Suddenly, I saw flashing lights in the sky.
It was a beautiful summer’s day on July 5, 2017. The temperature was perfect and there was not a cloud in the sky. My adorable young nephews, charming brother-in-law Joseph, and his lovely wife had all left the day before. My husband Peter had left to fly back to Houston, Texas where we live, and my eldest son Jack was at work nearby in the General Store in the small town of Dorset, Ontario. My two younger sons Andrew and Luke had just made smoothies and we had walked down the steps to sit on the lower deck and contemplate an imminent boat ride. What a perfect day at the cottage! As I settled into a Muskoka chair and we discussed the fun we’d had the last few days, I looked out over the sparkling lake and suddenly saw flashing lights in the sky and a strange pulsating movement. “I don’t feel very well”, I remarked to my boys. The next thing I remember is waking up lying on the deck. I could see the legs of paramedics and firemen around me and hear my friend Karen’s voice calmly repeating, “Sweetie, it’s ok, you’ve had a seizure”. This was my first grand mal seizure.
My boys will tell you that, after what I now know was an aura, I shook very violently, holding my arms stiff and straight out in front of me, my eyes slightly rolled back and staring vacantly at the sky, I slid out of the Muskoka chair to the deck (no easy feat if you’ve ever been in one of these deep, sloped chairs!). Andrew (15) had the presence of mind to cradle my head as I fell to the deck, then shouted to Luke (11) to call for help. Luke ran the 50 feet up the cottage and grabbed the cordless phone and called 911. He ran back down to the deck as the call went through and they both gave the dispatcher a description of what happened and our address. Then these smart boys called Karen, a school teacher and my summer bestie, who has a cottage at the other end of the lake. She immediately jumped in a neighbor’s boat and came speeding up the lake to help. It took 20 minutes for the emergency services to arrive and I was unconscious that entire time. When I regained consciousness I had no idea what had happened. I was transported to the small, local hospital in Huntsville, Ontario where I was given fluids, told that I was probably just dehydrated and sent home. It would take the next seven months of becoming progressively more ill, to finally receive an accurate diagnosis of LGI1 Autoimmune Encephalitis.
Looking back from where I am now on this journey, I know that I started having symptoms back in September of 2016. I started experiencing numbness and tingling in my forehead that spread under my eyes, as if I was wearing a mask. The neurologist at the time prescribed medicine for migraines and told me to take it when I felt a migraine coming on. I never took it as I never had a migraine. In late May 2017, I also started having numbness and tingling down my left side, affecting my left arm and left leg. I would have moments where I would be walking or talking or eating and I had to pause to let a strange feeling kind of wash over me. As a family, we started calling these moments “episodes” and I would just pause what I was doing for a few seconds until it passed. I’m a public health nurse, and I wouldn’t know until months later that these “episodes” were actually Partial Focal Seizures. That would be discovered, when, shortly after the Grand Mal Seizure and the diagnosis of dehydration in July 2017, my husband Peter knew in his heart this wasn’t right and flew me back to Houston from Canada for further investigation. A 24 hour ambulatory EEG showed that I was having over 40 partial focal seizures a day. I was diagnosed with Epilepsy and was started on the seizure medication named Vimpat. Fycompa was added a few months later.
My seizures decreased to about 8-10 a day but still, they persisted. Some days I would sleep for 20 hours a day. I hadn’t been able to drive since the Grand Mal seizure and a new school year was starting for my boys. Jack (17) had just received his driver’s license and was suddenly the main driver for me and his brothers to school, activities and appointments. Thankfully they were old enough to help with dinner, laundry, and chores and getting themselves to school and their homework finished. A myriad of friends jumped in to help drive me to appointments and brought meals and groceries when my husband was travelling for work and I was unable.
In September an MRI suggested Limbic Encephalitis and a Lumbar Puncture was done with samples sent to the Mayo Clinic for testing. My neurologist started to treat based on my symptoms and I received four days of IVIG in October 2017. My last known seizure was October 25th, 2017. Hurray! We were finally onto something and finally some relief from the constant assault on my brain. Unfortunately, it wasn’t to last. Leading up to a scheduled booster IVIG infusion in November, symptoms of numbness, tingling, weakness and fatigue were returning. My teeth ached and the tip of my tongue was all pins and needles. The IVIG in November gave me no relief.
The results eventually came back from the Mayo clinic, but unfortunately, the doctor did not collect enough CSF (cerebrospinal fluid) for the Mayo Clinic to run the full antibody panel, so the test was inconclusive. My neurologist ordered a blood test to check antibodies and on December 6(the day after my 46th birthday), I was told that I had tested positive for Voltage Gated Potassium Channel Antibodies (VGKC). We were devastated by the diagnosis but also relieved to finally have one. Over the next few days, I experienced an even more rapid decline. I experienced gait changes and was now limping and dragging my left foot, I had trouble choosing the right word when talking or texting (aphasia), my fatigue had come back with a vengeance, and my short term memory was abysmal and driving my children and husband around the bend as they had to repeat and remind me over and over of things.
I was admitted to Houston Methodist Hospital on December 11, 2017. I was admitted through the ER and will never forget the young, bearded, strawberry blond ER Doctor who, after reading my history and diagnosis, came into my curtained cubicle, plopped down in the chair and said “WOW”. Not very reassuring and only mildly terrifying. My husband Peter arrived to the ER late that evening. I had said goodbye to him early in the morning as he took a short business trip. He flew back urgently when he learned that I was to be admitted. In the ER just before midnight, I asked him to help me up to the toilet. He was shocked to see my physical deterioration as I leaned heavily on him for support, no longer able to walk unassisted. That night I began a five day course of IV steroids and was admitted to the Neurology unit on the 10th Floor. December 11th marks the beginning of one of the most challenging years of my life.
Early on the third day, Physical Therapy came and performed and short assessment. They recommended that I start using a walker. I tried to convince them that I didn’t need one, that I wouldn’t be sick for long. They insisted I be issued with one so that I was less of a fall risk. I depended on that walker for the next three months. The disease had affected not only my memory, but also my motor function. That week I endured multiple needle sticks as my veins kept failing due to the assault of the steroid infusions on my veins and my IV had to be reinserted a number of times. I was sent for another Lumbar Puncture, this time with sufficient fluid drawn to be sent to the Mayo clinic for antibody testing. As I lay in the bed, multiple doctors, residents and medical students visited me on rounds, most of them unable to hide their shocked, worried expressions.
I was discharged on December 17, 2017. My mum had flown in from Toronto, Canada to help look after me. It was my son Luke’s first ever Houston Youth Symphony Concert and I was determined not to miss him playing the violin. Clothes and a hat were brought to the hospital for me and the nurses gave me a bag full of surgical masks that I would wear in public to help prevent infection due to my weak and immunosuppressed state. Discharge went smoothly, the nurses all knew how important this was to me and I was driven by Peter, with my mum Lynda and my boys to the concert. We planned to arrive just as it was starting (friends had taken Luke early for dress rehearsal) and sit at the back, hoping to not be noticed but still able to hear the beautiful music these children had prepared over the last four months. It was possibly the most difficult walk I have ever made. The ramp into the Middle School where the concert was held was so steep and so long to me. The walk to the auditorium seemed interminable, even though I was able to see if from the entrance. The concert was outstanding and enjoyed it with tears rolling down my cheeks, proud of my young son and grateful to be alive. That night, finally back at home, Peter and the kids set off on vacation to spend Christmas with family in British Columbia – it had been planned for months. This is the way I wanted it as I did not want my kids robbed of the magic of Christmas. I knew that Auntie Laurie and their cousins would be good for their souls and they would be showered with love and reassurance that their mum would be ok.
My mum Lynda, a retired nurse, got to work getting me settled at home and getting my house adapted to allow me to move around safely with a walker. In the two weeks that she looked after me, she had to help shower me as I was too weak to stand in the shower or get in the bath. She shopped, cooked, cleaned, drove me to appointments and cared for me 24/7. I’ll never forget her love and care or her worried expression. Living in Toronto, she’d had no idea how sick I had become. On December 26 I had my first assessment at TIRR, the Texas Institute of Rehabilitation and Research, whose mission it is to “improve the lives of people who have sustained central nervous system damage through injury or disease”. Jeffrey conducted my new patient assessment that day with patience and incredible kindness. I was an emotional wreck as it was finally sinking in that it was going to take a lot of work to regain what I had lost. That day I burst into tears when I was even unable to walk with the walker for 2 minutes.
Thanks to the remarkable staff at TIRR, I would spend the next 6 months attending five hours of therapy a week, gradually reducing to two hours a week by May 2018. The therapy was very physically challenging and I spent many hours with Michelle doing physical therapy on land, Charlotte in the pool, Amy for Speech Therapy and Imelda for Occupational Therapy. I will be forever grateful to my friends in Houston that quickly organized a driving sign-up sheet so that I never had to worry about how I would get to and from my many therapy sessions. It was often the highlight of my day to catch-up with friends who were driving me. Friends also stepped in and began delivering lovely home cooked meals to our doorstep three or four times a week for five months.
I began seeing a skilled Naturopath once a week who I still see and continues to perform acupuncture, make chiropractic adjustments and suggest nutritional supplements. With his guidance, I adopted a modified Autoimmune Protocol Anti-inflammatory diet, which I continue to follow and may never change. I began practicing daily meditations. Peter and I began contemplating getting a second opinion. Everything we were learning about AE indicated that early and aggressive treatment led to the best outcomes. Were we doing enough only with the hours of therapies each week? We were very fortunate to have my brother in law Joseph, a practicing neurologist and my father in law, a retired neurologist on our team. They have both been excellent counselors, educators and a source of great encouragement through all of this chaos. The problem was they both live in North Carolina and we live in Texas.
Thankfully, Joseph was able to get us an appointment to see the highly regarded Dr. Christopher Eckstein at Duke University Medical Center in the next few weeks. Friends stepped in to look after our 3 children and my husband and I made the trek to North Carolina. One of the only pictures I have of this devastating period of my life is the photo of me and my 87 year old father in law “racing” our walkers down the hall on that trip.
We knew we had made the right decision as soon as Dr. Eckstein completed the most thorough neurological assessment I have had to date. He and his team spent hours with us, taking an extremely detailed history. We also saw Dr. Saurabh Sinha who assessed my response to the seizure medications in an effort to get a full picture of what was going on. Labs were drawn and sent for antibody screening and a few weeks later Dr. Shah, Dr. Eckstein’s Fellow called me to tell me that I had tested positive for LGI1 Antibodies. LGI1 Autoimmune Encephalitis is known to affect 0.83 in a million people. I have never been so relieved and terrified in the same moment. Relieved to finally have a diagnosis but terrified as to what it all meant. Dr. Shah patiently listened to me sobbing until I collected myself and then explained what she and Dr. Eckstein recommended next.
I was to begin infusions of Rituximab (Rituxan) every 6 months. Back in Houston, after consulting with Dr. Eckstein, my neurologist concurred with this new diagnosis and agreed to implement the recommendations. So far I have had two infusions of Rituximab in the outpatient ward and aside from causing some moderate headache for a day or two and extreme fatigue that lasts about two weeks, I have suffered no serious side effects. As I understand, the Rituximab works to destroy the LGi1 antibodies that are attacking my brain. Repeat Infusions are needed (every six months in my case) to teach my body to stop making these antibodies. Hopefully, one day I will no longer need these costly infusions but current research indicates that the relapse rate is high and we just don’t know how long I’ll need treatment. Some people ask about the long term side effects of the treatment. I’ve never asked the question because, to be honest, the alternative of not treating this disease is pretty grim. We are just grateful that I have a diagnosis and treatment plan, good health insurance, and a local neurologist who, so far, has agreed to implement Dr. Eckstein’s recommendations.
It is now January 2019 and I am proud of the huge gains I have made over the last year. I no longer use a walker or a cane and recently started walking about 2 miles about 5 times a week. I continue to go to TIRR for Physical Therapy twice a week to work on regaining the strength that I have lost. I now weigh 106 Lbs. when I used to weight about 125 Lbs. before becoming ill. I know I’ve lost weight through elimination of sugar, gluten, red meat etc. from my diet but I’ve also lost muscle mass. My legs were unrecognizable to me in December 2017. I continue to see my beloved Naturopath, Dr. Joe Lindley. I rely on my friends, children (now two of them drive) and my husband to do most of the driving, although I do drive in a bubble between my home, WholeFoods and my children’s school (about 7 miles end to end).
This stage continues to be challenging, but in different ways than 2018. Now my disease is largely invisible, except to my family and closest friends. I suffer from Brain Fatigue and have to be very careful to not over commit or over extend myself daily. I have become good about saying “that doesn’t work for me” when managing my commitments. I get headaches more frequently than I ever did and I still battle fatigue. When I am physically tired, Brain Fatigue affects me by causing aphasia and short term memory issues as well as gait changes where I begin to limp and drag my left leg again. I tire very easily driving and even being a passenger in a car can be exhausting for my brain. Being in a room where multiple conversations are occurring is very challenging for me as it is difficult to filter out the extraneous noise and focus my attention. But most of these effects aren’t outwardly visible and is what makes it challenging.
Still, I will be forever grateful for my ultimate diagnosis and recovery to date. I will be forever grateful that my children called 911 and my husband and family never gave up hope and advocated tirelessly on my behalf for diagnosis, treatment and aggressive rehabilitation. And lastly, I will be eternally grateful that I never, even in my darkest days, gave up hope for recovery. I somehow always believed that this wasn’t going to be the end of my story.