Watching my mother die

My mother was recently diagnosed with Creutzfeldt–Jakob disease. This is a rare, always-fatal, incurable, rapid-onset, neurodegenerative disease.

First it ruined her body. Now it is taking her mind.

A few months ago she was fine. Now I’m writing this post in the small hours of the night, whilst sat by her bedside, and she has maybe a few days left. At this moment she’s asleep, though fidgeting and muttering to herself.

I don’t know if I will ever share this post publicly. I’ll likely share this with a few friends, at least. To explain to them why I fell off the face of the Earth for a while.

But mostly, right now: I’m just trying to deal with the grief, and writing helps.

You probably have an image of her lying in a hospital bed. This is only partly accurate: right now she’s in a hospital-style bed, but at home for end-of-life care. In previous days she was in both an NHS hospital (a couple of months ago, then with only polyneuropathic symptoms: they sent her home with a diagnosis of “functional neurological disorder” i.e. “we don’t know”) and a private hospital (last week, after the cognitive symptoms started, and where the diagnosis was made: this time the more advanced state of her disease made it visible on an MRI scan).

We’ve never considered finding a hospice for her – with on-call nurses and the like – as it’s been her express wish that she die at home with her family.

I’m quietly wondering what’s going to get her first. It could be the CJD itself (=at some point the autonomic nervous system shuts off and she stops breathing, I think?) But most CJD patients die of an untreated infection before it gets to that point. Blood loss and/or lack of sustenance are another two contenders in this race for who gets to kill my mother first.

The past two weeks have been spent firefighting. She has hallucinations and these are not always successfully controlled; we are trying three different medications. She must sometimes be moved for sanitation, and each time she screams in disorientation. We are doing battle with a fungal infection that started in her mouth. She was panicking last night that she wanted to be able to see me… but her eyes don’t really work any more and I was right in front of her face.

Yesterday she started bleeding out – blood literally pooling on the floor – for absolutely no reason whatsoever. (And bleeding is not a symptom of CJD. What caused that?? For any experts – this probably doesn’t mean the root cause is actually cancer. The paraneoplastic autoantibody screen came back negative and the DW-MRI shows cortical ribboning.)

She is on anti-emetics, anti-psychotics, anti-seizures, anti-fungals, painkillers, sedatives, proton pump inhibitors, and even a coagulant. No, not an anti-coagulant (like you’d normally get when lying bedridden). We just have to accept the risk of stroke because the bleeding is the more serious concern. None of these will help her underlying condition, these are all just to manage the symptoms.

My mother’s care has been a full-time job for two people. Prior to the past two weeks it was my father on his own, and he was at breaking point. Now we have my father, myself, my sister and her fiancé. Between four of us we find enough time for the everything else: like showers for ourselves, a rota for who stays with her throughout the night, or the self-therapy of writing this post.

As a family, we’ve gotten pretty good at crisis management. Something happens, we react, she stabilizes again.

This is how we pass our days.

Is this even worth it? You’d be forgiven for asking that question. Let her drift off on an overdose of morphine. It might be kinder.

But, despite the hallucinations, the screams, the difficulty she has speaking or moving her body… there are moments when she’s there. Her eyes come alive, and she’s lucid. She can nod in response to questions. If we’re doing well then we might even exchange a few slow slurred sentences: and smile, laugh, make a joke. The woman who raised me, still there amidst the wreckage.

What breaks my heart the most is when she is crying. I’ll cry too. In her better moments then she knows what’s happening to her, and she is terrified of the end.

Just as I got to writing this point, she woke up. Screaming. She can’t see anything. This happens sometimes.

How does one handle this?

My mother terrified, because I dropped a bottle and the noise scared her…

…her shouting ‘wake me up’ at the top of her lungs…

…sometimes, the paranoia and accusations that we’re trying to hurt her…

…the moment I was surprised not to recognize my own face in the mirror, from how it has been twisted into a rictus of grief?

Every day, our victories become smaller. Once, a good day meant managing an assisted walk. Then she became wholly bedridden, and a good day was one with enough food. Then we compromised, and it became the days with at least a few spoonfuls of jelly. Then the days when we can still coax a smile out of her with some music.

Every day, we race against the clock. Once, this meant spending the available hours to shuffle in inch-long footsteps between bedroom and toilet. Now, it means taking advantage of every moment of lucidity: to spoon in enough food and fluids to keep her going another day.

At some point we will lose this race, and our victories will be over.

This is where I’ve spent the past few months: first, trying to understand what the possibilities were for what had befallen my mother (CIDP, Guillain–Barré syndrome, Lyme disease, paraneoplastic syndrome, autoimmune disorder, Wernicke’s encephalopathy, Sjögren’s syndrome, heavy metal poisoning, Creutzfeldt–Jakob disease, …). Then figuring out a diagnostic plan (oligoclonal bands, check for albuminocytologic dissociation, nerve conduction study, anti-Hu screen, …). Determining the barest traces of a possible therapeutic plan (physiotherapy, IVIG for autoimmune, chemotherapy for cancer, …)

Then working our way through one, two, three, four, five different neurologists (four of them private, two of them full professors) until we find the right one.

Getting the diagnosis. Diving into the CJD literature. Convincing myself that this really is the correct diagnosis this time. That there is no treatment available. Nothing that can stop this. Nothing that can even slow it. Or given that I literally work on developing immunotherapies… nothing that I can invent in time.

So nothing I’ve done will have changed much, in the end.

Now, all I can do is be there for my mother… at her end.

I can’t save my mother.

But maybe I can save someone else.

This is the reason why I do biomedical research. Why my current work is the development of therapeutics. Why I made this subject-switch from mathematics to computer science to biology. How could anything else be more important? Because every single one of us will find ourselves in a hospital bed, just like my mother, eventually, one way or another.

To my friends Agata, Asia, Johanna, Kat, Spiro, Stef: thank you to all of you. Each of you played some important part in making sure we did the best that we can, and I can’t thank you enough.