The following sections summarize recommendations on symptom management in
ALS and medical management during the last days and hours of life.
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.
- 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
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
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
of emergency intubation by emergency medical training staff or emergency
- 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
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).
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
- Adhere to Practice Parameter recommendations for respiratory care
for patients with
- Increase awareness and improve sensitivity and compassion among physicians
needs for management of respiratory symptoms and their needs for respiratory
assistive devices, such as NIPPV and In/Exsufflator.
- 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
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
- 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.
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
- 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
- Discuss Advance Directives regarding PEG and hydration,
including self-determination of withdrawing nutritional and hydrational
the end of life.
- Prospectively investigate survival benefits and quality-of-life benefits
- Assess nutritional needs during the end of life in patients with
and without PEG.
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.