The Final Signs of ALS

How ALS reaches its final phase

ALS steadily weakens the muscles under a person's control, and in its final phase that weakness reaches the muscles that power breathing. This is usually what drives the end. As the diaphragm and chest muscles fail, breathing becomes shallow and effortful, especially lying flat, and a person can feel short of air — a symptom sometimes called air hunger.

This is the center of end-of-life care in ALS. The American Academy of Neurology's care guidance for ALS addresses exactly this arc: multidisciplinary support, breathing assistance such as noninvasive ventilation, and symptom management with early palliative care to relieve breathlessness and other distress 1Ref 1American Academy of Neurology (Quality Standards Subcommittee) (2009).Practice Parameter Update: The Care of the Patient with Amyotrophic Lateral Sclerosis - Multidisciplinary Care, Symptom Management, and Cognitive/Behavioral Impairment.That end-of-life care in ALS centers on the weakening breathing muscles, addressed through multidisciplinary care, noninvasive ventilation, feeding support such as a gastrostomy tube, symptom management, and early palliative care.. Air hunger looks frightening, but it can almost always be eased.

The early signs of this final turn are often subtle and easy to miss. Breathing that is fine sitting up becomes hard lying flat, so a person starts sleeping propped on pillows or in a chair. Morning headaches, daytime drowsiness, a foggy or unrefreshed feeling on waking, and a weakening cough can all come from the breathing muscles tiring, especially overnight. Read together, these point to the same thing: the signs the end is near in ALS are, above all, signs of the breathing muscles giving out.

Eating, swallowing, and weight in late ALS

As ALS advances, the muscles used to chew and swallow weaken, meals take longer, food and liquid go down the wrong way, and weight falls. Earlier in the illness, a feeding tube placed through the abdominal wall can help maintain nutrition and ease the effort of eating; the AAN guidance addresses this option as part of ALS care 1Ref 1American Academy of Neurology (Quality Standards Subcommittee) (2009).Practice Parameter Update: The Care of the Patient with Amyotrophic Lateral Sclerosis - Multidisciplinary Care, Symptom Management, and Cognitive/Behavioral Impairment.That end-of-life care in ALS centers on the weakening breathing muscles, addressed through multidisciplinary care, noninvasive ventilation, feeding support such as a gastrostomy tube, symptom management, and early palliative care..

The very end of life is different. In the final days, appetite and intake naturally fall away as the body shuts down, and at that point artificial nutrition and hydration generally does not prolong life or add comfort 2Ref 2Peer-reviewed article (see publication) (2006).Artificial Nutrition and Hydration at the End of Life: Ethics and Evidence.That in the final days of life, when intake naturally falls, artificial nutrition and hydration generally does not prolong life or increase comfort.. Distinguishing the two moments — maintaining nutrition earlier versus the natural decline of the final days — is worth talking through with the care team, because the right answer changes as the illness moves.

What the final days can look like

Beyond the breathing changes specific to ALS, the last days bring the signs shared by the end of many serious illnesses. A person sleeps more and is awake less, and may not rouse at all. Interest in food and drink disappears. Breathing may fall into an irregular rhythm with long pauses, and a rattling sound can come from secretions too weak to clear. Hands, feet, and knees grow cool, and the skin there may take on a blotchy, purplish mottling 3Ref 3Hospice Foundation of America (2023).When Death Is Near: Signs and Symptoms.The family-facing signs of the final days shared across serious illnesses: increased sleep, reduced intake, irregular breathing, noisy secretions, and cooling and mottling of the skin..

None of these is an emergency in a person expected to die. They are the expected shape of the final days. Knowing them in advance is part of what keeps the time from feeling like one alarm after another.

Restlessness and confusion near the end

Some people become restless, agitated, or confused in their final days — moving without settling, calling out, seeming frightened. This is common near death and has a name, terminal delirium or terminal restlessness. It is often not fully reversible in the last phase, but it can almost always be settled 4Ref 4Peer-reviewed review (see article) (2020).Improving the Management of Terminal Delirium at the End of Life.That terminal delirium and restlessness are common near death, are often not fully reversible, and can be managed with both nonpharmacologic measures and medicines..

A hospice team looks first for treatable contributors — pain, a full bladder, constipation, breathlessness — and then uses a calm room, a familiar voice, and medicines to ease the distress. In ALS, where a person may be unable to move or speak yet still aware, the reassurance of touch and a quiet voice matters greatly. Someone who seems unreachable can still be soothed.

Comfort care and the fear it hastens death

The purpose of comfort care in ALS is to relieve suffering, and breathlessness is its central target. A common fear is that medicine given for air hunger will speed the end. The World Health Organization describes the palliative approach as one that affirms life and regards dying as a normal process, intending neither to hasten nor to postpone death 5Ref 5World Health Organization (2020).Palliative care.That the palliative approach affirms life and regards dying as a normal process, intending neither to hasten nor to postpone death, while relieving suffering such as breathlessness..

Medicine for breathlessness is given to relieve the symptom and is adjusted to the person in front of the team. A family that holds back relief out of fear can leave someone struggling for air when they did not need to. Whatever the medicine on the label, the anchor is the hospice team — and a nurse line reachable around the clock — to guide how and when it is given for this individual.

Planning ahead for the choices ALS forces

ALS forces decisions that most illnesses do not — whether to use a breathing machine, whether to place a feeding tube, how much intervention a person wants as the muscles fail. Because these choices arrive as the disease progresses, thinking them through early, while communication is easier, spares a family from deciding in a crisis. Early palliative involvement is part of the recommended care for ALS 1Ref 1American Academy of Neurology (Quality Standards Subcommittee) (2009).Practice Parameter Update: The Care of the Patient with Amyotrophic Lateral Sclerosis - Multidisciplinary Care, Symptom Management, and Cognitive/Behavioral Impairment.That end-of-life care in ALS centers on the weakening breathing muscles, addressed through multidisciplinary care, noninvasive ventilation, feeding support such as a gastrostomy tube, symptom management, and early palliative care..

One way to make wishes portable is a medical-order form, sometimes called a POLST, that turns a person's treatment preferences into orders a clinician will follow across settings; research finds care is largely delivered in line with these orders 6Ref 6Peer-reviewed systematic review (see article) (2021).Are We Getting What We Really Want? A Systematic Review of Concordance Between POLST Documentation and Subsequent Care Delivered at End-of-Life.That a POLST turns a person's treatment preferences into portable medical orders, and that end-of-life care is largely delivered in concordance with those orders.. Written down, honored, and revisited as the illness moves, these decisions let the person's own wishes lead.

Many people with ALS choose to keep a way to communicate as speech and movement fade — a letter board, an eye-gaze device, or an agreed set of signals — so that consent and preference can still be voiced late into the illness. It is also worth deciding, in advance and with the team, what should happen if a breathing crisis comes at home, so that a frightening night follows a plan the person chose rather than a reflex to call an ambulance. These conversations are hard, and they are also a gift to the people who will otherwise have to guess.