Death from ALS — It May Not Be as Peaceful As You’d Like

[Originally published on September 29, 2011 by Harwell.  Reprinted by permission of the author.]

If you knew Sharon, don’t read this post — it will just upset you.  But if you didn’t know Sharon, and you’re just trying to understand how death comes to an ALS patient, then read on.

Sharon died from ALS on July 31, 2011. In the months prior to her death I had tried to get an understanding of how someone with ALS would die.  I wanted to know because I wanted to prepare for what was going to happen to her, just as I tried to prepare for everything else in Sharon’s ALS decline.

The only article I found on death from ALS said that it was peaceful and quiet: the patient just gradually sleeps more and more as carbon dioxide builds up in the patient’s system.  Then the patient goes to sleep at some point and doesn’t wake up.

The reality is very different.

During advanced stages of ALS there are four things a caregiver is fighting:

• Communication — how to communicate with a person who can’t speak and who has limited use of arms and legs
• Movement — how to move the person to keep them comfortable and to provide for basic bodily functions
• Pain — mostly from the person being stuck in one position or from not being able to move head, neck, arms, legs or body
• Shortness of Breath — like the sensation of drowning, the person is gasping for air as the body reacts to the lack of oxygen and the build-up of carbon dioxide

Communication and movement are things that have progressively gotten worse throughout the duration of ALS, and I talk about those problems elsewhere.

Pain is something that’s dealt with using medication.  Sharon first started using traditional over-the-counter medications like ibuprofen and acetaminophen.  As the pain increased, she started using heavier, prescription doses of those medicines.   Then when she became more advanced in her ALS and went under a home hospice plan, she started using a low-dose Fentanyl patch to bring her pain to a perceived zero on a scale of zero to ten.  As pain increased, the dosage on the Fentanyl patch was increased to keep her pain at zero.

Shortness of Breath

Shortness of breath is the problem I didn’t anticipate.  Somehow I thought that shortness of breath was like pain and therefore would be treated with pain medication.  As it turns out, this isn’t the case.  Pain medication like the Fentanyl patch does seem to have some impact on decreasing shortness of breath, but the patch alone doesn’t go far enough.  And when I only used pain medication, Sharon would occasionally and suddenly go into what seemed to be panic attacks of shortness of breath — her eyes would get big, you could see the distress in her face, and she would give all of the symptoms and behaviors you would expect from someone who was drowning.

I learned that shortness of breath has to be treated through a combination of three different approaches:

• Use anxiety medication like lorazepam (generic Ativan) to help relaxation.  This doesn’t help her breathe better, but it makes her care about it less. Sharon took a low-level of alprazolam (generic Xanax) during her last six months, then added lorazepam as the shortness of breath attacks got worse.  We started with recommended dosages and had to adjust them to keep her awake when she wanted to be awake.

• Use fast-acting pain medication like morphine sulfate to treat the spikes of pain that occur during a breathing attack.  This medicine takes effect in a few minutes and reaches full effect in about 20 minutes.  But it doesn’t last more than about an hour so it has to be repeated frequently.

• Use BiPap through a mask or oxygen through a nasal cannula to increase the oxygen level in the blood.  BiPap is better than oxygen because it helps not only increase the oxygen level but also help the lungs move the waste carbon dioxide out of the body.  Oxygen through a nasal cannula will increase the oxygen level and therefore reduce the drowning symptom, but it won’t help relieve the buildup of carbon dioxide.  Oxygen through a mask should be avoided because it actually inhibits the ability of the body to remove carbon dioxide. Oxygen in conjunction with BiPap is not something I tried.

The downside of every one of these treatments except BiPap is that they cause sleepiness.  Too much lorazepam will cause the patient to get sleepy due to the increased relaxation.  Too much oxygen will cause the patient to get sleepy due to the buildup of carbon dioxide as a result of the increased oxygen level in the blood.  Too much morphine sulfate will cause the patient to get spacey and sleepy due to the medication’s effect on the brain.

So the battle you’re fighting toward the end with an ALS patient is a trade-off between consciousness and distress.  Distress from the sensation of drowning is never a good thing, but on the other hand, an unconscious patient is not able to derive much enjoyment from life.  You need to constantly adjust medication levels based on how the day is going and who’s visiting.  And you still occasionally get drowning fits that breakthrough your current level of medication and require more medication all of a sudden.

Sharon’s Last Day

On the day before Sharon died she started the day like any other in recent history.  She was on BiPap while she was asleep for the night.  I gave her lorazepam and morphine sulfate when I woke her up, and then I switched her from BiPap to oxygen while I got her ready for the day. She stayed on oxygen all day, and I gave her recommended doses of lorazepam and morphine sulfate on schedule during the day.

She was OK going into the evening, and then she started having drowning fits in spite of the medication.  I increased medication, called the hospice nurse for approval, and then increased it again.  The drowning fits lessened but she was still having difficulty breathing.  I called the hospice nurse again to see what medication I could give Sharon that would help her survive the night without distress, and was told to give lorazepam because it lasts for four hours or so.  So I gave lorazepam before bed, and got Sharon ready to sleep in the normal way, putting her on BiPap.  I set an alarm for 3:30 AM and gave her more lorazepam.  She seemed to be sleeping peacefully, quieter than she usually was at night.

I woke up early, did some writing, then went to wake Sharon.  I gave her a morning dose of lorazepam and morphine sulfate, then tried to wake her up by gently talking to her.  She wouldn’t wake — a  first — but her BiPap was still helping her breathe.  I held her in my arms for a few minutes, she breathed rapidly for a few seconds, then stopped breathing altogether.  The end was peaceful, but not without a few panicky moments the evening before.

Major disclaimer: I’m giving you the benefit of my experience, but your situation may be different and I’m not a doctor.  Please discuss any of these treatment options with a licensed medical professional before implementing any of these ideas.

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From the blog “Sharon has ALS,” written by Harwell Thrasher, who is an author and retired technology expert who became the primary caregiver for his wife Sharon when she was diagnosed with ALS.