Neurobehcets – When it Doesn’t Make Sense, but Does

Prepare yourselves for a little breakdown of my neurological funkiness, and what may or may not be related to the Behcet’s (but probably is). The reality is we don’t have a way of knowing exactly when my Behcet’s began, though likely in childhood. We also have no way of knowing when my neurological issues began with Behcet’s, though the thought process right now is somewhere in my teens.

My oral ulcers started in late elementary school, and we just figured I was a kid who got a lot of canker sores. I also got a lot of strep throat. Then in middle school I had these annoying episodes where one arm would jerk, and I would make a squeaking noise. I never saw a doctor, because it didn’t happen often enough to create concern, but it was annoying. (I would later be diagnosed with Tourettes, however, the occurrence is so infrequent, I never even remember to mention it in my medical appointments.) In my late teens I started to notice I had crappy balance.

This is where it gets important: the only reason I noticed, was because my friends pointed it out.

When we would all walk together, the ongoing joke, which was also the truth, was that everyone needed to walk on my right side. I tended to veer right, and if the group didn’t stay on that side, I wouldn’t walk straight, and would end up missing the conversation, or even trip. At 16 I’d torn a disc in my back (at the time we didn’t know the disc was injured, it took five years to get a scan, but that’s a whole different story). When I’d get wobbly or off, we just sort of figured it was back related.

Then in college, at 18, something scary happened. I got up out of bed, and my right leg was useless. I could move it, but I couldn’t really control the movement, and I couldn’t keep my foot up. A friend helped me get to the health center, where we found out I had no reflexes in my right leg. He hit my knee over and over and nothing. I was told I likely had a pinched sciatic nerve, aggravated from the prior back injury (which still hadn’t been scanned) and sent back to my dorm with crutches.

Yay student health systems! 

By 20 I knew I had something autoimmune going on, but my brain wasn’t really a concern. I had a seizure, but I’d been drugged, so the thought was obviously that the drug had induced the seizure. I developed PTSD, and future seizures were assumed to be psychosomatic. Except, they weren’t. I had also begun developing issues before my PTSD, where I would get dizzy, lose feeling and coordination on my right side, and begin to slur my words, or forget them all together. It was terrifying, and I’d often just have to sit in stores and wait for it to pass.

Eventually I saw a neurologist who misdiagnosed me with juvenile myoclonic epilepsy. The drugs made me sick, and my balance issues and lapses in focus continued. I would be okay, then have intense headaches and be stuck inside for days. Sometimes they’d say it was a migraine, but other times they couldn’t sort it out. There times when it was so bad I refused to go to the ER because I didn’t want to get up off the bed or floor. Once I dropped in the kitchen from the pain, and just laid there for nearly four hours.

A new neurologist saw me, and looped back to psychosomatic issues. They diagnosed the Tourettes which was random, but inconsequential with all the other issues. Thus began a decade of neurological struggles. Some neurologists believed me, others insisted it was a psychological issue causing seizure like episodes. We took videos of muscles spasms I couldn’t be controlling. We took videos of me trying to walk during episodes. We did whatever we could, but if it wasn’t on an MRI, they didn’t care.

I was actually blacklisted from a neurologist’s office because he was sick of me demanding that he figure out what was going on. Another told me I had MS, before later telling me he was wrong.

My current neurologist is amazing, and has stated that he believes my issues are 100% because of my Behcet’s, but he won’t diagnose neurobehcets because I don’t meet all the criteria. The only criteria I’m missing is sign of brain stem atrophy, or lesions. I’ve been receiving treatments since before neurological issues presented, and in fact, the neurological issues worsened and became noticeable, when I was on a hiatus from immune suppressing therapies.

I never really thought my neurological issues could be related to my Behcet’s, until I noticed the lack of coordination coincided with flares. Then I thought back to all the years, all the signs, and my heart sunk.

So now I’ve seen the new rheumatologist, and he, without a moment’s hesitation, declared neurobehcets as the culprit. True, I don’t have lesions (yet) or brainstem deterioration (yet), but isn’t that a GOOD thing? Even textbooks on the issue of neurobehcet’s have admitted that the criteria that exists is based on limited patient samples. Plus the reality is that it’s a rarer complication of an already rare disease. In my case, having the HLA-B51 subtype does put me at an increased risk of certain neurological impacts of the disease.

The question becomes what are my issues from NB (neurobehcets):

  1. Ataxia
  2. Seizures
  3. Fatigue
  4. Lack of coordination
  5. Muscle weakness (especially in heat)
  6. Hyperactive reflexes (makes anything that triggers involuntary movement in my legs extraordinary challenging to deal with. Shivering? Game over.)
  7. Headaches
  8. Autonomic neuropathy – which impact me thus far via:
    1. Issues with regulating heart rate and blood pressure
    2. Digestive issues, primarily lower GI issues involving intestinal dysmotility, and also aggravation of my gastroparesis
    3. Temperature regulating – this becomes a problem with things like hot baths/showers/tubs, or if I have a seizure. It’s normal to get warm during a seizure, but I struggle to come back down temperature wise. I also don’t get fevers when normal people do, sometimes. It’s frustrating to have a severe and painful kidney infection, but no fever. Doctors don’t tend to take you seriously

I’m sure that there are other issues I’m not even aware of, because I’ve just lived with them as they’ve shown up in my life. A friend was staring at me as I missed the keyhole several times. It’s normal for me. I didn’t realize until that moment, that most people can put the key, into the keyhole, without several bother attempts. I’d simply been doing it that way for a long time. The odd thing is, I never missed a vein when I was a phlebotomist, but I also would shake violently after each patient, and be exhausted after my shifts. I was also healthier at that time.

Acute NB tends to hit, and then go away. It can come back, but a course of heavy steroids, often IV, tends to knock it out. This is what was assumed to be happening when I was hospitalized a year and a half ago. Now, at the time, they didn’t know what was going on, so for six days we tried to deal with my heart rate being all over, from too high, to way too low. I couldn’t walk, because I shook to hard. When they stopped my Neurontin, my jaw locked shut. Finally they gave into rheumatology’s suggestion that we throw steroids at it, just in case it was related to an autoimmune issue. They’d done spinal taps, MRI’s, I’d coded, it was time to just do something.

Three days on IV steroids, and I was discharged with a walker.

I tried to point out that it was a classic acute NB flare, but because my spinal tap had been normal, as had what they could see on the MRI before I had my seizure in the machine, they refused to diagnose me.

As the steroids tapered down, the instability, and coordination issues returned. That was when I went to NYU, started Remicade, and then promptly ended up on Cytoxan.

Now I’m ready for my next round of IVIG. I notice the disappearance of the non-permanent neurological issues. I’ll always have ataxia, but the really obvious manifestations don’t come during the first 3 weeks of IVIG. The headaches are gone. Knock on wood but I haven’t had a seizure since starting IVIG, either. My hyperactive reflexes are permanent, but they appear to be limited to my legs right now, and mostly my left leg which makes sense. (My right leg is somewhat dumb from the back injury.)

I’m just happy to start over with a rheumatologist who is ready to fight as hard as I am. I loved my old rheumy, but her refusal to consider that I may have issues that were NB, because I missed the imaging to support it, was upsetting.

Why should I be forced to get sicker, when I’m clearly responding to treatment, and have all the symptoms of the disease?

Advocate for yourselves. Fight hard. The wanted to put me in a nursing home after I was discharged from the hospital. I had friends help me so I could get to NYU, and get options I wouldn’t otherwise have. You are stronger than you know.

Advertisements
Neurobehcets – When it Doesn’t Make Sense, but Does

Don’t Ignore Your Service Dog

So I’ve been casually seeing this guy, and when I spend the night I don’t always stay on top of grabbing something to eat in the mornings, or before bed. Lately I haven’t had an appetite at all, and since I haven’t been on steroids, the weight has been coming off massively. I didn’t really realize how little I was eating until recently, because honestly I haven’t been hungry. I was heavier than I wanted to be, and I’m at a nice weight right now, so it’s not like I’m unhappy with the weight I’m at, it’s just scary that I got here the way that I did, and it’s scary to know that I will continue to lose weight (rapidly) if I don’t eat more.

Anyhow, there are a lot of problems when you have no appetite, and an autoimmune disorder that makes you nauseas. First off, I have seizures, and while my medication works, if I’m not sleeping well, or eating well, I’m putting myself at risk for seizing. After two abysmal nights of sleep I was pleased that I at least got better sleep over the guy’s place than I had been at home the prior nights. Still, I’d eaten a whopping 400 calories for the entire day, and while I had noshed on a few potato chips at his place, it probably only got me to around 600, less if you consider calories burned.

I didn’t get home until almost noon today, which was later than I’d wanted. I was tired, and kind of hungry, but I had a ton of stuff to do for work, so I threw myself into working without even changing out of my clothes into something comfortable. I knew I wanted caffeine, but didn’t even stop to get my caffeine fix. My mind was on work…except my dog.

When I got PTSD 9 years ago, my dog stepped up in a big way. He learned to stop panic attacks, motivate depressed me out of bed, and check doors and windows if I was feeling twitchy. He also began to alert in the last few years to rapid heart rates associated with black outs, as well as begin to act funny during my pre-seizure auras. In the last few weeks I’ve noticed something else he’s on top of…low blood sugar.

So, as I sat there, trying desperately to get my work done, my dog was nudging my hand, whining, and licking my hands. I thought maybe he wanted me to move to the couch, but that didn’t do anything, in fact, me moving made it worse. I stopped what I was doing and did a body check. I wasn’t having an aura, so I knew I wasn’t going to seize unless I was about to have one totally random seizure which *knock on wood* has never happened to me. That’s when I realized it was pushing 12:30, and I hadn’t eaten more than a couple potato chips in over 12 hours. The last time I ate before that had been 12 hours before. In total, that was about 600 calories in 24-hours, with physical activity. I took stock of how I felt and realized I was kind of dizzy. I felt a little drunk, and confused, and was working slower than usual.

I ate a granola bar, with protein, and drank some vitamin water. I rapidly felt better, albeit nauseas because I wasn’t hungry but needed to eat. The dog, satisfied with himself, curled up under the blanket and slept while I finished my work.

People see me, and they’ll say, “you don’t look sick,” unless I’m flaring of course. (That’s when I’m told I look like death.) They see my little dachshund and wonder how he could possibly be a service dog…but he is. I need to remember he’s more in tune with me than I am sometimes, most of the time in fact, and I need to trust him to alert me to things I may not notice on my own.

I’ve been accused recently of having an eating disorder. Just because I’m glad that I’ve lost steroid weight, doesn’t mean I have an eating disorder. There is a huge difference between having an issue with food because your body doesn’t process it well, and wanting to be thin so you ignore the hunger. I rarely get hungry, and even when I’m hungry, I can only usually eat half of whatever it is I try and eat. I know I have that blocked celiac artery, and I’m sure it’s more blocked than the doctors think, because if it’s 60% blocked all the time, it must be more blocked when the vessels swell.

In any event, I feel like digestive garbage. Food doesn’t have pleasure for me. Lactose, gluten, alcohol, meat, and anything high in fiber? Yeah all of  that is out. I used to love salads. Now it’s a few bites and I’m in excruciating abdominal pain. Doctors push fiber so I can be regular, but anything with too much fiber and I’m doubled over and need prescription strength Imodium. This balancing act becomes such a pain that you double up on vitamins, and live off of bland foods because it’s the only way you can survive. I can eat like a normal person, or live like a normal person, but I can’t do both. I would love to eat at restaurants. Go on dates with guys and not have to explain why I’m only having a Sprite.

Anxiety obviously makes it worse, and I’ll admit that I do have IBS. My GI issues aren’t all Behcet’s related, though the bleeding isn’t necessarily a common thing in IBS patients. Still, the level of GI issues I battle? It’s intense.

So yes…I’m wearing smaller jeans. Half of my Facebook feed is congratulating me on the weight loss, while the other half is concerned. Me? I’m just glad my dog noticed my low blood sugar before my friend and I took off to buy new jeans. Passing out from low blood sugar is a drag. Seizing from it is worse. Doing either of those things at a crowded outlet mall by the California/Mexico border? Not ideal.

Apparently I should really be considering medical marijuana as a more regular thing instead of an occasional thing. I guess I’m still thinking about it as a drug drug, instead of a medication. It would be nice to be in a better mood, and have an appetite!

Don’t Ignore Your Service Dog