Pebbles and Boulders

I'll get right to it since I am sure that's why you are all here. I do not have Cancer, Hallelujah! However, before you congratulate me, I did not only receive good news this week. Honestly, that was the only good news I received...

I feel like trash. A dumpster on fire filled with hot garbage. This week started out with a nice big infusion of Lactated Ringers, D5, and a Zofran push. My GI tract has been doing extremely poorly the past two weeks and I have become dehydrated to a level that makes everyday life extremely difficult. My blood pressure, which is already naturally low, plummeted, along with my blood sugar. When the nurse started my IV she uttered, "wow, your blood is almost black. You must be really dehydrated! You look like you need some sleep too, honey." I love it when the nurses are honest. Actually, I love it when anyone is honest. Please, don't tell me I look great if I don't, it is extremely devalidating and confusing for me! lol

Midweek I saw the Immunologist. He was incredible; very thorough and clearly interested in my vastly complicated case. He reassured me that my recent labs do not seem to indicate Cancer but instead damage to my bone marrow caused by years and years of immune suppression. Unfortunately, when you're immune system is trying to kill you, you must suppress it. Immunosuppressive medication, while it suppresses your disease, also suppresses vital immune function. A double-edged sword. Without these vital functions, you are more susceptible to infection and Cancers. The drugs I have primarily been taking the past 5-6 years specifically depletes B cells, which are where Immunoglobulin is created. He believes this is why my Immunoglobulin was so severely reduced. Unfortunately, there is nothing that can be done to raise my IgA and IgM as there is no medical treatment to do so. The only Immunoglobulin you can raise is IgG via IVIG. However, my IgG is still 50ish points above the level the immunologist requires to begin IgG replacement therapy. The best way to increase your IgA and IgM would be to halt all immune suppression - and that is clearly not an option for me. We will have to keep a very close eye on these blood counts in the future as the IgG will likely continue to decline - meaning I will eventually require IVIG infusions. He decided to run a gambet of additional blood tests to assess the rest of my immune functions as well. These tests will give us a better idea if my bone marrow is permanently altered, or if perhaps it still contains the humoral memory to reproduce on its own. I will see him again in 4 weeks for the results. 

Next, I saw my Rheumatologist. He was very reassured by the Immunologist's findings and mentioned he truly through I had blood cancer, which is why he called me in such a panic. He also expressed feeling a bit guilty for the destruction of my bone marrow - but we both acknowledged without these years of immune suppression I would already be dead. I knew treatment for this disease had risks, but I also knew not treating it was the bigger risk - so I of course told him he should not feel any blame! Dead vs dealing with side effects seems like a pretty easy choice to me. It was clear that we needed to switch treatment to give my B cells a break from constant depletion. So, instead of continuing Rituxan, he has decided to switch to a drug called Actemra. This immunosuppressive drug works differently and should not deplete my Immunoglobulin as severely as Rituxan. Actemra is an IL6-Cytokine inhibitor. The IL6-Cytokines mediate and modulate immunity, inflammation, and hematopoiesis. They aid cell to cell communication in immune responses, infection, and trauma. For autoimmune patients, IL6-Cytokines go into hyperdrive and disrupt the balance in the immune system. Rituxan has been a miracle drug for me over the past year, improving my lung function and general quality of life tremendously. I am honestly terrified to discontinue it. However, I know my poor B cells need the brake. Trialing different drugs to see what works best for your particular immune dysregulation is something patients like myself become very accustomed to - though it never gets easier or less frightening. While Actemera will come with its own potential side effects, it seems to be the best option at this time. It will be a once a month infusion. 

On top of all this, I currently have an extremely unhealthy amount of inflammation in my body. (I have little sausage fingers again.) After physical evaluation, my doc said about 60% of my muscles and joints are in a state of heightened sensitivity. Plus, I have a raging sinus infection - likely a result of the depleted IgA which normally protects your mucosa from infections. He decided to do a 1000mg Solu-Medrol infusion to immediately calm the inflammation while I was in office. (If you have never had high dose IV steroids just know, it's hell. Everything, even water tastes like metal and you feel like a mentally unstable chipmunk on Adderall for a few days.) He actually wrote orders for one 1000mg Solu-Medrol infusion a month for the next six months to get ahead of this general autoimmune flare before it completely derails my progress over the last year. We are also going to run fluids three times a week for the next month to combat the chronic dehydration. If you're keeping count, that's 14 infusions a month - a number my already damaged veins could not possibly handle. The decision was made to place a port. I had my last port removed back in 2017 and it served me well. While I am devastated to receive another, I know it will save me from so much pain and vascular damage over the next 6 months to a year while my body recovers from this flare. Surgery to place the port, a central line which gives vascular access to the heart, will be Monday. 

Later this week I saw the Oncologist/Hematologist. She agreed with the Immunologist's general impression of my blood work. That my abnormal results are likely due to years of immunosuppression and not Cancer. She was, however, interested to receive the results of the further immune assessments the Immunologist ordered. She assured me if she saw anything off she would order a bone marrow biopsy asap. Honestly, she was my fav physician of the week. She was incredibly knowledgeable and took a whole hour to go over my case in office. Most of my physicians are so overwhelmed by their specialty it's not often I get a doc who genuinely tries to acknowledge my body as a whole instead of separate pieces in a larger cog. She was deeply concerned at the lack of follow up post my Autologous Stem Cell transplant and made a list of doctors and tests that a post-transplant patient like myself should be monitored for. So helpful!

So, it doesn't seem to be Cancer at this time. Of course, I'm THRILLED and incredibly relieved! However, please remember chronic conditions are just as devastating, just as expensive, just as deadly, and just as intensely strenuous. So please don't be quick to congratulate me on all of this mess being, "only a medication side effect." There was no good outcome in this situation, unfortunately. I am now emotionally processing major changes to my treatment plan, 14 infusions a month, upcoming surgery, and an extremely physically tasking flare. Moral of the story, the next few months are going to be full of a lot of adaptation and changes for me. Plus a lot of roid induced crying sessions hehe ;) I know many of you reading this who have followed my journey for years are thinking, "This is nothing! Chanel has dealt with so much worse!" While that is technically an accurate thought process, I am still human. I still feel disappointed. I still feel angst when changes arise. I still don't enjoy being cut into and having medical devices placed into me. Having a chronic illness never gets easier. It never gets less exhausting. Over time each little set back begins to feel so heavy. Pebbles don't weigh much alone but added up can be heavier than a boulder. 

I am still pretty jittery and emotionally fragile from the massive steroid dose, so please excuse this very frank, jumbled, and extremely angsty blog post. It is hard to keep my thoughts coherent and pleasant when I haven't slept in almost 48 hours... 

Xoxoxo, Chanel