The journey so far

My name is Sonia and I am Sara's daughter. My mother has been sick for a very short time. My intention is to give the details from the start of what we’ve seen to the present and then update when things change.

First week of May: Mom called my brother, Dave, in a panic saying that she couldn’t find her car… in her driveway. It worried him enough that he went down to check on her. It was obvious to him then (and to me on the phone) that she was having what we all thought were memory loss episodes similar to Alzheimer's disease, roughly stage 3. It was clear when he went down there then that she needed help, as she wasn’t remembering to feed herself and was having problems finding her way around her house. She was coherent, but not all of the time. She started having visual anomalies in the form of tracers of colors around that time as well, but she was chalking that up to new glasses. She had no problem with knowing who her children or friends were, but did not remember all of the things that had happened during the day. She was describing how she was feeling as “foggy”.

Second week of May:  In the short time of about a week her cognitive state started to decline so rapidly that I took her to the emergency room in her small coastal town of Seaside, Oregon. They ran basic diagnostics consisting of a mini mental exam (or, really, a mini mini mental exam), a CT scan (to look for bleeding) and a blood and urine screen. They suspected stroke but found “nothing” (to quote the ER doctor). Since she had an appointment with a GP the next week, and her mental state wasn’t terrible enough to admit her to the hospital (well, and she hadn’t had a stroke), he released her with the advisement not to drive and to have family close by to help her. He also called the GP and updated her so she’d have the information when the appointment time came.

Third week of May:  During the appointment the GP gave her a longer mini mental exam, ordered a chest x-ray (not sure why) and told us that she would be referring her to the University of Washington Medical Center as soon as possible for more testing (since both Dave and I live in or near Seattle, we wanted to have her there with us). The GP mentioned during the visit that she suspected it might be Alzheimer’s (note: although Alz is a unique as the patient, NOTHING I found in any Alz literature indicating that the disease could progress as rapidly as it was appearing to progress with her, so I wasn’t completely satisfied with her suspicion). At this time mom’s confusion was growing dramatically, and her short term memory was very sketchy. We worried very much about her wandering away from the house since she was so confused about getting where she wanted to be in her house and was sometimes going outside to find her bedroom, the bathroom or the kitchen. She also at this time was having a little trouble being steady on her feet. Not much, but she seemed to need to walk a little slower than normal and I noticed that she was holding on to things while standing still. She couldn’t sign her name – well, she got her first name but then was having a lot of trouble remember what letters were in her last name and how to physically write them.

May 21st:  Her GP orders an MRI. The test is easy, according to mom, and we await the results. She verbalizes that she is feeling more and more “foggy”. They call the next day and schedule ANOTHER MRI for the following week.  Since the hospital in Seaside is a small hospital, they don’t have a resident MRI machine (it is driven in from Portland for 2 days a week), we have to wait this long. They said they needed another one to make sure that she hadn’t moved during the MRI.

Memorial day:  My brother called from Seaside, where he’d been staying with her pretty much without a break since the beginning of the month, and he and mom told me that she was feeling even more disoriented, evidenced by trying to show her nephew around her house and not being able to come up with the words for the rooms. I suggested they go back to the ER, since when I took her they said to come back if it gets worse. I also thought that they would be able to read the MRI results, since nobody had given us the results from the first one. The visit to the ER was short – they looked through her chart and explained what the radiographer had written in the chart: there were a couple of possible reasons for the bright spots seen in her frontal lobes, most likely among them Creutzfeldt–Jakob disease (CJD). CJD is a degenerative neurological disorder that is very rare, incurable, and invariably fatal. Dave is in shock and disbelief, mom is just in disbelief. He calls me, and I am likewise in shock and disbelief.

May 27^th: This is the day of her second scheduled MRI. We had gotten advice from several people, including her GP, that going to the Oregon Health and Sciences University (OHSU) hospital ER in Portland would get us a quicker path to a neurologist, as we were having a problem getting a neurologist to schedule an appointment within the week. Why this was so troublesome is that the progression of her symptoms was so rapid and we were seeing dramatic changes daily. We wanted them to find her a diagnosis and cure before whatever was wrong took over (still in disbelief about the CJD diagnosis). By this time she had a shuffling gate, was “out of it” most of the time with stretches of clarity that only last a few hours at a time, and used words that were inappropriate to the conversation pretty consistently. After the MRI we took her directly to the ER at OHSU. This is a real ER in a big city with big, real emergency medical problems. Because of this we started to get a little nervous that it would take a long time to see us, and that once they saw us that they would send us away and tell us to wait for a neurology appointment. Luckily that didn’t happen.

We brought all of her medical information with us, including both MRIs, for the new crew of doctors to look at. They asked the same mini mental exam questions that the other doctors had, and it was clear that mom had lost more cognition since the last exam. She had a problem remembering her full name, for example, whereas the week before she could. We spent a few hours in the ER and thankfully they didn’t want to just send us home, but instead had their on-call neurologist come in. We were SO excited that we were finally getting the attention we needed. The neurologist tested her with a much longer mini mental exam than she had been having, as well as physical challenges to test her balance and strength. After these tests and after he reviewed her MRI and medical information, the neurologist admitted her, and told us he wanted to make sure that the neurology team at the hospital did every test possible to rule out some of the more simple explanations for what was happening, as well as try to rule out CJD.

The hospital stay was brutal on everyone. We took turns sleeping in her room with her. Some nights were better than others, some days better than others, but she was clearly getting worse each day of the 7 day stay. There was a team of neurologists, there was a team of therapists (cognitive, speech/language and physical), and endless rounds of nurses and nursing assistants. All of the doctors asked her the same questions every day, and it made her angry every time she got the questions wrong (which she was doing with more and more frequency). They gave her a full body CT because they said sometimes cancer somewhere in the body can make the brain do what her brain was doing. They took more blood. They gave her an EEG. They gave her a spinal tap (aka lumbar puncture or LP). All of the tests so far have come back inconclusive for any disease, but unfortunately the nature of CJD is that a positive diagnosis is not really possible without a brain biopsy or, worse yet, an autopsy. Every day, though, the team of professionals told us it is most likely CJD and that there’s really nothing they can do for her. There is no cure nor is there a treatment for this horrible disease. They wanted to make sure that she wasn’t being sent home to be by herself (duh) and helped us greatly in figuring out what our next steps were.

Latest information:  We have found her a residential care facility in Seattle that is lovely (relative to a nursing home, that is). The house is designed / architected for dementia residents and is therefore a very safe place for mom to be so we don’t need to worry about her wandering away at any time. The staff seems to really love what they’re doing and love the residents. We’re planning on taking her there on Monday, June 8^th.

What you can do:  If you can, visit her. Expect to only stay with her for a few hours at most, but know that even if she’s not engaging with you in an expected way, we believe having friends and family around really does make a difference. Please do talk about your memories of things you’ve done together and share with her how much you love her, but please do NOT mention her disease.  If you want directions to her new place, send her an email as I check it daily.