Terminal2 · Diagnosis Card #00

[Diagnosis Name]

A hospice-first, evidence-based clinical reference for clinicians, families, and patients navigating this diagnosis at end of life. Built for the team beside the bed.

S01 Everyone

What Is It

Definition, mechanism, and the clinical reality of ALS at end of life. What the hospice team needs to understand on day one — a disease that destroys every voluntary muscle while leaving the mind fully intact.

Americans Living with ALS
~32,000
Approximately 5,000 new diagnoses per year in the United States. ALS is rare but devastating — roughly 2 per 100,000 population per year. Incidence increases with age, peaking between 55–75. Lifetime risk is approximately 1 in 300–400.
From Symptom Onset
2–5 yrs
Median survival from symptom onset. Highly variable — approximately 10% of patients survive beyond 10 years (long survivors). From diagnosis: typically 2–3 years. Survival is influenced by site of onset, rate of progression, respiratory function at diagnosis, nutritional status, and access to multidisciplinary care.
Bulbar Onset
~25%
Approximately 25% of ALS patients present with bulbar onset — speech and swallowing affected first. ~70% present with limb onset (arm or leg weakness). ~5% present with respiratory onset. Bulbar onset carries shorter median survival of 2–3 years and earlier communication and nutritional crises.
Die from Respiratory Failure
~80%
The overwhelming majority of ALS deaths are from respiratory failure as the diaphragm and intercostal muscles weaken beyond compensation. The ventilator conversation is not an end-of-life emergency — it is a longitudinal clinical obligation that begins at diagnosis and must be revisited at every stage of disease progression.

Amyotrophic lateral sclerosis (ALS) is the progressive degeneration of both upper and lower motor neurons — the cells that carry movement commands from the motor cortex through the brainstem and spinal cord to every voluntary muscle in the body. As motor neurons die, the muscles they innervate atrophy, weaken, fasciculate, and ultimately fail. This process is progressive and irreversible. It moves through the body in a pattern that is partly predictable (spreading from the region of onset to contiguous spinal cord segments) and partly unpredictable (the rate and sequence vary widely between patients). The end result is the same: progressive paralysis of limb muscles, trunk muscles, bulbar muscles controlling speech and swallowing, and ultimately the respiratory muscles — the diaphragm and intercostals — that sustain breathing.

What makes ALS unique among all neurodegenerative diseases — and uniquely cruel — is what it does not destroy. In the majority of ALS patients, cognition, sensation, personality, and emotional capacity remain fully intact throughout the disease course. The patient feels every touch. They understand every conversation. They remember every face. They know exactly what is happening to their body, what is coming next, and what the prognosis means. They have often read the same medical literature that the clinician has read. They watch themselves disappear one muscle group at a time — first the hand that held a pen, then the legs that carried them, then the tongue that formed words, then the throat that swallowed food — while the mind behind those failing muscles remains clear, present, and aware. Note: approximately 15–20% of ALS patients develop frontotemporal dementia (ALS-FTD), and up to 50% show some degree of cognitive or behavioral change on formal testing. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) should be reviewed in the patient's records.

For the hospice team, ALS presents a convergence of clinical demands unlike any other diagnosis: complex respiratory equipment management (BiPAP, cough assist devices, suction), nutritional support through PEG tubes, communication through augmentative and alternative communication (AAC) devices, progressive total physical dependence requiring 24-hour caregiving, and pharmacological management of spasticity, pain, sialorrhea, pseudobulbar affect, and dyspnea. All of this occurs while the person inside the body is fully present, fully aware, and watching the team work. That awareness changes everything about how care is delivered.

🧭 Clinical Framing

The clinical team managing ALS must hold both dimensions simultaneously: the extraordinary physical management complexity of a progressive multisystem motor disease — respiratory support, nutritional support, communication technology, medication delivery routes, secretion management, spasticity control — and the unconditional respect for the fully intact personhood of the human being living inside the body that is failing. Every clinical interaction must be directed to the patient, not about the patient. Every decision must include the patient's voice — whether that voice comes from their mouth, their AAC device, or their eye-gaze system. The person is not the disease. The person is watching the disease. That distinction defines every visit.

From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"When you walk into an ALS home for the first time, stop in the doorway and look. The BiPAP machine on the nightstand. The suction catheter on the shelf. The eye-gaze device mounted on the wheelchair tray. The PEG tube supplies stacked in the corner. Every single piece of equipment in that room tells the story of a decision that was made — a conversation that happened, a window that was caught in time, a loss that was anticipated before it arrived. And somewhere in the middle of all that equipment is a person who is completely, fully, exactly who they have always been — watching you take it all in. Talk to them first. Not to the caregiver. Not to the equipment. To them."
— Waldo, NP · Terminal2
S02 Clinician

How It's Diagnosed

Diagnostic workup, classification criteria, and what to look for in hospice records. Most ALS patients arrive with an established diagnosis — this section helps you read the record and understand where they are in the disease.

Diagnostic Workup
  • EMG / Nerve Conduction Studies (gold standard): Electromyography demonstrates active denervation in multiple body regions — fibrillation potentials, positive sharp waves, fasciculation potentials. Chronic denervation shows large-amplitude motor unit potentials (MUPs), long-duration polyphasic MUPs, and reduced recruitment. Must show denervation in at least 2–3 body regions (bulbar, cervical, thoracic, lumbosacral) to support the diagnosis. Nerve conduction studies (NCS) show normal sensory nerve conduction — this sensory sparing is critical and distinguishes ALS from peripheral neuropathies, multifocal motor neuropathy, and other mimics. Motor conduction velocities may be mildly reduced due to loss of fast-conducting motor fibers.
  • Clinical Neurological Examination: The diagnostic signature of ALS is the combination of upper motor neuron (UMN) and lower motor neuron (LMN) signs in multiple body regions. UMN signs: spasticity, hyperreflexia, Babinski sign, clonus, Hoffman sign, pseudobulbar affect (pathological laughing or crying — involuntary emotional expression disorder). LMN signs: weakness, atrophy, fasciculations, hyporeflexia or areflexia in affected segments. The presence of both UMN and LMN findings in the same limb is strongly suggestive. Progressive spread from one region to contiguous regions over time solidifies the diagnosis.
  • MRI Brain and Spine: Primarily used to exclude structural mimics — cervical spondylotic myelopathy (the most common misdiagnosis), brainstem tumors, syringomyelia, multiple sclerosis, spinal cord compression. ALS is often a diagnosis of exclusion alongside positive EMG. Corticospinal tract hyperintensity on T2-weighted MRI may be seen in ALS but is not diagnostic and not reliably present.
  • Pulmonary Function Testing (PFTs): FVC (forced vital capacity) is the single most important prognostic and monitoring tool in ALS after diagnosis is established. FVC <50% predicted = significant respiratory muscle weakness, triggering NIV initiation and PEG timing consideration. FVC <25% = near-total respiratory failure, approaching terminal respiratory phase. SNIP (sniff nasal inspiratory pressure) is more sensitive than FVC for detecting diaphragm weakness in patients with bulbar involvement who cannot form a seal on the spirometry mouthpiece — SNIP <40 cm H₂O predicts respiratory failure.
  • Genetic Testing: C9orf72 hexanucleotide repeat expansion — most common genetic cause (~40% of familial ALS, 5–10% of sporadic); also the most common genetic cause of FTD. SOD1 mutation — second most common; target of tofersen therapy. TARDBP (TDP-43), FUS, NEK1, TBK1 — additional genes. Family history is positive in approximately 10% of cases. Genetic counseling and testing is a clinical obligation that affects living family members.
  • Cognitive / Behavioral Assessment: Edinburgh Cognitive and Behavioural ALS Screen (ECAS) — validated ALS-specific cognitive screen assessing language fluency, executive function, memory, visuospatial function, and social cognition. Up to 50% of ALS patients show some cognitive or behavioral change. 15–20% meet criteria for ALS-FTD. ALS-FTD significantly affects prognosis and decision-making capacity — this result must be known at hospice enrollment.
What to Look for in Hospice Records
  • El Escorial Classification: The revised El Escorial criteria classify ALS as clinically definite, clinically probable, clinically probable with laboratory support, or clinically possible — based on the number of body regions showing combined UMN and LMN signs. The classification affects prognostic certainty. "Clinically definite" ALS has UMN and LMN signs in bulbar plus two spinal regions, or three spinal regions.
  • FVC Trend: The most critical data point in the hospice record. Look for FVC trajectory over the last 6–12 months. A declining FVC tells you exactly where this patient is in the respiratory timeline. FVC at enrollment determines eligibility for PEG placement (≥50% threshold), need for NIV initiation (<50%), and proximity to terminal respiratory failure (<25%).
  • Current Respiratory Support: Is the patient on NIV (BiPAP/VPAP)? How many hours per day? Is NIV providing subjective relief? Is NIV tolerance declining? Has the invasive ventilation (tracheostomy) conversation occurred? What was the documented decision? If the patient is tracheostomy-ventilated, what are the current settings and is there a documented discontinuation plan?
  • PEG / Feeding Status: Has a PEG been placed? If yes, what is it being used for (medications, supplemental nutrition, primary nutrition, hydration)? If no PEG and the patient has dysphagia, was PEG declined or was it never offered? If declined, is the oral route still functional for medications?
  • Communication Method: Is the patient speaking? If so, is speech intelligible to unfamiliar listeners? Is an AAC device in use? What type — communication board, tablet-based app, eye-gaze device (Tobii Dynavox, MyTobii)? Has voice banking been completed? This determines how every conversation in hospice will happen.
  • Genetic Testing Results: Was genetic testing performed? If positive for C9orf72, this affects FTD screening, family counseling, and may explain cognitive or behavioral changes. If positive for SOD1, tofersen therapy may be relevant. If not tested and family history is suggestive, genetic counseling referral is still appropriate even at hospice enrollment.
  • ALS-FTD Screen / Cognitive Assessment: Was ECAS or other cognitive screening performed? If ALS-FTD is documented, this fundamentally changes capacity assessment, advance directive reliability, and communication approach. If not screened, bedside cognitive assessment by the hospice team may be warranted.
  • King's Clinical Staging: If documented — Stage 1 (symptom onset, one region), Stage 2 (diagnosis, second region), Stage 3 (third region), Stage 4A (gastrostomy), Stage 4B (NIV), Stage 5 (death). Helps frame the trajectory.

💡 For Families

💡 Para las familias

Your loved one's ALS diagnosis was established through a combination of nerve testing (EMG), neurological examination, and ruling out other conditions. By the time hospice is involved, the diagnostic workup is almost always complete — the focus now shifts entirely to comfort, function, and quality of life. If you hear the team mention FVC numbers, that is a measure of breathing muscle strength and is the most important number we track in ALS. If genetic testing was done and a mutation was found, that information may be relevant to blood relatives — ask the team about genetic counseling if this applies to your family.

El diagnóstico de ELA de su ser querido se estableció mediante una combinación de pruebas nerviosas (EMG), examen neurológico y descarte de otras condiciones. Cuando el hospicio se involucra, el proceso diagnóstico casi siempre está completo — el enfoque ahora se centra completamente en la comodidad, la función y la calidad de vida.

S03 Everyone

Causes & Risk Factors

Sporadic and hereditary causes, environmental associations, and genetic risk. Relevant for family conversations, genetic counseling referrals, and answering the hardest question families ask: "Why did this happen?"

Sporadic & Environmental
  • Sporadic ALS (~90% of cases): The vast majority of ALS cases have no identifiable single genetic cause. The leading hypothesis is a complex interaction between genetic susceptibility variants and environmental triggers — a "gene × environment" model. Despite decades of research, no definitive sporadic cause has been identified. This is the honest answer when families ask.
  • Military Service (1.5–2× risk): US veterans have a consistently replicated elevated risk of developing ALS — one of the strongest environmental associations in the ALS literature. The mechanism remains unknown. Hypotheses include toxin exposure (Agent Orange, heavy metals, depleted uranium), intense physical exertion, head trauma, and infectious disease exposure. ALS is recognized as a service-connected disease by the US Department of Veterans Affairs.
  • Athletic Activity Paradox: Elite athletes — particularly soccer players and American football players — appear to have elevated ALS risk in multiple epidemiological studies. The "athlete's paradox" of ALS remains under active investigation. Proposed mechanisms include intense physical activity, repetitive head trauma (CTE-ALS overlap), and selection bias. The Italian football (calcio) studies showing elevated ALS rates among professional players are among the most cited.
  • Occupational Exposures: Epidemiological associations exist for heavy metals (lead, mercury), pesticides and herbicides, industrial solvents, formaldehyde, and electromagnetic fields. The evidence is strongest for lead exposure. Mechanistic links remain unclear, and confounding is difficult to eliminate in occupational studies.
  • Head Trauma: Multiple studies show an association between traumatic brain injury and later ALS diagnosis. The risk appears to increase with repetitive head trauma, as seen in contact sports. The CTE–ALS relationship is an active area of investigation.
  • Smoking: A modest but consistently replicated risk factor for ALS across multiple studies and meta-analyses. The mechanism may involve oxidative stress and neuronal toxicity. Current smoking appears to carry higher risk than former smoking.
  • Geographic Clusters: The Guam ALS-parkinsonism-dementia complex (Lytico-Bodig disease) was historically 50–100× higher than mainland US rates, linked to cycad seed neurotoxin BMAA (beta-methylamino-L-alanine). BMAA has been hypothesized as a contributor to some sporadic ALS clusters beyond Guam. The Kii Peninsula of Japan and Western New Guinea showed similar historical clusters.
Familial & Genetic
  • Familial ALS (~10% of cases): Autosomal dominant inheritance in most families. First-degree relatives of familial ALS patients have a significantly elevated risk. Genetic counseling and testing is a clinical obligation — the results affect living family members, not just the patient.
  • C9orf72 Hexanucleotide Repeat Expansion: The most common genetic cause of ALS — found in approximately 40% of familial ALS and 5–10% of apparently sporadic ALS. Also the most common genetic cause of frontotemporal dementia (FTD). The C9orf72 expansion creates a spectrum: pure ALS, pure FTD, and ALS-FTD. Patients with this mutation have higher rates of cognitive and behavioral involvement. Repeat length correlates loosely with severity. The expansion is detected by repeat-primed PCR, not standard sequencing.
  • SOD1 (Superoxide Dismutase 1): The second most common familial ALS gene. Over 200 different mutations identified with variable penetrance and phenotype. SOD1-ALS is the target of tofersen (antisense oligonucleotide, FDA-approved 2023), the first precision therapy in ALS. SOD1 mutations cause a toxic gain-of-function, not a loss-of-function. SOD1 testing is now therapeutically actionable.
  • TARDBP (TDP-43): Encodes TDP-43 protein — the defining pathological protein aggregation in the majority of ALS (including most sporadic ALS). Mutations in TARDBP cause a minority of familial ALS, but TDP-43 pathology is central to the disease mechanism of nearly all ALS.
  • FUS (Fused in Sarcoma): Associated with aggressive, early-onset familial ALS. FUS mutations tend to present in younger patients with rapid progression.
  • NEK1 and TBK1: More recently identified ALS risk genes. NEK1 is one of the most common ALS-associated genetic variants and may contribute to both familial and sporadic ALS. TBK1 mutations are associated with ALS-FTD.
  • Demographic Factors: ALS has a modest male predominance (~1.3:1 male-to-female ratio). White Americans have higher incidence than Black or Hispanic Americans in most US registries — though this may reflect diagnostic access disparities rather than true biological difference. Incidence peaks between ages 55–75. Younger onset (<40) suggests familial or genetic etiology.

❤️ For Families: "Why Did This Happen?"

This is one of the most painful questions a family can ask, and the honest answer for 90% of ALS cases is: we do not know. Sporadic ALS has no identified single cause. Your loved one did not cause this disease. It was not caused by something they ate, something they did, or something they failed to do. It was not a lifestyle choice. ALS strikes across all populations, all socioeconomic groups, all levels of health and fitness. If your family member was athletic, physically active, or served in the military — those are associations that research has identified, but they are not certainties of causation. If genetic testing was done and a mutation was found, the genetics team can explain what that means for blood relatives. If genetic testing was not done and there is a family history of ALS or dementia, ask the team whether testing would be appropriate — it may provide information that protects other family members.

⚕ Clinician Note: Genetic Counseling Obligation

Even at hospice enrollment, genetic testing results — or the absence of testing — have implications for living family members. If the patient has documented C9orf72 expansion, first-degree relatives have up to a 50% chance of carrying the same expansion and are at elevated risk for both ALS and FTD. If the patient has SOD1 mutation, first-degree relatives should know that tofersen now exists as a therapeutic option. If the patient has a strong family history of ALS or FTD and was never genetically tested, discuss testing with the patient and family even at this stage — the result is not for the patient's treatment, it is for the protection of the next generation. Refer to genetic counseling. This is not an optional conversation.

S04 Clinician Everyone

Treatments & Procedures

Disease-modifying therapies, respiratory management, nutritional support, and communication augmentation — the complete treatment landscape of ALS at end of life. Understanding what the patient has received, what they are still receiving, and what decisions remain.

ALS treatment is unlike oncology treatment — there is no curative intent and no remission. Disease-modifying therapies offer modest survival extension measured in months, not years. The overwhelming majority of clinical management in ALS is symptomatic, supportive, and technology-dependent: respiratory support, nutritional support, communication support, mobility support, and pharmacological symptom control. At hospice enrollment, the question is not "what treatment can we stop?" — it is "which treatments and supports are providing comfort and function, and which are creating burden without benefit?" Many ALS-specific interventions (NIV, cough assist, PEG feeding, AAC devices) are primarily comfort-directed and should continue or even be initiated in hospice.

Disease-Modifying Therapies
  • Riluzole (Rilutek): Glutamate antagonist — the first FDA-approved ALS therapy (1995). Extends median survival by approximately 2–3 months based on the Bensimon et al. NEJM 1994 trial and subsequent meta-analyses. Dose: 50 mg orally twice daily, at least 1 hour before or 2 hours after meals. Hepatotoxicity risk requires LFT monitoring — monthly for first 3 months, then quarterly. Available as oral tablet, oral suspension, or oral film (Exservan). Hospice considerations: Can be given via PEG as liquid suspension (Tiglutik). If tolerated without monitoring burden, continuation is reasonable in comfort-focused care — the 2–3 month survival benefit is meaningful to many patients. Reassess whether the monitoring visits (LFTs) are consistent with comfort goals. If the patient has a PEG, the liquid formulation simplifies administration as oral swallowing fails.
  • Edaravone (Radicava): Free radical scavenger — FDA-approved 2017 based on the MCI186 study. Demonstrated modest slowing of functional decline (ALSFRS-R) in a highly selected early-ALS subgroup. IV formulation: 60 mg IV infusion for 14-day cycles followed by 14-day rest periods. Oral suspension (Radicava ORS) approved 2022. Hospice considerations: The IV infusion schedule is burdensome and often discontinued as disease advances. The oral formulation reduces burden but still requires monitoring. Most patients discontinue edaravone by hospice enrollment. If still receiving, discuss whether continuing aligns with patient goals.
  • AMX0035 / Relyvrio (Sodium Phenylbutyrate + Taurursodiol): Dual mechanism targeting mitochondrial dysfunction and endoplasmic reticulum stress. CENTAUR trial showed modest slowing of functional decline. Oral formulation (powder for oral suspension). Note: Amylyx withdrew Relyvrio from the market in April 2024 after the Phase III PHOENIX trial failed to confirm the CENTAUR results. Patients who were taking it prior to withdrawal may still reference it. Hospice considerations: No longer commercially available. Historical context for patients who received it.
  • Tofersen (Qalsody): Antisense oligonucleotide targeting SOD1 mRNA — FDA-approved 2023 under accelerated approval for SOD1-ALS. Reduces SOD1 protein and neurofilament light chain (NfL, a neurodegeneration biomarker). Intrathecal injection via lumbar puncture: loading doses every 2 weeks × 3, then every 4 weeks maintenance, eventually every 8 weeks. Only applicable to SOD1-mutant patients (~2% of all ALS). VALOR trial + open-label extension showed biological effect and suggestion of clinical benefit. Hospice considerations: The first precision therapy in ALS. For patients with confirmed SOD1 mutation, tofersen may continue in hospice if the intrathecal injection schedule is consistent with patient goals and the patient explicitly desires continued disease-directed therapy. Requires ALS center coordination. This is a highly individualized decision.
Respiratory Management
  • Non-Invasive Ventilation (NIV — BiPAP/VPAP): Initiated when FVC <50% predicted or symptoms of nocturnal hypoventilation appear: morning headaches, orthopnea, non-restorative sleep, daytime somnolence, nighttime awakenings with dyspnea. Extends survival by 7–13 months in tolerant patients (Bourke et al. Lancet Neurology 2006). The most important comfort intervention for dyspnea in ALS. NIV use typically progresses: nighttime only → nighttime + naps → daytime periods → near-continuous → continuous. Patients with bulbar weakness may have difficulty tolerating NIV due to mask leak from facial weakness — specialized masks (nasal pillows, custom-fit interfaces) required. Hospice consideration: NIV initiation is appropriate even at hospice enrollment if FVC <50% and the patient is not yet on NIV — this is a comfort intervention, not a disease-directed therapy.
  • The NIV-to-Invasive Ventilation Continuum: When NIV can no longer maintain adequate ventilation — persistent hypercapnia, worsening dyspnea despite continuous NIV, inability to tolerate the mask — the patient faces the defining end-of-life decision in ALS: whether to proceed to tracheostomy and invasive mechanical ventilation (IMV). Tracheostomy ventilation can sustain life for years to decades but requires 24-hour skilled caregiving, creates complete dependence on the ventilator, and fundamentally changes the character of daily life. This decision must be discussed and documented before the respiratory crisis arrives. Emergency intubation without a documented decision robs the patient of their choice.
  • Tracheostomy / Invasive Mechanical Ventilation: Approximately 5–10% of ALS patients in the US choose tracheostomy ventilation (higher rates in Japan, ~30%). Requires 24-hour caregiving, tracheostomy care, suctioning, speech valve management, and ventilator monitoring. Patients on invasive ventilation can live for years but are completely ventilator-dependent. Communication through speaking valve (if tolerable), mouthing words, AAC devices, or eye-gaze systems. Quality of life on invasive ventilation is highly variable and deeply personal.
  • Ventilator Discontinuation: The patient who is invasively ventilated and documents a desire to discontinue ventilation is exercising their autonomous right to withdraw life-sustaining treatment. This is legally and ethically supported. Ventilator withdrawal in ALS requires explicit palliative sedation planning: morphine for dyspnea, midazolam or propofol for sedation, glycopyrrolate for secretions. Death typically occurs within minutes to hours after withdrawal. The plan must be documented, the team must be prepared, and the family must be counseled in advance.
Nutritional Support
  • PEG (Percutaneous Endoscopic Gastrostomy) Placement: The most time-sensitive procedural intervention in ALS. PEG is safest when placed while FVC is ≥50% predicted — the procedural sedation risk increases significantly as respiratory function declines. Indications: significant dysphagia, weight loss >10%, meal duration >30 minutes, recurrent aspiration events, inability to maintain adequate caloric intake orally. PEG does not need to replace oral intake — many patients use PEG for supplemental nutrition, hydration, and medication delivery while continuing to eat for pleasure. Hospice consideration: If a patient enrolls in hospice with dysphagia and FVC ≥50% and no PEG, the PEG conversation is a clinical priority at the first visit — the window is open and it will close.
  • RIG (Radiologically Inserted Gastrostomy): Alternative to PEG when FVC is too low for sedation (<50%) but gastrostomy is still desired. Inserted under fluoroscopic guidance with local anesthesia only — avoids the respiratory risk of endoscopic sedation. Not available at all centers. Discuss with the ALS center or interventional radiology if PEG window has passed but tube feeding is desired.
  • Oral Nutrition Modifications: Speech-language pathology assessment guides diet texture modifications: thickened liquids, pureed foods, soft mechanical diet. Positioning strategies (chin tuck, head turn) reduce aspiration risk. Calorie-dense foods and supplements to maintain weight. As dysphagia progresses, the oral route becomes unsafe — aspiration risk increases, and the decision about PEG or comfort-focused oral nutrition must be made.
Communication & Mobility
  • Voice Banking: The patient's natural voice is recorded while still intelligible and used to create a synthetic voice for their AAC device. Systems: ModelTalker, Acapela my-own-voice, VocaliD (now Veritone Voice), Cereproc CereVoice Me. Requires hours of recording over days to weeks. This window is irreversible — once intelligible speech is lost, voice banking is no longer possible. Must be introduced at the first visit where speech is still adequate for recording. Message banking (recording specific meaningful phrases, jokes, terms of endearment) complements voice banking and can be done more quickly.
  • AAC (Augmentative and Alternative Communication) Devices: Low-tech: alphabet boards, picture boards, partner-assisted scanning. Mid-tech: tablet-based communication apps (Proloquo2Go, TouchChat, Predictable). High-tech: eye-gaze communication systems — Tobii Dynavox (I-Series, TD Pilot), Grid Pad with eye tracking. Eye-gaze introduction must happen before it is the only communication option — patients need time to learn, calibrate, and practice while they still have other communication methods available.
  • Mobility: Power wheelchair with tilt/recline/elevating legrests — essential for patients with significant limb weakness. Environmental modifications: hospital bed, Hoyer lift, ramps, bathroom modifications. Fall prevention is critical during the ambulatory decline phase — most ALS falls occur during the transition period when the patient is still attempting to walk but lower extremity strength is insufficient. Ankle-foot orthoses (AFOs) for foot drop can extend ambulation.
S05 Clinician

When Therapy Makes Sense

Evidence-based criteria for continuing, initiating, or introducing interventions in ALS at hospice enrollment. In ALS, many "treatments" are comfort interventions — the question is whether they are being offered at the right time.

In ALS, the distinction between "disease-directed therapy" and "comfort care" is less binary than in oncology. Non-invasive ventilation extends survival and relieves dyspnea. PEG feeding maintains nutrition and provides a medication delivery route. Cough assist devices prevent pneumonia and improve comfort. AAC devices preserve the patient's ability to participate in their own care decisions. These are not aggressive treatments to be withdrawn at hospice enrollment — they are the infrastructure of comfort in ALS. The hospice team that walks into an ALS home and assesses what is in place, what is missing, and what windows are still open is providing the highest level of palliative care.

  1. 01
    NIV initiation when FVC <50% — the most important comfort intervention in ALS. Non-invasive ventilation (BiPAP/VPAP) extends survival by 7–13 months and is the single most effective intervention for dyspnea in ALS. It is often initiated too late. The patient who arrives at hospice enrollment without NIV despite FVC <50% has not received appropriate respiratory care — initiate NIV at enrollment. This is a comfort intervention. Symptoms triggering initiation even if FVC is not yet measured: orthopnea, morning headaches, non-restorative sleep, daytime somnolence, anxiety about breathing, use of accessory respiratory muscles.
  2. 02
    Riluzole continuation if tolerated without monitoring burden. Riluzole provides a modest 2–3 month median survival extension. It is well-tolerated in most patients. At hospice enrollment, assess: is the patient taking riluzole? Is it tolerated (no nausea, no hepatotoxicity)? Can it be given via PEG as liquid formulation if oral swallowing fails? If yes to all, continuation is reasonable. The burden question is the LFT monitoring — if monthly blood draws are inconsistent with comfort goals, continuing riluzole without monitoring is a shared decision. If the patient values even modest life extension, riluzole is appropriate to continue.
  3. 03
    PEG placement if FVC ≥50% and significant dysphagia — the most time-sensitive procedural window in ALS. If a patient enrolls in hospice with ALS, has significant dysphagia (weight loss, prolonged mealtimes, choking episodes, aspiration risk), and FVC is still ≥50%, the PEG conversation belongs at the enrollment visit. The window is open. It will close as FVC declines — once FVC drops below 50%, the sedation risk of endoscopic PEG placement increases significantly. If the window is open, call the gastroenterology team that day. If FVC is already <50%, discuss RIG as an alternative (local anesthesia, no sedation). If the patient declines PEG, respect the decision absolutely — but ensure they understand the window.
  4. 04
    Cough assist device (MI-E) if cough peak flow <270 L/min. Mechanical insufflation-exsufflation is a primary comfort intervention in ALS respiratory management. It generates a simulated cough to clear secretions, reduces aspiration pneumonia risk, and extends NIV tolerance. Most ALS patients at hospice enrollment do not have one despite meeting criteria. Assess cough strength at enrollment — if the patient cannot produce an effective cough, prescribe MI-E, arrange DME delivery, and train the family. This is an enrollment-day intervention.
  5. 05
    Voice banking if speech is still intelligible — this window is irreversible. If the patient's speech is still intelligible at hospice enrollment, voice banking must be introduced at that visit. The patient's natural voice, recorded while still usable, becomes the voice of their future speech-generating device. ModelTalker, Acapela, and VocaliD systems require hours of recording over days to weeks. Message banking (recording specific meaningful phrases, pet names, jokes, "I love you" in their own voice) can be done more quickly. Once intelligible speech is lost, this window is permanently closed. Do not wait for the next multidisciplinary clinic visit. Refer to speech-language pathology immediately.
  6. 06
    AAC device introduction before complete communication loss. Patients need time to learn, calibrate, and practice eye-gaze communication systems (Tobii Dynavox, Grid Pad) before those systems become their only way to communicate. The patient who is introduced to eye-gaze for the first time at the moment of complete speech loss has a tool they do not know how to use. The patient who has been practicing for months has a tool they trust. Introduce AAC early, while other communication methods still work, so the transition is gradual rather than catastrophic.
  7. 07
    SOD1-ALS — tofersen discussion if not yet offered. If the patient has documented SOD1 mutation and has not been offered tofersen, this should be discussed at hospice enrollment and coordinated with the patient's ALS center. Tofersen is the first precision therapy in ALS — intrathecal antisense oligonucleotide that reduces SOD1 protein. The decision to continue or initiate tofersen in hospice is highly individualized and depends on patient goals, access to intrathecal injection, and coordination with the ALS neurology team. If the patient explicitly desires continued disease-directed therapy alongside comfort care, tofersen may be appropriate.
S06 Clinician

When It Doesn't

Knowing when interventions stop helping — and when documented decisions must be honored absolutely. In ALS, the transition from disease-directed care to comfort-focused care is defined by respiratory milestones and patient choices.

In ALS, the transition away from life-prolonging interventions is defined by two forces: physiological thresholds (respiratory function reaching a point where interventions cannot maintain adequate ventilation) and autonomous patient decisions (documented choices about invasive ventilation, PEG placement, and end-of-life care). Both must be respected. The patient who has documented a decision to decline tracheostomy has made one of the most important advance directives in medicine. The patient whose FVC has declined below 25% with refractory dyspnea is approaching the terminal respiratory phase regardless of what interventions are offered. The hospice team's role is to honor the patient's documented wishes, manage the respiratory transition with aggressive comfort measures, and ensure that the final phase is not defined by suffering that could have been prevented.

  1. 01
    Invasive ventilation declined — documented decision must be honored absolutely. The patient who has documented a decision to decline tracheostomy and invasive mechanical ventilation has made the most consequential advance directive in ALS. This decision represents weeks, months, or years of deliberation about what kind of life is worth living. It must be honored without question, without pressure to reconsider, and without second-guessing — even when family members disagree. If the documented decision is in the chart, it is the clinical anchor. If a patient who has declined invasive ventilation is emergently intubated during a respiratory crisis (in an ER, by EMS), this creates an immediate goals-of-care emergency requiring urgent family meeting and, if the patient can communicate, direct confirmation of wishes.
  2. 02
    FVC <25% with dyspnea refractory to NIV — terminal respiratory phase. When forced vital capacity has declined below 25% and the patient's dyspnea is no longer adequately controlled by non-invasive ventilation, the patient is entering the terminal respiratory phase of ALS. NIV may still provide some comfort and should not be abruptly withdrawn, but the clinical team must recognize that the transition to comfort-focused respiratory management has begun. This means: scheduled opioids for dyspnea (morphine 2.5–5 mg q4h, titrated to comfort), benzodiazepines for respiratory anxiety (lorazepam 0.5–1 mg q4–6h), aggressive secretion management, and honest conversation with the patient and family about the trajectory.
  3. 03
    PEG placement declined — respect unconditionally. The patient who declines PEG placement has made an autonomous decision about their body and their disease. This decision may reflect values about the kind of life they want to live, beliefs about artificial nutrition, or acceptance of the disease trajectory. Respect it without pressure. Provide the best possible oral nutrition support for as long as the oral route is safe. Plan the comfort medication delivery route in advance — as oral swallowing fails, medications must transition to sublingual (morphine concentrate), buccal (lorazepam), transdermal (scopolamine, fentanyl), or subcutaneous routes. Build this contingency plan at enrollment, before the crisis arrives.
  4. 04
    NIV can no longer maintain adequate ventilation + tracheostomy declined — comfort care transition. When non-invasive ventilation has reached its physiological limit (persistent hypercapnia despite continuous use, intractable mask intolerance, worsening dyspnea despite maximum settings) and the patient has declined tracheostomy, the transition to comfort-focused care is explicit. This is the moment the advance directive was written for. Begin or escalate opioids for dyspnea. Add benzodiazepines for anxiety. Ensure subcutaneous access is established. Ensure midazolam is available for acute respiratory distress. Continue NIV for comfort as long as the patient desires, but shift the goal from ventilation to symptom relief.
  5. 05
    Tracheostomy in place but patient can no longer communicate without documented wishes — ethics consultation. The patient who is invasively ventilated, has lost all communication ability (including eye-gaze), and has not documented wishes about ventilator discontinuation is in one of the most ethically complex situations in hospice medicine. The ventilator is sustaining life, but the patient cannot express whether they want it to continue. If no advance directive exists and no prior conversations about discontinuation were documented, the surrogate decision-maker must be engaged. Ethics consultation is appropriate. The goal is to reconstruct, as faithfully as possible, what the patient would have wanted — using prior statements, values, personality, and the surrogate's knowledge of the person.
  6. 06
    ALS-FTD with loss of decision-making capacity — advance directives are the anchor. If the patient has ALS with frontotemporal dementia (ALS-FTD) and cognitive decline has impaired decision-making capacity, the advance directives made before cognitive decline are the clinical anchor for all subsequent care decisions. The surrogate decision-maker (healthcare proxy, durable power of attorney) must be clearly identified and actively involved. If advance directives were completed before cognitive decline, they carry full weight. If no advance directives exist and the patient now lacks capacity, this is a clinical and ethical emergency that requires immediate surrogate engagement and likely ethics consultation.

⚠ Terminal Respiratory Failure: Comfort Management

The patient with ALS who is dying from respiratory failure must not suffer from dyspnea or the terror of suffocation. This is a preventable suffering. Morphine is the cornerstone: 2.5–5 mg q4h via PEG, sublingual, or SQ — titrate to respiratory comfort, not respiratory rate. Lorazepam or midazolam for the anxiety component — the fear of suffocation in ALS is one of the most profound existential experiences in hospice medicine. Glycopyrrolate for secretions (0.2 mg SQ q4–6h or via PEG). Scopolamine patch for ongoing secretion management. For ventilator discontinuation: pre-medicate with morphine and midazolam, ensure continuous infusion capability, have rescue doses drawn and ready. The family must be prepared: what they will see, how long it takes, that the medications will prevent suffering. This conversation happens before the day of death, not on it.

S07 Everyone

Out-of-the-Box Approaches

Evidence-graded integrative, technological, and complementary approaches specific to ALS. Grade A = strong evidence / clinical standard; B = moderate evidence / extrapolated; C = limited or controversial evidence; D = no proven efficacy / patient inquiry likely.

Voice Banking & Message Banking
Grade A
Systems: ModelTalker, Acapela my-own-voice, VocaliD/Veritone Voice, Cereproc CereVoice Me · Recording: 3–8 hours over 1–2 weeks · Message banking: specific phrases recorded in patient's voice

Voice banking is one of the most time-sensitive and most impactful comfort interventions in ALS. The patient's natural voice — their inflection, their accent, their personality expressed through sound — is recorded while still intelligible and synthesized into a personalized text-to-speech voice for their AAC device. Message banking records specific meaningful phrases, terms of endearment, jokes, bedtime stories, and greetings in the patient's actual voice — these recordings play back exactly as spoken, not synthesized. Both require intelligible speech and take days to weeks to complete.

Clinical imperative: The hospice nurse who walks into an ALS home and notices the patient's speech is still intelligible has a window that may close in weeks to months. Introduce voice banking at that visit. Do not wait for the next scheduled multidisciplinary clinic. Provide the resources. Connect to speech-language pathology immediately. Say: "While your voice is still strong, there are programs that can save it for your communication device later — has anyone talked to you about that?" Many patients and families do not know this exists. This is Grade A not because of RCT data but because it represents best-practice clinical consensus in ALS care, endorsed by every major ALS clinical guideline, and the window is irreversible once closed.

Eye-Gaze Communication Systems
Grade A
Tobii Dynavox I-Series / TD Pilot · Grid Pad with Eyetech · Calibration: 15–30 min setup · Learning curve: weeks to months for proficiency

Eye-gaze technology tracks the patient's eye movements to select letters, words, phrases, and commands on a screen — enabling communication, environmental control (lights, TV, phone), email, and social media access when all other voluntary movement is lost. Modern eye-gaze systems are remarkably accurate and responsive, with word prediction and phrase shortcuts that significantly increase communication speed.

Clinical imperative: Eye-gaze introduction must happen before it is the only communication option. The patient who has been using eye-gaze for months when speech completely fails has a trusted, familiar tool. The patient who is introduced to eye-gaze at the moment of complete communication loss has a device they do not understand, cannot practice with, and may reject out of frustration. Introduce eye-gaze systems early and deliberately — while the patient still has speech or other communication methods — so the transition is gradual. Insurance coverage through Medicare/Medicaid and private insurers typically covers speech-generating devices with medical justification from a speech-language pathologist. Tobii Dynavox provides loaner programs and funding assistance. The equipment evaluation and procurement process takes weeks — do not delay.

Mechanical Insufflation-Exsufflation (MI-E / Cough Assist)
Grade A
CoughAssist T70 or E70 (Philips Respironics) · Pressures: +30 to +40 cm H₂O insufflation / −30 to −40 cm H₂O exsufflation · 4–5 cycles per session · 2–4 sessions daily or PRN for secretion retention

The cough assist device generates a deep mechanical insufflation followed by rapid pressure reversal to simulate a forceful cough — clearing secretions from the airways that the patient's weakened respiratory muscles can no longer expel. This is the most underprescribed respiratory comfort intervention in ALS hospice care. Evidence shows MI-E reduces secretion retention, decreases aspiration pneumonia incidence, extends NIV tolerance, and improves respiratory comfort. Should be initiated when cough peak flow drops below 270 L/min — most ALS patients at hospice enrollment meet this criterion but do not have the device.

Hospice consideration: Prescribing the cough assist device at enrollment, arranging DME delivery, and training the family and caregivers is a primary clinical intervention. The family must learn the technique — when to use it, how to position the patient, how to manage the circuit and settings. Many caregivers become proficient quickly and report significant reduction in respiratory distress episodes after consistent use. This is a comfort device that should be in every ALS home with cough weakness.

Cannabis for Spasticity & Appetite
Grade B
THC:CBD combinations · Oral: 2.5–5 mg THC + 2.5–5 mg CBD, titrate to effect · Nabiximols (Sativex) 1:1 THC:CBD oromucosal spray (not FDA-approved in US but available in many countries) · Edibles, tinctures, or PEG-compatible oils

Evidence for cannabinoids in ALS spasticity is extrapolated primarily from multiple sclerosis trials — the MUSEC and CAMS trials demonstrated benefit for MS spasticity, and the pathophysiology of upper motor neuron spasticity is similar in ALS. Nabiximols (Sativex) is approved for MS spasticity in over 25 countries. ALS-specific evidence is limited to small studies, surveys, and case series showing patient-reported improvement in spasticity, pain, appetite, sleep, and emotional well-being. A Phase II trial of THC:CBD in ALS spasticity (CANALS) has been conducted with preliminary positive signals.

Practical considerations: Many ALS patients already use cannabis products by the time of hospice enrollment. In states with medical cannabis programs, facilitate access. THC:CBD combinations may address multiple symptoms simultaneously: spasticity (primary indication), poor appetite and weight loss, pain, insomnia, and anxiety. PEG-compatible formulations (oils, tinctures) are available for patients who cannot swallow. Monitor for sedation (respiratory compromise risk), dizziness, and psychoactive effects. Start low, titrate slowly. Grade B reflects moderate evidence extrapolated from MS spasticity trials with supportive ALS-specific observational data.

Diaphragm Pacing (NeuRx DPS)
Grade C
NeuRx Diaphragm Pacing System · Laparoscopic electrode implantation on diaphragm · FDA Humanitarian Device Exemption (HDE) for ALS · Conditioning protocol: gradual increase in pacing duration

Diaphragm pacing was initially hypothesized to maintain diaphragm function in ALS by providing electrical stimulation to the phrenic nerve motor points, similar to exercise for the diaphragm. The NeuRx DPS received FDA Humanitarian Device Exemption in 2011 for ALS. However, the DIALS (Diaphragm Pacing in ALS) trial — a randomized controlled trial published in Lancet Neurology 2015 — showed that diaphragm pacing was associated with decreased survival compared to standard care. The trial was stopped early for safety concerns.

Clinical position: Based on the DIALS trial results, diaphragm pacing is no longer recommended in ALS. Some ALS centers still implant the device in selected patients under the HDE pathway, but the evidence does not support routine use. If a patient has an implanted diaphragm pacer, it should be assessed for benefit vs. harm — if not providing subjective benefit, discontinuation is appropriate. If a patient or family asks about diaphragm pacing, explain the DIALS trial findings honestly. Grade C reflects the initial promising concept undermined by a pivotal trial showing harm.

Stem Cell Therapy
Grade D
No approved protocol · Multiple Phase I/II trials ongoing (NurOwn / MSC-NTF, NSI-566, others) · No FDA-approved stem cell therapy for ALS

There is currently no proven efficacy for any stem cell therapy in ALS. Multiple approaches have been investigated — mesenchymal stem cells (MSCs), neural stem cells (NSCs), induced pluripotent stem cells (iPSCs) — in Phase I/II clinical trials. The NurOwn trial (BrainStorm Therapeutics, autologous MSC-NTF cells delivered intrathecally) failed its Phase III primary endpoint. No stem cell product has received FDA approval for ALS.

Why this matters in hospice: Patients and families will ask about stem cell therapy. ALS patients — cognitively intact, highly motivated, and facing a terminal diagnosis — are among the most active medical information seekers. Many have researched stem cell clinics, some have traveled internationally for unproven treatments, and some will ask the hospice team directly. Address honestly and without dismissiveness: "Stem cell research in ALS is ongoing and there are active clinical trials, but as of today no stem cell therapy has been proven to work for ALS in a rigorous clinical trial. I want to be honest with you about that while also respecting how much hope this represents." Do not dismiss the patient's hope. Do not validate unproven claims. Redirect to clinicaltrials.gov if the patient wants to explore enrollment in legitimate trials. Be aware of predatory stem cell clinics that charge tens of thousands of dollars for unproven treatments — protect your patient from financial exploitation.

S08Everyone

Natural & Herbal Options

Evidence grading, dosing where supported, drug interaction flags, and explicit contraindications specific to ALS. Patients will use supplements — this section helps you have the right conversation.

From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"ALS creates the most complex supplement environment in hospice. Here's why: riluzole is metabolized through CYP1A2 — any inducer or inhibitor changes your drug levels. Dysphagia means half these supplements can't be swallowed and need PEG-compatible formulations. Respiratory compromise means anything sedating is a risk. And the person sitting across from you is cognitively intact and has been reading PubMed since their diagnosis. They know about CoQ10, they know about creatine, they've read the NAC papers. Don't dismiss them. Go through each one honestly, grade the evidence, flag the interactions, and let them make informed decisions. The ALS patient who is told 'just take what we prescribe' will find the information elsewhere — and you'll lose the clinical relationship."
— Waldo, NP · Terminal2
Herb / Supplement Evidence Grade Typical Dose Potential Benefit ⚠ Interactions / Contraindications
Coenzyme Q10 / Ubiquinol Grade C 100–200 mg daily Mitochondrial support; mitochondrial dysfunction implicated in ALS pathophysiology; Phase II trial did not show efficacy but safe and well-tolerated[30] Minimal CYP interaction; no riluzole contraindication; PEG-compatible in liquid formulation; if patient is already taking it, no reason to stop
Vitamin D Grade C 1,000–2,000 IU daily Deficiency associated with faster ALS progression in observational studies; correction of documented deficiency is appropriate[31] Safe; no CYP1A2 interaction; no riluzole interaction; PEG-compatible liquid drops available
Melatonin Grade C 3–10 mg at bedtime Antioxidant and mitochondrial protective properties; sleep disruption from respiratory symptoms and anxiety is profound in ALS[32] Safe; no riluzole interaction; PEG-compatible liquid formulation available; monitor for morning sedation in patients with marginal respiratory function
Creatine monohydrate Grade C 5–10 g daily Muscle energy substrate; extensive ALS trial data — no significant clinical benefit in multiple Phase II/III trials; however safe and reasonable if patient chooses[33] Safe despite lack of clinical benefit; well-tolerated; PEG-compatible powder dissolved in water; adequate hydration required
NAC (N-Acetyl Cysteine) Grade C 600–1,200 mg daily Glutathione precursor; antioxidant; neuroprotective signal in preclinical models[34] Mucolytic properties thin secretions — in ALS patients already burdened with secretions this may be beneficial or problematic; monitor secretion volume and consistency; PEG-compatible
Omega-3 fatty acids (EPA+DHA) Grade C 1–2 g EPA+DHA daily Anti-inflammatory; observational associations with slower functional decline; general nutritional benefit in cachectic patients[35] PEG-compatible liquid formulations available; mild anticoagulant effect at high doses — monitor if on concurrent anticoagulation; fish oil reflux may be bothersome
Medical cannabis (THC:CBD) Grade B Variable dosing; titrate from low Spasticity reduction, appetite stimulation, sleep improvement, anxiety relief; observational evidence in ALS populations[36] ⚠ Sedation monitoring critical in patients with respiratory compromise; avoid smoked/inhaled routes — use sublingual, oral, or PEG-compatible formulations; legal status varies by jurisdiction
Curcumin / Turmeric Grade D 500–1,000 mg daily Anti-inflammatory; preclinical neuroprotective signal; limited clinical data in ALS specifically ⚠ CYP interactions possible — curcumin inhibits CYP1A2 and CYP3A4, which may increase riluzole levels; bioavailability is poor without piperine; piperine itself has additional CYP interactions; discuss risk-benefit openly
🚫 Avoid in ALS
  • St. John's Wort: Potent CYP1A2 inducer — significantly reduces riluzole serum levels, potentially negating its modest survival benefit; contraindicated with concurrent riluzole use
  • Kava (Piper methysticum): Hepatotoxicity risk compounded with riluzole's own hepatotoxic potential — dual hepatic insult; avoid entirely in any ALS patient on riluzole
  • High-dose Vitamin E (>400 IU/day): Anticoagulant effect increases bleeding risk; no demonstrated ALS benefit at high doses; standard multivitamin doses are acceptable
  • Ephedra / Ma Huang: Sympathomimetic cardiovascular stimulation in patients with already-stressed respiratory and cardiovascular systems; tachycardia and hypertension risk; contraindicated
  • Valerian (high doses): CNS depressant with respiratory depression risk at high doses; in ALS patients with marginal respiratory reserve, any additional respiratory suppressant is dangerous; low-dose occasional use may be acceptable but requires monitoring
S09Everyone

Timeline Guide

A guide, not a prediction. ALS trajectory varies widely — bulbar onset is faster, limb onset slower, ALS-FTD changes everything. Use this to anticipate, not to promise.

ALS progression is shaped by onset type (bulbar vs. limb vs. respiratory), genetics (C9orf72 and SOD1 variants have distinct trajectories), age at diagnosis, and rate of initial decline. Bulbar-onset patients progress faster — median survival 2–3 years. Limb-onset patients average 3–5 years. The 10% who survive beyond 10 years are a distinct population. Use this timeline to front-load every intervention and every conversation — the patient who completes advance directives, banks their voice, and places a PEG in Phase 1 is infinitely better positioned for everything that follows.[2]

YRS–
MOS
Early to Mid ALS — First & Second Region Affected
  • First region involved — weakness in one limb, speech changes, or hand dexterity loss; second region beginning involvement
  • Riluzole started; attending multidisciplinary ALS clinic every 2–3 months — the survival benefit of the multidisciplinary clinic is itself an intervention[41]
  • FVC still >50% predicted — respiratory reserve adequate; pulmonary function testing at every clinic visit
  • Voice banking initiated now — ModelTalker, Acapela, VocaliD; requires intelligible speech and takes weeks to complete; this window is irreversible[24]
  • PEG conversation introduced — not placed yet unless dysphagia is advancing, but the patient understands the timing rationale and the FVC threshold[18]
  • Advance directives drafted — ventilator decision conversation started; goals of care documented
  • AAC introduction — communication devices introduced while patient can still practice with intact speech as backup
  • Cough assist device prescribed if cough peak flow <270 L/min; DME delivered; family trained[22]
  • Front-load every intervention: the ALS clinic's greatest gift is positioning the patient for what comes next — not reacting to what has already happened
MOS–
1 YR
Advancing ALS with Respiratory Involvement
  • FVC declining below 50% — NIV (BiPAP) initiated or being introduced; nocturnal hypoventilation symptoms: morning headaches, orthopnea, non-restorative sleep[20]
  • Dysphagia advancing — modified diet textures; PEG placed if not already done; the FVC window for safe PEG placement is closing
  • Speech deteriorating — primary AAC device now in regular use; eye-gaze system being introduced before it becomes the only communication method
  • Limb weakness severely limiting independence — wheelchair-dependent; adaptive equipment maximized
  • Caregiver hours increasing exponentially — from assistance to full-time care; caregiver burnout risk accelerating[43]
  • The explicit ventilator conversation must happen before this phase ends: "When the BiPAP stops being enough, would you want a breathing tube?" — document the answer[44]
  • Hospice integration should begin alongside disease-directed care for patients not choosing invasive ventilation
WKS–
MOS
End-Stage ALS
  • NIV providing marginal relief or patient intolerant; FVC <25% — near-total respiratory muscle failure[20]
  • PEG-dependent for all nutrition, hydration, and medications; oral intake minimal or absent
  • Communication limited to eye-gaze device only — all other voluntary motor function severely compromised
  • Completely dependent for all ADLs; 24-hour caregiving required — this is clinical-level home care[43]
  • Comfort-focused goals of care established; hospice enrollment most appropriate at this transition
  • Hospice team and ALS center working in coordination — equipment, medications, and care plans aligned
  • Comfort kit prepared and at bedside: morphine SQ, midazolam SQ, glycopyrrolate SQ, suction machine
DAYS–
WKS
Active Dying from Respiratory Failure
  • NIV running continuously with diminishing effectiveness — the machine is working but the muscles cannot respond[20]
  • Secretion burden increasing — suctioning more frequent; glycopyrrolate or hyoscine butylbromide scheduled
  • Morphine for dyspnea — 2.5–5 mg SQ q4h around the clock; do not undertreat; respiratory depression at appropriate doses is not a clinical concern here[37]
  • Midazolam for anxiety — the fear of suffocation is real and must be addressed pharmacologically[38]
  • Family preparation for death from respiratory failure — explain what it will look like; describe the breathing pattern changes
  • Ventilator withdrawal if applicable: if patient documented withdrawal wishes, midazolam and morphine must be pre-drawn and the protocol reviewed before initiation[39]
HRS–
DAYS
Final Hours
  • Cheyne-Stokes or agonal breathing pattern; may transition to mandibular breathing — jaw movement without air exchange
  • Unresponsive or minimally responsive; auditory awareness may persist — speak to the patient, not about them
  • Medications via PEG (if functional) or subcutaneous route only — no oral medications
  • Morphine CSCI (continuous subcutaneous infusion) for sustained dyspnea management; midazolam PRN for agitation or breakthrough anxiety
  • Glycopyrrolate 0.2 mg SQ q4h for terminal secretions — reduces audible secretions that distress families
  • Family at bedside; ensure they know this is respiratory failure and it may be quiet — ALS death from respiratory failure can be peaceful when adequately managed with opioids and anxiolytics
  • Pronouncement; post-mortem care; bereavement follow-up for caregivers who have provided months or years of clinical-level care
S10Clinician

Medications to Anticipate

Symptom-targeted pharmacology for ALS. What to have in the comfort kit, what to titrate first, and what the evidence supports — with route contingency for every drug.

🚨 Medication Route Contingency — Build at Enrollment

ALS medication management is unlike any other hospice diagnosis: every drug must have a delivery route that accounts for current swallowing function. At enrollment, establish: (1) Is the patient swallowing medications orally? (2) Is there a PEG for medication delivery? (3) If neither — what is the sublingual, buccal, or SQ route for each essential medication? The patient who loses swallowing function overnight without a PEG and without a sublingual medication plan has a crisis that was entirely preventable. Build the route contingency plan at enrollment. Additionally: respiratory compromise means any sedating medication requires careful monitoring — start low, titrate slowly, and assess respiratory rate after each dose change.[37]

DrugClass / Target SymptomStarting DoseNotes / Cautions
Morphine Opioid / Dyspnea from respiratory failure 2.5–5 mg PO/PEG/SQ q4h First-line for respiratory failure dyspnea — the most important comfort drug in end-stage ALS. Around the clock for persistent dyspnea. SQ conversion when oral/PEG route fails. Do not undertreat — respiratory depression at appropriate doses is not a clinical concern; undertreated dyspnea is.[37]
Lorazepam Benzo / Dyspnea anxiety 0.5–1 mg PO/PEG/SQ q4–6h PRN Adjunct to morphine for the fear-of-suffocation anxiety component. Do not use as primary dyspnea treatment without concurrent opioid. The fear of suffocation in ALS is one of the most profound existential experiences in hospice.[38]
Midazolam Benzo / Terminal dyspnea crisis 2.5–5 mg SQ PRN; CSCI 10–20 mg/24h ⚠ MUST be pre-drawn for any patient who has documented ventilator withdrawal wishes. Ventilator discontinuation sedation protocol; terminal dyspnea crisis management. Have drawn and labeled at bedside before the event.[39]
Glycopyrrolate Anticholinergic / Secretions 0.2 mg SQ q4h PRN Sialorrhea and terminal secretions. Preferred over hyoscine in conscious patients — does not cross blood-brain barrier, no CNS sedation. Effective for both chronic sialorrhea and terminal secretion management.[28]
Amitriptyline TCA / Sialorrhea + pain 10–25 mg PO/PEG at bedtime Triple benefit: anticholinergic drying effect for sialorrhea + neuropathic pain management + sleep promotion. Start low — anticholinergic side effects accumulate. Monitor for urinary retention.[28]
Botulinum toxin Neurotoxin / Sialorrhea Salivary gland injection q3 months Grade A for drooling in ALS. Parotid and submandibular gland injections. Requires specialty referral but highly effective when anticholinergics are insufficient or poorly tolerated.[29]
Baclofen Antispasmodic / Spasticity 5 mg PO/PEG TID; titrate to 80 mg/day First-line for ALS spasticity. ⚠ Respiratory depression at high doses — titrate cautiously in patients with compromised respiratory function. Do not abruptly discontinue — risk of withdrawal seizures.[27]
Dantrolene Muscle relaxant / Spasticity 25 mg daily; titrate to 100 mg QID Alternative when baclofen insufficient or not tolerated. ⚠ Hepatotoxicity — compounded risk with concurrent riluzole; monitor LFTs if using both. Peripheral mechanism — less CNS sedation than baclofen.
Nuedexta (DM/Quinidine) DM/Quinidine / Pseudobulbar affect 1 cap daily × 7 days, then BID FDA-approved for pseudobulbar affect (PBA). STAR trial evidence.[25] The patient who is laughing at inappropriate times or crying for no apparent reason — this is PBA from bulbar involvement, not depression. Prescribe at the first visit where it is present. Meaningfully improves QoL.
Mirtazapine NaSSA / Depression + appetite + sleep 7.5–15 mg PO/PEG at bedtime Triple benefit in ALS: antidepressant + appetite stimulation + sleep promotion. The 7.5 mg dose is more sedating than 15 mg (antihistamine effect predominates at lower doses). First-line antidepressant in ALS patients with weight loss and insomnia.[26]
Mexiletine Sodium channel blocker / Muscle cramps 150 mg BID Grade A evidence — JAMA Neurology 2016 RCT demonstrated significant cramp reduction in ALS.[26] Muscle cramps are one of the most distressing symptoms early in ALS; often undertreated. Monitor QTc at baseline.
Dextromethorphan Antitussive / Cough 10–30 mg q4–6h PRN For non-productive cough that disrupts sleep and exhausts respiratory muscles. Not a substitute for cough assist device for secretion clearance. Available in PEG-compatible liquid formulation.
Hyoscine butylbromide Anticholinergic / Terminal secretions 20 mg SQ q4h PRN Alternative to glycopyrrolate for terminal secretion management. Does not cross blood-brain barrier. May be preferred in some formularies. Comparable efficacy to glycopyrrolate for death rattle.[28]

🌿 ALS Symptom Management Decision Tree

Evidence-based · Hospice-adapted
Select a symptom below to begin
What is the primary symptom to address?

🚨 Comfort Kit Must-Haves for ALS

  • Morphine 10 mg/mL SQ — pre-drawn syringes labeled with dose and route; for acute dyspnea crisis; 2.5–5 mg SQ doses
  • Midazolam 5 mg/mL SQ — pre-drawn syringes labeled and at bedside; for terminal dyspnea/anxiety crisis and ventilator withdrawal sedation
  • Glycopyrrolate 0.2 mg/mL SQ — for secretion crisis; have minimum 10 doses available
  • Suction machine — portable, charged, with extra catheters; family trained on use; backup plan if power fails
  • Backup NIV mask — if primary mask fails at 2 AM, a backup must be in the home; families must know where it is and how to switch
S11Clinician

Clinician Pointers

High-yield clinical pearls for the hospice team. The things not in the textbook — learned at the bedside over years of caring for ALS patients.

1
Voice banking at the first visit where speech is intact
This window is irreversible. Once the patient cannot produce intelligible speech, voice banking is no longer possible — ever. The hospice nurse who walks into an ALS home and hears the patient speaking intelligibly has a clinical obligation to introduce voice banking at that visit. Say: "While your voice is still strong, there are programs that can record it and use it as your electronic voice later — has anyone talked to you about that?" Then provide the resources: ModelTalker, VocaliD, Acapela. Connect to speech-language pathology immediately. Do not wait for the next scheduled appointment.[24]
2
The PEG window closes with FVC
The patient who comes to hospice with significant dysphagia and FVC still above 50% has an open window. The patient who comes with FVC 35% and dysphagia has an increasingly risky procedure ahead. Assess FVC at enrollment. If it is above 50% and PEG has not been placed, call the gastroenterology team that day. The PEG conversation is a clinical emergency in this context — not because the patient is dying today, but because the window to safely place it is closing and may not reopen. Do not leave the first visit without making that referral.[18]
3
Ventilator decision documented before respiratory crisis
This is the most important advance directive in ALS. The patient who is intubated emergently during respiratory failure without a documented decision has had their choice made for them by circumstance. At enrollment, ask directly: "Has anyone talked with you about what you want to happen when the BiPAP is not enough anymore — whether you would want a breathing tube?" Document the answer. If the decision has not been made, make it the priority of the next visit. This conversation belongs at the first visit, not the last.[44]
4
Cough assist device at enrollment if cough is weak
Most ALS patients at hospice enrollment do not have a cough assist device (mechanical insufflation-exsufflation). Cough peak flow <270 L/min indicates need. The cough assist device reduces pneumonia, reduces secretion retention, and extends NIV tolerance — it is a primary respiratory comfort intervention. Prescribe it at enrollment. Arrange DME delivery. Train the family on use, cleaning, and troubleshooting. Do this at enrollment, not after the first aspiration pneumonia.[22]
5
The 24-hour caregiver assessment
ALS caregivers provide clinical-level care: managing BiPAP equipment, administering PEG medications and feedings, operating suction devices, using communication technology, performing repositioning, providing all personal care. This is ICU-level work done by family members in a home with no call button. Assess the caregiver separately at every visit — their physical health, mental health, sleep, and coping. Depression rates in ALS caregivers exceed 50%. The caregiver who collapses is a medical emergency for both people in that home.[43]
6
Medication route contingency — build at enrollment
For every medication in the patient's regimen, document the current route and the backup route. Oral → PEG → SQ → buccal/sublingual. The patient who loses swallowing capacity overnight must have a complete medication plan that does not depend on swallowing. If there is no PEG and the patient is still swallowing, every essential medication must have a non-oral alternative identified, sourced, and stocked in the home. This plan is built at enrollment, not at crisis.
7
Equipment audit at every visit
At every visit, check: BiPAP — functioning, mask fit, backup mask present, humidifier working. Suction — charged, extra catheters stocked, tip clean. Cough assist — functioning, family comfortable using it. Communication device — charged, calibrated, accessible from bed and wheelchair. PEG supplies — adequate formula, flush syringes, spare extension sets, site clean. Every piece of equipment must be functioning, and the family must be trained on every piece. Equipment failure at 2 AM is a crisis that preparation eliminates.
8
Racial and socioeconomic disparities — advocacy is clinical obligation
Black and Hispanic patients with ALS receive less multidisciplinary care, are referred to ALS specialty clinics later, have lower rates of NIV and PEG placement, and have shorter survival — not from biology but from access failures. Rural patients face geographic barriers to multidisciplinary clinics. Uninsured and underinsured patients face equipment and medication access barriers. The hospice clinician who recognizes these disparities and actively advocates for equitable resource allocation is providing clinical care, not social work. Ensure every ALS patient on your caseload has equal access to equipment, specialty referrals, and interventions regardless of zip code, insurance status, or skin color.[42]
From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"Every ALS home I walk into, I do the same thing: I check the BiPAP, I check the suction, I check the cough assist, I check the communication device, I check the PEG site, and I check the caregiver. In that order. Because if any one of those six things is failing, the patient's comfort is failing — and five of those six things can be fixed before I leave that visit."
— Waldo, NP · Terminal2
S12EveryoneClinician

Psychosocial & Spiritual Care

The existential weight of ALS is unlike any other diagnosis. Cognition stays. The body goes. Every psychosocial intervention must honor that reality.

Spiritual / Existential Framing

ALS forces an existential confrontation that no other disease replicates: the progressive loss of every physical capacity while the mind watches, fully aware, fully present. The person inside the failing body is not diminished — they are imprisoned. Spiritual care in ALS is not about preparing for death. It is about honoring the life that is fully present inside a body that is progressively unable to express it. Every chaplain visit, every dignity therapy session, every legacy project is an act of recognition: you are still here, you are still you, and we see you.

1. The Terror of Being Trapped Inside

ALS is unique among all hospice diagnoses in the specific existential experience it creates: the person watches themselves lose every physical capacity while remaining fully cognitively present. They know what is coming. They have often researched their disease exhaustively. They watch their own progressive paralysis with a clarity that no other disease imposes. The hospice clinician who acknowledges this explicitly — "I want to say something directly: you are dealing not just with the physical losses but with the experience of watching them happen while fully aware" — provides a kind of recognition that most people in the patient's life cannot offer.[45]

2. The Communication Grief

The loss of voice in ALS is among the most profound identity losses in any medical condition. Voice is how humans express personality, humor, intimacy, and presence. As voice deteriorates, social interactions decrease. Family members begin speaking for the patient. The patient becomes an observer of conversations about themselves. Address this grief proactively: begin voice banking, ensure AAC devices are present and working, and explicitly advocate for the patient's continued full participation in every conversation about their care — even when that conversation takes longer with a device.[24]

3. The Ventilator Decision as Identity Statement

For many ALS patients, the decision about invasive ventilation is not just a medical decision — it is a statement about who they are, what they value, and what kind of life is worth living. Patients who choose invasive ventilation often do so from love for their family and desire to remain present. Patients who decline often do so from a belief that the life the ventilator maintains is not the life they want to live. Both decisions are expressions of deeply held values. The hospice team's role is not to guide the decision in either direction but to ensure it is made with full information, full autonomy, and full emotional support.[44]

4. The Caregiver as Unpaid Clinical Staff

ALS caregivers provide ICU-level care at home: operating ventilators, managing PEG feedings and medications, performing suctioning, using communication devices, repositioning a fully dependent adult, providing all personal care — 24 hours a day. Caregiver burnout is not a risk; it is an inevitability without aggressive support. Depression rates exceed 50%. Physical injury from lifting and transfers is common. The caregiver who collapses is a medical emergency for both people in that home. Assess caregivers separately at every visit. Prescribe respite proactively. Connect to ALS caregiver support groups.[43]

5. Sexuality and Intimacy

Progressive physical disability fundamentally changes intimacy — but it does not eliminate the need for it. ALS patients and their partners rarely raise this topic; clinicians almost never do. Address it proactively: "Intimacy often changes with physical changes. Is that something you'd like to talk about?" Touch, closeness, and physical connection remain possible at every stage of ALS. Occupational therapy can suggest adaptive positioning. The loss of sexual function is a real grief that deserves the same clinical attention as dysphagia or spasticity.

6. ALS-FTD: When Cognition Changes

Approximately 15% of ALS patients develop frontotemporal dementia (FTD); up to 50% show some cognitive or behavioral changes on formal testing. Behavioral variant FTD changes personality — apathy, disinhibition, loss of empathy, compulsive behaviors. The family grieves twice: once for the body and once for the person they knew. When ALS-FTD is present, advance directives made before cognitive decline are the clinical anchor. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) should be performed at enrollment to establish baseline. Surrogate decision-makers must be clearly identified early.[40]

💛 Goals-of-Care Framing for ALS

In ALS, goals-of-care conversations are not one-time events — they are a longitudinal dialogue that evolves as the disease progresses. The patient's goals at diagnosis (fight everything, try everything) may be different from their goals when FVC drops below 25% and communication is limited to eye-gaze. Revisit goals explicitly at each major transition: NIV initiation, PEG placement, loss of speech, loss of ambulation. The question is always the same: "Given where things are now, what matters most to you?" The answer may change. That is not inconsistency — it is a person living with a progressive disease and responding to their reality with wisdom.

S13FamilyEveryone

Family Guide

Plain language for families. Share, print, or read aloud at the bedside. You are providing extraordinary care — this guide helps you know what to expect and what to do.

You are caring for someone with ALS — a disease that progressively affects the muscles used for moving, speaking, swallowing, and breathing. What makes ALS different from many other serious illnesses is that your person's mind, personality, and awareness remain fully intact. They are completely present inside a body that is changing. They know what is happening. They need you to know that they are still fully there — and to treat them that way in every interaction. This guide will help you understand what to expect and what you can do to help.

What You May See
  • Progressive weakness spreading to new muscle groups: This is the disease advancing. You may notice new areas of weakness over weeks — a hand that was working last month now struggles. Your ALS care team will help anticipate the next changes and adapt equipment accordingly.
  • Increasing difficulty with speech: Voice may become harder to understand over time — softer, slurred, or slower. The speech therapist and your nurse will help set up communication tools before speech is completely gone. Be patient and give extra time for your person to communicate.
  • Difficulty swallowing: Your nurse will guide changes in food textures and will discuss when to consider the feeding tube (PEG). Do not push foods that cause coughing or choking — aspiration is dangerous. Small, appealing portions of soft foods are better than large meals.
  • Increasing shortness of breath or difficulty breathing: The breathing machine (BiPAP) manages this. Report any change in breathing comfort to the nurse immediately. Breathlessness that is not relieved by the machine requires medication — do not wait.
  • Muscle cramps and stiffness: Very common in ALS. Medications help significantly. Report cramps that are not controlled so the team can adjust treatment. Gentle stretching and positioning can also help.
  • Uncontrollable laughing or crying that seems out of context: This is a known neurological symptom of ALS called pseudobulbar affect. It does not mean your person is having a mental health crisis or losing their mind. It is treatable with medication — ask the nurse about Nuedexta.
How You Can Help
  • Learn the BiPAP/VPAP equipment completely: Know how to apply the mask, clean the equipment, respond to alarms, and use the backup plan your nurse reviewed. The breathing machine is the most important piece of equipment in this home. Practice until you are confident.
  • Use the communication device consistently: Give full attention and time for your person to communicate via their device. Do not speak for them unless they ask you to. Their voice through the device is still their voice. Include them in every conversation about their care.
  • Protect the PEG site: Keep it clean and dry as your nurse instructed. Give medications and tube feedings exactly as trained — the right amounts, the right flush, the right schedule. Call the nurse if you see redness, swelling, or drainage around the site.
  • Position for comfort: Reposition at least every 2 hours. Use pillows to support limbs and reduce pressure. Elevate the head of bed for breathing comfort. Your nurse will teach you the best positions for your person's specific needs.
  • Manage secretions with suction: Use the suction machine as your nurse taught you. Keep it charged, clean, and accessible. Extra suction catheters should always be available. If secretions suddenly increase or change color, call the nurse.
  • Give medications on schedule via the correct route: Know which medications go through the PEG, which are given under the tongue, and which are injected under the skin. Your nurse will train you on each route. Keep a medication schedule visible and follow it carefully.
  • Practice caregiver self-care: You are providing clinical-level care. This is exhausting physical and emotional work. Accept help. Use respite services. Sleep when you can. You cannot care for your person if you are depleted. Call us when you need support — not just when the patient does.
📞 Call the nurse immediately if you see:

Sudden breathing change not relieved by the BiPAP — increased work of breathing, gasping, or air hunger that the machine is not helping. Choking or aspiration event — food, liquid, or secretions going down the wrong way with prolonged coughing or color change. Fever — temperature above 100.4°F may indicate aspiration pneumonia or infection. Equipment failure — BiPAP, suction, or cough assist not working and you cannot troubleshoot it. Medication questions — any confusion about dose, route, or timing. New symptoms — anything you have not seen before, including new weakness, swelling, pain, or changes in alertness. When in doubt, call. We would always rather hear from you early than late.

🙏 What you are doing is extraordinary. ALS caregiving is among the most demanding forms of home care in all of medicine — you are providing the equivalent of intensive care nursing in your own home, around the clock, for someone you love. Research consistently shows that patients with dedicated, supported caregivers have better comfort, better symptom management, and more peaceful deaths. You are part of the clinical team. Your presence matters more than you know. And we are here for you — not just for your person, but for you.

S14Everyone

Waldo's Top 10 Tips

Clinical field wisdom from 12+ years at the bedside. The things you learn after doing ALS long enough. Not guidelines — real.

  1. 01
    Voice banking must happen at the first visit where speech is still intact. This window closes and it does not reopen. The patient who loses their voice without banking it loses their voice forever — not their electronic voice approximation, their actual voice. ModelTalker takes a few hours over a week. It is not a nice-to-have; it is a clinical obligation on the same level as the PEG conversation. If the patient is talking to you clearly today, the voice banking referral happens before you leave this visit. Period. I don't care what else is on your assessment form — this goes to the top.
  2. 02
    The PEG window closes with FVC, and it closes faster than you think. The patient with ALS and significant dysphagia and FVC above 50% has an open window — call GI that day, not next week, not at the next IDT meeting. That day. The patient with FVC 30% and dysphagia is looking at a higher-risk procedure with an anesthesia team that may not want to do it. Assess FVC at enrollment and act on what you find. I've seen too many patients miss the window because someone said "let's discuss it next visit." There was no next visit at FVC 50%.
  3. 03
    The ventilator decision must be documented before the respiratory crisis — not during it, not after 911 has been called, not in the ICU at 3 AM. At enrollment, ask directly: "When the BiPAP stops being enough, would you want a breathing tube?" I know it's a hard question. Ask it anyway. The patient who is intubated emergently during respiratory failure without a documented decision has had their choice made for them by circumstance and by a paramedic who doesn't know them. That is a clinical failure, and it is preventable. First visit. Ask the question. Document the answer.
  4. 04
    Pseudobulbar affect is undertreated because it is underrecognized. If your ALS patient is laughing at inappropriate times or crying for no apparent reason — especially with bulbar involvement — this is PBA, not depression, not anxiety, not "they're just emotional." Nuedexta is FDA-approved, it works, and it meaningfully improves quality of life. One cap daily for a week, then BID. Prescribe it at the first visit where you see it. The family has been confused and embarrassed by this for months. The patient has been humiliated. Name it, explain it, treat it.
  5. 05
    The cough assist device is the most underprescribed respiratory comfort intervention in ALS hospice. I walk into ALS homes and there's a BiPAP, there's a suction machine, and there's no cough assist. If the patient's cough is weak — and it usually is by the time they're on hospice — prescribe it, arrange the DME delivery, and train the family. It generates a simulated cough that clears secretions the patient can no longer clear on their own. It reduces pneumonia. It extends NIV tolerance. It improves comfort. Do this at enrollment, not after the first aspiration pneumonia.
  6. 06
    Mirtazapine is the triple-threat drug in ALS: antidepressant, appetite stimulant, and sleep aid — all in one pill at bedtime. Depression in ALS is real, appetite loss from dysphagia and disease burden is constant, and sleep disruption from respiratory symptoms and anxiety is profound. Mirtazapine 7.5 mg at bedtime addresses all three. The 7.5 mg dose is actually more sedating than 15 mg because the antihistamine effect predominates at lower doses. If your ALS patient is depressed, losing weight, and not sleeping — and most of them are — this is your first-line agent. One drug, three problems, one dose at bedtime.
  7. 07
    Assess the caregiver separately at every single visit. I mean separately — not "how are you doing?" as you're packing up your bag. Sit down with them. Ask about their sleep, their back pain, their mood, their support system. ALS caregivers provide clinical-level care — BiPAP management, PEG feedings, suctioning, repositioning, medication administration — 24 hours a day. Depression rates exceed 50%. Physical injury from transfers is common. The caregiver who collapses is a medical emergency for both people in that home. Prescribe respite before they ask for it. They won't ask.
  8. 08
    Build the medication route contingency plan at enrollment, not at the swallowing crisis. For every medication in the patient's regimen, write down: current route, backup route, and emergency route. Oral becomes PEG becomes SQ becomes buccal. The patient who loses swallowing capacity in the middle of the night without a PEG and without a sublingual pain medication plan has a crisis that was 100% preventable. I keep a route contingency card in every ALS chart. When the call comes at 2 AM — "they can't swallow their morphine" — I already know the answer because we planned it on day one.
  9. 09
    Equipment audit at every visit. Every. Visit. BiPAP — is it working, is the mask fitting, is there a backup mask, is the humidifier filled? Suction — is it charged, are there extra catheters, does the family know how to use it? Cough assist — same questions. Communication device — is it charged, is it calibrated, can the patient actually access it from bed? PEG supplies — enough formula, flush syringes, spare extension sets? I've seen ALS patients in distress because a mask seal failed, because the suction battery died, because the extension set cracked. Five minutes of equipment checking prevents hours of crisis.
  10. 10
    The patient is fully present. Address them directly. This is the most important thing I can tell you about ALS. The person in the wheelchair with the eye-gaze device, who cannot move, cannot speak, cannot eat, cannot breathe without a machine — that person is cognitively intact. They are listening to every word you say. They understand everything. They have opinions, preferences, humor, and frustration. Talk to them, not about them. Wait for them to respond through their device, even when it takes three minutes for one sentence. Do not look at the caregiver when you should be looking at the patient. The greatest indignity of ALS is not the paralysis — it is being treated as if the paralysis has taken the person with it. It hasn't. They are right there. See them.
— Waldo, NP · Terminal2
REFClinician

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terminal2.care content is for educational purposes and is not a substitute for clinical judgment. Based on articles retrieved from PubMed. All PMIDs hyperlinked. © Terminal2 | terminal2.care

📝

Private Notes

Session notes — not saved to any server. Clears when you close the tab.

Use this space for visit notes, clinical reminders, or patient-specific observations. This text is stored only in your browser session and is never transmitted to any server. Use it to track ALS-specific details: current FVC, NIV settings, PEG status, communication method, medication routes, caregiver status, and advance directive documentation status.