Ways to Do the End of Life
Since ALS is, as of 2024, a fatal disease, in some ways, care for ALS is completely palliative (aiming at comfort rather than cure) from the day of diagnosis.
That does not mean that life with ALS is nothing more than a journey to death. It can and should be so much more — time with family, friends, hobbies/passions/work.
Many PALS write books, music, poetry, or create art, or continue in their professions for years past diagnosis. They can watch as children and grandchildren grow, be present for events, travel, find wonder in nature, visit museums, attend concerts, etc.
But living with ALS does mean that there is no longer a need to measure, record, and limit any intervention that makes the PALS more comfortable while still retaining the capabilities that they want to retain in that moment.
A "good" death rests on foundations of autonomy, mutual agreement, and most often a peaceful passage at home.
PALS deserve the greatest voice possible in their own life and death. This is made possible when the PALS has specified wishes before or without dementia, and the CALS has agreed to support them, while allowing for possible changes.
Unnecessary suffering can and should be minimized to the extent desired by a PALS. But "natural death" often entails more suffering than a PALS wants.
Death anywhere can be comfortable, but for most, home is preferable.
Starting Points
A few basics to keep in mind:
• You don't need to "use hospice" to have a good death.
• If someone is suffering, you give drugs as needed, adjust positioning, BiPAP and nutrition, and work the problem to address the cause. Most PALS do not require opioid drugs for most of the journey.
- No PALS has to say they can't breathe or it hurts to breathe until the very, very end, when you can stop measuring morphine. If they do, it's a mask, machine, and/or settings issue.
• You can give O2 near death if it improves comfort, but most often opiates are sufficient.
• When someone says it’s the end of the end, it usually is. There is no shame and no dishonor in deciding not to continue “fighting” an immovable force anymore, and it is primarily a physical issue, not a mental one.
• When the end is near, contact anyone that should know about or be around for the final chapter
• Leave it all out on the field. Don’t harbor regrets you don’t need to.
Preparing from the Beginning
When first diagnosed with ALS, planning and paperwork that will be invaluable later, depending on what applies to your location, includes:
A full family discussion with anyone who'll be implementing the end of life plan. This is no time to put it off — any one of us could die tomorrow. Everyone, not just P/CALS, should have this chat.
Health care power of attorney
Advance directive
POLST or other locality-specific directive for EMTs
An Emergency Protocol for hospital staff and first responders
How the End of Life Begins
Gradually, PALS often use BiPAP more and more, sometimes continuously. However, even then, a PALS may gradually lose even the assisted ability to transfer, communicate, and/or engage with others because so much effort is required just for breathing, even when it does not appear that the person is struggling for air.
Some clinics will write an order for hospice at a certain point in progression. Depending on caregiving support, the PALS’ wishes, and available options, you can decide to enter hospice or not. Make sure all major equipment needs are met first, since Medicare will not fund DME for hospice patients.
The one thing to make sure of is that the BiPAP settings have been adjusted for maximum comfort (usually turned down to accommodate muscles that can't handle as much air any more), so you can be sure that the struggle is really “the end” and not just a signal that BiPAP settings have lagged progression.
Bringing Peace
When the choice becomes air hunger due to BiPAP's no longer being enough, and when there can also be increasing pain that arises with ongoing immobility, many PALS opt for a "self-directed death," which is different from Death with Dignity laws or asking hospice to manage their death.
Instead of a legal transaction or relying on third parties such as hospice agencies, "self-directed death" can be carried out by the PALS and CALS, with other loved ones attending as desired, at home, by adjusting medications such as morphine to full comfort while BiPAP is being used. As the medications take effect, BiPAP support (target volume, backup rate) may be gradually reduced to permit a peaceful death, over a period of minutes to hours.
Of course, the PALS and CALS should understand and agree in advance (if the PALS can no longer communicate or participate) or at the time, that this process will hasten death. The "advance agreement" should be reflected in writing, with a State-appropriate advance directive and health care power of attorney, as well.
This is also the time to summon any out-of-town family or friends that are needed for the final chapter, to call/email/text others, make any farewell social posts, etc. It is also the time to reinforce to those in the household, if needed, that no ambulance calls are to be made, and no drastic measures taken.
In much of the world, death is still a taboo subject and therefore a "don't ask, don’t tell” custom is practiced; thus, your choices may be legal, but it can be prudent not to discuss them openly so that other people's misconceptions of what is legal do not present any difficulty or concern for you.
When this time comes, CALS can freely administer morphine, codeine, alcohol, marijuana, psychedelics such as ketamine, mushroom, or anything else that helps the PALS feel better.
When air hunger, a feeling of breathlessness despite optimal BiPAP settings, first manifests, liquid forms are best and can be given orally or via tube.
However, if you use hospice, agency staff may be trained to deliver morphine as injections since most cancer patients, the bulk of their clientele, don't have a feeding tube and often find swallowing difficult.
When morphine and other comfort aids are no longer being measured, and life is winding down, the CALS can gradually reduce the backup rate and target volume, allowing breaths to become slower and shallower as the medication(s) work. But if the CALS does not know how to do this (and if you're not on the last mile yet, it's a really important thing to learn; ask me), it is not strictly necessary.
You may want to look at the screen to see "spontaneous trigger 0%," which means that the machine is initiating all the PALS' breaths. Or you may not. It does not matter.
When their PALS is comfortable and deeply asleep, the caregiver can stop the machine and remove the mask. That part is actually necessary to avoid prolonging what isn't really "living" any more. And in that moment, that person provides the last, best gift: a peaceful death.
Legal & Religious Considerations
Other than select countries and states, the laws in the US and other countries prohibit the administration of drugs or other means as a way to actively end life. However, major world religions, including Catholicism, Hindi, Islam, Judaism, Protestantism, and others are in accord with the principle of avoiding preventable suffering.
Though no professional legal advice is offered here, to my knowledge, no caregiver has ever been prosecuted for administering drugs with that intent, knowing that is the PALS' wish.
Having documentation in place as to the person's wishes, however, is always desirable, especially in cases where a coroner's representative or law enforcement officer may wish to see it. For example, when my husband died, since he was not enrolled in hospice, the paramedic that pronounced him dead did ask for a copy of his advance directive.
If/when all breaths are being triggered by a machine "backup rate" (where the machine starts breaths so the person is getting a minimum number of breaths per minute) substantively the PALS is being “kept alive by a machine.” To the extent that a PALS has previously specified that such is not their wish, ending BiPAP support in this scenario is fully compliant with the advance directive.
In ALS, especially if a PALS is relatively new to morphine, titration to "full comfort" may easily amount to end of life, which may be acceptable to someone who does not wish to survive indefinitely with any level of intractable air hunger.
Since severe air hunger that can no longer be controlled with BiPAP signals the end of life, arguably titrating morphine to comfort is not actually hastening death — it is simply making it more comfortable.
What to Avoid
Don’t call EMTs for transport unless you would be OK with hospital admission — most often, admission will be recommended once you arrive, and it's very unlikely that EMTs can fix the problem without transporting the PALS to the hospital.
Never leave a PALS alone in a hospital, least of all in ER. A joint can be dislocated by a careless blood draw. A BiPAP can be accidentally disconnected. A drug order can have the decimal point in the wrong place. Two of the three have happened to PALS I know, and the third was narrowly avoided by, you guessed it, a caregiver.
Don’t go to any ER where you wouldn't want to be admitted. You have the right to select any hospital for stabilization in an emergency, whether in or out of your network, and you can ask the EMTs to take you to the ED of your choice. However, you may have to fight your plan over what was a true emergency if you go to a non-network hospital.
Hospice
As noted above, you do not need to sign on to hospice for the death you want. I've helped people who used hospice and those, like us, that did not. If you are interested in what's involved, ask around and interview the agencies that you are interested in, that are in your network.
Remember that any physician and many other types of clinicians can prescribe the opioids that are often used to reduce air hunger, pain, and anxiety at the end of life. Any such legitimate prescriber will want to have seen you in person in the last year.
Get all major equipment before you sign on the dotted line. Medicare does not fund equipment for those in hospice, and hospice agencies get only a small monthly payment for hospice patients no matter how many resources you use.
VSED
The media have popularized the option of "Voluntarily Stopping Eating and Drinking" (VSED) as a gentle way to die.
It can be hellish. And it's completely unnecessary.
Is that the death you would impose on a dog or cat? A horse or cow?
VSED can be a long, grueling slog for both the PALS and CALS. And it is far from the only legal way to self-direct death.
Titrating Medication
If someone considers their life over, simply titrating medication to full comfort, basically taking pain and air hunger as symptoms that you are treating, may hasten death. But that is not the goal. The goal is lack of pain and lack of air hunger. That's the care plan, same as it would be if someone were dying in ICU.
Many of us were brought up to believe that "only God can take a life" or "we must wait for nature to take its course." I have worked in health care for more than 40 years and have never seen anyone but people deliver lifesaving interventions and withdraw life support. These are decisions that are made in hospitals, assisted living facilities, nursing homes, and yes, residential homes like yours every day. They're made by imperfect humans to ease an only-human passage into peace.
MAID & "Death with Dignity" Laws
With a willing CALS to work with you on "DIY hospice," titrating meds to full comfort, you do not need these laws. Some people may prefer to use them, but they entail a lot of steps, may therefore prolong suffering, and some still require self-dosing, pretty silly in ALS.
Nor do you need to go to Switzerland.
In every country, there is a doctor willing to prescribe appropriate medication to improve comfort at the end of life, if that is the request that you make.
So often, I see P/CALS dooming themselves to pointless suffering because they do not have the "death with dignity" laws wherever they are. When I fail to change their minds, I think it is because they truly do not believe they have that freedom. But this is a society of constant war and the most fearsome weapons. Whatever you believe created those or made them possible, wouldn't that same power apply to a more peaceful transition from life?
Whether supporting your CALS or supporting your PALS, no law can substitute for your own best efforts and sound judgment.