I am a Zebra and then Rare Disease Community is My Herd

The story of Lori

My life changed in an instant.

32 years old, mom to 5 kids aged 2-13, business owner, married mom. Our newspaper business had just gotten off the ground, after some 3 years of hard work . We had finally opened our own office, hired staff. My husband had gone back to school at 38. We were a very busy family.

November 30, 1999 changed my life forever. Coming home after a late night, my babysitter asked me to run to the store quickly for some more milk for my little one before she headed out. Gladly, I jumped into the car and drove the quick 5 minutes into town.

It’s amazing how quickly life changes.

Ten minutes later, milk in hand, I jumped into the car and headed home. Just a quick drive. Airbags were not common at that time. At 100 km/hr, I met a buck deer, head on.

At first I thought I was alright. Luckily my inlaws Volvo, which I was driving at the time, was much larger than my own. Had I been in my own little car, I likely would have been dead. Instead, the front end of the car ruined, I drove myself home thinking I had gotten away lucky.

The next morning it began. Horrible headaches, unable to move my arms, turn my head. “Whiplash” I was told, by way of a single xray. Go to PT, do your exercises, wait it out, it would heal. Only it didn’t.

The migraines and shoulder pain were horrible. I couldn’t sit at my computer the 48-72 hours straight as I had before when on deadline for the newspaper. Heck, I couldn’t even handle 3 hours. We changed the chairs, adapted the workstation, went for massage, did everything they suggested. In the end, the newspaper had to be sold and I went back to my first career in medical diagnostics. At least there I could stand up during the day, something I still could handle. 

Fast forward 3 years. Nothing has changed. I cannot tolerate sitting up at all, pain centers don’t understand why the pain seems now to be spreading. Eventually they decided to call it post traumatic FM.

But the headaches were blinding. I could no longer work. Despite the worsening and the beginning of the neurological symptoms, no one looked further once I had the FM diagnosis.

Because they felt the headaches were too unusual for FM, in the 6th year after my accident, they decided to do a lumbar puncture (LP). That was the straw that broke the camel’s back. The next day, I was numb and weak in one leg, had saddle weakness and incontinence. The perfect storm had just arrived but I still wasn’t aware it was on the horizen. It would take another 3 years until we understood what was going on, but that is the day that sealed my fate.

Things only got worse. The pain was becoming unbearable, and over time I could no longer tolerate sitting, standing or walking. Gravity was my enemy. My left leg was weak, first requiring a cane, then later a walker. My balance was terribly off, I couldn’t tell where my limbs were in proportion to the rest of my body. The pain was unreal, a mixture of hot lava, itching, lightening strikes, spasms, and deep pulling pain. The diagnosis remained FM, but they added Psoriatic arthritis to the mix to explain the pain in my tendons seemingly with little use.

In all that time, not one person sent me for anything more than that first intial xray. I had consulted doctors in Ottawa, Toronto, Montreal and London, yet once that FM diagnosis was made, no one looked any further. To explain the unusual neurological signs not normally associated with FM, they said I had a rare, progressive form, even though, as a career medical researcher by that time (having moved over the years from medical diagnostics to medical research).I knew the literature described nothing of the sort.

By this time I was homebound and bedridden. The pain was just too much with any sort of gravity. I had become extremely photosensitive, unable to process overstimulation, incontinent, unable to walk without a walker for balance. No one knew what was going on, and doctors were starting to treat me like I was some sort of malingerer. I would later find out this long odyssey for proper diagnosis and treatment by medical doctors is typical for those of us with Rare Disease.

Finally, one day I woke up paralyzed on my left lower body completely. My muscles were in tight spasms, unable to release. I couldn’t release my bladder nor bowel, and my saddle numbness had become incredibly worse. The only answer was an ambulance. 

We were away from home when it happened, staying overnight at my inlaws a few hours away from home. Luckily for me, I was out of my usual network so no one “knew” my dx was FM, or at least, they were willing to finally look further. Some 10 years after the accident and 3 years from that fateful LP, I finally had the MRI I should have had all along. What a story it told!

Adhesive Arachnoiditis was amongst the list of problems I was now dealing with. Apparently, I had indeed cracked a vertebrae in the original crash, and it had been compressing my spine for years. Because it had gone unrepaired, it had built up a large bony structure that too was impressing the thecal sac. The LP they did most have caused a rare reaction, a disease process called Adhesive Arachnoiditis. I had had congenital stenosis quite severly when they did the LP, and we suspect the degree of compression in the nerves had pulled the cord out of the expected line for puncture. Somehow, they must had pierced the inner sacred sanctum of the spinal cord, causing a rare reaction. 

It was the perfect storm. Because so little is known about Adhesive Arachnoiditis, no one can say for sure what caused it. I now have it both in my neck (cervical) and lower back (lumbar). It is progressive, and I now require the use of a reclining electric wheelchair, patient lift, daily caregivers and more to make it through my day. Over the years, we have created a multi model treatment plan that allows me at least a comfortable baseline function, although I am mostly bedridden, able to tolerate my wheelchair for only a few hours, a few times a week. 

It’s been nearly 18 years now since that first accident that started it all. We do not know, had the original injury been caught in time and repaired, if I would still be in this boat, as research is not year clear on the mechanisms behind the intiation of the Adhesive Arachnoiditis process. My spinal cords have become inflamed, stuck together, and now are adhered to the inside of the sac that covers the brain and CNS. I have big issues with CSF function, as do most with AA. There is no treatment at this time, and no cure.

I have joined the Rare Disease community because I am excited at the recent successes in Rare Disease research in a number of disease entities; they are employing a new collaborative research model that has shown success. I am now using those skills I learned, first as a writer and editor, then as a clinical researcher, to create our own Arachnoiditis and Chronic Meningitis Collaborative Research Network. We hope to use the new resources provided by Eurordis and other Rare Disease networks to turn our attention to finding out the incidence, physiology and possible treatment for Arachnoiditis.

Could you have Arachnoiditis? Although not everything is known about the development of the condition, it is being associated with Epidural Steroid Injections, Obstetrical Injections, Spinal cord surgery, Contrast agents, and more.

 

*Find others with Arachnoiditis on RareConnect, the online platform for people affected by rare diseases.

 

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