Parkinson’s Disease-An Insider’s Report

By Ken Gunston  

I believe most of what I have written in this paper is consistent with published Parkinson’s Disease self-help literature. Using that literature, I tried to write about how I experience Parkinson’s. Two sections in this document are not consistent with the literature. Instead, they are consistent with what I seem to experience in my body and what I think is going on. I tried to make these differences with the literature clear in the text of these sections.

These two sections should not be taken as truths, but instead as something I would like reviewed and discussed. These two sections are marked with asterisks in the Table of Contents page (over). If you, as either a professional or a Parkinson’s patient or caregiver, have the time to entertain such a discussion, please let me know what you think about what I have written in these sections.

Editor’s note: the author can be reached at kenneth.gunston@gmail.com       

Who Am I ?

I was diagnosed with Parkinson’s disease (PD) about ten years ago at the age of sixty-nine. I am writing this with the thought that I may be near the time when, due to this disease, I can no longer write or put my thoughts together in understandable sentences. My goal is to write down some of the feelings I have and mechanics I use to enjoy my days, possibly even extending them. I have only my own body to experiment with. If you have PD, I hope what I write will trigger your thoughts and that you will be so kind as to share them with me. I intend to write things out in perhaps too much detail, things you already know. If you can, please humor me on that. Engineers are trained to worry about every bolt, lest the bridge collapse.

My medical record would say I am a 79-year-old male with PD, about 150 pounds (was 170), badly stooped (was 6 ft.) with moderate dyskinesia (random movements), forgetful and sometimes confused. I have no other ailments. I am a husband, a father of four, a grandfather, ex-electronic engineer, ex-home handyman and now, a reporter from within this disease.

If, before birth, I was offered a life of 70 years, ending in a quick death, or alternatively, an extra 10 or 15 years to be spent dying of PD and whatever else I could do with those years, I would choose the latter. I think you would too. That thought is better than feeling sorry for myself that I caught PD. Approaching my 80th birthday, I am living that choice.

I am lucky to have a life partner, my wife Suzanne, who plans around my limits and somehow carries on. This is where the pain is, watching her burden. My sweetheart is my angel. She helps me every day, an hour-by-hour continuous gift from her heart. You can’t get this close to someone with PD without feeling grief, frustration, and  anger, at times.

Sometimes I feel low, but mostly I enjoy life and I hope to have some time before Suzanne and I need to go through whatever end of life bumps await us. I have seen the final days of others that have completely lost their reason and I do not want that for myself, nor for others to watch. I hope that my family will let me go without feeling guilty. I am not looking for and do not think in terms of a poison pill or a gun, but perhaps I will no longer want to take care of my body’s nutritional needs. Of course, no one knows what they will do until their time comes. I have made sure there is a DNR on the fridge door, so maybe God will make these, “how will I die”, decisions for me. My job for now is the same as everyone else’s, to enjoy life so those closest to me can see that I am doing that most of the time.

The Parkinson Progression

As everyone knows, PD progresses downhill, but my sense is that it does it in steps. I am familiar with this staircase because I went up it as a child, gaining skills and cognitive capabilities that I am now losing. When I step down from the step I am on, I know I will not visit this step again. So I try to do everything my body and mind will let me do on each step. Steps, as I am describing them here, are not something defined in the PD literature, but rather something I define for myself. I enjoy when I wake in the morning and see that things are pretty much the same as yesterday. I am on the same step.

The concept of PD declining in steps is also important to the way I manage my efforts. I do not step down a step willingly. I fight it. Stepping down is what happens when things that used to work, stop working. It is not unusual to have several things become difficult at once. When that happens, I need to determine how I can maintain myself on the new step. To me going from one step to the next is a process with a beginning, a middle and an end. At the end, I have adjusted to the new step and have maintained whatever functionality that I can, perhaps at the expense of effort required or speed.

Also, a step is not necessarily something going wrong and something going wrong is not necessarily a step. For example, last summer I broke my right hip and it took seven months to fully recover. After the walker, the rollator, the cane, finally I was walking free again! Suzanne and I went on two-mile hikes on uneven ground with confident balance. All that happened on the same step, just an accident and recovery.

Then, only four months later, one year after my fall, my PD advanced a big step. This time, PD introduced me to “freezing” (feels like my feet are glued to the floor) and a big loss of memory capability. At first these things seemed insurmountable. But then I determined what was possible and I found some success. That is what PD feels like to me. There is always a step down the life-staircase but there is, so far, always a next step to catch me, to pause at. I now use a cane and restrict my walking to fairly even ground, and I use a rollator for the first few minutes or so when I awaken. It was a big step down, but it was not a cliff.

My Understanding of PD

In PD as I understand it, the neurons in my brain where memories are stored and thinking takes place, are not dying as happens with Alzheimer’s. (Though there is no rule that says I cannot have both PD and Alzheimer’s disease.  I am thinking, hoping, just PD. PD is enough for now.) What is dying in the brains of people with PD are a particular kind of neuron (a cell) that is located in a part of the brain called the substantia nigra. The job of these cells is to make a substance called dopamine, which is a neurotransmitter. Neurotransmitters are chemicals used in the electro-chemical action that takes place in the synapses between neurons. Without enough dopamine, the neurons communicate more slowly which is called bradyphrenia[1] (slow thinking). Bradyphrenia is the cognitive equivalent of slow motor control, which is called bradykinesia (slowness of movement). 

Some of the cells in our body can be replaced. That is what happens when our skin heals after an injury. The dopamine producing cells in the substantia nigra are not replaced when they die. We are all born with about 400,000[2] of these dopamine producing cells. We lose about 2,000 each year as part of the normal ageing process, but with PD we lose about 5,000 to 20,000 each year. Movement disorders become evident when we are down to about 160,000 dopamine producing cells. I am apparently below that number. The carbidopa/levodopa I take four times a day replaces some of the dopamine I need. It helps, but not completely.

They call PD a movement disease with perhaps some cognitive loss in its later stages. I suspect that at least for me, it is instead a cognitive disease that was first diagnosed, by its impact on my ability to move. Everything else that is affected; slight changes in decision making ability, memory, even feelings, are not hidden, but are so subtle that the observer, the family, the neurologist, even the person affected does not recognize these as PD. These symptoms may have started years before the disease evidenced itself as a movement disorder.

No one wants to admit cognitive loss and I am no exception. But this admission to myself is crucial because it allows me to be more specific as to what my cognitive losses are. I use the simple idea that many of my motor and cognitive symptoms, can be explained as slow communication between neurons (or inappropriate synapses firing), because there is not enough natural dopamine in my brain. Then using that idea, I think about what can be done to minimize some of these deficits. In the rest of this paper, I will try to describe how I have addressed these challenges.

Some people reading this paper with its list of symptoms, and what I do about them, may find it depressing, but that is not what I feel. To me, finding solutions is uplifting and that is what I want to share with you. For the record, I take 6 ½ (25/100 Sinemet) tablets a day, spread over four intervals. I seem to be stable for beyond these intervals. My neurologist has tried to supplement this regimen with other PD medicines, but my body did not handle them well.

I split the list of symptoms into two parts, Cognitive Issues and Movement Issues. There are a few over-arching thoughts that apply to all things I do to address both Cognitive and Movement issues.

  1. When I say I do things a certain way, I don’t mean I am consistent, just that I try to be.
  2. I try to focus on one thing at a time. For example, if my goal is to walk across the room, I stop thinking about what I just did (get out of the chair), review why I want to walk, then focus on the details of my steps.
  3. I try to never, never give up. And then I try again and then again.
  4. I think, analyze and experiment. The experts are great and so are others who have PD but only you, only I, have this body. It is a source of valuable information that I cannot get anywhere else.
  5. My purpose in doing the things I am writing about is to keep myself functional so that I can be with, enjoy and possibly help the people I love. PD robs me of so much, as it does to people who help me, especially Suzanne. There is always a tradeoff between how much I work on my PD issues so I can live and how much I live, how much I take from others and how much I give.

Cognitive Issues and Bradyphrenia

Pre-diagnosis Issues

Some folks believe there is some relationship between PD and certain chemicals. For the record, in the summer of my 20th year, I worked for the mosquito control commission in Suffolk county New York. Sometimes I had a five-gallon tank strapped onto my back with a pump handle and a small hose. With a team of others, we walked through wooded areas spraying something that killed mosquitos, perhaps DDT. During that time, I suffered multiple one to two-inch diameter “boils” on the side and back of my head and neck, one, two or three at a time, new ones replacing the old. Each had to be lanced and drained. however, I am unaware of any lasting problem from this exposure.

I had a prostatectomy in my 57th year, a major operation. The operation took longer than predicted and I spent longer in the recovery room than expected, due to problems related to the anesthesia. I am not aware of any lasting effects from this but Suzanne is concerned that there may have been some cognitive loss, particularly in my sense of direction, something I was once good at. In any case there was definitely a loss in my sense of direction and my ability to retrace (remember) my steps when I was in my late 60s.

Before I had any idea, I had a named disease I found myself forgetting things that I did not realize I was forgetting, like promises and agreements, both at work and at home. It was embarrassing. I had a good reputation professionally but I was not renewing it. Because I enjoyed my work, I had expected that I would continue to work beyond age 65, but when my boss offered me a small bonus to retire at 64 ½, I realized no good would come from not accepting that offer. I was, at this point, not enjoying my work.

Staying in the Here and Now

As one person with PD told me, “I would give up a lot of movement dexterity for less cognitive loss”. That is not a choice we are given. It may not look like it from the outside but there is much that I can do to deal with cognitive loss and it is my responsibility to figure out what I can do and then do it. I owe that to myself, to Suzanne, and to my family. This is where I have to be.

It was about five years ago when Suzanne started telling me she has to make an assessment each time we discuss something as to whether she is talking to “Old Ken” or “New Ken” meaning rational or confused Ken. I need to make the same assessment on myself more often. Specifically, I need to think before I say something. Being slow is OK. Bradyphrenia (slow thinking) is not no thinking. The first comes with PD but the second does not have to. Being “stupid” is scary and Suzanne’s primary window on my sanity comes from listening to me. I need to do it as well as I can. It is OK for me to ask her to slow down, saying, for example, “You are ahead of me. What are you referring to?” to buy myself more time to think. Pauses in our conversation are good. If I ask her something, she has already told me, it helps to let her know I am now aware that I was asking her to repeat what she said.

I try to pay attention to what is going on. Avoid daydreaming. Keep track of plans, doctor appointments and such for both Suzanne and me. Suzanne does not need me to help on that but I need it to be useful in a conversation about plans. We keep a white board which I update regularly, so I can check it when needed. We make Suzanne’s datebook the master document so I just update the whiteboard from her datebook, two to four weeks ahead. Every once in a while, I notice some conflict or consequence (we need to do this before we do that) that helps Suzanne. Almost always, she is the one to notice, but I think she appreciates that I care about these little things so she is not alone, thinking for both of us.   

When we travel to a store or doctor or to just someplace nice, I make sure I remember our route or I take out the map and follow it.

I make it a point to always know what day of the week and date of the month this day is, every day. I am annoyed with myself if I catch myself unsure of the date and day.

Getting lost                                 

About three years after I was diagnosed as having PD, I experienced these confusing situations:

  • While driving (or Suzanne driving) arguing with Suzanne as to which exit to take from the highway to get to our destination.  Then realizing I was confused and that she was correct. That happened several times even though in the past I had a fairly good map in my head. Suzanne used to say I had a good sense of direction. Not anymore.
  • Going into a building and leaving by a different door without realizing I had done it. Then, not knowing why everything looked different and not knowing what happened to my car.
  • Route 1 between Bath and Brunswick is a limited access four-lane which I was driving when suddenly, I realized I had no idea where I was, even though I was 7 miles from my home on a routine errand and had driven this road once or twice a week for years. To me, this day, Route 1 looked like any other-four lane. I could have been in Ohio for all I knew. There was an off-ramp just ahead, so I took it. That off-ramp presented me with a familiar scene, so I could re-orient myself.

These were not simple mistakes, quickly recognized and corrected. They were confusing and, disconcerting and each took a minute or more to recognize before I could correct myself. Each occurred within a few months of each other and about three years after receiving the PD diagnosis.

But they did not keep happening. I thought this was something I could beat. I made progress by consciously keeping track of my movements. I am not lost if I know how I got here and why. I try to keep fewer things on my mind and focus on the “why” and “how” questions while I am moving. I practice this around the house so I will do it when I am out of the house. This does not come naturally, but I feel like I am having real success. With PD, confusion begets confusion and this kind of confusion, the kind that could once be considered acceptable, is no longer acceptable.  

Remembering to Remember

There is a common theme to most aspects of living with PD. What used to be handled automatically, subconsciously before I had PD, no longer works unless I do something about it. I am referring to things like “walking” and “standing from a chair”. The list of these things is long. In general, the solution for each involves moving what used to happen subconsciously to my conscious mind, including each and every detail. Doing that is difficult. It requires identifying each step of the action, learning the order that each of those steps need be done, and then doing them every time the action is needed.

Coping with loss of memory is like that except that, there is an emotional factor. Identifying the steps needed to “stand from a chair” does not sound like a cognitive problem. Everyone knows PD is a movement disease, right? I go to a neurologist who specializes in movement diseases and a physical therapist who shows me the steps I need to “stand from a chair”. No one is threatening my ego. No one is saying anything that could be interpreted as calling me incompetent. But there is no way cognitive loss can be dressed up to look like a movement disorder. Listening is something I learned to do at an early age. Not doing it well in old age is a cognitive limitation, but the good news is that I can do something that helps.

My thought on memory is that cognitive memory loss is exaggerated by not handling, the process of getting the thought into memory. When I don’t remember well, it is often a “failure to listen” or “failure to notice”. Of the two, “failure to notice” is more complicated because it requires making a decision about what is worth noticing. For that reason, I will focus on what I try to do to listen. These steps are perhaps, obvious, but not completing each, will cause me to fail to remember.

The process of “listening” includes “understanding” and “storing”. Storing has two parts, “storing what was said” and “storing the handles”.

 Suppose Suzanne tells me “I am going to the market”. Hearing that sentence involves my brain decoding the sounds into words and then recognizing the meaning of the sentence made of those words. My ability to understand is limited by my bradyphrenia. If I do not understand I will ask her to repeat the sentence. Suzanne often tries to help me to understand by using simple sentences, each containing a single fact, as in this example. She will not give me more information until she can see I have digested the first fact.  Then she might say “I need to get the birthday cake”.

Suzanne using single fact sentences is a big help in “understanding”, but it is my responsibility to be sure I have completed the step of “understanding” what was said.

The next step in “listening” is “storing” which is a two-part step. Storing something involves both sticking the thing I want to remember in my brain and “providing handles” to the thought so I can extract the information when I need it. Both the thing to be remembered (Suzanne going to the store to get the cake) and the handles must be stored in my memory. Storing takes place when I think about it and thinking about it takes place as I create the handles. Before I had PD, this second part (creating the handles) occurred almost automatically. Now I need to do it manually with my executive function, piece by piece. For example, I need to record that I now know where Suzanne will be for the next half hour, so I will not be looking for her. And I will need to connect the birthday cake to the event it is planned for and who will be a year older. I may also connect the thought that she is having to make a separate trip for this cake because the cake was not ready when she was at the market earlier. I might also notice where we were standing or what she was wearing when she said this; any facts I can connect to make the memory richer. You may think I am just adding to what needs to be remembered but that is not how it works for me. All these things form a single concept, complete with a touch of emotion (pleasure – I like her in what she is wearing and I like how the birthday person will feel). All those things form into a single image that is easier to remember. These things are the handles I will use to extract the information I just put into my memory. This is what I mean by remembering to remember, remembering to do this extra work.

Remembering takes time and often requires a patient talker to take the time to answer my questions and requests for repeating the sentence. However, that is much better than not bothering to understand and then seeing what goes wrong. It does not always require extra work by the speaker. Suzanne may say the sentence and then be putting her coat on while my mind is churning away, digesting the information in the sentence she said. It always requires me to go through each of the steps, methodically.

This is not as hard as it sounds. A person does not need to have PD to have experienced only partially hearing a key word of a sentence someone has spoken. The sound stays in your mind for a second while you try to match a word that would fit. When you get it, the sentence makes sense and the thought expressed by the sentence matches the expression on the speaker’s face. That is the step of “understanding”. It is only the process of “creating the handles” consciously, that is new to my listening job.

Avoiding Danger                        

With PD and bad memory, there is a risk of just doing something stupid resulting in catastrophe. I keep a list of these things. I do not depend on my judgement for the things listed below. I am especially careful to watch for them when I have just taken a step down my PD staircase, when I am most susceptible to making a mistake. 

  1. I never drive because I worry about dozing off while driving and I worry about not handling complex traffic situations well.
  2. Some winter days are deathly cold in Maine. I always make sure that at least one door to the house is unlocked when I step outside in winter. I encourage Suzanne to do the same. Not just unlatched. Unlocked, so that even if injured, even if the wind closed the door, we could get inside.
  3. During the winter months, not just on freezing days, I make it a habit to watch the dog while she is outside until I get her in. I do not do anything else while watching the dog, even on nice winter days, even if Suzanne is home, to keep my habit unbroken. Our dog is deaf and not likely to bark. In warmer months I can use electronic devices to remind me to bring the dog in, but that method is not sure proof for winter months.
  4. I do not put anything flammable on the glass top kitchen stove. I once put a potholder on the stove while the burner was on and did not notice it start to burn. Thankfully, Suzanne did. I have not let that happen since. 

Vision Issues, Hallucinations, and the Riddoch Twitch*

In my opinion, the eye-brain connection is surpassed in complexity only by the functions and internal connections between the parts of the brain itself. The eye-brain connection is a place where this disease can have real fun at my expense. It is also where my dysfunction and reality present themselves in full dress. Not all vision symptoms are subtle and some are just old age, helped along by PD. However, they are all mine to have and to hold so I will describe them here.

My eyes no longer present one image most of the time. I see two images, my eyes apparently not tracking together. I keep one eye closed while I am typing or reading.

I have blank places in my vision that seem to come and go. For example, I may have two pills in the palm of my hand but I only see one, the other comes and goes and comes back again. Staring at it, the image seems to settle down and I see both pills consistently.

I can no longer look down a piece of lumber from one end to see if it runs true or is slightly bent or twisted.

I am becoming color blind. I see blue and green as almost the same color and sometimes two things appear to be two distinctly different colors when they are not.

It takes me longer looking at something to discern what it is, at least in its details. That happens with many folks as they get older, with or without PD. I noticed with my parent’s generation and now with mine, a common type of automobile accident. At an intersection with a yield or stop sign, the driver stops, waiting to cross or to make a left turn. Most of the traffic is from the right. Finally, there is a break in that traffic. One quick check to the left, no cars, accelerate and bam, T-boned. The driver swears that they looked before proceeding and did not see the car. I suspect that they did not look long enough, not so much because they were careless or were hoping there would not be a car there, but because they had not yet accustomed themselves to old age and “slow vision”. They saw the scene but not the car in it. When young, it only takes a second to catch a detailed vision, but it takes longer when older and longer still for a person with PD. In low light, with PD, it may take several seconds.

One time, while looking at the wood floor, I saw a vertical wood door where there was none. This happened to me while using my rollator, walking to the bathroom, half asleep, in the middle of the night, in low light. I knew my brain had not made the correct interpretation of the image. When this happens, if I focus, in a short time I will see the scene correctly, a bit like a painting that appears to be one thing, but if you look at it longer you see something else.

I think that there is another vision issue that may be misdiagnosed as hallucinations, an early symptom of Parkinson’s psychosis. Notice this description[3] which was taken from a more complete description of hallucinations under the title “Symptoms of Parkinson’s Psychosis”.

“Typically, psychotic symptoms are subtle when they first start. In the evenings you may begin to see shadows fleeting by or hovering at the edge of your vision or a feeling that someone is standing behind you. Gradually objects at the corner of your vision start to look like something else, and the illusion goes away when you look straight towards it. Afterwards, the images at the edge of your vision may start to become vivid, distinct and stereotypical every time. For the most part in the beginning, you can distinguish these images from reality. These subtle symptoms can persist for months, or even a few years, but it is often unclear when these early symptoms get worse. Usually, an infection or anesthesia from a surgical procedure can worsen it.”

That quoted paragraph includes non-vision phenomenon, but it is the description of vision issues that concerns me.

At least from my experience with PD, a common attribute of the disease is that neurons or the synapses between them, fire inappropriately, spontaneously from time to time, perhaps as part of my dyskinesia, or from my long use of carbidopa/levodopa. For example, if I hold my fingers over this keyboard while I am thinking, there is a chance that a finger will type a key I don’t want typed. This occurs when a muscle in my hand twitches, I assume due to a spontaneous neuron synapse firing.  Or I may feel a pin prick (or several) on my foot or hand but on examination there are no pins. Again, I chalk this up to an inappropriate synapse firing.

If an inappropriate synapse firing were to occur in the neurons in my ocular nerve, that may be another kind of vision problem. The human eye has three primal functions that I am aware of, to recognize night from day (so we sleep), to image light (so we can see) and to detect motion (so we can avoid danger). The Riddoch phenomenon[4] describes a person who can see motion but is otherwise blind, named after George Riddoch, a neurologist who in 1917 wrote a paper describing brain injuries he had seen in soldiers. This phenomenon is rare but has received notice by researchers in recent years. One such person was able to catch a ball thrown at her but could not see the ball once caught, could see a hand coming to her but could not distinguish thumbs up from thumbs down. Although I can’t document it, I believe that I have read that about 10% of the neurons leave the ocular nerve and enter the brain separately from the other 90% of them. These may be the ones that detect motion in the vision field.

PD people do not experience the Riddoch phenomenon because like most everyone else, they have both image and motion sight. In fact, like everyone else, we do not  recognize that these are two different capabilities of our eyes. Our brains integrate these two inputs to form a single perception. You may notice that it is much easier to spot a pill or coin just dropped, if you see it while it is still rolling instead of after it stops.

I suspect others with PD like me, sometimes have spurious neurons firing in those neurons that detect observable motion. For lack of a name, I will call it a “Riddoch twitch”.

All my life, as with anyone, if I thought I saw something move off to one side, I would look directly at it to see what it was, the cat jumping onto a chair for instance. I was almost always rewarded with a recognition of what happened to make me look. However, with PD, if I think something moved off to the side and I look, I may see nothing there. Given the brain’s propensity to make images where there are none (my door example), after having this happen a few times, and if the light is low, there is a visualization, no shape (maybe it is round) and no color (maybe it is gray), but something. I can imagine it being described as a fleeting shadow as in the quoted paragraph above. When this occurs, but nothing is there, I am calling this a Riddoch twitch.

After experiencing this several times, I now get the same visualization every time, the “round gray thing”, even if I sensed a real thing, but didn’t see it. For example, one time in the car at night on a narrow dirt road, Suzanne driving, she said “do you see that?” She was seeing some deer in the woods ahead, just off the road on the edge of what the head lights illuminated. I said “yes” because I too saw something, but I did not see the deer. I didn’t get my eyes to the deer fast enough. They had run away. This was not a Riddoch twitch because it was a response to a real thing that moved, the deer. However, my brain, being accustomed to providing an image when there is motion detected but no image, I perceived the “round gray thing”.

The description in the quoted paragraph of early Parkinson’s psychosis is so much like what I experience with a Riddoch twitch. The “psychotic symptoms are subtle”, “in the beginning, you can distinguish these images from reality”, “the illusion goes away when you look straight towards it” (it was never there). It tends to be to the side (where it can’t be disputed by optical vision) and is “stereotypical every time”. I call this a Riddoch twitch and ignore it. It does not happen often and when it does it is usually in low light conditions. I think this is a physical phenomenon, not a psychological one.

I don’t know why we have motion vision. Its main function may be to alert us to things happening off to the side where our optical vision is greatly reduced and our motion vision may be stronger. That may be one reason why Riddoch twitches tend to be off to the side. Another may be that if they occur directly ahead where optical vision is strong, the brain may ignore them.  

I am not disputing Parkinson’s psychosis, just the interpretation of this symptom. I am concerned that I have not found references to the possible role this physical phenomenon can play in diagnosis of PD hallucinations.

Also, given the description in the published literature, it is reasonable to conclude that I am just experiencing early symptoms of psychosis. I don’t think that, but check on me later.

I am interested to know if you, having PD, have experienced what I am describing, and if you do, is it easy to ignore? I am concerned that for some, this thing I am calling a Riddoch twitch, may feel spooky. The definition of “hallucination” is “an imaginary sensation perceived without anything in the environment to provoke it, like seeing an imaginary dog in the corner of the room when there is nothing there”. I believe that what I am describing is not a hallucination because it is not imaginary. It is real and if I am even partially right, it is happening to people who are vulnerable, questioning their own sanity and possibly being told that they are hallucinating. Even if I am right, I don’t know how common this phenomenon is. It may be rare. My concern is that, given a wrong diagnosis, how many times does a person see nothing there before they put the dog in it?

Vivid dreams, known as REM Sleep Disorder

REM Sleep Disorder is a common symptom for people with PD. These vivid, even ferocious dreams where I am physically fighting some imagined creature, person or even a machine, are scary. Once it was a spinning top, bigger than me. What makes these dreams scary is that I am acting them out. In my mind I may think I am hitting a bad guy but in reality, I am hitting or kicking at the air. REM Sleep Disorder is what forces couples who have shared a bed all their married life, to move to separate rooms.

Unlike most PD symptoms, I do not think REM Sleep Disorder dreams are caused directly by lack of dopamine and they are not unique to PD. “Dreams express an individual’s core concerns, drawing on memories that are similar in emotional tone but different in subject matter. This contextualization is clear in post-traumatic nightmares.” says Tore Nielsen, Director of the Dream and Nightmare laboratory at the University of Montreal[5]. As I realize that PD is the real thing, not something I will recover from, I want to fight it back. I usually wake from the dream when I am about to be overwhelmed by my enemy. Suzanne says I am usually screaming.

Movement Issues and Bradykinesia  

Pre-diagnosis Movement Issues

I was losing my sense of smell many years before I was diagnosed with PD. I still enjoy the taste of good food and an occasional smell comes through. There are many things that cause loss of smell.  PD appears to be one of them.

Our two sons are almost 11 years apart in age, born in 1970 and 1980. Starting when the youngest was 4 or 5 years old until we were all grown men, getting together at family events, we used to form a triangle to throw a ball between us. The triangle was almost a straight line with the youngest in the middle the first year there were three of us. It grew to a big triangle for a few years, limited by how far we could throw the ball and then to a straight line again, now with me in the middle. I came to have as much trouble throwing the ball straight as throwing it far. That was 10 years before I was diagnosed with PD. Sometimes I just watched, enjoying seeing their strength.

I walked with my head bent down looking at the floor (the PD slouch) for at least five years before I was diagnosed with PD.

Another early indicator of PD was the “PD mask”, the loss of facial expression. That did not occur as early as the other early symptoms. Suzanne said she thought I often looked like I was angry. This may have just been three years before the PD diagnosis.

About three months before I was diagnosed with PD, one arm stopped swinging when I walked. This is what got me to the doctor who in turn referred me to a neurologist and the PD diagnosis.

Tremor   

After being diagnosed with PD, the tremor and arm swing problem were my only symptoms of PD. The tremor was mostly in my right arm, though I did have whole body tremor (violent shivering) when I was cold.

Once, when young, while trying to fix a machine, I had my head in a tight place between hard steel surfaces with 3” to spare front to back. Something startled me such that I bent forward quickly and banged my forehead causing me to pull my head back fast, which resulted in banging the back of my head, alternately forehead then back, continuously, painfully, until I got my head out of there. My neck muscles were powering the fun without consulting my brain. The Parkinson’s tremor is much like that but without the pain. I cannot stop it by willing the participating muscles to stop. In normal operation two muscles working in opposition allow precision movement, like an artist holding a small brush about to touch the canvas or a surgeon making a precision cut. Add a little of what engineers call “lag” to such a control loop and it will likely oscillate. That lag is the slow synapse, the missing dopamine. That is what I think is the Parkinson’s tremor.

So how can I minimize the tremor if I cannot remove the lag (cannot add dopamine to the specific synapse)? I have no way to get in there and take control but I might be able to reduce the available energy to the muscles. That must be what happens when I am dozing off. The tremor stops then.

I stumbled across an opportunity to experiment with a specific tremor. My right arm tremored often, but not always. I came up with a reliable way to start the right arm tremor and a somewhat reliable way to stop the right arm tremor. Stopping the tremor was done by relaxing the muscles involved. Starting the tremor was done by scrubbing our glass stove top with the other arm.

The tremor started quickly but it could take a long time to stop. When it did stop, I immediately started scrubbing again. I worked on this for a long time, months. I was encouraged by the fact that it was getting harder to start the tremor. At last, I could scrub the glass top stove with my left arm without causing my right arm to tremor.

I cannot tell you that what I did was responsible for removing the tremor from my list of symptoms. It may have disappeared anyway. But I can tell you my tremor does not occur often and eight years have passed. Now I routinely scrub the stove top with either arm, and experience no tremor.

I have not eliminated all tremor. For example, I still often tremor when I am trying to talk but cannot seem to get my message out, but I think I did something that was worth the effort.

Problems with Large Muscle Movements

I suspect that everyone with PD has spilled drinks and broken dishes. I do two things: purposeful movements, poorly controlled (put the glass down but miss the edge of the table) and random movements (knock the glass over while reaching for the butter). The former seems to come when I have not pushed hard enough to overcome the apparent rigidity in my arms. The second may be caused by dyskinesia. Both seem to occur when my PD has advanced another step down and I have not accustomed myself to this new step. I “fix” the first with practice and the second by slowing my movements.

Problems with Small Muscle Movements

By small muscles, I am thinking about fine motor skills like writing (micrographia), putting small things together including buttons, things with screw threads, keyboarding, eating, opening cereal boxes, etc.

I believe that if I want an occasional victory over this disease, I need to hone two personality traits; patience and stubbornness (though Suzanne might tell you that the latter of these two doesn’t need any honing). Some days I cannot put something together, for example, screw a lightbulb into a socket. It jams, cross threaded. I change the angle of the bulb and it jams again. The one thing I do not do is ask Suzanne to help. I am usually successful eventually. Some things are more important, like the screw threads on the container for the water filter in our domestic water supply. Until I get that together after changing the filter, we have no water. I do ask Suzanne for help but only after I have spent some time on it.  

My handwriting is not legible but I can slowly print neatly. I like lined paper. I use the lines to keep my writing horizontal. I also use the lines to keep the size of each letter the same. Lower case is usually ½  the height of the space between the lines. Upper case is ¾ the space between the lines. I hesitate after every letter to assess size and alignment. Very tedious! Terribly slow! I can print with unlined paper by imagining the lines but I am even slower. That skill is needed for addressing envelopes.

I was once a touch typist, meaning I did not need to look at the keyboard to use it and I used all my fingers. I now use just one finger and I often have trouble finding a letter. I keep the spell checker busy finding my typos (many on every line, usually extra or missing letters) and my misspellings. I use the touch screen only with a stylus to implement the touch. I cover the touch pad with a piece of cardboard, hinged with a piece of tape, so I can access it quickly. Worse, is accidently hitting the control keys. That can put me some place else in my document while I think I am in the middle of typing a word, or even inadvertently closing my document. But keyboarding is better than hand printing.

Gait Issues

When I first had trouble walking, I tried to correct my stride by thinking about each muscle group and what it should be doing or not doing. That was both frustrating and not productive. Then another PD patient told me how to simplify my gait (just 3 parts, stretch out forward leg, land it on the heel, push off with the back leg). To do that for each step took concentration, so I still could not walk and talk at the same time, but it improved my gait. My physical therapist told me he could still recognize my gait as Parkinson’s because I did not twist my torso when I walked. Too complicated, I thought and dropped the idea of adding another motion to my gait. Then one day, I tried it, the three parts to the step, the arm swing and the torso-twist, all together. What happened was unexpected, a complete surprise! Everything seemed to work as it did before I had PD. The torso twist seemed to tie everything together. I am sure I could not fool anyone into thinking I had a normal gait but for the first time in a while, I could carry on a conversation while walking. It was a victory the way you feel victorious as a child, that first home run, or just riding a bicycle around the block without falling. This surprise was a big deal because it hinted that there was much more to learn about how my brain worked that might help me cope with this disease.

Somewhere in our brain there is a place where we store muscle memories. Muscle memories tell us how to use muscles to do a thing with our body that we have previously learned to do. The list of stored muscle memories would be long but would include, how to walk, how to run, how to ride a bicycle, how to catch a baseball, how to bring an overfilled cup to the mouth without spilling, and how to put it down. In short, just about everything we ever practiced and learned to do. These muscle memory recordings are there so we do not have to think them out each time we do something. The muscle memory recordings are not static recordings but rather are modified as needed. I catch the ball differently depending on where the ball is coming relative to where I am standing. These muscle memory recordings remain in my nervous system even if they have not been used for a long time, as anyone who tries to ride a bicycle after not riding for years, finds out to their delight. I believe that with PD, these various muscle memories are still there. There may be a way to awaken some of them by practicing the original movements. And when I practice, if I do not get the feeling that I am getting help from an old muscle memory, at least this idea got me to practice something. No harm in doing that.

Failure of Gait Initiation, FoG (Freezing)*

I recently started having the experience they call Freezing or FoG. What a nasty experience it is! It took me a few weeks to begin to learn how to maintain function; that is, how to ambulate with confidence after experiencing FoG.

Generally, in the self-help literature, FoG is understood as an extension of other gait disorders, including short steps, shuffling and unsteadiness. The literature describes FoG as “failure of gait initiation” (feet stick to floor or will not move). According to what I read, gait disorders can be helped by addressing balance, strength and energy level. FoG can also be helped by addressing the environmental factors that cause it, such as clutter or poorly lit rooms or shiny floors[6].

There are a host of suggestions for coping with FoG, some of which are similar to what I do. For example, Dr. Lieberman[7] describes them as useful “tricks” which are used at the Muhammad Ali Parkinson Clinic. They include humming or playing a tune, snapping your fingers or your cane while chanting “Go, Go, Go”, stepping over real or imagined lines on the floor or spots made by a laser light that you hold.

I think about FoG differently. I think that when my gait fails in this way (FoG), particularly when it is failing for environmental reasons (like a doorway), I stop walking right away and use another way of ambulating until I get by the obstruction. My only goal in this situation is to maintain function, being able to move my body away from danger, or risk of falling, when I need to do that. In my opinion, removing those fears are paramount, before even working on non-fearful festination issues.

Consider this example of freezing. I was standing and decided to walk across the room. Normally it takes a little more than a second to form the thought and take the first step. But this time I did not start moving. Instead, both my heels lifted so that I was standing on just my fore-feet. Both knees started an unproductive rapid back and forth without moving my feet. I could not walk normally. I could not walk at all for several seconds. I almost fell. Gradually I realized that in my PD progression, this strange thing was happening and it was happening often. It was my notification that I was taking another step down the PD staircase.

The way a person walks is called their gait. Our gait changes as we grow, as our weight increases into adulthood and our muscles get stronger. A person’s gait is the most efficient way s/he has to move around. As we reach our later years, injuries and diseases like PD take their toll. Again, our gait changes as needed to accommodate the changes in our body. Like everyone else with PD, I never had to think about my gait until I got PD.  When this happened, when my feet felt glued to the floor, I was facing something I could not ignore. Clearly, execution of my gait muscle memory, which had once automated my walk, is failing to function well. Not being able to move can be fearful, and the fear feeds back to the attempt, degenerately.

What follows is my hypotheses of what I think may be going on in FoG and what I can do about it. Using this thinking, I was able to do something important. I regained the confidence, that if I needed to get out of the way of danger, like a car moving toward me in a parking lot. I could do it.

My contention is, that in FoG, my lower-level brain (the part of my brain which is trying to implement a gait, probably my cerebellum and/or basil ganglia), is being overloaded with more information than it can handle (low dopamine, bradyphrenia ). And, that this problem can be solved.

When my gait fails (FoG), particularly when it is failing for environmental reasons (like going through a doorway), I stop walking and pause for a second. The next movement I make, will not be to start walking again. Instead, it will be to move one foot out ahead and pause again.

I believe the pause does two important things.

  1. It relieves my “low level brain” from the responsibility of walking while leaving it with the responsibility of maintaining my balance.
  2. It allows my executive to take control of placing one foot out ahead.

Researchers have found the place in the brain where conscious thoughts take place, where the executive is located, in the cerebral cortex. After the pause, when I move one leg forward, I am using a different part of my brain. I am using my executive.

The idea of using two different parts of my brain in a cooperative effort to accomplish something that is normally done automatically by one part, is what I am suggesting. This is something we know how to do. Long before PD had taken hold of my body, I would have occasion to do something similar to what I am describing. For example, while walking on a trail through wooded terrain, I might come across a large rock in the trail with a tree alongside the rock. The trail beyond the rock is higher still. I might stop to assess the situation. Then, using the tree trunk for balance, I grab it and step up onto the rock and the higher ground beyond it before continuing my walk.

In FoG it may take more than one “executive” step, each time dragging my back foot up and then pausing. I have noticed that after I have been practicing this for some time, I can reduce the size of the pause. I think the key is to make sure the pause and the “put my leg there”, be accomplished by my executive. 

Just a note on maintaining balance when using my suggestion of avoiding problems with FoG. After stopping, when moving one foot out ahead, it is important to first rotate the foot that is not being moved so that it provides left and right support.   

I am struck by how beautiful, how graceful, this transfer of control between parts of my brain happened when I was young and walking that trail. I guess it took losing it for me to notice. 

Drooling

Not drooling is important socially, to save computer keyboards and for my own comfort. I do not think I have much to offer that you no doubt have already tried, but this is what I do.

Posture really helps. I try to keep my chin up, not looking down. “Try” is all I am saying.

I suckle my mouth often (almost continuously when I am with people or typing).

I have learned to sleep on my back and when I want to sleep on my side, I first wet my lips and then hold them together until they dry, using my dry saliva as glue. This works sometimes but mostly I give up and lie on my back.

Postural Stability

Refers to ability to balance as on one foot (static) and while moving (dynamic). For several years now I have not been able to stand on one foot. I used to practice that. As a morning exercise I put my pants and socks on while standing but touching (usually leaning against) the bed. Then I turn 180 and do the other leg. At the end of the day, I take these off while sitting.

As to dynamic abilities, I need to be careful not to make large movements quickly lest I fall. I can put on my shirt, sweater or jacket while standing but I am careful to have my feet both facing forward, wide apart and that no one is near that could bump me.

Walking is easier than standing, as long as I don’t walk too fast. That is because while walking there is always a foot in the air that can be placed were needed to keep me upright, the way a bicycle rider keeps the bike upright by steering the wheels to remain underneath.  Counterintuitively, I find standing still on a horizontal surface will eventually result in a fall, though feet apart and shifting my weight from one to the other, like a golfer, sometimes helps. For me, standing still on an inclined plane is dangerous.

Getting up from a Chair, Any Chair

For kitchen (hard) or folding chairs, I use the LSVT “stand from a chair” exercise which is, butt forward on the seat (or at least not too far back), feet back as far as possible, upper body forward, arms out in front, all to get my center of gravity (CoG) directly above my feet, and then straighten my legs to stand. For overstuffed chairs I have to do something more because my butt is so far back and low. So, I precede the hard chair technique with a strong push with my shoulders against the back of the overstuffed chair while pulling on the front of the chair arms with both hands to get my butt to the edge of the seat so I can use the hard chair technique.  When this does not work to get my butt forward enough, I try again with a stronger effort. I want my CoG as far forward as possible before I push off.

For a toilet I use the same technique as for a chair. However, the shape of the toilet provides another option to get my CoG over my feet. The base of the toilet is narrower than the legs of a chair which means I can get my feet back on each side of the toilet, under my CoG.

To repeat because it is important, in general, for all kinds of chairs, while getting up I am doing two things, (1) getting my CoG over my feet and (2) then straitening my legs to stand up. It is important to do them in that order or I will fall back into the chair.

Standing from a sofa is difficult because at best you have only one arm rest, possibly none. In that case I use the shoulder push technique to get my butt to the edge of the seat. Getting my arms strait out front and my upper body horizontal, balanced over my feet, sometimes works better for me than pushing off.

Soft voice

My voice is soft enough so that often people do not know I am trying to say something, particularly in group settings. “LSVT Loud” practice results in a sore throat and a softer yet voice. A speech therapist tried to tell me how to use my mouth cavity to resonate and amplify the sound and to get me to move the sound forward in my mouth when speaking, but I have to admit I did not understand all that she was trying to teach me. That effort was interrupted by the pandemic.

Boxing

No one hits a person or gets hit in PD boxing, but the idea is to develop speed and agility, something PD patients have trouble with. They train for boxing by wearing boxing gloves and hitting speed bags, big bags or trainer mitts held and moved by the trainer.  This is a popular activity particularly for people (men and women) whose PD is less advanced and still have good balance.  I never participated because I have REM sleep disorder. The last thing I want is to learn how to throw a better punch.

Last Thought

Those of us who have PD don’t normally talk about the issues I have described here in this kind of detail. For some of us, like me, the disease has taken away our ability to process spoken words quickly. Which is why I wrote this paper. My hope is that, it will serve as a conversation starter. I hope you find some parts of what I wrote to be helpful and possibly supportive of what you think. Again, I want to trigger discussion.

If some of the paper is understandable, you are seeing Suzanne’s hand. I could not have written this paper without her. For those parts that are not readable, that is when I didn’t listen to her.

References


[1] Every Victory Counts, article by Mark Mapstone, PhD, page 145

[2] 100 Questions and Answers About Parkinson’s Disease by Abraham Lieberman M.D. page 32

[3] Every Victory Counts, article by Cherian Karunapuzha, MD, page 104

[4] Scientific American, October 2018. Article by Bahar Gholipour, page 14

[5] Scientific American, Article by Tore Nielsen, page 30

[6] Every Victory Counts, article by Helen Bronte-Stewart, MD, MSE, Page 98

[7] 100 Questions and Answers About Parkinson’s Disease by Abraham Lieberman M.D. page 97

Are you ready to meet virtually?

As you must have noticed, medical offices and hospitals during the time of COVID-19 have postponed or canceled most non-urgent visits, tests, and procedures.  This has meant a massive backup in patient care and we have tried to reach many people by telephone.  Phones are not ideal for movement disorders, however.  It helps for us to see your tremor, dyskinesia, or what have you.  Fortunately, the Center for Medicare and Medicaid Services has approved virtual appointments with video.  This means that if you have a smart phone, tablet, laptop, or a computer with a camera, you can probably have a virtual office visit with your healthcare provider from own home. 

I am not sure what platform every office in the state is using.  MaineHealth (including my practice recently) is using Zoom, an app (application) that you can download onto your device.  I am sure many of you are familiar with this, as it is being used all over the country for other types of virtual meetings (offices, families, and so on). I understand that some Parkinson’s support groups are even meeting by Zoom.  If you are not familiar with this app, it is free (on the end user side) and very easy to download.  On iphones visit the App Store, on Android phones visit the Play Store, and if using a desktop computer go to https://zoom.us/download   to download the software.

We are not sure when we will be back to normal office scheduling, and there is still a risk of contracting the SARS-CoV-2 virus that causes COVID-19 in our state, especially when people congregate.   In order to avoid a second wave of cases we are planning on trying to gradually reintroduce safe office encounters, but it is going to come along in stages, and there may be a long delay.  One thing we want to avoid at my office right now is a group larger than ten people, or a failure of social distancing, which would occur if all five doctors starting booking full appointment days in the clinic again. A video “telehealth” appointment is a good way to see your provider for now without that risk.  And, that technology is likely here to stay.  The genie is out of the bottle, so to speak. Further, becoming comfortable with this software would mean that you too could visit love ones with the computer or your phone.  So, put aside your computer discomfort, embrace the future, and get ready to meet virtually. 

Convalescent serum for COVID-19

April 3, 2020 Johns Hopkins in Baltimore was given FDA approval to test a blood plasma therapy to treat COVID-19 patients.  The trial will use blood serum (a straw colored liquid left after the clotting of plasma), collected from recovered COVID-19 patients. This trial will evaluate convalescent serum as a preventive treatment for infection in people at high risk of contracting disease, and as a potential treatment for people critically ill with COVID-19. 

How does this work?

When we get better from an infection, we have made antibodies, and these tiny molecules protect us from reinfection at some point in the future.  Antibodies are found in the blood serum.  This therapy is therefore about harnessing the immune system of one person who has recovered from COVID-19 to help another person either prevent getting ill in the first place, or possibly recover from active infection.    

Passive antibody therapy occurs with administration of antibodies against a target such as a virus, to a person who is susceptible to that virus. This is done to prevent or treat the infectious disease the virus might cause. We could contrast that to active vaccination, which occurs when for example, a protein from a virus is injected into a person to provoke an immune response to that virus. Doing that takes time, and may vary depending on the biology of the person getting the vaccination. 

According to the Johns Hopkins based Health University News, Arturo Casadevall, M.D., Ph.D., Johns Hopkins Chair of Molecular Microbiology and Immunology has worked with physicians and scientists from around the United States “to establish a network of hospitals and blood banks that can collect, isolate, and process blood plasma from COVID-19 survivors.”  The FDA, which is allowing emergency authorization of convalescent plasma as an investigational new drug (IND), notes “it is possible that convalescent plasma that contains antibodies to SARS-CoV-2 (the virus that causes COVID-19) might be effective against the infection. Use of convalescent plasma has been studied in outbreaks of other respiratory infections, including the 2009-2010 H1N1 influenza virus pandemic, 2003 SARS-CoV-1 epidemic, and the 2012 MERS-CoV epidemic.”  (footnote 1, and 2)

The history of passive immunization

The concept of using convalescent plasma is not new.  Doctors have long recognized that with most illnesses there will be some people who do better than others.   In the case of an infectious disease outbreak the same is true. The question is why. 

Much of the answer has to do with the immune system, the body’s defense against infection.  It has been shown that some people mount a more robust, or more effective immune response, than other people who might succumb to the same infection.

The therapy proposed here goes back at least to the 1890s, when it was shown that serum from rabbits previously exposed to tetanus toxin could be used to prevent tetanus in other rabbits exposed to the toxin.  The technique was also shown to work with diphtheria toxin.  Building on this it was shown that increasing from small to larger doses of bacterial toxins such as those in diphtheria and tetanus could induce an animal to develop immunity against larger, lethal doses.  This led to mass production of serum in dairy cattle and horses for the treatment of diphtheria.  It also led to the concepts of active and passive immunization, and to the field of humoral (antibody) immunity.  Nobel prizes were awarded for these history-changing discoveries.

In the early part of the 20th century, physicians would often try to contain outbreaks of viral diseases such as measles, mumps, and polio by treating exposed people with convalescent serum. During the 1918 influenza pandemic, serum from recovered patients was used to treat some acutely ill influenza victims, and to treat people who had been exposed.  By the 1920s it became very common to treat pneumococcal pneumonia with serum form horses.  Over the next few decades clinical benefit was also seen with this technique in the treatment of Haemophilus influenza B, and meningococcus.  There were multiple related therapies over the years.

An expert in the field

Dr. Casadevall discussed how doctors of the early 20th century used convalescent serum to prevent outbreaks of infectious disease on the Johns Hopkins Bloomberg School of Public Health podcast Public Health On Call.  He noted that the antibodies we make with our immune system during an infection are “why people only catch measles once, and (why) they’re protected for the rest of their lives.”  When an epidemic decades ago was starting, doctors “would find somebody who’s immune, somebody who’s recovered from the illness, ask him to donate blood, they would separate the blood and the serum, and then they would give small amounts of the serum to people who are vulnerable, that is, those who have been exposed, those who  are likely to have caught the disease.  And, it was quite effective.” 

Convalescent serum was used in 1934 for example, to stop an outbreak of measles in a Pennsylvania preparatory school.  In that case the serum taken from one recovered boy was used to treat 66 other boys who had been exposed.  Each boy was given only 5-10 mL of serum.   “When you have a disease like measles, approximately 25% of the kids will get it.  It’s one of the most infectious contagious diseases that we have. And…only 3 children developed measles, even though they expected numbers would have been a quarter of the 66 children,” (17 of them).  The 3 boys that had measles wound up having mild cases due to the treatment.  “When vaccines came on board in the 1960s this practice was both stopped and forgotten.”    

With COVID-19 many people are going to do well, and are going to make antibodies to the coronavirus. The plan is to use blood banking processes to prevent the spread of other diseases, and give small amounts of safe convalescent serum to healthcare workers, first responders, people caring for others with coronavirus at home.  Convalescent serum therapy is more effective for prevention than treatment.   However, it can be given after disease has already started, but there were some hard lessons learned decades back.  

When antibody therapy was used to treat streptococcal pneumonia doctors found if it was given in the first few days of symptoms good results were seen, though it seemed to have much less, or no value if given later than that. One speculation as to why has to do with the amount of antibody needed. To prevent disease people need very little antibody because the number of organisms infecting a person is usually very low. Alternatively, once disease has developed in a person and cells are making copies of the infectious agent (bacterium or virus), the number of particles in the body is very high, which would require more antibodies and thus, more serum.

Conclusion

Convalescent serum sounds very promising, especially as a protective strategy for those who have likely been exposed to the virus that causes COVID-19. It seems less likely to be helpful in those with fulminant disease.

Larger doses of convalescent plasma may be helpful. Shen, et al. reported in the March 27 issue of JAMA that doctors in China treated 5 critically ill patients on ventilators with laboratory-confirmed COVID-19 by giving convalescent plasma transfusion. There were improvements in the first week, and by 37 days after infusion, 3 had been discharged from the hospital, and 2 were in stable condition. This is interesting, because the fatality rate of COVID-19 patients on a ventilator is high. It is a hopeful sign.

Given all of these points, even if convalescent serum is helpful in limiting the spread of an outbreak, it is probably a stopgap measure.  There is a massive worldwide research effort aiming to give stronger therapies, such as drugs to inactivate coronavirus, monoclonal antibodies-which are usually superior to convalescent serum (think silver bullet versus shotgun), and vaccines.  The problem is the time it takes to develop these interventions. Convalescent serum may be a much faster option. That is probably why this is also being tried around the world.

FOOTNOTE 1:  The FDA has significant “Considerations for healthcare providers interested in obtaining COVID-19 Convalescent Plasma for Use under IND.” These include using COVID-19 convalescent plasma collected from recovered individuals if they are eligible to donate blood under the Code of Federal Regulations used by blood banks.  Evidence of COVID-19 should be documented by a laboratory test either by a diagnostic test (e.g., nasopharyngeal swab) at the time of illness, a positive serologic test for SARS-CoV-2 antibodies after recovery, if prior diagnostic testing was not performed at the time COVID-19 was suspected.   The donors must have either complete resolution of symptoms at least 28 days prior to donation, or complete resolution of symptoms at least 14 days prior to donation, and negative results for COVID-19 either from one or more nasopharyngeal swab specimens or by a molecular diagnostic test from blood.   In terms of recipients of the blood serum, the FDA requires laboratory confirmed COVID-19, with a severe or immediately life-threatening COVID-19 (specific criteria on the FDA website).  

FOOTNOTE 2: This study in Northern China in 2003 evaluated 99 convalescent serum samples from patients 35-180 days after the onset of symptoms. Anti-SARS antibodies were detected with three lab tests in 87 of the samples. Zhang, et al., A serological survey on neutralizing antibody titer of SARS convalscent sera. J Med Virol. 2005;77(2):147-50.

When will the curve reach a peak in Maine?

That is something we would like to know on the medicine/public health side so that we can plan how to respond with vital resources and people to care for patients.  It would also give most of us a sense of relief to know that there is an end to this crisis here in Maine, and around the world.

To figure out when an epidemic with peak, we have to consider multiple factors.  There were early reports from China that wave of COVID-19 peaked at about 8-9 weeks. If that is true, it may have been due to excessive containment strategies, rather than a natural cycle of the outbreak. South Korea also was able to control the outbreak through a concerted effort to test huge numbers of people, track down contacts, isolate, quarantine, and the society was engaged in the ethos and practice of social distancing. It is less clear around the world whether the same period of time will occur, though according to news reports it appears Italy may be in a plateau, with a stable number of new cases over the last few days. I don’t think the duration of the outbreak is a universal number, though with infectious disease epidemics we often do see periods of time which seem to be the “season” for that disease, such as the range of time we see seasonal influenza. The SARS-CoV-2 virus that causes COVID-19 is a novel virus, and it spreads rapidly in populations. There are many unanswered questions about this pandemic.

One simple place to start in trying to determine the time a wave of a new epidemic will take is with the incubation period (the time from exposure to illness).  We know the incubation period is between 2 and 14 days, and the average time that symptoms develop in those affected is 5 days.  Using just this, we should be able to estimate that effective flattening of the curve would take up to two weeks to occur (if as many people as possible are social distancing, for example).  In other words, if we were able to completely separate all of the people in our state, it would take two weeks to see no new cases.  A virus needs a host to spread, and can’t do that if there is no one else around.  The problem is that complete isolation is not possible.

Complicating matters, there are many other factors at play, such as the stability of the population size.  Many cases of COVID-19 have been associated with travel, and we are seeing an influx of non-residents and residents coming back from out of state locations (more below). Although on March 16 educational facilities closed, and on March 25 non-essential services closed, essential workers are still in contact with people, in some cases, a lot of people.   Other factors include resources, the ability of health systems to withstand a surge of patients, what the size of that surge might be, and whether the percentage of healthcare workers who become ill is stable (17% of cases overall as of 4/3/20 when it was reported last), to name a few. 

There are many people in our state who are trying to model the pandemic within our regional outbreak.  You can be certain that every hospital system is trying to do this.  Dr. Nirav Shah, Director of the Maine CDC has said during his daily briefings recently that they are tracking various models, though none should be treated as completely predictive. 

“The modeling tools are only as good as the assumptions we put in, and then on the other side, how well Maine people follow those assumptions.”   

Nirav Shah, M.D.

The Institute for Health Metrics and Evaluation (IHME), part of the University of Washington, and the recent recipient of a 10-year, $279-million investment by the Bill & Melinda Gates Foundation, is tracking the COVID-19 outbreak across the U.S. and making models of how the outbreaks are projected to increase and peak in every state.  April 1, 2020 the IHME reportedly projected that Maine’s health care system might become overwhelmed by April 25, based on the concurrent trajectory of the outbreak, and social distancing and business restrictions in place at the time.  April 2 Governor Janet Mills’ Stay Healthy at Home mandate took effect.  There was however, much public concern about the influx of people coming in from out of state, including from hot spots such as the state of New York, the current epicenter of the epidemic in the U.S.  

A common facet in the history of epidemics is that those in the center of an outbreak flee.   However, this invariably spreads the epidemic while exhausting resources. Another commonality is a less than welcoming response by those in the receiving territory. 

On April 3 Governor Mills issued an Executive Order Mandating Quarantine Restrictions on Travelers Arriving in Maine to Protect Public Health and Safety which required that travelers arriving in Maine, regardless of state of residency, self-quarantine for 14 days.

“The Order directs the Maine Department of Transportation, the Maine Turnpike Authority, and others to post this guidance at all major points of entry into the State, exempts individuals who are providing essential services as defined by Governor Mills’ March 24 Executive Order.” 

Mills further instructed visitors not to travel to Maine if they are displaying symptoms of COVID-19 and advised them not to travel to Maine if they are traveling from cities or regions identified as COVID-19 hot spots. 

“To deter travel, the Order also suspends lodging operations, including hotels, motels, bed and breakfasts, inns, and short-term rentals such as those available through VRBO Airbnb, RV parks and campgrounds, and all public and private camping facilities as well as online reservations effective April 5, 2020 at 12:00 p.m.” 

There are some exceptions, such as vulnerable populations: children in emergency placements, persons at risk of domestic violence, and homeless individuals as permitted by the state, accommodations for health care workers, or other workers deemed necessary to support public health, public safety, or critical infrastructure.

The IHME projections for Maine as of 4/3/20 indicated hospitals would hit peak resource usage on 4/17/20, involving 1,179 beds (118 more than available), and 179 ICU beds (with a shortfall predicted).  Per Dr. Shah as of 4/3/20 at his weekday press briefing, there are 272 ICU beds in the state, but availability is a number which changes daily, based on more than just COVID-19 (we still have seasonal influenza, community acquired pneumonia, and many patients with chronic lung diseases in Maine).  IHME predicted we would need 143 ventilators for COVID-19 patients at peak.  Dr. Shah reported we have about 348 ventilators in the state, along with just under 200 “alternative” ventilators designed for medical procedures such as surgery.  IHME projects that by 4/15/20 deaths related to COVID-19 will peak at 12 per day in Maine.  Sadly, it is projected that by June we will see a total of 364 deaths due to this epidemic in our state.  That number appears to be flat through August.  That is of course, with assumption of only be one wave of this outbreak. 

While these numbers are frightening, they pale in comparison to some other parts of our country. In the U.S. 9,458 people have died from COVID-19, and over a third of the 331,234 cases in the U.S. as of this writing, are in New York. Over 60% of the states have case numbers in the thousands. New Jersey has over 37,000 cases, and several states have over 10,000. There are only 7 states in the continental U.S. with fewer cases than Maine: Nebraska, West Virginia, Montana, South Dakota, North Dakota, Wyoming, and Alaska, (in that order). We have fared better as a group of less densely populated states. Maine has the willingness of Mainers to pitch in to the effort. We need everyone on board with that effort.

We have still seen an unacceptable number of cases and should have had more testing a long time ago. The entire country is in that position. I will say again that we don’t really know how many people are sick. We, like the majority of the country are only able to test people who meet a narrow set of criteria. Many people have been, or still are sick at home with what is probably COVID-19, but have not qualified for testing. The CDC states that probably 25% of all of those infected are asymptomatic, but capable of spreading disease. It is imperative that we markedly increase testing.

Today in Maine there are 470 cases of confirmed COVID-19, and 86 people have been hospitalized.  There have been 10 deaths since the start of the outbreak, and all but one county, Piscataquis, have had cases. It is not time to relax. We could still overwhelm our hospitals. Take a close look at the graph. The number of cases has more than tripled in the last 10 days. What might that look like in 10 more days? We must flatten the curve. If we approached this pandemic the way experts have recommended, we could crush the curve.

Though our nation has the highest number of cases in the world, this is going to get better. On the other side of the peak is a downslope. We just need to continue to stay home, practice social distancing, wear cloth masks in public, wash our hands, to not touch our faces, and to help each other get through this. We are going to have to flexible, and we will likely come out of this a changed nation. May we all learn from this. May we be better prepared in the future. May we never allow our government to weaken the CDC and our defenses against infectious disease again. May we again learn to let the experts in science and medicine do their jobs. We are better and smarter than this.

Making cloth face coverings, instructions from the CDC

In the MPDN post yesterday, CDC recommends wearing a cloth face cover in public, I gave a link to CDC instructions for making a cloth face covering from a cotton T-shirt, along with a couple links to videos, etc. The CDC has added additional instructions which can be viewed online or downloaded as a .pdf. These instructions demonstrate how to construct three types of face coverings:

  • Sewn Face Covering from fabric
  • Quick Cut T-shirt Face Covering (no sew method)
  • Bandanna Face Covering (no sew method)

To put on a face covering, grip the cover by the draw strings or elastic material used over the ears. It is okay to adjust to tightness around the nose, but try not to touch the material through which you will breathe. In general, try to limit touching the mask while wearing, and if you do so, use clean hands. Please keep in mind that any cloth face cover should be washed regularly, depending on use. It follows then that any material you select should be capable of being laundered and machine dried without damage or change to shape.

The CDC notes “individuals should be careful not to touch their eyes, nose, and mouth when removing their face covering and wash hands immediately after removing.”

There are many other sites that show instructions as well. I am not sure all are equal and would generally treat the CDC instructions as the standard, especially where safety features such as material and thickness are concerned. An article in The New York Times also included instructions for a simple cloth face cover. If I see any more interesting articles on this topic in the coming days I might add an addendum to this one.

Remember, the purpose of wearing a face cover in public is to limit the spread of the virus. Do your part, cover your face. We in the hospital and clinic have been masking for every patient encounter to protect you. Now, we all need to cover our faces in public for the time being.

Finally, I will say it again, wearing a cloth cover does not mean you should stand closer than 6 feet with other people. Keep doing that. Let’s crush the curve.

CDC recommends wearing a cloth face cover in public

Friday, April 3 the CDC recommended:

“Everyone should wear a cloth face cover when they have to go out in public, for example to the grocery store or to pick up other necessities.  Cloth face coverings should not be placed on young children under age 2, anyone who has trouble breathing, or is unconscious, incapacitated or otherwise unable to remove the mask without assistance.” 

The CDC was clear that “the cloth face cover is meant to protect other people in case you are infected.”  More on that below.  The CDC also stated:

  • Do NOT use a facemask meant for a healthcare worker
  • Continue to keep about 6 feet between yourself and others. The cloth face cover is not a substitute for social distancing.

Dr. Jerome Adams, U.S. Surgeon General, gives a 46 second video about how to convert a simple winter scarf and two rubber bands into a face cover.   Also check here for more Q/A on cloth face coverings by the CDC.

This might seem to be a reversal of Dr. Adams’ February 29, 2020 tweet in response to Americans panic-buying all the surgical and N95 masks (specialized microfiltration respirator masks):

“Seriously people- STOP BUYING MASKS!  They are NOT effective in preventing general public from catching #Coronavirus, but if healthcare providers can’t get them to care for sick patients, it puts them and our communities at risk!”  

It is not, and he was right on more than one point.  If healthcare workers all get sick, there won’t be anyone left to care for the rest of the public.  If health systems fail, the epidemic curve steeply and broadly increases, and that is bad.  

He was also right that there was, and still is a big shortage of personal protective equipment (PPE) all over the country (and in many other nations). N95s are needed by medical personnel who perform procedures which might result in aerosolization of virus, such as intubation of people with COVID-19. N95s, or some other specific PPE are also worn by medical personnel in the presence of suspected and confirmed cases of COVID-19 generally, though I have heard from colleagues around the country who may be running out of those supplies. This places them at very high risk.

Paper surgical masks have a role also for medical personnel, and should not be worn by the general public. Take a moment to consider that when a surgeon and his or her team wear a mask, they are protecting the patient from their own respirations: respirations which contain droplets, droplets which might contain bacteria, viruses, or other pathogens. 

Dr. Adams was also right that with an airborne virus a paper surgical mask is not effective in preventing the general public from catching the virus.  This point is a complex thought contained in a tiny tweet.  A paper mask (even if you are wearing it correctly, and many people are not), will not prevent aerosolized particles from being inhaled because they are so tiny.  Even if you wear a face cover, stay away from sick people. 

Being in the proximity of a sick, coughing and/or sneezing person is the most likely way to catch this virus, because you will probably be exposed to high numbers of active virus particles, which means a better chance of getting some of them into your respiratory system.  It might also mean a more aggressive case of disease.  In medicine a general principle is that being exposed to higher numbers of infectious particles is usually a bad thing, and might explain why otherwise healthy medical personnel made up about 15% of cases in China (and a high number of cases in the U.S.).  It is common knowledge that droplets can travel much farther than 6 feet after a sneeze or a cough.  A very recent report demonstrated droplets up to 27 feet after a sneeze.  Click to see the video and read the report in JAMA.

The droplet range of 6 feet is meant for people who are not coughing or sneezing.  However, even breathing or speaking usually causes people to expire respiratory droplets.  And, louder talking requires more air, thus is likely to generate more droplets.  So… loud talkers, you know who you are (or at least everyone else does), it only makes sense to tell you to lower your voice for public safety.   And, go ahead and keep that in mind from now on. 

The most important consideration with wearing a cloth face cover in public is that you might not know you are infected, and still be able to spread the virus.   CDC authorities are reporting that asymptomatic carriers might make up about 25% of all infections. These are not people who qualify for testing under current federal or state guidelines.  These are not people who are even aware they have been infected.  Even among those who will become symptomatic, data shows they are shedding virus particles during the 48 hours prior to symptoms.  For these reasons wearing a cloth face cover in public can limit the spread of the virus. When wearing a face cover, put it on properly over your nose and mouth. Do not lower it to speak, and do not touch it a lot. Only touch your mask with clean hands. But please don’t forget, even if you wear a face cover, you should still stay at least 6 feet back from even seemingly well people.

Finally, there are many free patterns available on the internet if you want to make your own mask. One a friend gave me looks nice. You have to make sure there are enough layers and the right kind of material is used. It might surprise you to find out you may already have what you need. In 2006 the CDC posted a page about how to make an 8-layer thick cloth mask out of a Haines cotton T-shirt.

If you want to make a very strong DIY mask, a Maine physician posted this video about how to make a mask that contains a pocket, into which you can insert a HEPA filter cut from a vacuum bag.

COVID-19: update on the numbers and the rapid test

According to the Maine CDC, there have been as of today 344 cases of COVID-19 (up 41 cases since yesterday) and a case has been detected in Hancock County, bringing the number of counties affected to 13 (though we should assume there is virus in every county).  Community spread is present in York and Cumberland Counties, where cases are the highest. The number of healthcare workers infected remains at 43.  Since the onset of testing 63 people have been hospitalized, and 80 people have recovered.  Two more people have died from COVID-19, both women in their 80s who were hospitalized, bringing the total to 7 deaths.  A second positive case at the Oxford homeless shelter has been detected. Authorities have been working to identify potential spread at that shelter.

Testing

Maine CDC has supplies to test another 4000 people at present, and there have been 8400 negative tests so far.  As mentioned yesterday, there is a new FDA-approved (Emergency Use Authorization (EUA)) rapid test (taking less than 15 minutes) being manufactured by Abbott Laboratories (based in Illinois with production in Scarborough, ME).  This test will be used in the existing Abbott ID NOW device, a small piece of medical equipment about the size of a toaster.  The availability of a rapid test will be helpful in reducing the amount of personal protective equipment (PPE) used by medical personnel. Dr. Nirav Shah today noted that this week we received our third and final distribution of PPE from the Strategic National Stockpile.

To that end, Maine will be taking possession of 15 Abbott Laboratories ID Now devices, 100 test kits, each able to perform 24 tests, therefore 2400 tests.  Several labs around the state also have these machines, which were designed for rapid diagnosis of other diseases.  As noted yesterday, Abbott can produce 50,000 test kits daily, though as below, it sounds as though the majority will not be allocated for Maine. Miles White, the outgoing CEO of Abbott, told CNBC on March 30, 2020 that in addition to the rapid tests, more tests are coming. “There’s still more, and there’s a need for more.  We have a collection of technologies and formats, we’ve got multiple R&D teams at work.  There are more tests coming, there are serology tests, antibody tests, we’re looking at automated versions of that…all of those are a matter of weeks to a couple of months away.  Those teams are working around the clock…” They are also reportedly working on ramping up production to much higher numbers of tests daily.   “For a while we’ll be allocating and prioritizing to high need areas.”

Vital resources

More and more hospitals are reporting to Maine CDC, and the numbers of vital resources are going up:

  • 272 ICU beds, 124 available
  • 348 ventilators, 271 available
  • Alternative ventilators, approximately 128 available

Stay at home orders

Multiple governors around the country have instituted stay at home orders in the last few days. These orders are an effort to contain the outbreak in local communities, to prevent deaths, and to prevent an overwhelming surge of cases in hospitals and healthcare systems during this most severe pandemic the world has seen in the last 100 years.  I have read that some Mainers feel it is too soon to execute such an order. However, in medicine and epidemiology we know that this is precisely the time to act. And, we know that the numbers of seriously ill people with COVID-19 we are seeing today are people who contracted the disease an average of five days ago. Right now there are likely many more people who are infected but not yet showing signs of disease. There are also likely many asymptomatic carriers who are capable of spreading disease. Some experts have documented high levels of virus in the nasopharynx of people with no symptoms at all.

As an epidemic like this grows, it does so in an exponential fashion. For more on this please read my COVID-19 questions post. Looking at national trends on March 29, 2020 Dr. Anthony Fauci told CNN’s State of the Union audience that this pandemic could kill 100,000 to 200,000 Americans and infect millions. Many different authorities are making projections, and many are keeping track of the rapid spread of this pandemic. As it stands now Johns Hopkins notes that there are 190,740 cases in the U.S.  We are unfortunately the country with the highest number of reported cases worldwide.  Johns Hopkins further indicates a total number of positive tests worldwide of 887,067, with 44,264 deaths since the start of the pandemic.  Some cities such as New York and New Orleans are being particularly hard hit.  Stay at home orders and social distancing should be taken very seriously.   

Conclusion

Check the home page for links to other useful sites on the web. Know that people all over the world are working on this, and advances are happening daily. This is what science is for. And, caring for patients and constantly trying to improve how we do that is what doctors, nurses, and other healthcare workers are trained to do. We are not going to stop.

Please safely check on someone who might be isolated. Use social distancing, a telephone, texting, whatever works to say that you care. We need to stay connected in the ways that we can. Think about checking in on all of your loved ones. They might not be as strong as they seem. None of us has ever faced a worldwide crisis like this.  

Also, please keep up routines and sleep/wake schedule. Make sure you move your body in a healthy way daily with exercise, stretching, and other activities you enjoy.

Finally, I know many of these posts are about frightening topics. Try to also focus on something happy, meaningful, or beautiful to you. There is a reason we have poets, novelists, artists, and musicians. They center us, they bring us peace.  Know too that if you or someone you know is in a states of crisis, there is still help. Call your doctor’s office or call the Maine Crisis Hotline at 1-888-568-1112.

COVID-19, the numbers, an update, and a new mandate

Today the Maine CDC reports there are 303 confirmed cases of COVID-19 in fulltime residents of Maine. Since the start of the outbreak there have been 68 recoveries, 57 patients have been hospitalized, and 5 deaths. Among positive cases, 43 are healthcare workers.  There is a current backlog of 600 tests to run, all patients in the lowest risk category. High risk patient tests are prioritized (more below).  

Positive cases are present in 12 Maine counties with 169 cases in Cumberland County, and 59 cases in York County.  Cases are represented by every decade of life, though over 87% of cases are in people 40 and up.  

Non-residents who test positive in Maine are not listed in the total above, but are instead recorded in their home states. Those numbers (as far as I can tell), are not available on the Maine CDC website.  From an epidemiology standpoint it makes sense to count the number in the state where the disease was contracted.  If presumably a case was contracted in Massachusetts, but the person traveled to Maine and was tested here, MA needs to know not just the number of cases they have generated, but also the identities of cases.  Knowing the identity allows public health officials to track contacts and hopefully limit spread.

From the opposite view, it is not clear to me how many cases we are counting who are Mainers with COVID-19 in other states.  Many Maine residents are currently in Florida for example.  A week ago Governor Mills advised people in other states to stay put, as the risk of encountering the virus would be higher with travel than staying in place.

As far as the numbers go, the total count of infected people in one place still matters.  If an infected person is present in a state where they did not contract the virus, it can be important for the logistics of disease management to count those people, especially if the numbers of sick people become significant. Think of it this way: if you needed to feed a certain number people at a dinner it wouldn’t matter how they got there. All that would matter would be how many plates to set and how much food to cook.  Managing a disease within a population can be the same way.  You need to know how many cases there are so that you can plan on resources like hospital beds, ICU space, ventilators, supplies, and people to care for those patients.

There is another reason to know the total number.  If you have an epidemic of an infectious disease it grows to some maximum number (the peak) before numbers of new cases start to drop off.   Consider the total number of infected cases like a number that needs to fit into a math problem.  If you know that for every person who has disease the overall size of the epidemic will expand in a certain way, then it becomes necessary to count those people.  With relatively small numbers like we are seeing now, I am hopeful that the difference is negligible.   A lot of effort in Maine is being put into predicting the growth of the epidemic and what that will mean in terms of resource and response. 

Maine CDC

At his daily media brief today Dr. Nirav Shah of the Maine CDC noted that overnight two women in their 80s passed away in the hospital.   He noted that two new pediatric patients (who are not school-aged) have been diagnosed.  One person who spent some time at the Oxford Street shelter tested positive.  Health officials are responding. 

The Maine CDC is continuing with a plan to send some samples to an outside commercial laboratory (LabCorp) as a response to the backlog of tests.  There is also an order for a new piece of equipment which should arrive in 1-2 weeks.  Once that new equipment arrives it will take several days to calibrate and integrate into the workflow.  See below for a new test.  

In terms of the mechanics of testing, Dr. Shah noted that the way these numbers are collected is that overnight the commercial laboratories and the state lab combine results.  When this occurs the PCP is contacted for more information about the patients with a positive result, including phone numbers.  The patient is contacted by the Maine CDC and is asked about every contact they have had in the last two weeks.  There is community transmission in many parts of the state. 

“What we know about disease transmission, is that it occurs in places of congregation.” 

Nirav Shah, M.D., Director of the Maine CDC

However, cell phone companies have been reporting to what extent people are straying from their homes (much lower), and traffic patterns have changed to reflect this.  It appears social distancing is occurring, and it is having a curve-flattening effect on the rate of new cases.  To be certain, number of cases is growing, but there are many factors at play.  We are not done seeing a rise in the rate of cases, and we need to take it very seriously. There is of course a concern that if numbers continue to grow we will see a large rise in the numbers before the outbreak peaks in Maine.  We don’t want to overwhelm health systems in our state. This brings us to supplies.    

Yesterday Maine received a third distribution from the federal stockpile, including 60,000 N95 masks and other PPE.  Maine CDC authorities are to soon distribute, primarily to hospitals.   

Vital resources currently in Maine

  • 190 ICU beds, 90 available
  • 330 ventilators, 262 available
  • 89 alternative ventilators (primarily ventilators used in short term medical procedures such as surgeries that can be modified for use in COVID-19 patients)

If those numbers seem like a lot, they aren’t. The Maine CDC is asking hospitals to increase reporting, including available numbers of PPE, and other data.   “In any type of a situation, we have to know where were going, but in order to know where we are going we have to know where we are right now.”

A new COVID-19 test

In other news, Abbott labs has developed a new, FDA-approved rapid COVID-19 test which will give a positive results in 5 minutes, and a negative response in 13 minutes.  Production begins at the Scarborough facility tomorrow.  Abbott reports they will produce 50,000 tests daily, and will be working with the Trump administration in terms of distribution.

Stay Healthy at Home Mandate

Governor Janet Mills this afternoon announced “a series of substantial new mandates to protect public health and safety in the face of COVID-19, including a Stay Healthy at Home directive that requires people living in Maine to stay at home at all times unless for an essential job or an essential personal reason, such as obtaining food, medicine, health care, or other necessary purposes.” This new mandate will take effect 12:01 a.m. April 2, 2020 and will last until at least April 30, 2020 unless changed by the governor. 

In brief, essential businesses and operations that remain open will limit the number of customers in buildings at any one time, implement curb-side pickup, delivery options, and enforce U.S. CDC-recommended physical distancing requirements for their customers and employees in and around their facilities.  The mandate also prohibits the use of public transportation “unless for an essential reason or job that cannot be done from home and limiting the number of people traveling in private vehicles to persons within the immediate household unless transporting for essential activities.”  Classroom and other in-person instruction will not resume until at least May 1, 2020.  It is now mandated that when out of the home or at work at an essential business, “individuals shall maintain a minimum distance of six feet from other persons.”

Essential personal activities defined in the mandate “with relation to an individual, their family, household members, pets, or livestock” include obtaining necessary supplies for household consumption or use, medication or medical supplies, seeking medical or behavioral health, emergency services, providing care, traveling to and from an educational institution for purposes of receiving meals or instructional materials for distance learning, engaging in outdoor exercise activities, such as walking, hiking, running, or biking, “but, only in compliance with the social gathering restriction in Executive Order 14 and all applicable social distancing guidance published by the U.S. and Maine Centers for Disease Control and Prevention.”   For further details please read the mandate.

Conclusion

This is a virus, a deadly virus. For it to spread, it has to be spread from one person to the next. If we could all isolate for two weeks, it would be gone. Do your part, please stay home.

What to do if you think you have COVID-19, and your code status

If you have a fever, new cough, shortness of breath, sore throat, or muscle aches, call your doctor’s office.  Watery diarrhea is also seen in a minority of cases, and a new loss of sense of smell or taste has been reported frequently in mild and severe cases. 

Over 80% of cases of COVID-19 are mild, but in high risk groups (people over 60, and people with serious underlying chronic diseases such as COPD, asthma, cardiovascular disease, diabetes, kidney disease, or immunocompromised state), risk of serious illness or death is higher than the general public.  

Mild symptoms

Generally speaking, for MILD symptoms, we encourage patients to monitor their own health at home.   This is in part to avoid spreading the disease, and in part to avoid overwhelming the hospitals in our state.  People with mild symptoms are not usually going to qualify for a COVID-19 screening test at this time because there are not enough tests.  Hopefully that will change soon.  Whatever the case, it is still cold and flu season also, and most tests are coming back positive. Those who come in for a test are also running the risk of exposure to the virus that causes COVID-19.

People with mild symptoms should isolate at home for 14 days as long as the symptoms remain mild (and at least three days free of fever without medications).   One should call back for medical advice if the symptoms become severe, and call ahead before going to the doctor or the emergency room.  Health workers need to know if there is a chance you might have this highly infectious disease. 

If symptoms remain mild, stay home, isolate in one room, use one bathroom that is not shared, avoid contact with others, and do not share plates or eating utensils.  Have someone else bring you groceries and medications. If you must leave the house for any reason, avoid public transportation and wear a mask. Do all that you can to avoid spreading this disease. Wherever you are, cover your mouth and nose with a tissue or your sleeve (not your hands) when coughing or sneezing.  Throw all used tissues in the trash.  Wash your hands with soap and water immediately after coughing, sneezing, or blowing you nose.  Wash hands often with soap and water for at least 20 seconds to avoid spreading virus to others.   If soap and water are not readily available, use an alcohol-based hand sanitizer that contains 60% alcohol. Always was hands if hands are visibly dirty with soap and water.  Here is one resource to go over these issues: CDC

If you are sick, stay hydrated, and get good nutrition. Please let your doctor’s office know, and ask loved ones to check on you by phone if you do not live with someone.

For people with SEVERE symptoms:

Call ahead to your local ER that you are coming in, or inform EMS of your symptoms and that you might have COVID-19.  Wear a mask immediately upon entering ER or ER registration.  If you have called EMS put on a mask prior to seeing them.

If you haven’t put your code status in writing, it is time

There is no easy way to say this.  We should all know our code status.  For the uninitiated, code status refers to whether you would want heroic measures to be taken if your heart stopped, or if you had serious problems breathing.  In other words, would you want chest compressions, possible shocks to restart or normalize your heart (cardioversion), or a tube down your throat to help you breathe (intubation), and be put on a machine (a ventilator) that would breath for you?  Many of you have thought this through and have an advance directive, POLST form, or similar document.  That is good, as long as it covers code status clearly, and it is available to EMS or the ER. 

Under Maine law, advance directive refers to any spoken or written instructions you have given about the health care you want should you become too ill to decide.  An advance directive will let others know what treatments or interventions you want, and those you do not.  Having decided these issues well before you become ill is helpful to medical personnel, helpful to you, and helpful to your loved ones by sparing them the burden of making tough end of life decisions.

In Maine, anyone 18 years of age or older may use the Maine Health Care Advance Directive Form, (click here to download the .pdf). This form can be canceled or changed at any time.  This document does not take away your rights as a patient.   Once you state your wishes in writing, please let your loved ones know what you want.  The ER and the intensive care unit (ICU) are not good places for surprises.  The key thing to medical personnel is what you want. Your doctor and local hospital should have copies too.  Most people keep the document with their code status handy, perhaps on a refrigerator door, along with a list of medications.  

In our lifetimes there has never been such a threat in our communities.   The percentage of those who experience serious illness or die is much higher in people over age 60.  About 20% of people of all ages with COVID-19 have a severe case requiring hospitalization, with about half of them winding up in an ICU.  About half of those patients wind up on a ventilator (5-10% of COVID-19 cases overall).  We can break the risk apart by age to some degree.  On March 21, 2020 I noted here in MPDN that the state of Washington that day had 1793 confirmed cases with 95 deaths (today the numbers have climbed to 4,896 cases and 195 deaths).  Deaths up to March 21 occurred as follows by age and (percentage):  60-69 (10%), 70-79 (23%), 80 and up (60%).  If a person winds up in the ICU and on a ventilator risk of dying is high, much moreso in older people.

A research letter to JAMA March 19, 2020 noted that at Evergreen Hospital in Kirkland, Washington a case series of 21 people with COVID-19 was admitted to the ICU (average age, 70 years with a range of 43-92 years).  Mechanical ventilation was used in 15 patients (71%) and acute respiratory distress syndrome (ARDS) developed in all of those patients.  As of March 17, 2020, death had occurred in 67%, and 24% of patients remained critically ill. 

In a summary of 72,314 cases from the Chinese Center for Disease Control and Prevention published in JAMA, 14% of cases were defined as severe, and 5% of cases as critical.  The case fatality rate for all ages was 2.3%, but 14.8% for patients over 80, and 8% for those 70-79 years of age.   Of note 3.8% of all cases were healthcare personnel, and 14.8% of them were classified as severe or critical.  Healthcare workers risk exposure to high inoculation of virus particles, especially while performing intubation and other invasive procedures.  All patients in this group who were listed as critical died from COVID-19. 

A study published in The Lancet Respiratory Medicine reported that of 52 critically ill patients (average age 59) at a Wuhan, China hospital 30 (81%) of 37 patients requiring mechanical ventilation had died by 28 days. The age of those patients was not given.

The point is that older people, especially those with chronic illnesses, do not have good outcomes with this disease, especially if they become critically ill.  Please consider your code status carefully. 

COVID-19 questions

The following is a summary of questions I was asked by patients about COVID-19 this week. Please bear in mind that I am a neurologist.  My main concern in writing and posting this articles is to protect public health.  I hope this is helpful.  If you are not finding what you need here, try the Maine CDC COVID-19 FAQ page, or the CDC in Atlanta FAQ page.

Isn’t COVID-19 like the flu?

No, but it is easy to see why people might make the comparison.  COVID-19 and influenza are both respiratory illnesses that can be spread by droplets (coughs, sneezes, talking, dirty hands, dirty doorknobs, etc.).  However, COVID-19 is about twice as infectious as the current seasonal flu in the U.S.   

How do we know COVID-19 is more infectious than the flu?

One way to figure this out is the reproduction number, or R0 – pronounced “R-naught.”  This basically tells us the number of people to whom an infected person will likely spread the disease.  It is a way to describe how contagious a disease is. When R0 = 1 then and infected person will infect 1 other person. When the R0  = 10, then an infected person will infect 10 other people, and so on.  Therefore, a lower number is better than a higher number. As we will see, it is possible to lower the number. When the R0 is less than 1, the epidemic is likely to stop, because an infected person is unlikely to spread disease to anyone

The basic reproduction number of seasonal influenza is R0= 1.3.  Thus, each infected person spreads the flu to about one other person (1.3 people, but let’s round down to keep it simple).  That person spreads it to another person, and so on.  The spread of seasonal influenza is usually person to person.  So far, the data with COVID-19 is that the R0 = 2.2, close to twice as high: every person infected spreads it to two people, rounding down again, but it becomes person to people.   That is a real problem, because it means that if nothing stands in the way, the number of cases doubles when it spreads. Based on data from the Wuhan, China outbreak, that occurred about every week during the early stage of the epidemic. (1)  The spread might first go from 1 person, who spreads to 2 people, who spread to 2 each (4 more people), who spread to 8, and those 8 to another 16, to 32, to 64, to 128, to 256, to 512, to 1024, (keep doubling, and in ten more steps you have reached 1,048,576 new cases, and the aggregate  is about 1.5 million). However, there are even more factors that influence an “exponential growth curve.”

Wuhan, China is a city of 11 million people.  The city contains a large outdoor seafood market where the virus that causes COVID-19 is thought to have originated. There were several issues that fostered the growth of COVID-19.  It was the season of the Chinese Lunar New Year (the largest annual mass travel event worldwide).  There were huge crowds, people had come in from all over China, and all over the world.  In the City of Wuhan community spread first occurred in mid-December.   By mid-February, China reported over 77,000 cases.  However, people who traveled to Wuhan returned home, and flights left through the end of 2019 until at least the first week of March, many of them to the U.S., and bringing the virus with them. Remember that many people may not have had any symptoms during travel.  As of this am, Johns Hopkins reports there are 607,965 cases of COVID-19 affecting 177 countries.  There are 104,837 cases in the U.S. This is far worse, and far more rapid than influenza.  For a little more detail on R0, see footnote 1.

But doesn’t the flu kill more people?

This flu season from October 1, 2019 until March 14, 2020, the CDC in Atlanta estimated 38 – 54 million flu infections in the U.S., with 400,000 – 730,000 hospitalizations, and 23,000 – 62,000 deaths.  (footnote 2)  By comparison, in the U.S. there have so far been 1,711 deaths from COVID-19. (footnote 3)  But, consider the fact that influenza had a six week lead on COVID-19.  We don’t yet know how bad this will be.  And, the case fatality rate (the number of people who will die from an illness as percentage of all those who are infected) is only about 0.1% for seasonal influenza.  In Wuhan, China the case fatality rate was 2.5% (25 times higher than seasonal influenza in the U.S.), and in most parts of the U.S. the case fatality rate appears closer to 1% (10 times higher). In some outbreaks the case fatality has been as high as 5% (50 times higher).   The bottom line is that COVID-19 is much more lethal than seasonal flu. This is why we are trying so hard to avoid it.   

Even if COVID-19 is more deadly than the flu, please make no mistake, you should get a flu vaccine every year unless there is a clear medical reason not to get oneThe numbers I have given for the flu should shock you.  We lose an average of 30,000 Americans to the flu annually (some years much worse).  You can do your part to stop the spread of the flu by getting your vaccine every year.  As I mentioned in the last post, older people should also get a pneumonia vaccine.  Stop the spread of infectious disease.  

Will this be over before Easter?

I don’t think so.  But there is reason to be hopeful that we will get this under control before summer.  All viral illnesses seem to have a “wave” during which they pass through a community or population.  Cases go up, peak, then come down.  China claims to have leveled off the rate of new cases not long after mid-February, with a current 81,996 cases.  I hope that is true.  China also claims 75,099 recovered cases.  Remember also that about 80% of cases are mild.   In the U.S., which is several weeks behind China, we are still reporting growth in the number of cases and have seen nearly 900 recoveries (among those tested).  If the wave of illness lasts about 8-9 weeks, we might see a decline in the rate of cases soon on the west coast.   However, like everything else about this, it is complicated.  It also requires social distancing to flatten the curve.  As above, this disease is very contagious.  Stay home, and avoid sick people. 

Are there parts of the country, or maybe parts of our state that will be able to go back to business soon?

It is possible.  A reasonable strategy might be to identify and isolate all cases in a community if the number is low (such as Montana).  This would mean finding all contacts of sick people and isolating them, and not allowing new cases in. The problem is that we don’t have enough tests yet to do mass screening.  So, the strategy is still social isolation.  Stay home.  

When will we have more tests?

A lot of work is being done.  I understand that vendors are coming online as fast as possible.  The most common test, the rt-PCR, requires a special reagent which is in short supply around the country.  This test checks swabs from the nasopharynx for viral RNA.  As far as I know, home test kits I have seen advertised are not yet FDA approved.   There may soon be a blood test.

Do people develop resistance to COVID-19?

We think so.  We are not yet sure, and there is not enough data to be sure.  There is data that people who have recovered produce antibodies against the virus.  Opinions around the globe are that it is likely that people will be immune to COVID-19 if they have recovered from a prior infection.  The director of Allergy and Infectious Disease at the NIH, had this to say to Trevor Noah on March 27, 2020:

“I feel really confident that if this virus acts like every other virus that we know, once you get infected, get better, clear the virus, then you’ll have immunity that will protect you against reinfection.” 

Anthony Facui, MD

Is there a vaccine?

Not yet, but people all over the world are working on it. We need a vaccine.  For more about why we need a vaccine, see footnote 4. 

Are there treatments for COVID-19?

There are currently no proven treatments.  The strategy is largely supportive at this point.  Fortunately, in the general population, 80% of cases are mild about 15% are moderate, and about 5% are severe, requiring mechanical ventilation.  As discussed in prior posts, the numbers are not the same if we break it apart by age.  Above age 60 the frequency of severe illness increases steeply.

What about hydroxychloroquine?

There has been some data to suggest it might be helpful, by making it less likely for the virus to bind to targeted cells in the lungs and GI tract.   But there is no proof that this works, or that it is even safe to take during this illness.  A drug for any condition should be tested and thoroughly vetted before use.    Hydroxychloroquine is needed for rheumatologic diseases however, and it is unethical to take drugs out of supply that are needed by other people.  Those who are selfishly attempting to take hydroxychloroquine prophylactically are doing so at a great cost to people with chronic disease.

What about ACE inhibitors and ARBs?

There is an association between angiotensin-converting enzyme 2 (ACE2) and SARS-CoV-2, the virus that causes COVID-19. (2)  ACE2 is a co-receptor for viral entry for SARS-CoV-2 (is needed to help the virus get in the cell), and it might be related to pathogenesis (the evolution of disease) of COVID-19. ACE2 is seen in the human lung, GI tract, heart, and kidney.  ACE inhibitors, which are normally prescribed to lower high blood pressure, might directly inhibit ACE2. The problem is that ACE2 is not inhibited by clinically prescribed ACEIs. (3)  And, there is concern that the use of ACEIs and ARBs (angiotensin receptor blockers) might actually increase expression of ACE2, and that might increase susceptibility to the virus.  We know from many animal and human studies that these drugs increase expression of ACE2 in the heart and brain, and there is limited evidence that the same might happen in the lungs.  Still, as we try to find something that might help COVID-19, experts have called for both the use and the cessation of ACEIs, ARBs.  The data is not there yet. 

What about antivirals?

There is no data yet. Some of the antivirals used to treat HIV look interesting, but the same can be said about safety and appropriate use here. 

What about plasma or serum from recovered people?

Data is being collected.  This is a very promising area, and a very old idea. For about a century we have used the technique of collecting antibodies from recovered patients to treat the acutely ill.  I have also read about trails harvesting specialized white blood cells from recovered people. 

  

FOOTNOTE 1: R0 is not a fixed number.  Factors within a population such as whether vaccines are used (and to what degree), the status of nutrition in a population, whether people in a community have some resistance to this virus, or whether some other infections are going around all come to bear on how infectious something is.  That is why doctors, scientists, and epidemiologists discuss the effective R0.   It is also why we ask you to take vaccines (see footnote 4 below).

FOOTNOTE 2:  The reason for the lack of certainty is put by the CDC as follows: “Because influenza surveillance does not capture all cases of flu that occur in the U.S., CDC provides these estimated ranges to better reflect the larger burden of influenza. These estimates are calculated based on CDC’s weekly influenza surveillance data and are preliminary.” 

FOOTNOTE 3: Sadly, the first death due to this infection in Maine occurred this week.  Worldwide, there have been 28,125 deaths due to COVID-19 as of this am, 3/28/20. 

FOOTNOTE 4: Measles is one of the most contagious infectious diseases and can cause explosive outbreaks. It has an R0 of between 12-18 in the U.S.  The R0 can be higher in completely susceptible populations.  Consider 1875, when measles arrived in the Fiji Islands. According to Hans Zinsser, in his excellent 1934 book Rats, Lice, and History, the King of the Fijis and his son returned home from a visit to Sydney, Australia, apparently bringing the infection back with them, and killing 40,000 people from a population of 150,000 (28% of the population).  This population was naïve to measles.  If a disease has been present for a while in a population, some immunity develops.  Those people, who have survived an infection are either not susceptible or partially susceptible.  It becomes much more complicated when most adults, but no small children are immune.  And, there are many other factors such as hygiene, nutrition, and medical care that play a role.  Such was the case with measles in the U.S. prior to the introduction of a safe and effective vaccine in 1968.  Measles used to infect 3-4 million children in the U.S., resulting in up to 500 deaths annually. (4)  There have been multiple measles outbreaks associated with unvaccinated children in the U.S. since then, though in 2019 we saw a 25 year high of over 1200 cases in 31 states.  This was fueled by the anti-vaccine movement and those claiming religious exemption. Epidemiologists calculate that we need to vaccinate 95% of kindergarteners to reach a community protection threshold adequate to protect those who are not able to be vaccinated due to medical concerns.  It is the ethical duty of all parents whose children can be vaccinated to do so in order to protect not just their own children, but those who are more vulnerable, and cannot have the vaccine. 

REFERENCES

  1. Li, et al.  Early Transmission Dynamics in Wuhan, China, of Novel Coronavirus-Infected Pneumonia. NEJM 2020;382(13): 1199-1207.  
  2. Zhou,  et al.  A pneumonia outbreak associated with a new coronavirus of probable bat origin.  Nature. 2020;579(7798):270-273.
  3. Rice, et al.  Evaluation of angiotensin-converting enzyme (ACE), its homologue ACE2 and neprilysin in angiotensin peptide metabolism.  Biochem J. 2004;383(pt 1):45-51.
  4. Kohl, K, Gelline, B. Measles as Metaphor-What Resurgence Means for the Future of Immunization.  JAMA 2020;323(10):914-915