Amyotrophic lateral sclerosis  (ALS), is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. ALS, commonly known as Lou Gehrig’s Disease, attacks cells in the brain and spinal cord that carry messages from the brain to the muscles. As motor neurons degenerate and die, it leads to scarring — or sclerosis — in the spinal cord and brain — leading to a lack of control in muscle movement. When the muscles go unused, they atrophy, or waste away. This is when the symptoms of ALS, like the loss of ability to speak, eat, move, and breathe, begin to appear.

While patients often rely on families and physicians to speak for them during a health crisis, studies have shown that neither group accurately understands patient wishes for life-sustaining treatments. And fewer than 40 percent of ALS patients have recorded their attitudes and wishes regarding invasive procedures such as ventilation, resuscitation, artificial nutrition, and hydration. 

For diseases such as ALS, much of the relationship between health care clinicians and patients centers around ensuring they understand their symptoms, understand how to manage their functional impairments, and receive therapy on how to manage disease progression. Modern models of care involve curative and palliative care throughout the course of the disease with curative care at the onset of diagnosis and palliative (eventually hospice) care taking on a greater role as the disease progresses. 

Sadly, there is currently no cure for ALS which leaves patients and health care providers in a cycle of symptom management. This cycle makes the need for advance care planning (ACP) essential due to the need for caregivers to understand a patient’s wishes for their medical care through every stage of the disease. However, broaching the subject of ACP with the patient must be well timed. 

Improving communication between ALS patients and their healthcare team is an important way to respect patient wishes and help them continue to take an active role in their treatment despite disease advancement. Individuals with ALS want to be involved in end-of-life decisions, particularly those involving invasive and heroic procedures, however, some interventions render them unable to communicate effectively. 

At the time of diagnosis, caregivers should be cognizant of the numerous emotions a patient may be experiencing – many of these emotions follow the stages of grief including bewilderment, denial, distress, and sadness mixed with anxiety over disease progression. A discussion regarding ACP at this time could increase feelings of anxiety and cause the patient to tailspin into depression. 

Discussions regarding ACP should instead be initiated during follow-up visits and may be initiated by the physician, the patient, a caregiver, or any member of the medical team. The discussion should focus on the patient and his/her rights and preferences for care. It should also include clarification around issues surrounding power of attorney and a living will.  

When approaching the idea of ACP with an ALS patient, clinicians should make sure they are clear in what happens during each stage of the disease and what treatments are tied to those stages. Instead of telling a patient that they will be on a feeding tube, ask them if they have heard of a feeding tube and understand what it is. This will not only dispel myths but will allow clinicians to open the discussion on patient preferences that can then be documented in the patient’s ACP records. 

Remember that very few patients make decisions in a vacuum. Family (whether that be blood relatives or friends chosen as family) can play a huge role in a patient’s wishes. If a member of a patient’s family will be helping him/her with aspects of care, ensure that both that family member and the patient are aware of what goes into every treatment in which they will be assisting. This could play a vital role in the decision-making process. 

A 2017 study of people with ALS showed the use of a decision-making aid, such as offered by Iris, helps physicians improve their ability to predict a patient’s choice of care in a series of scenarios. Guided decision-making tools such as these can improve patient/clinician communication and therefore patient satisfaction. 

Physicians Michael Baer, M.D., and Colin Quinn, M.D., offer the idea of focusing on the “three Fs” when introducing the subject of ACP to ALS patients: Feelings, Facts, and Family. Focusing on these areas will help address all factors that may contribute to a patient’s decision making. 

Iris representatives have a variety of ways to ensure you manage patient expectations and wishes in the most respectful way possible. Our high-touch Advance Care Planning solution is designed to engage seriously ill members to better align their goals of care with an individual approach to healthcare services. Our team of specially trained ACP experts provide telehealth consultations when it’s convenient (daytime, evening or weekends) for individuals and their families to facilitate highly individualized care planning discussions and provide disease specific education. 


Our tech enabled consultation process allows our experts to document and generate state specific directives and goals of care summaries, which are then widely distributed to their care teams and family members. Our health care planning experts also address symptoms and other medical care needs, help uncover social determinants of health, and needs of caregivers. This approach enables better care coordination, enhances the member experience and improves healthcare outcomes