Change in diagnosis!



We went for our regular appointment at the Movement Disorder Specialist at Medical University of South Carolina, and she changed John's diagnosis to a different kind of Atypical Parkinson's--Multiple Systems Atrophy (instead of Lewy Body Dementia).  Her main argument was that he has not had a steep cognitive decline.  I have thought he did have fluctuating cognition, one of the two essential symptoms of Lewy Body Dementia, because sometimes he can have a sophisticated conversation (about politics) and sometimes he is confused.  But that is hard to define.

I'm shaken up by the new diagnosis for two reasons. One is that it makes me feel alone--I no longer fit in several of the communities that have been helpful to me. MSA is a much rarer disease. The other is what would we have done differently if we had had the correct diagnosis earlier? I don't think John would have had two surgeries for benign prostate enlargement, which gave little benefit. I took over more things because I expected more rapid progression to dementia, though I think my doing so has made his life more pleasant.

The new diagnosis does make sense. The key symptoms are rigidity, slowness, and problems with the autonomic nervous system such as postural hypotension, urinary retention, constipation, and both kinds of incontinence.  Consider a list of red flags for MSA:

• Early instability--John has only started falling several times a month recently, but he has had balance problems from early on
• Rapid progression--not so much so, but if defined as in a wheelchair in 10 years (the Parkinson's disease definition) that is likely to fit
• Abnormal postures--he has something called disproportionate antecollis where his head is way forward
• Bulbar dysfunction--various voice issues, which he doesn't have except a little hoarseness
• Respiratory dysfunction--snoring and another noise breathing in while sleeping, but these were issues for many years.  He often falls into a shallow rapid breathing pattern, though he reports no shortness of breath
• REM Sleep Behavior Disorder--this was an early symptom
• Emotional incontinence--not an issue

I had read about Multiple Systems Atrophy but had never considered that it fit John, because some diagnostic criteria specifically exclude anyone with dementia.  Cognitive issues were a key symptom when he was first diagnosed because he was having difficulty with his job, but those have gotten worse only slowly.  I found an article that says 11-32% of patients with MSA show significant cognitive impairment.

The relationship between the diseases is odd--most of these symptoms are seen in both diseases, the difference is which are predominant.  Yet they show different kinds cell damage on autopsy.  One article states: "Although the molecular mechanisms of misfolding, aggregation, and fibrillation of α-synuclein might partly overlap among the α-synucleinopathies, disease-specific cascades that are determined by genetic and environmental factors are likely to discriminate these." That is, Lewy Body and MSA may start with the same process that is damaging the brain, but the brain can fight that damage in several different ways, which define the different diseases.

Both diseases have treatment only for symptoms, no way to modify the course of the disease.  Because of the change in diagnoses the doctor did increase John's Sinemet dose, in hopes of reducing his stiffness and balance issues.  There doesn't seem to be anything more we can do about the constipation and incontinence issues.  Life expectacy is similar for the two diseases, perhaps with fewer people with MSA living more than 10 years from diagnosis.  With MSA, the body is likely to go before the brain goes completely, which is a good thing. 

(The image is a road sign from Alaska sent to me by a friend)

Emergency room visit

Friday morning a few minutes before I headed into class I got a phone call from John.  He was crying so that I couldn't understand what he said, beyond that he had fallen.  I grabbed a film for my teaching assistants to show and headed home.  Luckily I had gone in very early so my car was right in front of the building.

I found John sitting on the bench in the bathroom, with a mess on the floor and in his clothes.  Trying to take his clothes off he fell and hit the side of his face against magazine table.  He had a lump and a small scrape, but he wasn't in pain any more. I helped him get the rest of his clothes off and get in the shower and I cleaned up the floor.  I left the clothes to deal with later and went back and taught the second half of my class.

When I got home again I ate my own lunch and then cleaned up the floor and helped him get dressed.  I made him some lunch and then he discovered he couldn't eat it because it hurt so much to chew even something soft.  We decided that had better be looked at, particularly since it was Friday afternoon, so we went to the local urgent care center.  The wait there is usually very long--this wasn't too bad in comparison.  The nurse-practitioner said he needed an xray.

I explained that he couldn't lay flat on the table because his neck is bent forward too much.  It turned out they planned to do the xray sitting up and the problem was he couldn't turn his head to the side very far.  Eventually the technician had him sit on a stool and put his head forward on the table.  He wasn't very stable that way.  I suggested I could robe and hold his shoulders and to my surprise the technician went for that.  The technician said the xrays didn't show anything obvious.

We waited a while for the xrays to be read at the hospital.  The nurse-practitioner came in and said John would need to go to the hospital for a cat scan but it looked like his jawbone was shattered and he would need surgery.  We were surprised given what the technician had said and John's lack of pain, but we headed for the hospital, with a brief stop to get me some food and John a milkshake.

They took John back right away, though then we waited a while for the doctor.  The nurse said the radiologist hadn't seen much, and we began to realize that the nurse-practitioner might have misunderstood the report.  But they did want to do the cat scan.  After another wait they took us back and again they included me in the process of positioning John.  This time he was stable by himself and the technicians just asked me to go to the booth with them each time they took an exposure.

Eventually the doctor came and reported they didn't see any break at all, it must be just a bruise that made it hurt so much.  He gave John a  prescription for pain medicine and we got home about 8 pm.

I have had the principle "avoid the emergency room at all costs," but this was actually a good experience.  We weren't there much over two hours, and that on a Friday evening.  They were much more willing to let me accompany John for everything than I had expected.  I don't know if it was just because they saw he was on Aricept.

When we got home I made him some carrot soup, but told him he would have to ask Paul to help him get in his pyjamas.  Today he has not dared try eating anything that requries chewing.  I'm discouraged by the strain.  Can I really go away next weekend?

Two years

It is about two years now since we realized John had Parkinson's disease. He was diagnosed as soon as we saw his family practitioner and sent to a neurologist and then to the Movement Disorder Specialist who said Lewy Body Dementia.

John is doing better than I had expected. Our son, who isn't very observant, said he thought John hadn't changed significantly in those two years. The changes I see are that John is slower and gets confused more easily. But he is still taking care of himself except for wanting help putting on his socks and he still doesn't have hallucinations. He did a driving evaluation with the occupational therapist at the rehab hospital and passed. I think our new renovated house has improved our lives--he can deal with his sleep issues by watching tv or listening to books on tape in bed or sleep on his back and snore. We have a caregiver/cleaning person twice a week who does her best to keep his chaos under control. I'm happier anyway now that I have my own peaceful space.

He is in somewhat more denial about the cognitive issues, but he admitted recently he can't handle financial matters and the like when he asked for my help with a situation where he has been dropping the ball for years--serving as executor of the estate for some people who were important to him when he was young. I thought he had withdrawn a year or so ago but it turns out he didn't. I will play the illness card on that one--tell them he has early dementia and doesn't like to admit it. We also have arranged for an accountant to help aunt Florence with her finances so she doesn't keep asking John for help.

John and I talked a little about how he is doing better than I expected after two years. He wondered how much of it is due to his taking coconut oil and MCT oil? No way of knowing. He does have impaired glucose tolerance, so if some dementia is a kind of diabetes in the brain he is likely to be in that group. And that is what the oils are supposed to address by providing ketones as an alternative fuel for the brain.

We saw John's neurologist yesterday and the doctor was pleased with John's muscle tone. John is working with a better physical therapist and she has even motivated him to do some exercising on his own. He has even gone out for a walk a couple of times in the last few weeks. I just wish he didn't have such a tendency to decide to go for a walk or to go see his aunt Florence at dinner time.

I miss having someone to share the work

Yesterday I went with John to the urologist, who again recommended more surgery for his benign prostate enlargement (or the scar tissue from the first surgery). They did say they could do it without general anaesthesia. John wants to try a dilation procedure first, but if that doesn't last very long he wants to find a high-ranked doctor farther away. Sigh.

I'm wishing for help with Christmas. Our daughter and I went and got a tree today and I got it up, but no one has offered to help with decorating it so it isn't decorated. John hasn't been feeling well this afternoon/evening, so I shouldn't complain about today specifically. But more generally, Christmas is too much work to be trying to do mostly on my own. I'm ok with cooking a traditional meal even if I have to do most of the cleaning up too. But even if my daughter will help me some with decorating, having to do the cleaning up makes the decorating seem too hard. I need to think about what I would want in a simpler Christmas.

hand problem

We went to the neurologist last week and talked mostly about John's partial loss of use of his good hand, which was supposed to get better (it supposedly resulted from falling asleep in a desk chair and compressing the nerve). It has gotten only a tiny bit better, though I notice the last few days John is doing some things he had said he couldn't do, such as taking out the trash. The neurologist said recovery definitely is slower than it should be, and he was concerned by how the muscles in John's arm have wasted. He wanted John to get electrical stimulation at the physical therapists office first, and if that doesn't work in a couple of weeks he will try a cortisone shot in the elbow.

John's massage therapist is concerned it might have been a mini-stroke. I'm not following that up because I don't think the therapy would be any different and I am not up for a new round of doctors right now.

I'm tired of cutting John's food up for him, and this is only the beginning of the journey of helping him with activities of daily living. Caregiving does not come easily or naturally to me.

doctor's visit

John had a neurologist's visit Thursday. John reported that his balance is worse and we both reported that he is moving slower. He asked about increasing the Sinemet but the doctor said no because the doctor found his muscles not stiff. The doctor seems most worried about falls.

We asked about when the doctor thinks Namenda should be started. He said when the Aricept stops working. What I wonder is whether the Namenda would be any better than the Aricept, which John takes only a half dose of because it causes him balance problems.

John reported that he doesn't get enough air through his nose and needs to breathe through his mouth. The doctor asked about allergy symptoms, but John said it is true even when his nose is not running. The doctor suggested more exercise to strengthen his chest and diaphram muscles. John said he would go back to using our exercise bike. He does take a 1/2 hour walk several times a week, but I don't know how aerobic that is.

After we got back from Massachusetts it was several days before I got John to go visit the other house, and then he didn't say much. The renovations are in the stage where everything is torn out and some of the changes are framed in. I did get him to make a decision about whether he wanted a larger closet or more built in bookshelves. But it feels like he isn't interested, which is hard on me. It is mostly just one more thing than he can deal with--he has trouble getting the necessary things done in a day.

I'm having a tough time because the main part of the budget for the program I run at work just got eliminated. My job is safe but it is discouraging and hard to know what to do. We also got a lowball offer on our current house and they haven't responded to our counteroffer.

no way to know

I went with John and his aunt to her appointment with an orthopedist. Two weeks ago John took her without me and afterwards told me the orthopedist said she didn't have a new fracture, just arthritis from trying to do too much while healing from the old fracture. Today I asked about the arthritis and the doctor looked at me like I wasn't making sense and said she is healing from a fracture of her tailbone as well as the earlier fracture of her pelvis. So did John misunderstand two weeks ago or was the doctor covering up something he missed then? No way to know.

neurologist appointment

John had a neurologist appointment yesterday and I went with him. John reported that he felt things were stable, except that his feet and legs are more swollen (which is associated with Parkinson's). The doctor suggested Lymphedema Therapy for that and will check whether the physical therapist John already sees can do that. I was very pleased to have a doctor suggest something that could help without taking more medication. I think John's walking is on average worse, he shuffles more, but the doctor thought the swelling was contributing to that. I bought John at least 8 pairs of socks with no elastic at the top but he isn't currently wearing them--he says he lost them. That probably means they are in one of the baskets of his clean laundry either in the family room or in the laundry room.

The doctor was concerned about fall prevention. John said the last time he fell was over Christmas. It startled me to have the doctor see the possibility of John going rapidly downhill--I've been focused on not knowing whether that will happen with Aunt Florence.

sociability

I went with John for his annual appointment with his family doctor this morning. There really weren't any issues to discuss that John wasn't seeing other doctors for, so the family doctor said come back in a year. John's cholesterol numbers were improved.

We went from the doctor's office to the Alzheimer's early stages support group, so we were 10 minutes early. We talked for a few minutes in the car and then when other people started to arrive I said I was going to go in and be sociable. John said he was going to sit in the car and read until time for the meeting to start; 2 hours was enough sociability. I was startled by that, that he sees socializing as such a strain. The person he likes best in the group arrived late, perhaps it would have been different if that person had arrived earlier.

snow day

We had a snow day here, which allowed me to catch up on some tasks I had been avoiding. As the roads were still slushy I drove John to get some lab work done (he grew up in Texas and has never felt comfortable driving on snow). For the second time in his life they had trouble drawing blood. This time he insisted they use the back of his hand after trying his arm once and that worked.

It was quite late in the morning and he hadn't eaten, but he had been drinking water. I asked the technician if it could be a result of low blood pressure and she said yes. But it still seems strange that he has started having problems--his blood pressure isn't that low and he doesn't have small veins.

He called the credit card company and it turns out the credit card I was worried about is actually in both names rather than having him as primary. We lowered the credit limit--not as much as I would have liked but it is some protection.

neurologist visit

John has an appointment with the neurologist today. The neurologist is gradually getting to know John better and ask smarter questions. He asked John about constipation and sleep and in detail about exercise and he asked me if I thought going back to 5 mg. Aricept had made a difference. I said no--I saw a benefit with the 5 mg. but not a significant difference between 5 and 10, though that may be because John is less depressed recently. John felt the 10 mg. badly affected his balance and it is better again now that he is back to taking 5 mg.

John is concerned that his walking is getting more awkward. I guess that is the one place where there is clear progression in the last three or four months. I asked the doctor if it was time to get a handicapped parking permit and the doctor said yes and signed the form. That feels big to me--we are in a new category--but John seemed to just see it as a convenience.

The next question in my mind is when to start Namenda, but thought I would wait and ask it of the movement disorders specialist this summer unless John starts to have hallucinations, which would be a reason to start sooner.

Lewy Body Dementia Organization

A new issue of the Lewy Body Dementia Association newsletter is now available, with an article about the benefits of the right medication, in this case Aricept or Exelon. I have the impression that the organization is beginning to have a real impact on awareness of the disease. If you run into doctors who are not aware, the information to give them is on the web site, including a new article for emergency room doctors. Please, if you can, make a contribution to the association for its important work.

Parkinson's specialist

The Movement Disorder Specialist we saw in Charleston has moved to Washington DC to work for the FDA, so John's local neurologist recommended a specialist in Atlanta, which is a lot closer. We saw him yesterday and were not happy with him.

It was an interesting experience for me because the doctor was taking the approach that John's Parkinsonism was mild and he didn't yet show all the signs of Lewy Body Dementia. John's response was to argue with the doctor that he is more impaired than the doctor realized (John had been working on his Social Security Disability appeal so he had been thinking in those terms). For me, having John acknowledge what I'm finding hard outweighed having the doctor ignore it. The doctor just didn't seem to be interested in subjective experience, only in the measurable symptoms. He made a big deal of how smart John was (having read the neuropsych testing report from May) as if he didn't expect to see Parkinson's patients who are smart. I finally said if John was 80 we would think he was doing great but this isn't what we expected at 63.

At the beginning of the appointment a nurse took John's vital signs and medication information and then sent him back to the waiting room. The doctor himself came out to the waiting room to get John and introduced himself to John. But the odd thing was that the doctor didn't acknowledge my existence when I got up and walked with John. Initially when he asked John questions I didn't speak up because I thought it important for him to see where John got confused. But he never did ask me for my perspective, though I did start giving it anyway.

good news from the ophthalmologist

The bleeding in John's eye was just a bit of bad luck with the natural changes of age (the gel inside the eye shrinks). The doctor examined his eyes several ways, including with a lens pressed to his eyeball, and saw no problems with the retina. So that was good news--I was expecting John would need laser treatment. I did have to miss my class; the only time the doctor could fit us in was in the middle of the day and we waited for almost two hours.

John was very drained when it was over but he seems to be feeling better now.

new issues

John called from the airport where he changed planes on the way home to say that he has what is almost surely bleeding inside his eye. He talked to one ophthalmologist and I spoke to mine and both said come in in the morning. So far as I can tell the problem is related to nothing but age and bad luck.

Reading the book on Families, Illness, and Disability over the weekend I had focused on the idea tha we are out of the crisis/adjustment stage now and I need to change my approach to focus on the long haul. I started to think about what do I want to get done while I still can, and this morning I was seriously thinking about signing up for an Iron Distance triathlon next fall (signups don't open until early December so I don't have to decide yet). And now here we are back to crisis mode--not a big crisis but I've cancelled several appointments tomorrow so that I can take John to the doctor.

my health

When my family doctor saw me in July she was concerned--my A1c (a measure of diabetes control over several months) was up significantly and my LDL cholesterol was up as well. She was also concerned about my emotional struggles with caregiving and told me that menopause is a very vulnerable time for depression. She suggested either an anti-depressant or estrogen. I said give me two months and see if I can find a solution for myself.

Today she was very pleased with my numbers. My A1c is back to its usual excellent level (5.6), my LDL was lower (still somewhat higher than recommended but with an HDL of 78 I refuse to worry about it much). I also reported that while it is still hard I don't have days when I feel hopeless.

I actually give a lot of the credit to the supplement I am taking, which lowered my fasting blood glucose 20 points (from a high level due to stress back to my usual level) and seems to take the edge off the depression. My exercise is also back up some from what I had time for in the spring and I am being careful to stick to my low carb way of eating.

The doctor asked me if I was worried about my weight. I said I've tried to take the attitude that if an extra 20 pounds is what my body wants to get me through menopause with nothing more than mild hot flashes, that is fine with me. But I've gained some more from stress and I am disappointed that it hasn't come off when I brought my A1c back down. I don't believe in dieting (see this for why) but I wish I could go back to the weight my body settled at when I started controlling my blood sugar (which was at the top end of the normal range for BMI).

After I talked defensively for a while the doctor said: "I'm not worried about your weight, I only asked if you were worried. If I were you it is the last thing I would be concerned about; I wouldn't even weigh myself." It was such a good feeling to hear that from a doctor, particularly one who was also telling me that she was very pleased with how I was doing in other respects.

sleep doctor

A belated report on our appointment with the sleep doctor on Monday.

A year an a half ago I had urged John to get a sleep study, because he would occasionally gasp and snort as if he was struggling to breathe. That study showed that he was having 3 apnea episodes an hour--not frequent enough to need treatment. The doctor said to come back in a year and see if it had gotten worse.

By the time John got back, our key concern was not the sleep apnea, but the REM sleep behavior disorder (RSBD). A year ago John was waking me up every night crying out in fear or anger in his sleep. He hadn't hurt me, but he had hit at me a few times and I didn't feel safe. He had been taking Imipramine to help him sleep and the Movement Disorder Specialist wanted him off that because it makes the RSBD worse. John wanted a substitute medication to help him sleep and the specialist said he should see a sleep doctor for that. The medication most often used for RSBD is Klonopin (Clonazepam), which would help him sleep but can make sleep apnea worse.

John didn't think he could do without a sleep medication (in addition to Clonidine) but he ended up weaning himself off the Imipramine about a month before the sleep study. The RSBD became less of a problem because I started moving to another bed as soon as he woke me, but also became less frequent once he was off the Imipramine. The sleep study showed less apnea than a year and a half before. The sleep doctor started focusing on telling John he slept ok even in the sleep study, he should just try to go to bed within 30 minutes of the same time every night.

I pointed out that our main concern had been the REM sleep behavior disorder. That hadn't showed up in the sleep study, which the doctor thought not surprising. I do think it has lessened considerably (though I still move to another room because of John's snoring). We agreed that if it wasn't bothering him or me it didn't need treating, but John asked for and got Klonopin for occasional use when he does need something to help him sleep.

visit to the specialist

Yesterday John saw a Parkinson's specialist at Medical University of South Carolina, Dr. Bergmann. In our last visit, he said he wanted neuropsychological testing before he saw John again. John had that testing at the end of May, and I was upset when the psychologist presented the results as showing that John was not significantly impaired.

Dr. Bergmann viewed the test results quite differently. He said that a 37 point difference between verbal and performance IQ was not something you would see in normal Parkinson's. He said the block design test is the canary in the coal mine for Lewy Body Disease, and that was John's lowest score. He also thought John's 40th percentile on letter and category fluency was signficant.

He increased John's sinemet (to one 100/25 four times a day) to help his increasing Parkinson's symptoms but told us to be careful about dopamine discontrol syndrome and said that punding is particularly characteristic of Sinemet. He said to John that he needs to understand that one of the symptoms of the disease is lack of insight and that he needs to trust me.

Dr. Bergman didn't increase the Aricept above the 5 mg. John is taking. Dr. Bergman said that Aricept tends to makes the Parkinson's symptoms worse so he keeps the dose low, and that its positive cognitive effect builds up gradually over several months.

urology appointment

Early last winter, when John complained about urinary frequency, I was surprised by how willing he was to go to a doctor and do something about it. The diagnosis was benign prostate enlargement, and Flomax didn't help. So the urologist recommended surgery, which John had at the end of February. The surgery wiped him out terribly--it took a week before he was really functional and longer than that for him to not need a catheter. Looking back on it, we were probably seeing a Lewy Body reaction to anesthesia.

The discouraging thing about the whole process was that once the immediate healing from the surgery was over, John wasn't any better off than he had been before. The doctor suggested more surgery but John didn't like that idea at all. So the doctor ordered several further tests and then tried another medication, Proscar, and an office procedure to stretch the area where scarring from the surgery was causing continued problems. The combination worked quite well and John now feels his situation is acceptable. He wants to stay on Proscar because it has stopped his hair loss (a lower dose is approved under another name as a treatment for hair loss).

All the difficulties of the prostate surgery coming right at the same time as John was diagnosed with Parkinson's disease has been a very stressful situation. So it was very nice today to have the urologist tell John to come back in 6 months because his situation is now satisfactory and hopefully stable.

The bad news was a call from John's general practitioner's office asking him to make an appointment to discuss some test results. The two tests were of levels of testosterone and vitamin B12 (the second actually ordered by the neurologist). So another doctor's appointment just when I thought that the amount of time we spend going to doctor's appointments was finally going to decline.

Sleep Doctor

John had an appointment with the sleep doctor today. A year and a half ago I got John to go to this sleep doctor and he had a sleep study which showed very mild sleep apnea, not serious enough to treat. The doctor wanted to do another sleep study in a year, but this visit was more motivated by the Parkinson's specialist we saw in April, who wanted John off the Imiprimine he has taken for sleep for many years because it makes the REM Sleep Behavior Disorder worse. John has always had trouble sleeping, and the combination of Clonidine and Imiprimine is the first solution that has worked for him long term. The sleep doctor wanted a new sleep study before changing the medication and that can't be scheduled until early September. He said he would then try John on Klonopin and assured John that that would help him get to sleep.

With the specialist in April, John said he was willing to try to change his terrible sleep patterns, but now he has decided he is too set in his ways. He falls asleep in a chair around midnight watching TV or reading or using his computer and doesn't come to bed until sometime between 3 and 5 am. He also drinks a half a bottle of wine about three nights a week, starting about 10 pm. I hadn't expected him to change his ways--my thought instead is to get him a really comfortable recliner so that he sleeps in his chair in a more comfortable position. The wine is clearly problematic but it is his major hobby so he isn't going to give it up. He drinks late rather than with dinner because I don't drink and am somewhat uncomfortable when he does (I think it makes him less aware of other people's needs and feelings). If the doctor had pushed the point I might have volunteered that John should have his wine with dinner instead, though I wouldn't have liked that. The sleep doctor did raise the issue of alcohol disrupting sleep but he backed off when John asserted that he doesn't see any difference in his sleep between nights when he drinks and when he doesn't.

We see the Parkinson's specialist at the end of July so there will be chance to run this all by him before making any changes. I don't have strong feelings about what needs to happen. Because of the REM Sleep Behavior Disorder I'm just as glad not to overlap in the bed very much with John (I get up at 5 am to swim two days a week, between 6 and 6:30 other days). When John does wake me up crying out and thrashing in his sleep I just move to another room.