Posted on May 17, 2018
“I’m sorry, you have motor neuron disease…”
“…type: Primary Lateral Sclerosis.” My neurologist looked at me sympathetically and I just stared back stunned. I had heard of ALS but PLS didn’t even register as a thing that existed. After I composed myself I asked the question every person dreads asking, “So how much time do I have?”
“Maybe 10 years,” he said. “Many patients become ALS patients over time.”
I don’t remember much else about that visit. I had some blood work done and I made another appointment. My neurologist was kind and supportive, he offered to fill out any documents I needed, “because you shouldn’t be working right now. You should be proud that you worked this long. I’m actually surprised you did.” He gave me a note for work.
Mr. Tucker had always come to my medical appointments but this one he had to miss due to an important work meeting. I had ubered over from my job with the full intent of heading back to work right after. I had left my work cellphone and some food scattered around and had to jot off a quick email to my bosses and a colleague telling them I wouldn’t be coming back and asking them to clean up my desk. I called Mr. Tucker in a panic, “Come get me. I have bad news.”
In 2013 I started falling. Nothing too worrisome at first but over time my legs were constantly twitching and my balance got worse. My GP at the time was dismissive and it took two years of mentioning it over and over to her – and finally just weeping hysterically – before she ordered a peripheral nerve test (all clear) and then referred me to a rehab doctor. He checked my reflexes and said, “You definitely need an MRI…” When the results came in it seemed pretty clear to the neurologist that I had a spinal compression, I was booked in with neurosurgery in April 2016 for a decompression and a fusion. I also got myself a new GP.
In some ways my life is so incredibly lucky: I have a great husband, great kids, a great job, and a wonderful home. In all things medical however, I am a complete lemon. Four days before my neurosurgery date I slipped on a scarf and broke my ankle. I had orthopedic surgery the next morning followed by my neurosurgery three days later. I was a bundle of sexy complete with collar and cast and everything I did required assistance for a couple of months. It was a super low period of my life but I was determined to keep things as normal as possible: two weeks later I started working remotely and a month later I was off to Italy for our 10 year wedding anniversary. Medical science had done its part and it was just a matter of time before I healed and could get back to running, biking, dragon boat, and skiing. Right?
(SPOILER ALERT: WRONG)
By August we had realized that the ankle wasn’t healing correctly. By September I had a second orthopedic surgery. By December it was clear that it wasn’t taking and I had to be referred to a surgeon who specialized in reconstructive surgery. In July 2017 I had my final surgery and finally, FINALLY, the ankle seemed to be healing.
Of course during this time my muscles seemed weak and the healing I thought would happen after the neurosurgery just wasn’t coming as quickly. I dismissed it mostly because I had been off my feet for so long due to the ankle, and I was convinced as soon as that healed up I could get back into physio and start working on the neurological challenges. Besides, work was slammed and there were tons of changes happening there, we had just learned we had to move and were searching for houses in earnest, and all the stress was causing me to lose sleep and have terribly unhealthy habits. I was exhausted all the time so I just chalked everything up to stress and exhaustion.
I started physio for the ankle in the fall of last year but around that time I started to see new, stranger symptoms: my speech seemed slurred, and my arms were beginning to twitch and ache. “Just stress,” I thought. Still, I was concerned, I headed back to my neurosurgeon. He ordered an MRI and confirmed that my neck was fine. “It wouldn’t be your neck that was causing speech issues anyway, I think you should go back to see your neurologist.”
…and that’s where this story began.
Here is the thing about PLS: it’s super rare. Only about 50 people in Canada have it, and about 500 in the US. It’s also a diagnosis of exclusion, which means that you have to eliminate all the other options before landing on the conclusion that it is PLS. It’s also a diagnosis that takes time: before 5 years, it may be just upper motor neuron dominant ALS (and still could be, time is the determinant). I had an EMG to rule out ALS (for now) and remain hopeful that I may be a statistical outlier given my slow progression.
So what does this mean to my life now? I head to the ALS clinic every six months so they can monitor my progression and offer support with around six different specialists, as needed. They tell me that since the inception of the clinic eight people have had PLS: one has seen no progression in sixteen years, two have become ALS patients, and the rest have had varying degrees of degeneration. I’ve also joined up with online groups for people like me, and have met the one local woman who hasn’t seen changes in years, her advice was to continue with movement, stretching, and physio as much as possible. So there is hope!
If you google PLS it will tell you that people have normal lifespans but what they don’t tell you is that quality of life can vary exponentially. There are people who have many interventions daily, and some who have very little. There is such a range of abilities within the same diagnosis even over time that it is hard to determine what the trajectory will be for me. All I know is that seeing you brain on a screen with dark squiggly lines where your motor neurons should be was one of the most difficult moments of this entire process.
I will end by saying though that the nurse at the ALS clinic told me that, “only nice people get ALS,” which is my saving grace because it means I will never get it. So I remain hopeful that I will become one of these outliers and continue to live my life as best as I can for as long as I can.
Tomorrow I will have some words on the financial implications of this diagnosis – or any disability diagnosis, really. If you aren’t prepared, you should stat thinking about becoming prepared. I wish I had been more prepared.