By Dr. Ferdinando Mirarchi, DO — Board-Certified Emergency Physician, Founder of MIDEO Health
There is a moment in every case of ALS or progressive cognitive or neuromuscular disease when the person can no longer clearly state what they want. The day before that moment, they could speak for themselves. The day after, somebody else has to.
That single shift is one of the most consequential things that can happen to a patient, and most people never plan for it. ALS may take your voice. Dementia may take your ability to weigh a complex decision. But neither one should be allowed to take your choice.
This article walks through why a standard advance directive is not enough for a progressive neurodegenerative disease, how other people’s emotions and assumptions start filling the silence when a patient can no longer speak, and why your wishes belong in a medically guided video directive that captures your actual voice while you still have it.
An Example of Why an ALS Advance Directive Is Non-Negotiable
Here is a real example of what could happen.
You are 38 years old and you have just been diagnosed with ALS.
Previously, you had a history of high blood pressure, high cholesterol, and a family history of cardiac disease. It is a normal day and you develop chest pain. Because it is severe, you go to your local emergency department.
You are seen by an emergency medicine physician and they determine you are having a heart attack. Not just any heart attack. This one is called a widow maker. This means about 30% of the time we know that person is going to go into cardiac arrest.
Now the emergency medicine physician, who is a medical stranger to you, knows that you have ALS. And even though you are at an early stage of your diagnosis, the physician has a vision of what your future life would be like as far as how the ALS disease will progress.
This bias is already playing into their decision making on how aggressively they should treat you. The physician thinks about it as if they were the patient with ALS. This is what we call a “projection bias,” and then they apply that projection bias to how they are going to treat you in an emergency.
So say this heart attack, this widow maker, turns into cardiac arrest. This physician is thinking, should I save this person’s life, or should I just let it end now so they do not have to experience what is going to happen to them in the future?
That projection bias can cause delays in your care and prevent them from saving your life.
What no one in that room actually knows is what you want
What we do not know in this equation is what “you” really want as far as treatment wishes. Maybe someone told you to document your wishes in the form of an advance directive or a living will, not knowing that those types of documents are not safe for the general population, as shown in the TRIAD Patient Safety research, and are surely not safe enough for those with ALS or a neurodegenerative disorder.
What we see happening with those types of documents is that the medical providers and physicians, who are medical strangers to you, think you are a do not resuscitate patient.
Remember, at this point medical strangers are just guessing, because they do not really know what you want.
Now, you were just diagnosed, or maybe you have a moderate stage of your ALS diagnosis. You may have set goals for yourself, and those should be respected and honored. For example, maybe you have young children and want to spend more time with them and watch them grow up. Maybe you have family goals, such as a son or daughter graduating from school. Or maybe you have already decided that you do not want to experience the kind of progressive course you know is coming with your ALS diagnosis, and you are ready to die.
The point here is that these should be your decisions, and not left up to a medical stranger to decide. ALS can take your voice, but with newer patient safety technologies like MIDEO, ALS should never be allowed to take your choice.
Do not let ALS take your voice. With MIDEO you can preserve your voice and appearance now to control future aspects of your care.
Why You Need More Than an ALS Advance Directive
When ALS or dementia does take your voice and your ability to make your own choices, you will need to appoint something called a healthcare agent (HCA). It is extremely important to do this as soon as possible.
This appointed HCA is usually a spouse, an adult child, a sibling, and sometimes even a close friend. Each of those people loves you as the patient and fears for you as the patient. Each of them wants to make the right decision for you, and many of them, if not prepared, will feel a sense of guilt or fear in making decisions for you.
If you create a standard paper based dementia advance directive, then you are setting them up for a guilt ridden process and future decisions that will afflict them with turmoil.
This is another reason that embracing patient safety technologies can assist them now and in the future, when they have to care for you and make medical decisions for you. We have an entire section dedicated to how MIDEO sets the process up for success below.
Why a Dementia Advance Directive Has to Happen Before Your Window to Speak Closes
Both dementia and ALS give the patient a finite window during which they can still articulate what they want, and then that window is slammed shut.
In dementia, cognition fades first. Speech often remains intact long after the patient has lost the underlying capacity to make complex medical decisions. A patient may sound like themselves while no longer being able to weigh trade-offs, project consequences, or hold a coherent picture of their future preferences.
In ALS, the inverse happens. Cognition stays sharp in the vast majority of cases. The voice is what fails. Speech remains functional for an average of about 60 months from disease onset in spinal-onset ALS, but for bulbar-onset patients, the time to speech loss can be as short as 23 months.
In both cases, the window for capturing the patient’s actual voice is shorter than people expect.
The Biggest Risk Is Not the Silence. It Is Other People Filling It In for You.
When a patient with dementia or ALS can no longer clearly state their wishes, the silence does not stay silent. This is particularly true for people with ALS. When a person with ALS starts losing the ability to speak, other voices try to fill the void.
For example, a spouse may speak from fear. An adult child may speak from hope. A friend may speak from guilt. A clinician may speak from habit, training, or what usually gets recommended in similar cases.
And when the patient’s own voice is silenced, other people’s emotions and assumptions can start shaping decisions in ways the patient never intended.
Family bias
The spouse who married you forty years ago is not a neutral interpreter of your wishes. They are someone facing the loss of the most important person in their life.
The adult daughter making decisions about her father’s care is carrying her own guilt, her own grief, her own complicated history with him, and her own assumptions about what “Dad would have wanted,” assumptions that may be more about who she needs him to be than who he actually was.
The son who lives three states away and only comes home for emergencies will arrive with his own version of the patient’s wishes that may not match what the local caregiver has been hearing for two years.
Each of these family members loves the patient. Each of them is also a person whose own fears and needs will color how they hear, remember, and represent what the patient said.
Clinical bias
The path of least legal risk is usually “do everything possible.” The training of most physicians, especially in emergency and acute care settings, is oriented toward saving life, which is exactly what we want most of the time, but it is not always what the patient actually wanted in a specific situation.
A clinician reading an ambiguous advance directive at 2 a.m., with a family in the waiting room, has institutional incentives to resolve that ambiguity toward more treatment.
For the patient with dementia or ALS whose wishes were specifically about not having certain interventions, that same system overrides their wants.
A dementia advance directive can do the speaking
These conversations should happen while the person with ALS or dementia is still able to express the type of care that they want.
Why is it so important that the patient’s voice gets heard? One major systematic review found that surrogates incorrectly predict patients’ end-of-life treatment preferences about one-third of the time. That is a staggering number when the stakes involve ventilation, feeding support, hospital care, or comfort-focused care.
This is why an ALS advance directive or dementia advance directive is the best way of protecting the patient.
Waiting Can Cost You the Chance to Be Clear
A lot of people delay advance care planning for understandable reasons. For one, the diagnosis is overwhelming and the future feels hard to face. Oftentimes, families want to stay hopeful and patients do not want every conversation to sound like surrender.
But in ALS, or in any type of progressive dementia, waiting puts you and your family at risk.
Because it is progressive, this is one reason ALS advance directive planning matters so much. Advance care planning is not supposed to be a one-time or an all-or-nothing conversation. In fact, advance directives can be changed as the situation changes. In other words, acting early does not trap a person in one permanent decision.
Why an Advance Directive for Dementia or ALS Needs to Be Detailed and in Video
A standard written advance directive is a start, but it is not enough for people experiencing dementia or ALS.
The legal ethicist Rebecca Dresser, in a widely read Hastings Center Report critique, argued that dementia-specific advance directives are too simplistic to serve patients well. Her critique is uncomfortable to sit with, and it is not wrong about the documents she was critiquing. Checkbox advance directives genuinely struggle with the complexity that Dresser names.
What checkbox advance directives also struggle with is the bias problem we just discussed above. A written line that says “I do not want artificial nutrition” requires someone to interpret it, and as we just saw, the people doing the interpreting are bringing their own grief, fears, and institutional defaults with them.
A family member may hear “keep me comfortable” and assume that means no feeding tube. Another may hear “I still want to fight” and assume that means full-time invasive ventilation no matter what.
A line on a form provides almost nothing for the patient’s actual reasoning to push back against. The family member who wants to override it has only a sentence to push against. Unfortunately, the patient’s voice is nowhere in the document.
Standard advance directives leave too much of the patient out when it comes to progressive diseases like ALS and dementia.
These diseases can force decisions about:
- Noninvasive breathing support
- Invasive ventilation
- Feeding support
- When to voluntarily withhold food and drink
- Resuscitation
- Hospital transfers
- Medical Aid in Dying (MAID)
In both ALS and dementia, the directive should not just state preferences in vague moral language.
A strong directive explains what those words mean to the patient. It draws lines. It gives context. It helps loved ones understand not just the choice, but the reasoning behind it. That is what makes a directive useful when ALS has already taken away the easiest way to speak.
There is a way for people with ALS or dementia to create an advance directive in their own words and on video, so nobody can mistake what they mean. We discuss it in the section below.
MIDEO Preserves Your Voice and Your Choices, in Your Directive and in Your Own Words
The truth is that a MIDEO video advance directive does what a written form structurally cannot.
“I do not want to be kept alive on a ventilator if my doctors believe my condition is irreversible, and the reason is that I watched my father go through that and I would not want my children to live through it again.”
When a person sits down and records themselves saying something like that, the family member who wants to override that decision is no longer arguing with a sentence on a form. They are watching the person they love explain themselves, look the camera in the eye, and own their reasoning.
For dementia and ALS specifically, this matters more than in almost any other condition, because the window for capturing it is closing.
A MIDEO video advance directive is medically guided and lets everyone hear your voice stating your wishes. The video is paired with a legally compliant written advance directive and made retrievable at the bedside through a QR code in seconds.
If you or someone you love has been diagnosed with dementia, ALS, or another progressive condition, MIDEO can help you preserve your wishes. Talk with a board-certified MIDEO doctor, or get started on your own through the self-guided platform.
