In my last blog post, I mentioned that I had two back to back Rituximab infusions to try to annihilate my immune system in hopes of coming down further on my steroid dose, but was nervous because the reaction risk with that medication is particularly high. And good news-- the two infusions successfully wiped my B-Cell count to zero, which means I shouldn't need another infusion for a little while! I did have a reaction at the end of the last infusion, but it thankfully responded to the Epi Pen and other medications, so I didn't have to be admitted.The side effects during and after that infusion were not too fantastic, but in the long run, well worth it. For me, it causes flu like effects-- so aches, chills, body pain, fatigue, headaches, etc.-- but thankfully they usually go away in a few days. My back has still been pretty painful from the compression fractures, but I started physical therapy, which has been helpful.
Since starting the Rituxan back in December, things overall have been far better in terms of my angioedema (swelling) and mast cell symptoms. I still have reactions and episodes, but they have been far less frequent and more controllable. I was hospital admission free for about a month. My energy and stamina had dramatically improved. However, I have to be really careful because I have absolutely 0 b-cells, on top of multiple existing immune deficiencies.
To try to combat my not so fantastic immune system, my doctors said that it was extremely important that I re-start immune globulin therapy. For those of you who have been following my story for a while, I was on IVIG (intravenous immune globulin) for a while to help treat the autoimmune component of my health issues. However, we had to stop it because I was getting angioedema and reactions to IVIG. We suspected that it was because I have zero igA, which protects the body's mucosal surfaces. This is something that I was born with. It makes any form of immune globulin or blood products risky for any IgA deficient patient (not just me) because we can develop anti-igA antibodies and/or react to the IgA in these blood-based products because our bodies are not accustomed to having IgA.
We were hoping that the Rituximab would surprise my immune system enough to try SCIG, which is subcutaneous immune globulin. It's similar to IVIG, but instead of IV administration, it is administered more frequently through needles that infuse the IG below the skin. SCIG usually is better tolerated and often can be done at home on a weekly basis. It also usually isn't as difficult as IVIG for people like me with no IgA. Because of insurance logistics, my first dose was administered with a nurse from the pharmaceutical company at home as opposed to a hospital or infusion center. I was a bit nervous given my history, but I was given a pre-medication routine to follow, and I would be with a nurse through the full infusion.
Placing the needles wasn't comfortable, but wasn't as terrible as I thought it would be. Both needle placement and infusion are a little tricker on me because it is supposed to be infused into an area with enough body fat to absorb the medication, which at the moment, I don't have much of. I was warned that this could be a problem, but was told that it should be ok. During the infusion, I was reacting, but again, was responding positively to medications, and was able to complete the infusion. After the infusion, the nurse pointed out that my stomach was really distended (sticking out). She said that it likely was that my body didn't have a place to put the large amount of IG liquid, but that it should go away within a few days.
It was like 9/9:30 PM by the time she left, so I went to sleep shortly thereafter. I was pretty uncomfortable with the big belly and usual IG side effects, plus recovering from the reaction I had, but fell asleep. I woke up an hour later with much worse abdominal pain, rashes, flushing, nausea, stomach cramping, a headache, horrible body pain, itching-- you name it, I had it. I was SO uncomfortable. I felt like my abdomen was going to explode. I tried my best to control it at home, but was unable to do so. My home health nurse came to change the dressing on my PICC line, and had me use my EpiPen. When it didn't work, she sent me to the ER.
I was not a happy camper about going to the ER, and once I got there, they started me on an Epi drip really quickly because of my swelling. That also meant a one way trip to the ICU. I was so disappointed that I have been doing so well, and then still had this major reaction and setback. This one was particularly frustrating because we had to wait the 4-5 days as the medication was slowly absorbing, and I was continuing to react to the medication as it was doing so. They wanted to transfer me to CHLA so I could be at a facility with an allergy team, but because of my age, the transfer was denied. They then were going to transfer me to UCLA, as they have more respiratory equipment that's non invasive, but they were full. I was on the epi drip until Saturday morning, and moved out of the ICU Saturday evening.
This setback was also really challenging on me emotionally. I was disappointed and frustrated that I failed this treatment. My back pain worsened significantly because I wasn't really allowed to get out of bed much during the 6 days on the epi drip. I had one nurse that caused a lot of distress for me, and 2 or 3 pain crisis type episodes which also made me swell.
However, one major positive from this last week is that it's pretty clear that the Rituxan is making a HUGE positive difference. Yes, I had a major reaction to the SCIG, which was an "error" to say the least," but once the SCIG absorbed, I dramatically improved, which shows that this was more of an allergic reaction versus an angioedema attack. They only had to increase my steroids for 3 days, versus the insanely high doses for weeks that I usually need. This also means that it could be related to the IgA in the SCIG.
In terms of moving forward, it's pretty clear that SCIG is a no go. However, because of the state of my immune system, I need to have some immune globulin on board. They want to re-attempt the same low IgA IVIG brand I was on before, but at a lower dose or this same SCIG med (which ain't happening). But of course, the IVIG is now on a national shortage. Thankfully, they're letting me go this evening while we figure out a game plan for the future.
Ok, and finally, non-medical update-- I have been working with the state's Department of Rehabilitation (DOR) to help me figure out options for school and work online. I encountered an online Patient Advocacy Certificate program through UCLA Extension online. It's a three quarter program, with 2-3 courses per quarter. This is right up my alley, and can help me with my role at CHLA. The DOR has been an amazing resource-- they're paying for the program, and helping me with supplies, books, etc. I started last Monday, and so far, have loved having the distraction and intellectual stimulation beyond my tutoring. Most of the learning happens through an online discussion board, but there are also online lectures that I watch and quizzes. The classes I am in at the very moment aren't my favorite topics, but are part of the certification curriculum-- Fundamentals of the US Healthcare System and Introduction to Healthcare Finance, the Advocate's Role. The hope is that this program will keep me occupied until I have a better idea on my prognosis and what type of work I want to (and am able to) do.
I'm looking forward to getting home later today, and back up and running later this week!
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| Playing tourist in LA with a friend from the Bay Area on one of my good days! |
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