Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder that targets the motor neurons, the nerve cells controlling voluntary muscle movement. This deterioration leads to muscle weakness, twitching, and atrophy throughout the body. The disease affects a person’s ability to walk, speak, swallow, and eventually breathe. Preparing families to recognize and respond rapidly to sudden, life-threatening crises is a necessary part of managing this condition.
Defining the ALS Emergency
An ALS emergency is defined as any sudden, severe, or rapidly progressing deterioration of a patient’s physical state that compromises vital functions and requires immediate advanced medical care. This situation moves beyond the typical, gradual progression of muscle weakness seen in the disease. The most frequent and serious crisis involves the respiratory system, as the muscles responsible for breathing become increasingly impaired. Other events can also constitute an emergency, such as a sudden and profound worsening of general muscle weakness or a quick decline in the ability to manage saliva and phlegm.
The inability to clear the airways can lead to choking or aspiration, which can quickly become life-threatening. Sudden confusion, unresponsiveness, or a significant change in mental status also signals a medical crisis. These events often stem from a sudden drop in oxygen levels or a dangerous buildup of carbon dioxide (hypercapnia). Recognizing these warning signs is the first step in seeking appropriate help.
Recognizing Acute Respiratory Distress
Respiratory failure is the most common cause of death in people with ALS, making the recognition of acute respiratory distress the most important aspect of emergency preparedness. Early signs of respiratory muscle weakness can be subtle, sometimes presenting as shortness of breath (dyspnea) only with mild exertion or when lying flat. This difficulty lying down, called orthopnea, occurs because the weakened diaphragm must work harder against the abdominal contents. As the disease advances, shortness of breath may become apparent even at rest.
Changes in breathing patterns can provide observable evidence of a crisis. Shallow breathing is common, but a particularly concerning sign is paradoxical breathing, where the abdomen moves inward instead of outward during inhalation. This indicates that the diaphragm is severely weakened, and accessory muscles are struggling to compensate. Behavioral changes also signal reduced oxygen intake or carbon dioxide retention, often manifesting as severe anxiety, restlessness, or an inability to complete full sentences without pausing for breath.
Less obvious symptoms indicating chronic respiratory insufficiency worsening into an acute event include waking up with morning headaches or feeling “fuzzy-headed.” These symptoms result from hypoventilation during the night, leading to carbon dioxide retention. Visual cues of a severe lack of oxygen, such as a bluish discoloration of the lips, fingertips, or pale skin, are late-stage signs of hypoxia. For those using monitoring devices, a sudden, sustained drop in pulse oximetry readings below 95% indicates a medical emergency.
Immediate Response and Emergency Protocols
The immediate response to an ALS emergency is to contact local emergency services by calling 911 or the local equivalent without delay. When speaking with the dispatcher, it is imperative to clearly state that the patient has Amyotrophic Lateral Sclerosis and is experiencing a respiratory emergency or inability to breathe. This information alerts first responders to the unique physiological needs of an ALS patient, who may have difficulty communicating due to bulbar involvement.
Caregivers should be prepared to convey medical information immediately upon the arrival of the emergency medical team. This includes specifying any existing respiratory support equipment, such as a BiPAP or ventilator, and its current settings, as well as the presence of a gastrostomy tube or any Do Not Resuscitate (DNR) orders. This information should be pre-written and readily accessible, perhaps laminated and stored near the patient or on their mobility device.
Before medical help arrives, position the patient to maximize their breathing capacity. The person should be placed in an upright or semi-sitting position, as lying flat (supine) can further compromise the weakened diaphragm. If the patient has suctioning equipment available, it should be used to clear obstructing secretions. Supplemental oxygen should generally be avoided unless specifically prescribed, as it may mask respiratory failure signs and does not address carbon dioxide retention.
Caregivers should remain calm and communicate clearly with the emergency team. The medical team must be informed that the patient’s lungs are healthy, but the muscles controlling them are weak. Preparing for transport, including gathering any respiratory equipment and a medical information file, will help ensure a smoother transition to the hospital.