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Amy's Story

In early 2017 shortly after the birth of my fifth child, I began to have some short-term memory loss.

For example, my husband would ask me to make him a cup of coffee, then I would switch the kettle on, walk away, and forget that he had even asked me. Or my mum would show up at the house and I would say ''What are you doing here?" and she would say that we made arrangements the night before to see each other. I would miss birthdays and other events I was invited to - simply because I forgot. I would go upstairs to retrieve something for the kids, and then forget why I had gone upstairs. I would forget that I had added ingredients to a meal as I was cooking it, and immediately add them again. I would be talking to someone and forget mid-sentence what I was talking about. Or I would frequently hear people remind me that I had just already told them something - which was always a surprise to me as I could have sworn I hadn't told the story before.

Because I had five children under six years of age at the time, I just put it down to being tired and over-worked. But I do remember asking mum multiple times whether early dementia ran in the family.

At about this time, I was becoming increasingly paranoid. I had severe obsessive-compulsive tendencies (repeatedly checking locks, the stove, the oven, hand washing) with bouts of mania and hypervigilance. I was starting to pace around the house in the middle of the night. My thoughts were racing constantly. Reading became a problem for me as I could not settle my mind or focus on sentences, let alone even remember what the previous paragraph was about. I remember wondering whether I had bipolar disorder or some sort of adult attention deficit disorder... I was always on edge.

My mum would constantly exclaim that I needed to see a psychiatrist as she thought I was having a major personality change. I was becoming increasingly anxious, controlling, agitated, and aggressive, with severe mood swings and angry outbursts. My husband was constantly exasperated with my inability to multitask and organize things. He would often argue with me as I was becoming erratic and impulsive. I was unable to stick to decisions, and he would also accuse me of being inappropriate with people in my mannerisms and conversation. I am a mild-mannered and polite person, so this was not normal behaviour for me.

Again, I put it down to sleep deprivation (I was up breast-feeding most nights, and unable to go back to sleep) and the stress of having five children under six years of age. Also, my dad was battling stage 4 stomach-cancer at the time, so I was under enormous pressure.

Then the onslaught of physical symptoms started which made me put the cognitive issues on the back-burner.

First up, my vision had a change. There was a darkening, and I could see what looked like black patches and smoke rising up in my visual field. Sometimes I could see television static. Sometimes it was as though I was looking through a white smoky haze. I was terrified that I was going blind, but every test that I had in regards to my eyes e.g., vision testing, orbit CT scans, MRI scans, came back normal.

Then I started to get prickling, buzzing and burning in my arms, legs, hands, and feet. At times it felt like I was walking on broken glass or gravel. My hands would go numb. I had difficulty picking things up. I had various muscle weaknesses. My left leg and arm were becoming increasingly heavy and I would start dragging my leg. Sometimes my legs would collapse under me and I would fall.

And I was becoming nauseated. Every time I brushed my teeth I would gag. There was a point in time when I couldn't brush my teeth at all as I was unable to rotate my forearm and wrists. I was in agony in my wrists, arms, shoulders and I was also lacking coordination. Things like washing my hands became difficult and wiping down surfaces when I was cleaning the house. I had difficulty coordinating my arms to bathe and dress my kids.

I had episodes of not being able to walk up and down the stairs, and my husband had to carry me. Even turning in the bed became incredibly difficult. I just couldn't do it. I couldn't stand up from a sitting position unless I had assistance by someone to pull me up. I couldn't wash my hair unless I leaned my arms on the walls in the shower as my arms were easily fatigued. My husband would have to blow dry my hair for me as I couldn't do it. Writing became painful and difficult. Typing, using a computer mouse, and texting on my phone also became difficult. I had issues controlling the mouse cursor on the computer screen and I couldn't press TV remote control and EFTPOS buttons.

I would turn my head from side to side and feel like throwing up. My neck and the back of my head ached constantly. I had difficulty holding up my head. I would have to lie down a lot, or prop my head up with my hands with elbows resting on the table.

I had issues with my voice too. It would be tiring to talk and my voice would become tight and hoarse with repeated use.

I would often feel light-headed and sometimes would faint or fall when I would stand from a seated or lying position. I would lose balance easily.  If I squatted to tie the kids shoe laces or pick something up, I would tip over backwards. Sometimes I couldn't even stand up from a squatting position as my thighs lacked the strength to do so.

Just randomly, my heart would start racing (even at rest) and I'd feel very dizzy.

My muscles started twitching.

I would slightly drool from the corners of my mouth and swallowing was becoming difficult. My jaw and neck muscles would clench and stiffen. At that time, I was having mild difficulties saying certain phrases and words e.g., 'hour/shower', 'turn it off.' My mouth was struggling to make the shape to pronounce certain vowels and consonants, so I would avoid saying them as they felt so unnatural to pronounce. I often had episodes of difficulty chewing. I had to cut food into tiny pieces as I struggled to chew it and swallow it down. Sometimes having a cup of tea and a biscuit resulted in a coughing and spluttering mess.

Speaking of food, certain foods started to taste like chemical solvents. Coffee (which is something I enjoy greatly) became repulsive to me. Many times, I would think the food I was eating was expired, although everyone else thought it was fine.

My hands and ankles would swell.

I was becoming increasingly short of breath on exertion and rest, and would wake up at night breathless. I couldn't lay flat on my back at all. Many times, I was sitting up in the middle of the night leaning forward trying to regulate my breathing. Out of all my symptoms, this one caused me the most distress.

I could feel this perpetual internal tremor sensation - and it was more pronounced when I would lie in bed.

I had constant low buzzing in one of my ears.

I had (and still do) so many variable and fluctuating symptoms and this made it very difficult for me to get a diagnosis. By the time a long-awaited specialist's appointment rolled around, some of my symptoms would start tapering off with new ones starting. Eventually the old symptoms that had tapered off, would return again. I suppose having an impaired memory didn't help the situation either when it came to listing symptoms during consults.

I was a frequent flyer at my family doctor. I saw so many different doctors. I showed up at emergency departments multiple times. I saw several eye doctors, rheumatologists, neurologists, a gastroenterologist, and, finally, a neuroimmunologist who diagnosed me.

Diagnosis

After having multiple and extensive testing done - frustratingly, with the majority of them coming back normal (e.g., repeated various MRIs, CT scans, ultrasounds, bone scans, respiratory function testing, extensive blood tests, an EMG, other extensive neurological testing, and a lumbar puncture), I was diagnosed with antibody-negative autoimmune encephalitis. This is because I finally had findings on a repeated brain MRI, and my lumbar puncture results indicated that there was inflammation in my cerebrospinal fluid.

The worst thing about this illness is the lack of awareness of it. At the early stages of my diagnostic journey I was frequently told that I was a stressed, anxious, hypersensitive, over-worked mother of five young children with likely fibromyalgia, and psychosomatic symptoms 'fuelled by persistent Googling' and 'having a sick father'. One doctor suggested I take up meditation and yoga, and then gave me a referral to see a clinical psychologist for biofeedback therapy.

I was highly embarrassed coming home and telling my family and friends what the doctors had said. Everybody thought I was crazy. In the meantime, I was really struggling and desperate. Deep down I knew there was something really wrong, and I was beginning to lose hope. By then I was convinced I was slowly dying from a neurodegenerative disease. Imagine dealing with this stress and also managing a household of seven, with five kids under six years of age, and minimal family support. My husband was working full-time, mum was caring for my dad, and my kids could see me breaking down physically and emotionally. Between doctors appointments I was pretty much couch-bound. Many times, I would just lie on the floor with my kids crawling over me waiting for my husband to come home. I was so sick.

But towards the end of 2019 I was finally given a diagnosis and treatment was commenced. I was sent for a PET scan to check for tumours/cancer which thankfully came back normal. I had a central-line inserted, and I was administered several rounds of high dose steroids, and started on monthly cycles of plasmapheresis as an out-patient. I am on monthly intravenous immunoglobulin, and immune-suppressing tablets which I take daily. I've also had Rituximab infusions.

I still have certain residual physical problems e.g., left sided heaviness, difficulties with movements and issues with motor dyspraxia. There are some lingering cognitive issues too. But I am much better than I was before treatment started. Some days are better than others. Occasionally, I feel like I'm relapsing, but these episodes (since being on treatment for over a year) are becoming fewer and further between.

I didn't realise I would be needing continual treatment over a long period of time and this has taken some time to adjust to. The side effects of these treatments are unpleasant. A handful of times I have gone back to the hospital's emergency department seeking relief or reassurance because of the crappy side-effects. I also get frustrated at times because I'm not really sure what the plan is. It kind of feels like a waiting and watching game. And admittedly, when some symptoms do return, I kind of panic.

Despite all this, I am living a full and productive life.

Given the fact that this disease is a rare disease, I do understand why obtaining a diagnosis is difficult. More awareness and research of the disease is needed. But I'm thankful to God that He coordinated my steps and eventually led me to the right doctors that listened to me and wanted to help me. I'm thankful for the team (doctors, nurses, administrative staff) and their combined effort in looking after me. I'm especially thankful for the neuroimmunologist who diagnosed me.

A dear lady friend of mine (with the same illness) said this great quote that I reflect on frequently:

'Not every day is good, but there is good in everyday.'

And that has been absolutely true.

Each day presents itself with its own challenges and even though I don't know what the future holds, I am most calm when I focus on the good one day at a time.

Amy 31/01/2021