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5 Things to Know When Caring for Someone With Creutzfeldt-Jakob Disease

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So the worst has happened. After a horrendously fast onslaught of weird symptoms, after the specialists and neurologist rule out absolutely everything else, after days or weeks on a roller coaster of probable diagnoses, someone said it…

Creutzfeldt-Jakob disease (CDJ) — a degenerative brain disorder. The conversation that follows is devastatingly simple… no treatment, no cure, only one possible outcome… death.

So what do you do now? How do you help someone in times like this?

This story is for you, the caretaker. So you know that you are not alone. That this path has been walked and survived by others before you and will unfortunately be walked by others after you until scientists and doctors find another path for us. I have been where you are. And I hope that my experience offers some usable advice for you on this journey.

So here goes… Five things to know when caring for someone with CJD:

#5. There Really Is No Cure

I know you heard the doctors say it, but I want you to take a second and let that soak in. Essential oils or special vitamins are not going to make it stop. There is no secret diet to help symptoms abate. If you search the internet far enough you will find examples of experimental treatments, but so far none have succeeded. At this time, this disease is 100% fatal.

That doesn’t mean you stop. Instead the fatal diagnosis means it is time to switch your priorities. Quality of life, not quantity, becomes the focus from here forward.

#4. Have the Hard Conversations Now

You need to be able to ask your loved one these questions. (If your loved one is no longer able to make decisions for themselves, read through these questions then apply them in number #3.)

Who do you want making your medical decisions when you are no longer able? Pick one person to be the primary and a second person to make decisions if that person is unavailable. It will be best to have these decisions in writing. There are forms available online if you don’t want to go through an attorney.

What kind of life-sustaining/extending treatments do you want or not want? Be specific. Do you want a tube in your stomach to provide you with nutrition after you can no longer swallow? Do you want IV fluids to provide you with fluids after you can no longer swallow? Do you want a machine to help you breathe? Do you want us to perform CPR if your heart stops? These treatments will not save your life, but I want to make sure we have a plan that you feel is best for you.

What kind of palliative care can we give you? Are you OK with pain medication? What about medication to help you sleep or keep you calm? These treatments will make your quality of life better towards the end.

You will eventually need round the clock care. Your care will include help going to the bathroom and showering, and eventually diaper changes and bed baths. Do you want that to be done by family at home if possible or would you prefer to live in a care facility?

Do you want an autopsy? We can arrange an autopsy with the National Prion Disease Pathology Surveillance Center (in the U.S.). If you consent, your autopsy will tell us exactly what kind of CJD you had. It will also tell us if it is the familial or genetic kind, which could mean something to your kids and grandkids as well as your other relatives.

What kind of funeral do you want? Do you want it to be planned completely by us or would you like to share in the planning? Do you want it in a specific church or funeral home? We will need to contact them as soon as possible in order to educate them about CJD.

Do you have a will? Do you want to update it? What other important things do you think I should know?

#3. Hard Choices Without Them…

It might be too late for these conversations by the time your loved one gets the diagnosis. This means if they don’t have a Power of Attorney for Medical Decisions or a Living Will you and your family will be making these decisions without their input.

This can be hard. But all the questions above still need to be answered. Families can have trouble getting along and agreeing during the best of times, and these are not those times. Don’t be surprised if feelings get hurt and people disagree. The final decision will usually fall to the loved one’s guardian, spouse, adult children, parents or siblings in that order.

Hopefully your family is able to focus on what is important, the quality of your loved one’s life.

#2. The Disease Progression

If I had to describe the progression of this disease and the role of a caretaker in it, I can only compare it to caring for an infant in reverse. Your loved one’s path may not follow exactly like mine, but I think the analogy will still apply.

At first, they are the person you know, only slightly altered. Maybe they have a hand tremor, or a different walk, or trouble remembering a word, but they are still them. They like the same foods, have the same habits and only need a little help to function reasonably normally in their environment. Respect and a focus on as much independence as you can offer them is the priority at this stage. Try to do things together that you both enjoy. My mom and I were still doing yoga together at this point. We were still preparing meals together and eating them in the dining room. It was early winter and we would walk around the yard together as my mom explained every plant and flowerbed.

Then things change. They might start to fall all the time. And a walker might help. But then when they wake up from a nap, they won’t remember that they need a walker, so they get out of bed and fall. Safety becomes a priority. Look for tripping hazards and eliminate them. No rugs or cords that might trip up someone who is already unsteady on their feet. Respect and independence are still important, but getting harder to maintain. The first time I had to help my mom into the bathroom wasn’t a big deal, but pretty soon I had to help her get her pants down and make sure she was wiped well. This was not a problem for me (I had a toddler at the time and it wasn’t much different), but for my mom it was hard. But as with all things, we muddle through. During this time, we would sit at the table and watch the birds through the kitchen window. Winter had come for real and we could watch the birds at the feeder with fresh snow as a backdrop. We swapped out her regular bed for a hospital bed to make things easier. We started watching movies lying next to each other with the bed sitting up. We snuck chips in bed, even though my sister was totally against it.

And then things change. You swap out the walker for a wheelchair and get a shower stool for the bathroom. You learn your way around a gaits belt to help make transitions from bed to chair and chair to toilet easier on everyone. Your loved one starts taking more naps. All the sharp edges of their personality have softened. Their smile is different and unfortunately so is their anger. This might be a time when you start wondering what causes sundowners and how to avoid it. You keep doing your best, because there is no other option. Your main focus from here on out is keeping them content and cared for. We had a hard night where mom was so sure that she needed to go upstairs. She yelled at us and when it was obvious we couldn’t be convinced, she threw herself out of her wheel chair at the bottom of the steps.

We were balanced on a knife edged line between us making decisions for her health and her making decisions for herself. We got her up the stairs. She sat in the empty bedroom on what used to be her bed looking around at a room she couldn’t remember. She calmed down and asked for a picture album sitting on a shelf. We looked at the pictures together smiling at pictures of me as a baby and her in bell bottoms. At every point we were just making the best decision we could.

And then things change. They don’t get out of bed as much as they used to. And their dementia seems to ebb and flow like the tides. You start realizing that diapers are a lot cleaner than trying to keep up with accidents. You learn the art of washing someone’s hair in bed with trash bags and plastic tubs. Their wardrobe has probably changed from regular clothes, to soft elastic wasted outfits, to now PJ’s that are easy to get on and off when needed. Their diet has softened and their interest in food has started to wane. During this time, my mom’s dementia was a daily changing thing. I was heartbreakingly called “nurse” for the first time and then a few days later she was cognizant enough to play Scrabble with my little sister. I still smile when I think back on that day because the point of the Scrabble game in my opinion was to entertain Mom, but my sister was obsessed with the rules. “No mom, you can only have seven tiles. Seven tiles, Mom, put two back.” And then for some reason my sister kept trying to keep score and even though mom couldn’t really write anymore, she took the notebook away from her. And when my sister tried to subtly try to keep score on another sheet of paper, mom took that away too! The first spring daffodils started blooming and we managed to get her wheelchair on the deck to enjoy some warmish sunlight.

And then things change. You might notice a bunch of dry diapers in a row. You don’t know it yet, but the ability for the muscles in their bladder to work has stopped. You learn about catheters. And the nurse comes out to put one in. And then put it in again after they yank it out. You learn how to give bed baths or more specifically how to give someone a bath, change their clothes and change their sheets at the same time all while not getting them out of bed. While they sleep, you notice their hands doing weird movements almost like directing an orchestra or crocheting a big blanket. Dementia has stolen every part of their personality. You become an expert in caring for their physical body. Making
sure they are clean, comfortable, that bed sores don’t form. Times when your loved one is awake grow farther and farther apart. For us this happened as spring began to turn the whole world green. Every day the world outside was getting brighter and brighter, while the world in our home was growing dim and still.

And then things change. You notice their breathing is different. They cry out when you turn them. It is only time they open their eyes anymore. The nurse who comes out to check isn’t surprised. You have been giving them pain medicine for a while, and now it is pretty much all you give them. They haven’t wanted or been able to swallow food or water
for a few days. You are wetting their mouth with swabs. Over the next few days their breathing continues to change. It sounds harsher and harsher and spaces out with huge gaps in between each breath. At this point both my sisters and my brother had gathered together. My sister and I slept in the same room with her that last night, and for part of it I curled up on her bed with my hand on her chest. The next day, the nurse came out again to check and while he was there she stopped breathing. We all sat with her as he checked for a heartbeat. And there wasn’t one. Mom’s battle with CJD was over. Unfortunately for those of us left behind, we carry it with us.

#1. Living After CJD

I wish I could say it gets easier. But I found the weeks and months after my loved one died of CJD to be hardest part. At least during the caretaker phase, I had something to do. And I considered what I was doing to be the most important thing I had ever done. And then even that was gone. I went back to my normal life a broken person.

I saw a counselor, I saw my doctor, I even took medicine for depression. None of that made it better. But it all helped a little. I spent that summer staring at the wall and avoiding my own children. We ate every meal from a takeout window and lived in a house that didn’t get cleaned. But I got through it.

I went back to work and then took off more time because I told my boss he could either give me a few weeks leave or I would quit. Luckily for me, I was surrounded by a loving community including my husband and coworkers. I went back to work again, and this time it stuck. I kept moving forward until eventually I got through an entire day without crying. And then a few weeks without crying. And that got me to now.

Now I can talk about CJD without wanting to scream. Now I can remember my mom before her illness. Now I can parent my children and do my job and live my life, a changed person, but not a broken one.

So my advice for you is to keep living until you get to this point. See the doctor, talk to a counselor, join a grief group, start a CJD Awareness group.

Just keep living until you find your way back to you.

Getty image via Anna Minkina

Originally published: May 5, 2020
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