Info for Emergency Professionals

Emergency Medical Services/Emergency Room Information

  • Oxygen given alone may depress respiratory drive in ALS, causing elevated CO2, leading to respiratory failure. Often lungs are healthy, but respiratory muscles including the diaphragm, may be weak.
  • If shortness of breath or low oxygen saturation (SpO2) is present, may use supplemental O2 with close monitoring of patient.
  • Use pulse oximetry and capnography for monitoring.
  • Non-invasive ventilation (i.e. Bipap™ or Trilogy™) or bag-valve-mask assisted breathing should be used to expel CO2 and increase SpO2. CPAP is contraindicated as it may increase the work of breathing.
  • Bleeding in oxygen to non-invasive ventilator is best for adding oxygen, thereby increasing SpO2 while keeping CO2 down.
  • Non-invasive ventilation may help avoid the necessity of invasive ventilation.
    Note: Weaning/extubation is often difficult or impossible once invasive ventilation is initiated.
  • If patient is on non-invasive ventilation at home, bring equipment, tubing and mask and use enroute as required. The caregiver will know how to use it and may better understand speech while wearing it. If possible, caregiver should accompany patient on the ambulance.
  • If using ambulance or hospital non-invasive ventilator, consider beginning use at same settings as home equipment to start, then titrate.
  • If new to non-invasive ventilation, therapy might be started with beginning pressures of 10/5 and backup respiratory rate of 10 via mask, then titrate as needed for efficacy.
  • If using a BIPAP™ instead of Trilogy™, be aware that pressure support will not self-adjust for changes in body position and that patient may be at risk of aerophagia leading to increased dyspnea.
  • When possible, avoid paralytic or general anesthetics, narcotics, muscle relaxants or other sedation unless patient is on non-invasive ventilator support with a back-up rate as these medications may dangerously decrease respiratory drive.
  • If meds must be used, the ability to rapidly assist ventilation should be available.
  • Lying supine may cause SOB due to respiratory muscle weakness, perhaps resulting in CO2 retention and low SpO2.
  • If lying flat is necessary, wearing non-invasive ventilator (i.e. Bipap™, Trilogy™) would likely help breathing. If patient has excessive secretions, protection of airway should be monitored.
  • Aspiration is a danger and may be the cause of SOB. Many patients have decreased ability to protect their airway.
  • Have a suction machine available and set up, oral secretions may be excessive.
  • If available, a Cough Assist (insufflator/exsufflator) is preferable to suction as it can more forcefully “pull” secretions and mucous plugs out of the airway.
  • If a gastrostomy tube is in place, consider using for administration of “oral” medications, as appropriate.
  • Swallowing may be compromised, evaluate prior to PO intake. Patient/caregiver may have prior recommendations.
  • Dysarthria is common including slurring, slowness, inability to speak at all. Do NOT assume alcohol or drug use.
  • Speak in a normal voice and allow time to communicate. Yes/no questions may be best. Speech boards, paper and pencil, and other devices may be utilized.
  • Avoid separating patient from caregiver, they are knowledgeable about speech, treatment needs and equipment.
  • If possible, allow caregiver to be present during interventions to assure appropriate handling and communication.
  • If patient is left alone, ensure they have call system that they can use. Many patients cannot push a button, consider alternative system, for example pillow, tent or microlight switch for use with head or other body part.
  • Use caution moving; muscles may be atrophied or stiff and range of motion limited. Do not lift by the arms if weakness/atrophy present as this may lead to shoulder dislocation.
  • Gait may be unsteady from ALS, take precautions as needed. When mild-moderate assistance is needed, suggest using a gait belt which will give greater support without injuring weak shoulder girdle.