Title: Fast Fact and Concept #73: ALS: Management of Respiratory Failure
Author(s): Ajmal Gilani, MD; Albert Hinn, MD; Peter Lars Jacobson, MD
Respiratory failure is the most common cause of death from ALS. Except for rare patients who present with respiratory failure, respiratory muscle weakness develops insidiously during the course of the disease. Options for ventilatory support should be discussed long before the development of respiratory insufficiency. A power of attorney for health care should be obtained and/or healthcare proxy identified by the patient and family.
ASSESSMENT OF RESPIRATORY INSUFFICIENCY
Early signs and symptoms of respiratory muscle weakness are subtle: dyspnea w/ mild exertion, supine dyspnea, insomnia, morning headache, reduced appetite, weight loss, dizziness, depression, anxiety and marked fatigue. There are no standard tests to detect early respiratory failure.
The following tests may be helpful:
CHOICES FOR RESPIRATORY MANAGEMENT
Bi-level Positive Airway Pressure (BiPAP), a type of Non Invasive Ventilation (NIV), can improve symptoms of hypoventilation, quality of life, and survival by several months. Some patients, particularly those with bulbar involvement and difficulty in clearing secretions, cannot tolerate BiPAP. The sole use of oxygen at night is not sufficient; patients with oxygen desaturation and chronic hypercapnia require nocturnal BiPAP machine to avoid suppression of respiratory drive during sleep. During the day BiPAP can be used to alleviate respiratory muscle fatigue. The use of Intermittent Positive Pressure breathing machines has been reported to expand the lungs and reduce atelectasis.
Full time ventilator support is sometimes considered when NIV is no longer effective or tolerable Treating reversible problems (e.g. acute respiratory infection) is important to avoid the need for invasive long-term ventilation w/ tracheostomy. Most patients who select a ventilator become completely dependent on it and are unable to communicate verbally. Many patients will choose not to be ventilated if comfort is assured and advance care planning occurs.
Regardless of assisted ventilatory choices, compassionate and effective palliative care must be implemented. A calm environment, the reassuring presence of relatives, trunk elevation and chest physiotherapy may all provide relief. The sensation of shortness of breathing is reduced by morphine (2.5-10 mg by mouth or 1-2 mg IV/subcutaneous every 1-4 hours). Titration of the morphine dose against the clinical effect almost never leads to a life threatening respiratory depression. (see Fast Fact #27: Dyspnea at End-of-Life) Anxiety due to respiratory insufficiency can be treated with lorazepam, (0.5-1.0) sublingually. When co-morbid medical complications develop, or patient determined quality of life deteriorates, ventilator support can be withdrawn and symptom control provided to allow for a comfortable death. See:
Fast Fact #33: Ventilator Withdrawal Protocol (Part I)
Fast Fact #34: Symptom Control for Ventilator Withdrawal in the Dying Patient (Part II)
Fast Fact #35: Information for patients and families about ventilator withdrawal (Part III)
Carver, Alan C., Foley, Kathleen M. Neurology Clinics, Palliative Care. W.B. Saunders Company 2001;835-836.
Bradley, Walter G., Daroff, Robert B., Fenichel, Gerald M., Marsden, Davis C. Neurology in Clinical Practice Vol. II. Butterworh, Heinemann 2000;2005-2014.
Bach, JR. Amyotrophic lateral sclerosis: predictors for prolongation of life by non-invasive respiratory aids. Archives of Physical Medicine and Rehabilitation 01-Sep-1995; 76(9): 828-32.
Pinto AC, Evangelista T, Carvalho M. Respiratory assistance with a non-invasive ventilator (Bipap)00 in MND/ALS patients. Journal of the Neurological Sciences 01-May-1995;129 Suppl:19-26.
Howard, RS, Wiles, CM, Loh, L. Respiratory complications and their management in motor neuron disease. Brain 01-Oct-1989;112(5):1155-70.
Oliver D, Borasio GD, Walsh D; Palliative Care in Amyotrophic Lateral Sclerosis. Oxford University Press 2000; 45
Copyright and Referencing Information: Users are free to download and distribute Fast Facts for educational purposes only. Citation for referencing Fast Facts and Concepts #73 ALS. Ajmal Gilani, MD; Albert Hinn, MD; Peter Lars Jacobson, MD July 2002. End-of-Life Physician Education Resource Center www.eperc.mcw.edu.
Fast Facts and Concepts was originally developed as an end-of-life teaching tool by Eric Warm, MD, U. Cincinnati, Department of Medicine. See: Warm, E. Improving EOL care--internal medicine curriculum project. J Pall Med 1999; 2: 339-340.
Disclaimer: Fast Facts provide educational information, this information is not medical advice. Health care providers should exercise their own independent clinical judgment. Some Fast Fact information cites the use of a product in dosage, for an indication, or in a manner other than that recommended in the product labeling. Accordingly, the official prescribing information should be consulted before any such product is used.
Creation Date: 7/2002
Purpose: Instructional Aid, Self-Study Guide, Teaching
|Training: Fellows, 3rd/4th Year Medical Students, PGY1 (Interns), PGY2-6, Physicians in Practice|
|Specialty: Anesthesiology, Emergency Medicine, Family Medicine, General Internal Medicine, Geriatrics, Hematology/Oncology, Neurology, OB/GYN, Ophthalmology, Pulmonary/Critical Care, Pediatrics, Psychiatry, Surgery|
ACGME Competencies: Medical Knowledge, Patient Care
Keyword(s): Cardio-pulminary, Neurologic diseases, Treatment withdrawal, Treatment withholding
The Fast Facts series is distributed for educational use only and does not constitute medical advice. For the most current version of Fast Facts visit the EPERC web site at www.eperc.mcw.edu. This mirror version is provided subject to copyright restrictions for educational use within the Inter-Instutional Collaborating Network on End-of-Life Care (IICN).