One night in late October 2014, Priscilla was awakened around midnight by pain spreading throughout her lower back. She couldn’t get back to sleep and at 4:00 she woke me up for help. I gave her a heavy dose of pain killers and we both slept a few more hours. Then we called her nurse practitioner and home health care nurse.
They sprang immediately into action, because they’d already become concerned about her. The nurse practitioner had noted on a recent visit that Priscilla’s heart was beating rapidly. She’d suspected this was due to failing lungs: the blood was oxygen-poor, and so the heart was trying to send more blood around to deliver what oxygen it could. The nurse, for her part, had witnessed the decline in Priscilla’s breathing on her weekly visits and had already been thinking it was time to get her into hospice. She just hadn’t said anything to us about it yet because she knew families had to accept what this meant before they could consider it fairly, so she was waiting until we brought it up ourselves.
The nurse practitioner got to our house as soon as she could and found Priscilla breathing rapidly and shallowly, with her heart racing. She called her supervising doctor and he agreed that Priscilla was likely “compensating” for oxygen-poor blood. But this was not sustainable. “Sooner or later she’s going to ‘de-compensate,’” he warned, meaning that the heart would become worn out by the exertion and slow down, leaving the body starved for oxygen. The heart might even stop entirely. The nurse practitioner later told us that she’d thought this could be the end.
But it wasn’t. Somehow Priscilla’s body worked out a different way to “compensate.” (No one ever figured out exactly how; “our bodies are marvelous things” was the only explanation the nurse practitioner could offer.) She pulled out of the crisis, but it was agreed that she would need some new kinds of help.
Priscilla had gotten the VPAP in April. Four months later, by August, the symptoms of carbon dioxide buildup had returned, so the respiratory therapist (RT) increased the pressure. Now, only two months further on, the symptoms were returning again (the back pain, for example). It seemed the weakening of the lung muscles was accelerating. The RT now recommended another pressure increase and switching the VPAP from “spontaneous” mode (responding when Priscilla initiated a breath) to “timed” (breathing for her if she didn’t initiate within a certain period). Her caregivers also felt that the pressurized air should be supplemented with oxygen.
Priscilla agreed to give these measures a try, once she was assured that they’d only provide comfort and not artificially prolong her life. But she found that the oxygen dried out her throat and made her terribly thirsty, while the “timed” breaths kept waking her up. She decided she’d rather die during a good sound sleep, if it came to that, than suffer through thirsty, broken nights, so she went back to plain “spontaneous” breathing.
Priscilla’s medical team now all encouraged her to enter a hospice program. This could be at-home hospice if she didn’t want to move into a residence. They explained that sooner or later, as her lungs weakened, she would experience “air panic”—the sensation that she was drowning—and that she’d need liquid morphine to turn off this signal to her brain. Home health care agencies didn’t have it; hospices did. “And you really don’t want to be without it when you need it,” they warned.
By then it was early November. The entrance into home hospice was delayed as we investigated whether Priscilla could keep her current caregivers. Unfortunately, she couldn’t keep the nurse and aide from home health care, but she would be able to keep her case manager and nurse practitioner, who were contracted through our medical insurance, and the RT and mobility specialist, who “came with the equipment.” So the transition wouldn’t be completely disruptive.
By the time this was settled, caregivers were already shuffling their schedules to accommodate Thanksgiving and Christmas vacations. “You might as well wait until the new year,” the aide advised Priscilla. “That way you’ll get your ‘regulars,’ and you’ll only have to train them once.” Her condition was stable at this point and this seemed a safe enough course. But we did do all the preliminary paperwork so that Priscilla could go right into home hospice in the first full week of the new year.
Once again, she almost didn’t make it. In mid-December she had another episode in which she started hyperventilating, and this time she was also shaking uncontrollably. We still had the supplemental oxygen, but it made no difference. Somewhat desperately we called our home care nurse, who was supposed to come by sometime that day. “I’m parked right outside your house!” she told us. She’d arrived the moment we needed her. She consulted the nurse practitioner and they figured out how to bring Priscilla’s symptoms temporarily back under control. But they had to find out what had caused them in the first place, to prevent any recurrences.
A battery of blood tests ruled out the most likely possibilities, such as an infection. This left, as the probable culprit, a somewhat rare but well documented complication of ALS, “autonomic dysregulation.” The disease, especially in its late stages, can affect the branches of the nervous system that control things like heart rate, breathing, and involuntary muscle actions such as shaking from cold. Autonomic “storms,” like the one Priscilla had just had, could be triggered by poor oxygenation. She’d had a shower earlier that day and the exertion may have been enough to cause this.
So she was walking a tightrope. ALS was weakening her lung muscles, but her autonomic nervous system was mysteriously improvising some effective response via heart rate, blood pressure, respiration rate, etc. to keep her body oxygenated. At the same time, the disease was also throwing off this very system that was otherwise keeping it at bay. I discovered that autonomic dysregulation is implicated in the sudden death of ALS patients, most often at night, due to things like a catastrophic drop in blood pressure or a sudden cessation of breathing.
The RT had told me earlier that she’d discovered, by downloading the data from the SD card in Priscilla’s VPAP, that she had “central apnea.” I’d wondered why she kept her voice low until I did some research and discovered what this was. Obstructive apnea, the kind we are perhaps most familiar with, is when you can’t breathe, because of a blockage of some kind; central apnea is when you simply don’t breathe. In the night, Priscilla’s breathing had been stopping for anywhere from 20 seconds to 2 minutes at a time. At any point it might have not started again.
From now on I would go to bed each night not knowing whether Priscilla would still be with me when I woke up. We adopted a new way of saying good-night to each other. One of us would say, “See you in the morning,” and the other would respond, “Either ‘this one’ or ‘that one.’”
For nearly another 400 nights, it was “this one.” Now I’m looking forward to “that one.”