I can't believe it's already been two weeks since I started my 5 day loading dose of IVIG. It's a total blur at this point, but I wanted to try and document some of what I remember before it completely fades, and before all the steroids are out of my system (since they're still making my hands a bit more functional right now!)
It'd been a couple months since I got diagnosed with Autoimmune Autonomic Neuropathy (AAN, aka. Autoimmune Dysautonomia, or its more technical name Autoimmune Autonomic Ganglionopathy, which is a neurological complication or comorbidity with my more systemic case of Sjogren's Syndrome), and I still hadn't heard anything from the hospital about starting IVIG. My doctor's office had told me a couple times to keep waiting, but as I lost more of my ability to swallow and the mysterious muscle weakness got worse every week, I finally got impatient enough that with a little prod from a friend who's dealing with similar health issues, I took a chance and called the hospital directly. It turned out my file was stuck in booking, as it's a smaller hospital and they were having trouble getting me in for 5 consecutive days.
Two days after I called, on a Thursday, they called me back and asked if I could start Monday morning the following week - they were going to have me do the loading dose in the chemotherapy clinic where they had more space. And that was that, suddenly all the waiting was done and it was time for the hurry up. Fortunately, Bruno and I had done some preparation ahead of time since we didn't know how much notice we'd have when the call came, so we just had to spend the weekend preparing food and getting organized, and Monday morning bright and early off we went.
Day 1: The calm before the storm
The first day went okay - I had an amazing nurse and we got the IV line put in (that was hopefully going to carry me through at least a few days), and started the first infusion which they ran extra slowly, always after Benadryl by IV first thing to help stave off reactions to the IVIG infusion. They also ran some saline as I'd asked if it could help with warding off potential drug induced aseptic meningitis - a condition I've been prone to from other medications in the past, and knew IVIG was a more common trigger of. I'd mentioned this to my neurologist as well, but I think since it's considered a rare complication, nobody (other than me) was too worried about it. The saline isn't standard there, but many doctors use it for patients with a propensity for headaches when giving IVIG. (I don't actually have much of a problem with headaches, outside of drug induced aseptic meningitis, ironically...)
Day 2: When the shit hit the fan in every way possible
Day two, things went downhill quickly. I had a different nurse who I found more difficult to work with, and the saline in the morning started giving me some pressure in my head, and also I began having repeated vagal reactions (feeling like passing out and barfing from my nervous system being overstimulated), and then had a big bowel flare up out of the blue, which the Benadryl seemed to calm back down - not sure what that was about, but next time I have a bowel flare, you bet I'm going to try some Benadryl! We're not sure if it was all the fluids, or the IVIG, or the IV line starting to go bad, but by afternoon we had to stop and the chemo clinic nurses were suddenly talking about getting a port placed (minor surgery to put a permanent IV line in the chest) or a more temporary but still longer use picc line installed in my arm, since my IV wasn't working well and they didn't see a lot of other viable veins. I was NOT up for discussing semi-permanent IV access only two days in, as they can bring a range of other risks and complications up! Luckily the nurse I had the first day stepped in a bit and advocated for me, and they had someone from the IV team come down and use an ultrasound to start a better, deeper IV line that was in a spot with less movement (this IV line was a champ and lasted the rest of the week, I was so grateful!)
Unfortunately, the IV nurse who had not been involved in my care otherwise, and seemed not to know anything about IVIG, cranked up the drip rate (a big no-no, as slow rates help prevent adverse reactions) to test the IV was working. I told her not to turn it up and that it wasn't safe to run it quickly, and I guess she didn't listen or turn it back down? Next thing I knew, I looked up like 20 minutes later and the whole second half of the IVIG bottle had drained into me already. CRAP. I knew this was bad, and called my nurse over, but she brushed it off as no big deal, while I could already feel my head starting to hurt.
I got discharged for the day and sure enough by dinnertime my headache was increasing. A couple hours later, probably an hour too late already (I was in denial, and really didn't want to go spend the night in the ER), we hauled off back to the hospital as I knew I was getting a whopping case of aseptic meningitis. I've had it in the past from other medications, but this was escalating faster than I could have expected. The ER was totally slammed and I had to sit in absolute delerious pain while dry heaving for an hour before a doctor was able to get to me - luckily I already had an IV and they got the meds into me pretty quickly once I saw him. But the pain was off the charts - it was literally the worst night of my life.
Luckily, the ER doctor was a gem, and despite not knowing much about my condition or aseptic meningitis from IVIG, he really listened to my own expertise and corroborated my story with my file (we went back to the hospital I'm getting treatment at exactly for this reason). He treated me immediately for the meningitis with steroids, benadryl, tylenol, and anti-nausea meds, letting my waive the usual standard spinal tap they'd normally do to confirm it isn't infectious. We did this all out in the hallway of the packed ER, in the middle of the night, and after a few hours on the IV drip I became a lucid human again. He and the nurse on that night were saints, and he made sure we were allowed to sleep the early morning hours off in the ER hallway before rolling back up to the chemo unit to start the next day's infusion, instead of having to drive back home. (Since my neurologist is in a different municipality, it's about a 30-60min drive each way depending on traffic.) Less than 12 hours after I'd been ranting deleriously about never doing IVIG again, I was hooked back up for another day of treatment.
Day 3-4: Onwards...
The wonderful chemo nurse I had the first day was with me again (thank goodness) day 3, and she called my neurologist and arranged a new pre-medication regimen that would hopefully allow me to tolerate the three remaining days of treatment while still recovering from the aseptic meningitis - and hopefully not sparking it back to full blown life. Bruno was also really hurting by this point, from being up most the night, and they even made him a bed in one of the chemo chairs so he could rest and recover a bit himself. I still feel so lucky we were able to do the loading dose in the oncology unit - it was peaceful there, and they were just amazing to us the whole week. I had to stay in the bed area (rather than the recliner chairs) the rest of the week, and basically just went in each day in survival mode, laid there and got all this stuff pumped into me, went home, slept in 4 hour chunks between medication doses and diarrhea, and rinsed and repeated.
Day 4, the nurse I had on day 1 + 3 was away, and I had another wonderful one, who I spoke with about the transition to my subsequent treatments. I had gotten my schedule the day before and realized some things didn't look right. (The hospital had just moved to a new computer system that was driving them all up the wall, and so we knew there might be some mistakes.) It turned out they had indeed mistakenly booked my next treatment only a few days after the loading dose - thank goodness I had learned enough on my own accord to know that didn't sound right! I confirmed with my neurologist's office and I wasn't actually supposed to get another infusion for at least two weeks, and if I had done it as scheduled I would have been given way too much in too short a time!
Then I had to call the regular infusion clinic that my subsequent treatments would be at and sort that out - the woman there seemed really annoyed with me, my doctor's office, the computer system, etc. and took it out on me quite a bit, so it was pretty stressful dealing with that while doing treatment and recovering from the night in the ER... Also, because I ended up having these reactions, meaning my infusions require a bunch of extra time and pre-medications, she was not happy about all of this and the chemo nurse did some advocating for me that if it was a problem maybe I could come back and do the infusions there... This is still up in the air as far as I know - they apparently are trying to stop having non-oncology patients in the chemo clinic, even though they're much more set up to deal with complex and lengthy infusions - but they're managed by the same team, so may end up moving me around depending on what happens.
Anyway, eventually that got sorted out enough and we cancelled the too early infusion scheduled the next week, actually leaving me a few extra recovery days before my next dose. It turns out I'll be going every two weeks for a one-day infusion through the entire summer before seeing my neurologist again and regrouping.
Day 5: If only this was the end...
Day 5 went off pretty well, with my day 1 + 3 nurse back with me - she and the great nurse from day 4 were so lovely but are also only working there temporarily, so I guess they were just a gift from the universe for loading dose week, even if I won't get to stay with them long term. I decided to bring the nurses a card and a treat to thank them, because they had really gone above and beyond helping me out with all the crap that came up, and advocating for me with the IV issues and the meningitis. I felt a bit silly since I'm shy doing things like that, and thought they must get this sort of thing all the time, but they were actually really surprised and thought it was so nice!
My two favourite nurses even gave me big hugs and the one who I'd had for the 3 days that was soooo helpful and supportive got all teary, and it was just a really nice moment in a hard week. They cared so much about how I was doing, and really validated what I was going through as a younger person with such a difficult condition. I was so, so, so incredibly grateful to have gone through such a hard and scary week with that kind of support. I was so relieved just being done the week as we said our goodbyes and closed out the clinic one final day... that is, after spending an hour prying the 4 day stuck Tegaderm (IV adhesive) off my arm. The last bit just wouldn't come loose, and though we tried to be careful (as last year I developed two kinds of "physical urticaria" - dermatographism and delayed pressure hives, I knew that aggravating the skin on top of my adhesive allergies was not going to go over well...) by 10 mins past clinic closing time, I finally just let the nurse pull the last bit off, and within a minute I had a huge edema patch swell up on my arm. UGH. So my arm was sore and swollen and bruised for days afterwards... not the nicest way to wrap up the week, but little did I know there was more to come anyway.
If only that had been the end of the story! The day after my last infusion, I had a little "cardiac episode" that had us back in the ER - I don't really know what happened, but some combo of my ever low potassium levels, my autonomic system freaking out from the treatment, and tachycardia from all the steroids they'd been pumping into me made my heart rate spike at an alarming level even for me. I felt like passing out and barfing and my heartrate tracking watch had some scary numbers on it, so they then gave me a 24 hour holter monitor... I hadn't showered in many days at this point and just wanted to rest but instead ended up with itchy horrible holter stickies on me all night. UGH. I basically spent the weekend in bed otherwise. My blood tests were also just really off and I'd barely been able to eat for days from the treatment and meds upsetting my GI system at this point - I was just really feeling rough, and we kind of wrote that blip off as a blip.
Sunday wasn't too bad... but then Monday the aseptic meningitis came back. Apparently after the steroids from Friday were out of my system, the round the clock Tylenol and Benadryl wasn't enough to keep it at bay. We called the chemo nurses as they'd said I could call if I had any issues in the subsequent days, and they sent me back to the ER again. Unfortunately, Monday's ER nurse was not so accepting of my "drug induced aseptic meningitis" story, and they stuck us straight into a quarantine room with the masks and everything, until the ER doctor had spoken with my neurologist and become adequately convinced I wasn't bringing something infectious into the hospital. I actually appreciated their precaution in a way, but it was not pleasant at all, and I was more worried I was going to catch something from the icky quarantine room!
This time they gave me a stronger type of steroid by IV, and while it helped calm the meningitis back down quickly, it made me feel absolutely horrible. By the time we were discharged and through the rest of the day, I was feeling really dizzy and my eyes were hurting like crazy. I still had the head pressure I'd been feeling since the second day of the IVIG infusions, and it seemed like it actually got worse after this stronger steroid... I managed to speak with my neurologist on the phone (she's been really wonderful through all this, but it was the first time I'd talked to her directly since my intake appointment where I got diagnosed), and we sorted out a plan to manage this at home as much as possible going forward. I got switched back to the weaker steroids I tolerated better, to do a week long taper off them, and she gave me a bit of a pep talk just about how hard the high dose loading dose can be for patients with certain autoimmune diseases, and that it should hopefully get easier with time and the lower doses of the ongoing treatment.
I also had the more common side effects of feeling like I had a horrible flu for several days towards the end and after the end of the loading dose - back aches, fluey feelings, "malaise". I had to take Benadryl and Tylenol around the clock for two weeks straight, and it wrecked my GI system so I also had diarrhea and could barely eat for over a week. Which meant losing more weight, etc. It's been rough.
Slow, roundabout recovering...
It's been just over a week since that last ER visit, and I'm on the lowest dose of steroids now for a couple more days. The pain has definitely improved, but I'm still having some intense pressure on the side, and also my vision isn't 100% back to normal, so I waited it out through the weekend and left a message at the neurologist's yesterday morning to make sure it's ok to just keep doing what I'm doing at home...I haven't heard back, so I take it they're not too concerned. Either that or the admin assistant forgot about my message (this happened before, so it's not super reassuring, though she was very apologetic - it's just, it's my freaking BRAIN, ya know?) This is all still very much a process right now - while I had a feeling the infusion week would be hard, I really didn't realize how long afterwards it could take me to recover, and I'm pretty antsy for things to get back to "normal" before I do my next treatment day.
I'm hoping it's nothing too alarming, and "just" a little residual swelling or fluid imbalance or steroid side effects, or who knows what. My body has been through A LOT these last two weeks, so I'm trying to be gentle with myself even though I'm getting pretty cabin fevery now, and we were hit with a big heatwave over the weekend. (At least it wasn't during the loading dose?)
In the meantime, I also dragged my butt to my endocrinology 6 month follow up last week (I didn't want to cancel since he books 6 months in advance and I knew he'd have some things to say about all these steroids and my bones), and saw my GP (who's going to be on leave for a year starting in the fall *sobs!*), and am juggling all this other ongoing health stuff with my potassium levels, osteoporosis, etc. Everything else is really on the backburner and I'm only dealing with what is absolutely necessary, at least until things seem to calm down. It really feels like a full time job right now, but hopefully through the summer we'll settle into new routines with all the new meds, treatment schedules, etc.
The new normal - it's really not a fixed thing, but an ever changing landscape that I (and we - Bruno has been an absolute rock for me through this, I can't even... *sobs some more*) are figuring out how to navigate and adjust to with each day and week that passes.
The long game
Basically I've been repeating to myself these past couple weeks, "play the long game, eye on the prize"... It feels and maybe sounds like it was a choice to embark on this treatment, but it really wasn't. When you know you have a progressive illness and it finally gets properly diagnosed and matched up with a treatment plan, you just have to do it. I just had to suck it up and do what this doctor - this doctor who I finally have who actually understands what's wrong with me and how to treat it - says to do. The alternative is WORSE. No matter how hard this past couple weeks have been, or how well I tolerate the upcoming treatments, I have to keep going. Because progressive neurological disease is BAD. Things were already bad, but now that I know exactly how much worse it can get - feeding tubes and being bedridden - honestly, it's terrifying. And I don't think it would have taken me a lot longer to get there.
While my condition is rare, there are a few dedicated research groups in the US working on it, and the latest study that came out has really encouraging results treating AAN from Sjogren's with IVIG. Not everyone responds, but those who do can see recovery to the point where they can work part time again after about a year of treatment (and then of course actual rehabilitation from all the damage and deconditioning will be a whole other challenge). It's long and slow and not a magic bullet, but if I'm dedicated and motivated to do what needs to be done, and actually tolerate the treatment, even getting half my old functionality level back would be a miracle. So I'm trying to play the long game, keep my eye on the prize, and look at all this present suffering as part of the process.
It sure hasn't hurt that between the loading dose and all the steroids, I had about a week where my hands actually worked again (yesterday they really started reverting to pain balls as I'm down to a low dose of the steroids now), I'm not constantly losing my voice, I had a big break from the pressure hives, and various other nagging autoimmune symptoms have just faded away. I know it won't last as I'm already tapering off the steroids, but as my GP agreed, it's really encouraging to see that treating this as autoimmune is already showing results. And hopefully with time, the IVIG will make all these early improvements stick, bring the neurological function back up to a more normal level, and become a permanent improvement...again, as long as I can tolerate the treatment. That feels like the biggest hurdle right now, so the coming months will be telling, once I recover from loading dose and see how the regular infusions sit with my sensitive system.
It's going to be a difficult, but very interesting summer, and a telling year. I have a lot of hope, and a lot of fear, but you know which one I'm going to let win as long as I have any reason to.