Huntington’s Disease – The Auld Skald's Fudgel

Terminal care failures and a “peaceful” end

Warning: this discusses failures in terminal care, and death

On Saturday, 29 June 2024, I observed the fifth anniversary of becoming a widow, noting that it was also my first anniversary as a widow knowing I was autistic. Having recently recognised my “need for cognition”as another autistic trait, in my growing library of traits, I turned to the internet to research autism and grief.

I found, among several research papers, this article, in Psychology Today. This paragraph stood out to me, almost shouted at me, and suddenly, the manner of my grief made sense.

In the autistic mind, grief doesn’t neatly follow a logical pattern. Instead, it’s a tangled bundle of trauma, isolation, depression, and sadness—a heavy knot that resists untangling. Grief for autistics isn’t a straightforward cause-and-effect scenario, as often seen in behavioral therapy. It’s a maelstrom of pent-up emotions, recurring emotional tsunamis, outbursts, and disorientation, all of which can manifest at the most unexpected times and significantly impact an autistic’s daily life on multiple fronts. (Srinivasan, 2024).

My response was to say, let’s add another aspect, that of a whirlwind, a tornado, that picks you up, spins you around until you don’t know which way is up, then dumps you, tear-sodden, croaky of voice, and no less bereft.

My late husband’s last days

My late husband’s last four days on this Earth saw two failures in care. I’ll give you warning, now, this is a graphic account.

Wednesday 26 June, 2019
Mid afternoon, nursing home staff looked in on Allan, my late husband, and thought he was asleep. They left him be.

Thursday, 27 June 2019
Nursing home staff attempted to wake my late husband around 8am, but he was non-responsive. The RN and Clinical Manager were called, and then they called me, telling me that he was unconscious, respirations were down, and fingers and toes were showing cyanosis. At this point, he was put on a two-hourly morphine cocktail.

Friday, 28 June, 2019
The night nurse finished her shift at 6am, just as the horror of her failures were becoming evident. That night nurse had failed to give him any of the two-hourly morphine cocktails during her shift, despite a clinical order from the Clinical Manager.

As a result, his bodily pain brought him out of his unconscious state. With complete dysphagia, a loss of control of the muscles in the throat, he was unable to speak, only moan. At the time, he weighed around 25kgs, on a 180cm frame. He had refused a feeding tube for nutrition and had also refused a drip for hydration. But, those moans; I can still hear them. Hearing your husband making those sounds and being unable to do anything, feeling utterly helpless, is a heart-breaking state to find yourself in.

The Clinical Manager and a small team swung into action, trying to soothe and settle him. That’s the cruelest thing. You can’t catch up on all those missed doses of morphine at once. It has to be slowly reintroduced in a controlled way. But oh, my, the things they don’t tell you.

At 25kgs, a body is literally skin and bone. There is very little muscle or body fat. Palliative care drugs are usually delivered subcutaneously, but when a body is skin and bone, where subcutaneously? The decision was to inject into pockets of body fat, in his abdomen and thighs. But consider this. Those drugs sitting in a small pocket of body fat don’t work. So the staff were trying to gently massage the injected drugs out of the body fat, rubbing directly on his bones in the process. His moans became wails, nonverbal crying from eyes too dry to produce tears. Gut-wrenching, again, excusing myself from the room because I just couldn’t take it. To see my husband, my life partner of 31 years, nearly at the end of the horrible journey that is Huntington’s Disease.

They tell you about Huntington’s Disease, but they don’t tell you about the death of a Huntington’s Disease patient. If not suicide, then catastrophic accident; if not in an accident, then aspiration pneumonia; if not aspiration pneumonia, then wasting away in the absense of a feeding tube and drip.

He was pain-free and unconscious again around midday. Supplier issues delayed the delivery of morphine pumps. These arrived around 3pm, and they were installed and drip feeding the morphine in regular, controlled doses.

By that time, I was utterly exhausted. The emotional and cognitive load of that day were extraordinary.

Saturday, 29 June, 2019
I awoke around 4am, the usual time for me. I checked my phone and saw, with a sinking heart, that I had missed a call from the nursing home. You see, after that horrendous day, I had gone back to my hotel room, had dinner and perhaps a bit too much whisky, and I had forgotten to take my phone off silent mode, which it had been all day in the nursing home.

When I rang back, the male nurse answered and told me that Allan had passed. He had had the two hourly “turning”, shifting a pillow from under one hip to under the other hip, at 1.30am. He continued to sit beside Allan’s bed, observing. He reported that at 1.44am, Allan had breathed out, and not breathed in again. That was it. Game over, life over.

I’ll post another day about the comedy of errors and the multitude of compassion I found, on that day. But for now, let’s look at the failures in care.

Nursing home staff failed in their duty of care to Allan. This included something as basic as recognising significant changes in his health status, such as the difference between sleeping and being unconscious. Additionally, where was the attempt to administer “comfort feeding”” for dinner on Wednesday night?
The RN on the night shift failing to administer morphine as prescribed contributed to no doubt significant harm. The pain and suffering that could have been prevented no doubt took minutes, if not hours off his life.
Both I, as his spouse, and he himself had the right to competent care and timely medical intervention.

What could or should have been done?

These days, Queensland Health has clinician resources for Residential Aged Care End of Life Care Pathway. This was available in 2013.

End of Life Directions for Aged Care has a website and a Residential Aged Care Toolkit.

Palliative Care Australia has published Principles for Palliative and End-of-Life Care in Residential Aged Care.

What difference does it make to me know?

Knowing that I am autistic, with a new understanding about autism and grief, made Saturday, 29 June, 2024, a little easier. Yet, I will still stand by what I said last year, on Facebook.

Everyone’s journey through grief is different; become, once again, a student of your own life, then, once again, author of your own life as you regain the strength and compassion to go from reaction to response in your new state of being. But remember that grief is social. Don’t grieve alone and don’t ever let anyone grieve alone. Give them space and let them talk, and above all, hug. Human touch is vital.

Look after your bereaved friends. They need you.

Failures in palliative care

Discussion of Terminal Illness
End-of-Life Care
Pain and Suffering
Medical Negligence
Loss and Grief
Emotional Distress

It is coming up to the fifth anniversary of my husband’s death. Regrettably, the last 7 weeks of his life were not the smooth, pain-free days that he should have had.

On 15 May, 2019, I received a call from the nursing that Allan, my husband, had had a choking incident during lunch and had been taken by ambulance to hospital. For context, Allan had Huntington’s Disease (HD), a dreadful, incurable, degenerative brain disorder. You can read more about it here.

After getting that phone call, I was immediately on the phone to the emergency department at the local hospital. As Allan’s Enduring Power of Attorney (EPOA) for health matters, and his Statutory Health Attorney as his wife, I knew I had to get on to the emergency department to make sure his wishes for treatment were known. No antibiotics, no drips, no restraints, no feeding tube. Just pain management.

Why was I immediately going to palliative care mode? In 2019, Allan was 15 years in to HD, and in the palliative phase of the disease. He had been on a “moist and minced” diet for about four years, and spoon fed (comfort feeding) for about two years. In the advanced stages of HD, he had lost all ability to speak and control swallowing. Anything he ate had a 50/50 chance of going into his stomach or lungs. He had complete dysphagia. We knew that meant a high risk of aspirational pneumonia.

Later that evening, I got a phone call from the ED that his scans were clean with no foreign substances in his lungs. He was on his way back to the nursing home. I was relieved, and exhausted. The stress on caregivers is staggering.

Imagine my distress, then, when I got a phone call from the nursing home the next morning. It was a nurse. This is what I posted to Facebook.

OH. MY. GOD. An RN at the nursing home rang to tell me that speech pathologist has told GP, no food by mouth for Allan. “We can’t starve him and there’s no palliative care plan. What do you want us to do?”.

The Clinical Manager is in essential training, and the admin person can’t find the advanced care plan we agreed on back in March of last year.

Thanks to a very dear friend, I will soon be on my way down to the nursing home to a) write a new care plan, b) raise hell that a nurse could say that to a resident’s spouse, c) raise hell that the nursing home could misplace such an important document, d) establish said speech pathologists’s experience to make such a call without any consultation with the spouse, other carers and clinical manager.

The rage is cancelling out the tears for now. Worst case, nil by mouth and no feeding tube. Best case, someone made an ill-judged decision and Allan will be enjoying pureed steak and veg for dinner.

Not much more I can say right now. Love you all.

A very calm version of these events is here, in a blog post called “The Practice Run“.

Can you imagine Allan’s anguish on the afternoon of Tuesday, 16 May, as both I and the Clinical Manager at the nursing home, confirmed his wishes for only pain management? He was sprawled on a mattress on the floor – his floor was covered in mattresses for when he fell. He had no voice, so I crouched next to him, fighting back tears, asking him to try to touch me if he wanted to say “yes”, and not try if he wanted to say “no”.

Three times, with the Clinical Manager as witness, I asked him the same questions. Three times, he answered no to everything except pain management. By this stage, he had not eaten anything since the interrupted lunch on Monday.

Away from his room, I joked to the Clinical Manager about checking if the morphine sulphate that was in the dispensary for him was still in date. She had no idea. And so started the horrible roller coaster of the next 12 days.

At day 6 of 12, I posted this on Facebook.

So, apparently it was all a false alarm because the speech pathologist made an over-zealous call. (Too bad the GP signed off on it.) So things continue as they were two weeks ago and this can be considered a false alarm. AND IT TOOK 8 DAYS FOR THEM TO FIGURE THAT OUT, putting me through the wringer in the meantime.

After 12 days, I could take no more of the questionable actions of the nursing home. Five years on, it pops up in my Facebook memories every year, and for the fifth anniversary, it deserves a detailed examination.

This is what I wrote on Facebook after those twelve days.

We’ve all seen those Facebook memes about walking away. I have’t walked away, but I have turned away. I cannot, for my own health and wellbeing, continue to engage with the management team at the nursing home.

The clinical manager said, on Friday 17/5, that she would get a blood test done to check kidney and liver function. The blood wasn’t drawn until Thursday 23/5.

Three times, I have asked for a copy of the speech pathologist’s report of Tuesday 14/5 and the GP’s sign off on it. I still haven’t received it. That was the one where it was amended from ‘nil by mouth’ to ‘comfort feeding’.

I have told them several times that Allan is in pain. He reports a constant 4/10 all over and 5/10 for his posterior, being on it all the time. Yet, his pain medication has not been increased, except the two days where I demanded an additional dose for him. I have told them that his pain levels are the first thing they should be checking.

This has lead to the facility manager checking on Allan’s legal rights. Not checking his expectation of dignity and freedom from pain, as set out in his advanced care directive.

So, I will still be there for Allan, funding his needs, and as his EPOA when he no longer has capacity to decide things for himself. But I cannot continue to engage with nursing home management. They have my contact details, so they can contact me if and when they want to discuss his condition.

Some will not agree with this decision; it is my life and my decision to make. While discussing this, I have no churning in my gut, no threat of tears, no apprehension, no increasing muscle tension, no shaky voice. I have to take care of myself, and it does him no good at all if I have a breakdown.

Perhaps, when it’s over, I will ask for his case file and take it to a solicitor or medico-legal expert for review, and then provide it to the nursing home’s corporate office. I understand about fixing it for others for the future, I do, but I cannot do that at this point in time.

In the intervening period from “nil by mouth” to me “turning away, I had taken leave from work. I had worked with his NDIS Plan Manager to ensure he had someone with him for 10 hours a day under his NDIS funding. I had notified the insurance company, contacted funeral directors, everything that you do when you know a loved one is in their final days. Except, he wasn’t.

I was kept in the dark about his alleged change of mind to refuse food on Wednesday, 17 May, and that the speech pathologist had amended her report, changing it from “nil by mouth” to “comfort feeding” sometime on Tuesday afternoon/Wednesday afternoon.

Key Failures in Care

Delayed Diagnostics

The significant delay in obtaining blood tests, from Friday 17/5 to Thursday 23/5, is concerning. In palliative care, timely diagnostics are crucial to manage symptoms effectively and provide appropriate care. If these organs aren’t functioning, medications can build up instead of being excreted, causing toxicity.

Lack of Communication and Documentation

Repeatedly requesting the speech pathologist’s report and the GP’s sign-off without receiving it is a clear failure in communication. This documentation is essential for coordinating care and ensuring that all team members are informed of the patient’s needs and treatment plan.

The key document I requested was the amended speech pathologist’s report, and the GP’s acknowledgement of the amended report.

Inadequate Pain Management

Allan’s persistent pain, with a reported constant level of 4/10 and specific areas of higher pain, indicates insufficient pain management. In palliative care, ensuring comfort and minimizing pain is a priority. The lack of appropriate adjustment to his pain medication, despite repeated reports of his pain levels, is a significant oversight.

The need to demand additional pain medication highlights the failure of the nursing home staff to proactively address Allan’s discomfort. Effective advocacy should not require repeated demands from family members.

Remember, I had asked the Clinical Manager if the morphine sulphate procured for him in March 2018 was still in date.

Ignoring Advance Care Directives

The facility manager’s focus on Allan’s legal rights rather than his stated expectations in his advance care directive is troubling. Respecting a patient’s wishes, especially regarding dignity and freedom from pain, is fundamental in palliative care.

Emotional Toll on Caregivers

The situation clearly took a toll on my emotional and psychological wellbeing. You need only read the raw Facebook post from the time to see what state I was in. I had to disengage with the management team and this underscores the systemic issues within the nursing home that prevented effective communication and care.

Related posts

I wrote a post a few days ago about advanced care directives. Back in 2019, they weren’t legally binding, which is why I had to advocate so strenuously on Allan’s behalf.

Conclusion

The failures in care identified above include delayed diagnostics, poor communication, inadequate pain management, failure to respect the advance care directive, and ineffective advocacy.

These significant failures in care likely took days off Allan’s life. Just seven weeks later, he breathed out for the last time.

Palliative care is about quality of life. Terminal care is about quality of death.

Palliative care is about enhancing the quality of life for patients with serious illnesses by managing symptoms and providing comprehensive support.

Terminal care, a critical aspect of palliative care, focuses on ensuring quality of death by prioritizing comfort and dignity in the final stages of life.

Resources

https://palliativecare.org.au/im-a-carer/

https://carersqld.com.au/carer-program/

https://www.carersaustralia.com.au/information-for-carers/palliative-care-and-end-of-life-care/

https://www.racgp.org.au/clinical-resources/clinical-guidelines/key-racgp-guidelines/view-all-racgp-guidelines/silver-book/part-a/palliative-care

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7404818/

https://www.health.gov.au/topics/palliative-care/palliative-care-contacts

The practice run

On a seemingly ordinary Monday afternoon in May 2019, my phone rang, and it was the nursing home calling to inform me about yet another choking episode for Allan. The speech pathologist, who happened to be on-site, had made the decision to call an ambulance, and Allan was en route to the emergency department. Little did I know that this would be the start of a rollercoaster of emotions that would define the coming days.

The hours that followed were filled with nerve-wracking phone calls to the emergency department, as I advocated for Allan’s wishes and his intention to refuse certain medical interventions. It seemed like everything was back to normal when I received a call from the ED, reassuring me that Allan’s scans showed clear lungs, and he was on his way back to the nursing home.

However, the next morning, the nursing home threw me a curveball by asking what they should do with Allan. It turned out the speech pathologist had declared him “nil by mouth,” and the nurse who called me said, “there’s no care directive, we can’t starve him, so what do you want us to do?” To say that I was shocked and angry would be an understatement.

Imagine my frustration, knowing that was indeed an advanced care plan that had been developed in consultation with the Clinical Manager and the GP. This plan had been established after the neurologist declared Allan to be in the palliative phase of his illness. I was left shaking, unable to drive, and in dire need of support. Thankfully, a dear friend came to my rescue, driving me to the nursing home.

During a challenging afternoon, we confirmed Allan’s intentions, which included refusing a feeding tube, intravenous hydration, and antibiotics, opting only for pain relief. At this stage of his disease, Allan was nearly non-verbal, suffering from severe dysphagia, and had dropped to around 35 kilograms in weight. As someone with a background in occupational health and safety, I knew that without nourishment and hydration, he had only a few days left.

I began preparing for the inevitable, reaching out to Allan’s NDIS support coordinator and service providers, who proved to be invaluable. His NDIS plan was adjusted to ensure that a support person would be with him for 10 hours a day, so he wouldn’t be alone during his final days. The outpouring of support from these individuals was truly commendable.

I also started making arrangements with the funeral insurance provider and the funeral director, even organizing a removalist in advance to pack up Allan’s room and transport everything to my home. I took two weeks off work, anticipating the worst. I checked into the Mercure Gold Coast and prepared to make his last days as pleasant as possible.

Allan slept most of the following Tuesday, but when I called the nursing home on Wednesday morning, I was bewildered to learn that he had been fed and was resting comfortably. It contradicted the “nil by mouth” decision, and my anger grew over the lack of adherence to Allan’s wishes. I rushed to the nursing home, only to be told by the Clinical Manager that he had changed his mind, with no witnesses except her, and that the speech pathologist had amended the report to allow comfort feeding. Allan had not been provided with any additional pain relief.

This contradicted the GP’s previous approval of the “nil by mouth” decision, and the lack of proper documentation left me frustrated. It also meant that the 10 hours of daily personal care funded by the NDIS had to be discontinued.

After a whirlwind of events, I had to disconnect from the nursing home, although I remained committed to Allan. The experience had taken me on an emotional rollercoaster, swinging from preparing for the worst to a confusing state of uncertainty about Allan’s long-term outlook.

What were the failings in palliative care? Was it that a palliative care plan was not put in place, at that time, after his decisions on the Tuesday afternoon? Was it that I wasn’t sure if his wishes weren’t being adhered to? Was it that the confusion over the speech pathologist’s report? Was it that they didn’t take Allan’s wishes seriously at that point? Was it the GP’s failure to implement a terminal care plan?

In retrospect, this tumultuous period became a devastating “practice run” for Allan’s end of life, just six weeks later.

What is Huntington’s Disease?

Huntington’s Disease (HD) is a serious and currently incurable brain disorder that affects how people move, behave, and think. In most cases, it starts showing symptoms in a person’s 40s, and their life expectancy after that is around 15 to 20 years. However, it can also affect younger individuals in a more devastating way.

People who have the gene for HD are at a much higher risk of suicide, and sometimes their changing thinking abilities can lead to problems with the law.

It’s estimated that in Australia, there could be between 1,657 to 3,000 people with the HD gene in their DNA.

This disease is passed down through families, and if one parent has the faulty gene, their children have a 50% chance of inheriting it.

HD was first observed in families back in 1872, and we didn’t understand its genetics until 1983. In 1993, scientists found the huntingtin gene, which is the key to HD.

This gene has a repeating pattern of three of the four DNA building blocks (C, A, and G) on a specific part of chromosome 4. This is called a “trinucleotide repeat.” When this repeat is too long, it leads to the creation of a harmful protein called mHTT, which causes brain cells, especially neurons, to die.

Today, doctors can diagnose HD by examining a blood sample and checking for this specific repeating pattern on chromosome 4.

Why do I care to write about Huntington’s Disease? My late husband, Allan, had this condition. In his case, his blood test showed 49 CAG repeats, which is a clear sign of HD. Thankfully, we didn’t have children.

That is the path to me becoming a widow.