We checked into the hospital this morning, bright and early, to get started on our first dose of Rituximab.
Even after 2 hospitalizations in the last few weeks, it’s never any easier, especially for Maddi. I couldn’t sleep last night; so full of anxiety and worry. The last two hospitalizations results in respiratory failure, intubation, and an ICU stay. Again, in my rational adult brain, I knew she was going in much healthier than she had been before, but I still couldn’t shake the worry.
Would she end up in the ICU again?
Would Maddi be aggressive and irritable?
Would she develop some rare side effect from the Rituximab?
So many thoughts ran through my head and prevented me from sleeping. It made my stomach sour. It made my heart race. Even as I write this, I can’t shake the feeling of wanting to vomit.
When that pregnancy test turned positive 6ish years ago, I immediately accepted the responsibility that came along with being a parent. I knew and accepted with such fervor, my role in raising Maddilyn to be a wonderful, productive, healthy, functioning person. I accepted my duty as her Mother to love, protect, nurture, and guide her. I also accepted that sometimes this role meant I would need to make difficult decisions on her behalf, because as her Mom, there would be times that I knew better.
Honestly? I always thought “those” moments would be moments like: “Your dress is too short! Go change!” “No you cannot date a guy who is 3 years older than you!” “Don’t do drugs. Don’t have sex. I’m the mom, I know what’s best!!”
If the duty to raise her in all the right ways doesn’t already feeling like an unmanageable duty, go ahead and pile on the weight of having a life altering illness, because I checked, there is no written guidelines to prepare you for that.
If I’m being even more honest here, I don’t have this parenting thing figured out. I’d like to think that on most days I’m a pretty competent parent, but there are many other days that I’m just at a loss of what to do, or how to handle things…and I mean everyday things like being sassy or taking toys away from her little brother.
Nothing prepared me for this. Nothing prepared me for a critically ill child. Nothing prepares you for the countless decisions you’ll need to make on their behalf. Decisions that could alter their lives in ways that is irreparable.
Here we are now. Making more decisions on Maddi’s behalf to get her well, and I can’t help but feel extremely anxious about it. I don’t want to make the wrong decision. Is Rituximab the right decision? Is a hospital stay the right decision? Is Risperdal the right decision? I don’t know, and the not knowing is the part that gives me the most anxiety.
The unpredictability of NMDAR Encephalitis is what gives me anxiety. It is the source of my stomach aches, the source of my inability to sleep, the source of my difficulties with concentrating, focusing, or even forming thoughts most days. I don’t know if Maddi will have a relapse. I don’t know if she’ll continue to get better. I don’t know if she’ll have another seizure again. I don’t know. We don’t know.
Ugh. I hate that phrase, “I don’t know.”
After Maddi’s seizure on Friday, Jason and I felt really comfortable with saying yes to the Rituximab, despite the long list of side effects, one of those being the activation of the JC Virus (which we all carry, btw!) JC Virus is potentially fatal, and when it’s not, has significant impacts on brain functioning. It makes NMDAR Encephalitis look like a walk in the park. As the rheumatologist Dr. Elder, explained the side effects to us this morning, she briefly mentioned the JC Virus and added in, “it’s extrememly rare, and very unlikely she’d develop that.”
Um, excuse me for a second…
Can I remind you why we’re here again?
Oh yeah, My kid developed a RARE DISEASE!
I giggled and told her at this point, I wasn’t dismissing the possibility of anything anymore.
Through-out the morning and early afternoon, they prepared Maddi to receive the Rituximab, and we were told that it could take 12+ hours to infuse it, depending on her reaction to the infusion. Dr. Elder assured us that when/if people have reactions, it’s almost always during the first infusion, so if Maddi tolerated this one well, she would tolerate the others just as well.
So, how does Rituximab work? I’m going to explain this in my non-doctors terms as best as I can, so excuse me for errors.
Dr. E explained that in the bone marrow, b-cells are developed and go through a maturation phase. A chemical, CD20 helps the B-Cells mature, and once they are mature they divide into two categories: Memory B-Cells or Plasma B-Cells. The NMDAR mutation happens during the maturation stage, and once the B-cells mature, some of them go on to produce the anti-NMDAR antibody in Maddi’s plasma, and others go on to be stored as memory B-cells with the anti-NMDAR antibody in her lymph nodes, etc. Now, the Rituximab will do nothing for the memory B-cells. Those are already formed and stored. It’s unknown how long they’ll last, but some last forever. The plasma B-Cells die off very fast, so any plasma b-cells that have already matured will eventually die off. Rituximab works at the maturation phase. It binds to the CD20 on the B-Cell and turns on a kill switch, effectively killing the cell off before it reaches maturation. The idea would be that at some point, Maddi will no longer produce the B-Cells that trigger the Anti-NMDAR antibody, and thus the progression of the disease will stop.
So, what does this mean for her immunity?
Although Maddi is immunocompromised, she is not as bad off as say someone with cancer, who is on multiple immunosuppressant drugs. Thankfully, all of her Memory B-Cells stored in her body for viruses, colds, etc that she has already been exposed to in her life, will help in the future if she were to be exposed again. Her body will be able to make anti-bodies for them. Also, IVIG gives a sort of “acquired” immunity, and as long as she keeps up with the IVIG treatments, she will be largely protected. At the same time, she is still immunosuppressed, and the cases of fatality in NMDAR Encephalitis were resulted from illnesses caught from immunosuppression, so we will be taking precautions. This situation has taught me to never dismiss the possibility of anything.
Maddi tolerated the Rituximab infusion very well at first, and then around 5pm that evening, she started to develop a really dry cough. Once it was ruled out she wasn’t going into anaphylaxis, the Rituximab infusion was lowered to a slower rate, and she was able to tolerate it throughout the night, with no sleep disruption.
I was so incredibly proud of Maddi during this hospital stay. It’s very hard for her to understand why we are going through this, because for the most part, her body is healthy, and the processes that are at play are not visible to the naked eye. All I can do is explain,“Your brain is sick, and we have to get it better.” I know she can understand that, because I know there is a part of her that recognizes that something isn’t right. The hardest part of the entire stay was holding Maddi in my arms while she got her IV put in.
The whole time she was just looking up at me and crying out, “Mommy please make them stop. Mommy it hurts. Mommy make them stop.” Talk about feeling incredibly helpless, and making me question everything again.
I think I will ask myself daily whether or not Jason and I are making the right decisions on Maddi’s behalf.
I have to again resolve myself to believe that yes, for today, we are making the right decisions.
Maddi will return in two weeks for her second Rituximab infusion, along with two doses of IVIG
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I’m praying for a full recovery for your daughter. My niece was also diagnosed with This terrible disease just over 3 years ago at the age of 4. I’m glad to see there is more info becoming available about this as there was not a lot to go on when she was diagnosed. You are definitely in my thoughts!!