Promoting Excellence : ALS Report to the Field : Symptom Management at the End of Life


аи Executive Summary
аиаReport to the Field

The following sections summarize recommendations on symptom management in ALS and medical management during the last days and hours of life.

Respiratory Care

Woman Watering Plant Illustration

Identification of Existing Resources

Respiratory insufficiency represents one of the most critical issues for the majority of patients with ALS. The Practice Parameter reviews assessment of respiratory insufficiency and provides practice recommendations for management and is included in Appendix F. However, in addition to the clinical questions addressed in the Practice Parameter, there are several other questions in respiratory care that are critical in evaluating end-of-life care for patients with ALS. These include:

  • What is the ideal way to manage patients who do not have a plan regarding respiratory ventilator support? Some patients cannot make a decision and will by default end up on ventilatory support. In discussing the initiation of non-invasive or invasive ventilation, the ethics and logistics of withdrawal of these interventions should be part of that discussion.
  • How can health care providers work optimally with those patients who have arrived unexpectedly at the decision for respiratory support (e.g., those where monitoring was not performed, or who were unable to make a timely decision, or in whom the diagnosis was made concurrently with the detection of respiratory failure)? For many, the decision-making process in these patients is deferred to health care providers.
  • While withdrawal of ventilator support is legal and ethical, studies need to be done to assess the optimal way to do this in practice.
  • What is the best way to care for patients who change their minds regarding ventilator support? Advance Directives stipulating no ventilator support may be insufficient if not accompanied by a concrete plan of management of respiratory symptoms. This may include overt involvement of palliative care or hospice, education and counseling regarding emergency services and the risk of emergency intubation by emergency medical training staff or emergency room staff.
  • What is the best way to manage expectations and raise quality-of-life issues for patients opting for ventilatory support? The quality of life of those on ventilators needs to be studied to see if there are differences in those with planned and unplanned transitions to ventilator status.

Clearly, the management of respiratory issues in ALS is complex and involves a number of areas of concern outside of the decision regarding initiation and discontinuation of respiratory support. A review of the published evidence, existing resources and helpful tools that address many of these questions is included in Appendix D.

Identification of Existing Gaps

Despite the near universal occurrence of respiratory failure in the patient with ALS, management of respiratory care at the end-of-life period has not been well studied. Several areas remain unexplored, particularly those relative to the use of non-invasive ventilatory support and the current availability of smaller portable invasive ventilators and other mechanical devices. Management of distressing symptoms and signs of choking, aspiration, stridor and dyspnea have not been systematically analyzed. The effects of medications that may offer therapeutic benefits on respiratory parameters also require systematic analyses.

Despite the strong relationship between respiratory dysfunction and death in ALS, great variability exists in the diagnosis and treatment of respiratory failure. Similarly, there is a lack of uniformity in the timing of NIPPV and invasive ventilation (IV). This substantial variability from patient to patient suggests that for a subset of patients respiratory care is suboptimal. Invasive procedures, such as placement of a PEG tube, have not always been performed with an understanding of the pulmonary status of the patient. This oversight carries significant morbidity and mortality risks.

Another gap is that for some health care providers the use of permanent ventilation is not a favorable treatment option for a variety of reasons. Physicians' opinions regarding respiratory support limitations and benefits might influence patients who may have a desire to stay alive and choose to have appropriate tertiary support (or vice versa, where patients choosing not to use respiratory support may be persuaded or influenced to start respiratory therapy).

Respiratory support is very expensive, and inadequate financial resources, including payment from third party payers, may influence the type of care patients with ALS receive. For some patients, funds are provided, but these are usually insufficient to cover all the costs of care-leaving a large financial burden on caregivers and families.

Recommendations to the Field

Practice Recommendations

  • Adhere to Practice Parameter recommendations for respiratory care for patients with ALS.
  • Increase awareness and improve sensitivity and compassion among physicians for patients' needs for management of respiratory symptoms and their needs for respiratory assistive devices, such as NIPPV and In/Exsufflator.

Research Recommendations

  • Conduct prospective studies to determine whether NIPPV improves quality of life and prolongs survival for patients with ALS. Assess how the use of respiratory devices facilitates or interferes with meeting the patient's goals for the end of life.
  • Identify the following:
    • Whether early ventilatory support alters prognosis;
    • The optimal timing of ventilatory intervention; and
    • Better noninvasive methods of ventilatory support.
  • Study patients' wishes and feelings about living with a ventilator and identify how much the insufficiency of current insurance coverage interferes with patients' wishes to live with a ventilator.
  • Study methods of withdrawing both invasive and noninvasive respiratory support in ALS and assess how respiratory symptoms should be managed clinically.

Policy Recommendation

Current Medicare criteria for NIPPV are too strict. Lobby Medicare to liberalize the criteria allowing NIPPV. Pressure Medicare to lower the barrier for reimbursement for NIPPV and tracheostomy.

Nutrition and Hydration

Management of nutritional care for patients with ALS becomes more challenging with disease progression as patients lose motor function, particularly the ability to feed themselves and to swallow. As a result, non-oral routes of providing nutrition and hydration are implemented. Guidelines on how to monitor the nutritional state and provide nutritional support are reported in the Practice Parameter. The literature reviewed in Appendix D suggests that patients are very reluctant to proactively accept PEG. When the decision is finally made to move forward with a PEG, the disease severity often is more advanced and the benefits of PEG are less evident. The consensus is that PEG should be placed earlier in the disease process-yet consensus defining the optimal time for PEG placement has not been established.

Identification of Existing Gaps

One gap is addressing the patient's reluctance to proactively accept PEG. Within the medical community, there may be considerable variation on how rigorously PEG is recommended. Additionally, as the illness progresses and patients approach the end of life, decisions need to be made regarding withdrawal of nutritional and hydrational support, yet optimal timing in the context of end-of-life care is not known.

Recommendations to the Field

Practice Recommendations

  • Proactively discuss the importance of nutritional care with patients with ALS, including earlier initiation of PEG.
  • Adhere to the recommendations made for nutritional care in the Practice Parameter recommendations.
  • Discuss Advance Directives regarding PEG and hydration, including self-determination of withdrawing nutritional and hydrational support during the end of life.

Research Recommendations

  • Prospectively investigate survival benefits and quality-of-life benefits of PEG.
  • Assess nutritional needs during the end of life in patients with and without PEG.

Policy Recommendation

Increase coverage for supplemental medical equipment needed for feeding patients with ALS. This includes feeding tubes, special equipment for eating and drinking and nutritional supplements.

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Promoting Excellence in End-of-Life Care was a national program of the Robert Wood Johnson Foundation dedicated to long-term changes in health care institutions to substantially improve care for dying people and their families. Visit for more resources.

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