Marks Story, Age 62, U.S.A.
My wife Sherry loves Ocean City, NJ more than anyone I know. It might be for Mallon’s many different types of sticky buns or an awesome slice of pizza from Mankos & Mankos. Her beach- bag packing skills are legend. We try to visit there every summer and make day trips anytime of the year just to “restore our souls” and to get a slice of Pizza. When the caller ID showed that my neurologist of some twenty years was calling me at nine o’clock at night in the middle of August 2015, I suspected that our summer vacation was about to be interrupted in a significant way.
Earlier that month, at my regularly scheduled appointment at the MDA clinic, I mentioned that my brain was “not right”. My neurologist ordered a bunch of tests including a blood test for some illness I never heard of–something about antibodies.? We “needed to return home” from our vacation because “I scheduled a lumbar puncture for you tomorrow at noon”. How thoughtful of her! We were planning on going deep sea fishing the next day at six in the morning! That blood test had come back “positive” for some kind of encephalitis, and it was “necessary to do a spinal tap to see if my spinal fluid tested positive for the same antibody”.
My biggest concern when I hung up the phone was how disappointed Sherry would be about leaving early. Instead, what I encountered, was not disappointment; it was fear, with a capital F. Yes, I had been having some mild visual hallucinations and yes, my short term memory was absent, as if there was a loose wire that was keeping me from being able to make connections that even a youngster would be able to make. My mind was definitely not “running on all cylinders” but seeing my wife fearful was new to me…and very sobering. You see, Sherry is a critical care nurse of over thirty-five years. As they say in the military, “she eats interns and residents for breakfast”. One of the intensive care physicians once told me, “we call her the ‘Alpha Nurse’ ,when we see that she is working we breathe a bit easier.” Sherry is very smart, kind of like a walking medical dictionary smart. She knows what critical thinking is, especially as it relates to a critically ill patient, she has the ability to “see where the patient will be” two hours from now if they are given, or not given this or that drug or fluid. She is also “down to earth” and is a very vocal patient advocate.
Finally, my wife is the most caring nurse I have ever met. Her sense of caring is genuine and comes from a place deep within her. It is a trait that I have had the good fortune to watch “mature” over the years of knowing her. She is known to cry and or pray for, and with, her patients when it’s appropriate. Once she was found singing with a patient who asked her if she knew the words to Amazing Grace. Most days before she leaves for work, I remind her to “be a light”, she knows well what that means. So to see this incredibly strong woman express fear about some vague health issue was unsettling to me. Was this about to get serious, these antibodies?…heck if I knew. We packed hastily and then quietly made the two and a half-hour drive home as instructed.
“Mark, I want you to know that we are using fluoroscopy to do this procedure so please don’t worry”. “First I’m going to numb the area and then all you will feel is some pressure when I do the tap-OK?” I thought who am I to argue wearing nothing but the proverbial hospital gown. The guy sure seemed nice- I later learned that the PA was nice, and good at what he did, and of course, like most of the hospital staff…. he knew Sherry. It took a few weeks to finally get the news that the cerebral spinal fluid also contained these antibodies. My neurologist, the best neurologist in the world, informed me of the significance of these findings but I swear it just didn’t sink in the first time. I had to do what?.get what medicines… why?
I went to the internet and typed in antiNMDAreceptorencephalitis –I didn’t particularly like what I found. After some additional researching everything from medical literature to my insurance coverage we finally, and reluctantly, get me checked into the large hospital that we are expected to use if we expect our health insurance to cover the expenses. ” and what brings you here”? the admitting physician asks. I have always wanted to answer “our car” to that question but I figure this was no time for levity. “I have anti NMDA receptor encephalitis”. Well one would have thought I had just uttered the worst stream of obscenities ever heard based on the physicians’ response. It was hard for me to tell if he was shocked or angry. “You have WHAT?” …and …. “How do YOU know this?” Somehow, probably because of my wife’s diplomatic entreaties, he agreed to admit me and consult the Neurology group to see what in the world they should do with this patient who claims to be having hallucinations while also being able to pronounce big words that should be reserved for the sane.
“Where did you say your neurologist works?” The question came from one of the four neurologists standing at the foot of my bed all looking rather stern. I knew only one attending physician was the norm for a consult– so I sat up straight and tried to look my best! I knew they were the neurologists because one or two of them had occasionally done EEG’s and EMG’s on me over the past twenty years because of the muscular dystrophy that I have had since age 16. Within a minute of pressured introductions, the sternest of the group almost yelled—“Did you CONSULT with the University of Blank Hospital?……my friend is a physician there! They are the leading experts with this disease!” I apologized for not doing so, while attempting to re-direct their attention back to the serum and CSF results that tested positive for the illness that I thought we were discussing. Instead, I quickly learned we were actually dealing with a different illness; one that I had learned about in Graduate school: pure unadulterated Narcissism. I was discharged two days later after the mandatory X-ray and MRI of my brain were completed. “Like we told you, your husband couldn’t have anti-NMDA receptor encephalitis and be able to have the clarity of mind to tell us”! One physician in the group told Sherry that “If your husband has ANMDARE I will personally apologize to him”. Still waiting for that one,but I’m not holding my breath. Somewhere around this time, while searching the Internet, I found The Anti-NMDA Receptor Encephalitis Foundation,INC. in Ontario, Canada. There was a “Contact US” button on their website, so I tapped it and sent off an email that basically said, I have this illness and I’m not sure what to do next. I received a rapid response from some very nice person in Ottawa, Ontario who was actually willing and very able to give me expert guidance about what to do next. She had an impressive wealth of knowledge about ANMDARE. There ended up being at least six back and forth communications that helped me to know what the next step “Should probably be”. I will always be in Nesrin Shaheen’s debt because I know I would not have pushed as hard for rapid treatment had I not sent and received emails from her. After a few more very frustrating attempts to obtain help at our local hospital, I finally agreed to be admitted to Hahnneman University Hospital where my neurologist is on staff. She is the best physician I have ever known, and I have known more than my fair share.
Finally, I was going to receive treatment for ANMDARE from people who knew what they were doing and how to do it. I would simultaneously be receiving a “work up” for any tumors that I might have since they are the primary cause of the disease. Comforting thought: I have this illness with a very long name and now you tell me there’s a good chance I have a tumor somewhere? Seriously God, “What did I do to piss you off this time”? Just kidding. This admission was a bold/desperate step since we had every belief that our insurance would not cover it. “But what’s the good of our retirement money if you are dead”? Sherry could always get right to the point.
I remember very little from my twenty- one day stay at the University Hospital, probably because I was quite psychotic most of the time. Psychiatric symptoms are usually the heralding sign of this illness. Sadly, many folks are thought to be “crazy” and are confined to psychiatric wards where they remain untreated for the neurological illness that is wreaking havoc with their brains and causing the psychiatric symptoms. Their family members are usually told that their loved one has Schizophrenia. How tragic for patient and family alike.
I do remember the day that I came close to pulling off my “escape” out onto the chilly streets of center city Philadelphia! This was necessary because I was quite certain the staff was preparing my body in order to harvest my organs to sell on the black market! Even though I was experiencing full blown paranoia, I was sane enough to question their wisdom since, as far as I could tell, my sixty-two year-old organs weren’t really in that great shape! I also remember calling my wife and oldest daughter to warn them that the phones were tapped and that I thought text messages were probably compromised as well. …I’m rather sure they both really appreciated those calls! I abandoned my escape plans for a later date when I realized I had no shoes no cloths no money and no charge on my cell phone.
No tumors were found, Yeah! I continued treatment with intravenous infusions of 50 Grams of IVIg and 1 Gram of Solu-Medrol daily. I will never forget the headache that I had even prior to any of the hospital stays. It felt as if my brain was hot; like molten lava had somehow been poured into the top of my skull and it oozed down my spine into my butt and down both legs. I remember asking the nurses for a large bucket of ice water and a snorkel. The nurses thought I was kidding -I wasn’t. I was able to get ice packs and Ace bandages with each pain shot. That was my routine every 3-4 hours for days. I tried to fall asleep early each night so that I was awake when Dr. T made rounds at 0500 +or- 30 minutes. She always asked me how I was doing and always seemed to be able to tell one of her residents that we needed to check this, that or the other thing because “his blood work showed some sign of something”.
I was very impressed with the attitude of all of the hospital staff: It was basically “We care about you and are here to help you as much as we can.” Every nurse was superb, even with me being psychotic and I’m sure a bit of a pain in the butt on more than one occasion. I was very fortunate to be discharged after only twenty-one days. I had to be re-admitted three more times, once in February and again in March and April 2016, in order to receive daily IVIg and Solu-Medrol to treat severe headaches and auditory hallucinations caused by the encephalitis. The hallucinations had “progressed” from just visual stuff to auditory command hallucinations: the most dangerous psychotic condition that I can think of. They seemed so real and their message was nothing short of evil; “Hurt yourself or we will hurt Sherry”! Those voices compelled me to leave my home immediately, (the March admission) and drive myself to the hospital. When I got there I was completely mute for the first 48 hours or so. I had some hospital employee sitting in my room twenty-four hours a day because the staff was concerned about me hurting myself. My physician somehow had the IVIg and Solu-Medrol running in my veins within an hour of me walking through the doors of that hospitals’ emergency room. In my case, the infusions of steroids and IVIg seem to work like magic in making the symptoms lessen significantly.
Currently I receive weekly home infusions of 50 grams of IVIg and 500mg. of Solu-Medrol. I have to take an antipsychotic medication to control hallucinations and mild, intermittent paranoia. Most days I have a burning hot pain in my neck that runs down my spine, and it does not collect $200 as it ends at the bottom of both legs! I experience huge variations in my blood pressure and have fallen several times because of it. The last time I broke a few ribs On my left side. This usually happens right before my infusions. After the infusions I feel steady and think clearly, I call those my “action days”! I was guided to a support group on Facebook that has been very helpful because the people in the group have all been through, or are going through the same thing. On the not so good days I have zero energy and I don’t feel safe to drive a vehicle or even use a kitchen utensil. I wrote a note on my calendar on the date of June 1,2016: It simply says-CURED, because I have a lot of summer plans. I figure you always need to have a goal if you expect to keep moving forward.