We met with Maddi’s Neurology Team last Tuesday to talk about her progress so far, Jason and I expressed our concerns that Maddilyn had seemed to be getting no better since her last round of IVIG and Steroids. For the last 4 weeks she had been consistently irritable, aggressive both verbally and physically, and starting to exhibit signs of anxiety and sleep dysfunction. Now, there are so many factors that compound this matter, it’s really hard to decipher her behavior and to figure out if it’s 1) disease, 2) steroids, 3) brain damage. I mean, the reality is that it could be all three factors, but I’m weary of adding in more medications or treatments, if the solution is simple. After talking at great lengths, Dr. Kang, who is the head of her Neurology team, felt that steroid and other factors aside, the fact that she was still exhibiting symptoms was evidence that her disease was still at play, and unless we treated the underlying cause, Maddi wouldn’t get better. At that point, he brought up the recommendation for plasmapheresis and/or exploratory surgery to look for an ovarian teratoma. At the beginning of this whole treatment process, Dr. Kang had mentioned those two options, but mentioned them as “last ditch,” efforts to try if nothing else seemed to be working. He had explained that he was always nervous performing plasmapheresis on someone as small as Maddi, but he had recently treated an autoimmune case on a small child, similar to Maddi’s, with great success. I remember sitting there and listening to everything he was saying, resisting the urge to vomit all over the exam room. I felt myself slowly starting to panic. My brain was racing in a million different directions. She’d have to go under general anesthesia. It was major surgery. They’d be touching her vessels- her heart. What if she got an infection? What if there was a complication? They’d have to cut open her abdomen. What if she never wakes up? What if they find a teratoma and have to take out her ovaries? What if we are over exaggerating and she is getting better? What if they do all of this, and something happens, and it was all for nothing?! Traci, the ARNP who we work directly with, was in the room and must have noticed that I had turned green was starting to panic, because she intervened and asked if we’d like some time to think about our options. Yes. That’s what we needed. Jason and I needed time to process; time to think; time to prepare. Although the reality is, I don’t think I could ever really prepare. Sure, we read about the procedures. I reached out for support from fellow Autoimmune survivors and caregivers. I scoured the medical journals. NMDA is can be a paraneoplastic disease, meaning that it can result because of a tumor. I could find zero documentation about the tumor rates in cases such as Maddi’s, in fact, it’s very rare, yet not entirely unlikely. We tossed around the idea for days. If she has a tumor and we don’t operate, she could get worse…way worse…if she doesn’t have a tumor and we operate, then we run the risk of surgery complications for nothing. So many decisions to make, and all the while I’m feeling terrified of making the wrong one. It’s such a heavy load to bare, making decisions that impact others. We talked and decided over the weekend that the risks were low enough for both the procedures, that the benefits were worth it, especially if this was the treatment that would push her over the edge of wellness. We also decided to go ahead and put her back on a low dose of Risperidal to help with her irritability and agitation until we could get her well. So, after talking with the office, the game plan is to get admitted Monday morning for a 24 hour EEG; Tuesday she’ll have an MRI and Ultrasound (requested by Peds Surgery), Wednesday surgery for the central line placement and x-lap, and Thursday she’ll start the Plasmapheresis (3 days, then once every other day for two more treatments; total of 5).
This is going to be an extremely long hospital stay, minimum of 10 days. That is a level of stress that Jason and I don’t even know how to address at this point in time. Thankfully, we have a great support system, and we’ll make it work!