|Some of my Beads of Courage-- I'm behind a few months. Each bead represents a medical hurdle I have overcome (http://www.beadsofcourage.org)|
Despite my immune system being unbelievably stubborn, the team here hasn't given up on me, which I'm immensely thankful for. It absolutely was a blessing in disguise that both UCLA ICUs were full, and was taken to USC/Keck. We may not have any answers as to why this episode occurred, but the team here has been incredibly patient to try to identify any information that could help us get me out of this flare, and hopefully, to improve my quality of life overall. Unfortunately, immediately after my last blog post, there was one mishap with the kitchen and an exposure to one of my primary food allergens, tree nuts. This required them to dramatically increase the doses of a lot of my medications back to where we started to avoid a breathing tube. We have also had countless other setback which have been quite frankly, exhausting-- I tried and failed two different formulations of Gleevec, the mild oral chemotherapy drug I was on a couple of years ago, found a safe formulation, then magically developed a sensitivity to that formulation, have had spontaneous swelling episodes for literally no reason 4-5 times a day, tried and failed 2 entirely different medications, had additional doses of IVIG (limited due to my reactions to it and a national shortage), 5 days of iron infusions, had the PICC line (the IV that was in my arm that went up through my shoulder to my heart for medications) crack, replaced in the other arm, and then replaced by a longer-term line in my chest (in addition to my implanted port, which I have mentioned in other posts), have been on high dose IV steroids this entire time (which have given me major major moon face despite no change in body weight), random electrolyte imbalances, the list could go on and on and on. I'll spare you all the details. But, the point is, when I thought I was hitting a metaphorical wall before, I never could have imagined the wall that I have been hitting.
I've been struggling, which isn't always easy to admit. I'm exhausted. I have had moments of unimaginable pain. I want to go home so badly. I'm struggling with the steroid "moon face" that makes it so I barely even recognize my own face in the mirror, despite the fact that my actual body weight hasn't changed. I want to see my dog. My bed. Again, I could go on. But for now, I know that the safest place for me to be is here, and that I couldn't ask for better care with such challenging circumstances. So, in terms of gains-- the team here has been integrating as many opinions and views from specialists within the relevant fields around the world, and doing anything and everything they can to keep me comfortable and understand my disease process better. On many days, they have taken me outside for a bit to get some air. The nurses and doctors have been incredibly attentive, understanding, and patient, and all gone above and beyond to show their compassion, and hold my hand both literally and figuratively when the going gets rough. The pharmacy students here were tasked with the significant burden to collect lists of inactive ingredients in all prescription and over the counter medications over the last few years to identify potential allergens, and actually successfully identified 3 "fillers" in medications that I appear to be allergic to. We are waiting a re-analysis of my genetic testing results. My GI symptoms have improved a bit with a 2 week course of a medication to treat small intestine bacterial overgrowth, which is common in people with my conditions.
|I've been able to meet 3 others with MCAS while inpatient!|
In terms of "big picture" progress-- they rechecked my autoantibodies while I have been here, which basically show if may body is creating antibodies that is attacking itself. However, with where modern science is, there are probably thousands of autoimmune diseases and variations that don't show up on lab tests. I have tested positive for multiple autoimmune diseases/antibodies before (Sjogren's, Hashimoto's, and a type of autoantibody common in Type 1 Diabetes), however, I don't actually have the symptoms usually associated with these antibodies (as in my thyroid intermittently is a hair off, but not consistently enough to treat, I don't have any indicators of type 1 diabetes). In addition, the Rituxan infusions I had earlier this year completely wiped out my B-Cells, which are the cells that typically make these autoantibodies, and all of the detectable autoantibodies in the blood. My B cell count is still 0 from the Rituxan, yet, when they rechecked my auto antibodies here, they came back. Having no B cells but having positive autoantibodies can happen, but its pretty uncommon. The interpretation from the teams here has been that this indicates that my body is in a full blown autoimmune flare, but that there likely is a whole lot going on that is "behind the scenes" on top of what we do see that is causing things to be so severe.
Since some detectable autoantibodies came back, the discussion of plasmapheresis resurfaced (description of plasmapheresis). This treatment is something I mentioned in blog posts a long time ago, but is similar in concept to dialysis. Because I have failed literally every single med trial except for 1, and things have continued to decline despite very reasonable intervention, they decided to move forward with the plasmapheresis. However, it has literally never been used in a situation like mine-- it has been used experimentally in some mast cell patients, and more regularly in different autoimmune diseases, but since my constellation is rather unique, there is no protocol or expectations to follow. Everything is to some degree on the fly day to day based on my response. Plasmapheresis requires an extremely large IV that is usually used in dialysis, as the machines are very similar. I have a Hickman line in my chest, which is like the PICC I described earlier but in my chest instead of my arm (and a material that Im not allergic to like the one I had last October, and a port in my chest, but neither can handle the pressure of the machine. This meant that they had to place a dialysis line, which is usually in the chest. However, because I have the Hickman and port already, my risk of developing a life threatening blood clot was too high to put it in my chest, which meant they had to place it in the only other location with a big enough vein-- the groin. The placement was more on my leg than I thought iw would be, but my god, both the placement (which happened 8/16) and the site now is absolutely excruciating. I currently can't walk more than a few feet without breaking into tears from pulling at the site. I had my first pheresis session yesterday afternoon, where 68% of my body's plasma was removed and replaced with albumin, a type of protein, over a little less than 2 hours.
The current plan is to do a total of 3 sessions over 6 days (one day on, one day off) to see if it makes a difference. There are a few sucky parts in addition to the painful dialysis line. Since this is uncharted territory, they have no idea if the pheresis will make a positive difference. If it does, they will extend it to a two week course, and if it doesn't, they will stop after 6 days. However, if it does make a positive difference, that change is only temporary-- my body will still produce the same faulty antibodies again. Therefore, if I do respond positively, that means that my care team will have to be far more aggressive than they have been with immunosuppresants and chemotherapies, since improvement on the pheresis confirms that my body is finding rather innovative ways to continue to produce ways to attack itself. If I don't respond, then we're kinda just back to square one. I don't really win either way. Phereis isn't particularly sustainable long term because of the time commitment and frequency that it is required, the fact that it is essentially a band aid, and that very few facilities have the proper equipment to do it. It isn't something that can be done at a local hospital or dialysis center.
Even though it looks like my treatment course will be highly highly unpleasant if the pheresis is successful, I'm really hoping that the phersis is successful at least temporarily-- we so desperately need both a break and answers. And when I say we, yes, I'm referring to myself and my medical team, but also to my family, immediate and extended. They have been here come hell or high water, driving 2+ hours each directions to hold my hand during painful procedures, keep me company while I work on an assignment for my online class, to play a card game, etc. It is an absolute nightmare to live in the hospital for 2+ months, but I'm incredibly thankful to my family (and friends) for trying to help make it a little more bearable. Well, more than just bearable, more like preventing me from losing my mind entirely! I am surrounded by my silly little projects (which I suppose have been improving in caliber) and sloth themed everything. I have now learned how to incorporate rhinestones into my IV cap art, and successfully supplied "badge buddies" to many of the nurses on my unit as well as the one next door. I may or may not have sustained a few "crafting injuries" due to my clumsiness and trying to use a glue gun in bed.
|Some of the cap art/badge buddies.|
|Literally 68% of my body's plasma in a bag at the end of the first round of plasmapheresis.|
I have somewhere in the neighborhood of 3-4 weeks left for the quarter on my patient advocacy certificate, and then one quarter left to go until I finish the program entirely. Tutoring has been a little slow given its the summer (and that I feel like crap so I haven't been going online much) but it's there. I'm doing some remote work for my role at the Children's Hospital whenever and wherever I can to keep my position. If you have called me and I haven't responded, please forgive me-- I promise I am so incredibly appreciative of the contact, and want to respond, but voice calls in particular are really exhausting for me at the moment. However, I looooooove to text, and usually respond pretty quickly! I also love in person visitors, but texting me ahead of time is much appreciated just to make sure the visit doesn't happen to fall right in the middle of me having a rough moment (or getting the dressing changed on my dialysis line, which looks rather, um, interesting shall I say?).
Also, public service announcement-- GO DONATE BLOOD. Both IVIG (which my immune system hates but I still need intermittently to replace my confused immune system) and the plasmapheresis require a significant amount of donated blood. I have not been asked to see if any friends or family can donate on my behalf or anything like that, but donating blood in general is critical both for myself and thousands of others. There is currently a national shortage of IVIG that is worsened by a shortage in blood donations (check out this article about the shortage).
Thanks again for all of your love, support, and well wishes!