to take oral medications — syringe driver (subcutaneous continuous infusion) required
Profound weight loss, cachexia
Recurrent aspiration events despite maximal management

Symptom Management in the Last Days

Symptom	Management
Dyspnoea / breathlessness	Morphine 2.5–10 mg SC via syringe driver over 24h + 2.5–5 mg SC PRN breakthrough. Add midazolam 2.5–5 mg SC via syringe driver if persistent.
Secretions (death rattle)	Glycopyrronium 200–600 µg SC via syringe driver. Repositioning (semi-prone). Avoid suctioning — distressing for family. Hyoscine butylbromide 20–60 mg SC as alternative.
Pain	Morphine titrated to effect. Consider musculoskeletal pain from immobility (regular paracetamol 1 g via PEG/RIG PRN). Pressure area care essential.
Anxiety / agitation	Midazolam 2.5–5 mg SC PRN or via syringe driver. Haloperidol 0.5–1 mg SC PRN if terminal delirium (assess reversible causes first).
Nausea	Metoclopramide 10 mg SC TDS or haloperidol 0.5–1 mg SC BD. Levomepromazine 6.25–12.5 mg SC if refractory.

✅

Place of death: With appropriate community palliative care support and equipment (syringe driver, suction, hospital bed, NIV if desired), many people with MND can die at home. State palliative care services, MND Association support, and GP involvement are key. Document preferred place of death in the advance care plan and ensure after-hours crisis plans are in place (e.g., Palliative Care After-Hours helpline in each state/territory).

Ethical Considerations

Voluntary assisted dying (VAD): VAD legislation is now enacted in all Australian states (Victoria 2019, Western Australia 2021, Tasmania 2022, Queensland 2023, South Australia 2024, New South Wales 2024). Eligibility requires decision-making capacity, an expected death within defined timeframes (typically 6–12 months), and meeting other legislative criteria. MND patients may be eligible. Clinicians with conscientious objection have a legal duty to refer. Detailed discussion of VAD is beyond this guideline but is a legitimate topic for discussion within advance care planning.
Withholding and withdrawing treatment: Withholding or withdrawing NIV, antibiotics, or artificial nutrition/hydration at the patient's competent request is ethically and legally permissible in Australia, provided it is well-documented and the patient is supported.
Palliative sedation: For refractory symptoms (severe dyspnoea, agitation) unresponsive to maximal pharmacological therapy, palliative sedation may be considered. This is distinct from euthanasia and is ethically accepted when the primary intention is symptom relief. Requires specialist palliative care involvement and clear documentation.

Aboriginal and Torres Strait Islander Health

Aboriginal and Torres Strait Islander Health Considerations in MND

MND has a unique epidemiological significance in Aboriginal and Torres Strait Islander populations. The Tiwi people of Bathurst and Melville Islands in the Northern Territory have among the highest recorded incidence rates of MND in the world (approximately 70–140 per 100,000 per year in some cohorts — up to 15–20 times the national average). Elevated rates have also been observed in parts of Arnhem Land and among some Torres Strait Islander communities. The reasons for this clustering remain incompletely understood, though genetic factors (SOD1 mutations and other variants) and environmental exposures have been investigated. This epidemiological reality demands that palliative care services in northern and remote Australia have specific MND expertise and cultural capability.

Cultural safety in end-of-life care

Death and dying are deeply significant in Aboriginal and Torres Strait Islander cultures. Sorry Business (bereavement and mourning customs) requires that clinicians respect cultural protocols — including avoidance of speaking the deceased person's name, specific burial and ceremonial practices, and community grieving processes. Palliative care teams must work closely with Aboriginal health workers and liaison officers (AHLs) to ensure culturally safe care. The Australian Indigenous Doctors' Association (AIDA) and Palliative Care Australia provide guidance on culturally responsive palliative care.

Remote and very remote access

MND care requires specialist neurology, respiratory medicine, speech pathology, dietetics, and palliative care — services that are scarce or absent in remote NT, WA, and QLD communities. Telehealth (MBS items 99201–99215 for bulk-billed telehealth consultations) is essential for MDT reviews. The Royal Flying Doctor Service (RFDS) and visiting specialist outreach programs provide periodic face-to-face consultations. Equipment (NIV devices, cough assist, hospital beds, hoists, AAC devices) must be freighted to remote communities and maintained locally — a significant logistical challenge.

Family-centred decision making

Aboriginal and Torres Strait Islander approaches to health decisions are often family- and community-centred rather than individual-focused. Advance care planning must involve extended family and, where appropriate, community Elders. The concept of individual autonomy in medical decision-making, while legally important, should be balanced with cultural values of collective decision-making. Sufficient time must be allowed for community discussion — "yarning" — before major decisions such as PEG insertion, NIV initiation, or ventilation withdrawal.

Language and health literacy

English may be a second, third, or fourth language for many Aboriginal and Torres Strait Islander people in remote communities. Health information about MND, palliative care, and advance care planning must be provided in plain English and, where possible, in the person's first language using interpreter services (Aboriginal Interpreter Service in NT, Aboriginal Interpreting WA). Visual and culturally adapted resources (e.g., MND Australia's Aboriginal and Torres Strait Islander resources) should be used. AAC devices may need to incorporate local language vocabulary.

Primary health care coordination

Aboriginal Community Controlled Health Organisations (ACCHOs) are the primary providers of health care in many remote communities. MND care coordination must be integrated with the ACCHO model — Aboriginal health workers and practitioners are often the key point of contact between the person with MND and the broader MND MDT. Education and support for remote health workers in MND management, NIV troubleshooting, and palliative symptom management is critical. The Central Australian Aboriginal Congress (CAAC), Miwatj Health Aboriginal Corporation, and Tiwi Health Board have developed local MND care pathways.

Spiritual and cultural care at end of life

Country (connection to land) is central to wellbeing for many Aboriginal people. Wherever possible, supporting a person with MND to return to or remain on their Country for end-of-life care is a powerful act of palliative care. This may require creative solutions for equipment provision, carer support, and remote clinical oversight. Traditional healing practices may complement Western palliative care and should be respected as part of a holistic care approach. Sorry Business after death may involve prolonged community mourning — bereavement support must be culturally appropriate and sustained.

⚠️

Research priority: The extraordinarily high MND incidence in Tiwi and Arnhem Land communities represents a global research priority. The MND Research Australia consortium, in partnership with Aboriginal community leaders and the Menzies School of Health Research, continues to investigate genetic, environmental, and lifestyle risk factors. Community consent and governance over research participation is essential under the NHMRC guidelines for ethical conduct in Aboriginal and Torres Strait Islander health research (NHMRC 2018).

📚 References

1. Kiernan MC, Vucic S, Cheah BC, et al. Amyotrophic lateral sclerosis. Lancet. 2011;377(9769):942-955. doi:10.1016/S0140-6736(10)61156-7
2. Talman P, Duistermaat E, Pamphlett R, Mathers S. Longitudinal registry of motor neurone disease in Australia — the ALS/MND Registry. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2016;17(5-6):370-376.
3. MND Australia. National MND Clinical Practice Guidelines. MND Australia; 2023. Available at: www.mnda.org.au
4. Connolly S, Bedlack R, Genge A, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Neurology. 2009;73(15):1218-1226.
5. Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) — revised report of an EFNS task force. European Journal of Neurology. 2012;19(3):360-375.
6. National Institute for Health and Care Excellence (NICE). Motor neurone disease: assessment and management. NICE guideline [NG42]. Updated 2022. London: NICE.
7. Oliver DJ, Turner MR. Some difficult decisions in ALS/MND. Amyotrophic Lateral Sclerosis. 2010;11(4):334-338.
8. Radunovic A, Annane D, Rafiq MK, Brassington R, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews. 2017;10(10):CD004427.
9. Aoun SM, Bentley B, Funk L, Toye C, Stajduhar KJ. A 10-year literature review of family caregiving for motor neurone disease. BMC Palliative Care. 2013;12:24.
10. Pamphlett R, Ward S, Kiernan MC, et al. Motor neuron disease and frontotemporal dementia in Australia: clinical and epidemiological perspectives. Internal Medicine Journal. 2020;50(12):1456-1463.
11. Talman P, Henderson RD, Mathers S, et al. Environmental exposures and motor neuron disease in Australia: a systematic review. Australian and New Zealand Journal of Public Health. 2019;43(6):519-525.
12. NHMRC. Values and Ethics: Guidelines for Ethical Conduct in Aboriginal and Torres Strait Islander Health Research. Canberra: National Health and Medical Research Council; 2018.
13. Palliative Care Australia. National Palliative Care Standards. 5th ed. Canberra: Palliative Care Australia; 2018.
14. Bede P, Oliver D, Stodart J, et al. Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives. BMC Neurology. 2011;11:127.
15. Australian Institute of Health and Welfare (AIHW). Amyotrophic lateral sclerosis (ALS) and other motor neurone diseases in Australia. AIHW; 2023. Cat. no. PHE 320.
16. Van den Berg LH, Sorenson E, Gronseth G, et al. Airlie House ALS Clinical Trials Guidelines. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2019;20(5-6):317-338.
17. Berlowitz DJ, Howard ME, Fiore JF Jr, et al. Impact of non-invasive ventilation on clinical outcomes in motor neuron disease: a prospective Australian cohort study. Respirology. 2016;21(5):879-886.
18. Blackhall LJ. Amyotrophic lateral sclerosis and palliative care: where we are, and the road ahead. Muscle & Nerve. 2012;45(3):311-318.

for PBS scripts. Utilise ACCHS pharmacies and Remote Area Aboriginal Health Worker programs for medication supply in remote areas. Avoid initiating benzodiazepines; support holistic pain management including community-based exercise programs.

Preventive health

Promote bone health: encourage vitamin D supplementation (1000 IU daily in deficient individuals), smoking cessation support, reduction of alcohol intake, and weight-bearing exercise. MBS Item 715 health checks provide a structured opportunity to assess bone health, screen for osteoporosis risk factors, and discuss musculoskeletal health in a culturally safe context.

Quick Reference: Differential Diagnosis at a Glance

Costovertebral dysfunction

Paracetamol ± NSAID; manual therapy

2–6 weeks

Provocable on palpation; no red flags

Thoracic compression fracture

Paracetamol; ± calcitonin; DXA + osteoporosis Rx

6–12 weeks healing

Elderly; osteoporosis; acute onset

ACS (posterior MI)

Aspirin 300 mg, GTN, heparin; urgent PCI

Time-critical

ECG, troponin; CV risk factors

Aortic dissection

IV labetalol; urgent CT aortogram; surgery (Type A)

Time-critical

Tearing pain; BP differential >20 mmHg

Vertebral osteomyelitis

IV antibiotics (vancomycin + ceftriaxone initially); ID consult

6 weeks IV antibiotics

Fever, elevated CRP, IV drug use

Biliary colic / cholecystitis

Paracetamol ± morphine; lap cholecystectomy

Surgical within 72 h (cholecystitis)

RUQ/infrascapular; post-prandial; RUQ US

📚 References

1. Briggs AM, Smith AJ, Straker LM, Bragge P. Thoracic spine pain in the general population: prevalence, incidence and associated factors in children, adolescents and adults. A systematic review. BMC Musculoskelet Disord. 2009;10:77.
2. National Health and Medical Research Council (NHMRC). Evidence-based management of acute musculoskeletal pain. Canberra: NHMRC; 2003 (updated 2020).
3. Australian Institute of Health and Welfare (AIHW). Aboriginal and Torres Strait Islander Health Performance Framework: Summary report 2023. Canberra: AIHW; 2023.
4. Deyo RA, Rainville J, Kent DL. What can the history and physical examination tell us about low back pain? JAMA. 1992;268(6):760–765.
5. Stochkendahl MJ, Kjaer P, Hartvigsen J, et al. National Clinical Guidelines for non-surgical treatment of patients with recent onset low back pain or lumbar radiculopathy. Europ Spine J. 2018;27(1):60–75.
6. Erwin WM, Jackson PC, Homonko DA. Innervation of the human costovertebral joint: implications for clinical back pain syndromes. J Manipulative Physiol Ther. 2000;23(6):395–403.
7. Royal Australian College of General Practitioners (RACGP). Guidelines for preventive activities in general practice. 9th edn. Melbourne: RACGP; 2018 (updated 2023).
8. Hirsch JA, Singh V, Falco FJE, et al. Thoracic facet joint interventions. Pain Physician. 2016;19(4):E581–E593.
9. Erwin WM, Jackson PC. The costovertebral joint: anatomy, biomechanics, and clinical significance in thoracic back pain syndromes. J Can Chiropr Assoc. 2003;47(2):112–120.
10. Strayer RJ, Gunnerson JM, Brown LH, et al. Aortic dissection: clinical features, diagnosis, and management. Aust Crit Care. 2019;32(2):144–153.
11. Ombregt L. A system of orthopaedic medicine. 3rd edn. Edinburgh: Churchill Livingstone Elsevier; 2013. Chapter 18: Thoracic spine.
12. Lin CC, Chen KH, Li DM, et al. Characteristics and outcomes of patients presenting with thoracic back pain to the emergency department. Emerg Med Australas. 2020;32(5):805–811.

for PBS-listed medicines at participating pharmacies.

Cultural safety

Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.

Medication adherence

Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.

Specific conditions

Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.

Referral pathways

Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).

for PBS-listed medicines at participating pharmacies.

Cultural safety

Medication adherence

Specific conditions

Referral pathways

📚 References

1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).