IVIG Day #2, TPN Day #4

Thanks again for everyone's well wishes and support over the last couple of days! It certainly has felt like a bit of a time warp, which I guess is kind of to be expected when you're in the same room for a while. Since my last post, I had my first three days of Total Parenteral Nutrition (TPN), and two days of IVIG (intravenous immunoglobulin).

For those of you who have been following my story for a while, I have been receiving IVIG monthly, and then bi-weekly since November. My first admission of the past couple of weeks occurred after an adverse reaction to my standard IVIG infusion. IVIG has helped immensely, so we were not ready to give that up unless there was really persuasive evidence that I could no longer tolerate IVIG. To test that, we have been doing 1/3 of my usual IVIG dose each day, with the third and final day tomorrow. With the high dose IV steroids I have been receiving for almost 2 weeks, these infusions went smoother, which overall is a good sign. I still react to the infusions, but have responded well to the meds to treat the reaction symptoms.

Awesome surprises!

The first 24 hours on the TPN was kind of rough. I felt even more run down/beat up than usual, almost as if I had the flu, and some reaction symptoms. My body seems to have adjusted well to it, and now that I'm actually getting the nutrition that I have been so short on for so long, I am already noticing a difference in my strength, energy, and thinking. When I came into the hospital, I could barely walk because I was so weak, and experiencing arrhythmias from low electrolytes, which have now almost entirely resolved.

We are still working my way up to a "full" daily dose of TPN. Because of how malnourished I was, they have to slowly add in nutrition to avoid what is known as Refeeding Syndrome. Refeeding Syndrome occurs when the body is overwhelmed by nutrients if they are introduced too quickly after extended periods of malnutrition, and can cause issues with electrolytes, metabolism, etc. They watch for it closely through daily labs, and adjust the "cocktail" of vitamins, minerals, calories, and electrolytes in the TPN accordingly.

My labs are still showing significant issues with electrolytes, which again, is absolutely no surprise because of the levels I was starting with and the continued auto-immune process impacting my kidneys and gut. In addition to the TPN, I have been getting additional infusions of potassium and phosphorus.

Yellow= TPN, White= Lipids, Clear = Benadryl

I haven't gained weight (supposed to take time, not overnight), and my electrolytes are improved but certainly not resolved, which means that the plan is to continue TPN after I am discharged. I mentioned this in my last post as well, but usually TPN is used for individuals with little to no ability to consume food or use their GI tract. In my case, it is being used as a supplement to get me closer to a healthy weight, maintain electrolytes, and a minimum of 1,000 calories a day of intake. The hope is that over time, we will wean me off the TPN in a few months. I still have concerns of infection, practicality, and the impact on the rest of my organs related to the TPN, but at this point, it's what we need to sustain me in order to even explore other treatment options down the line.

After the final day of IVIG tomorrow, the plan is to over the weekend to slowly start decreasing the IV steroids. I'm still on a higher dose of steroids than I was re-admitted on, so that also is going to take time and patience. Real conversations of discharge haven't started yet (other than confirming that the TPN is sticking around), so realistically speaking, we are looking at mid next week.

For those of you who are curious what goes into TPN. Lipids are a separate bag.

I'm of course still majorly bummed about graduate school and the overall conversations about treatment options and such, but I'm glad we are at the very least seeing progress. My def The team here that is part of the UCLA network has been amazing, compassionate, patient, and realistic about the situation at hand. They have taken the time to listen to us/our concerns, and helped me continue to maintain confidence when I bring about concerns or questions. I am a little irked that nobody was more aggressive about nutritional support up until this point, but there's not much that can be done about it.
In the mean time, the snacking/Netflix/coloring/sticker book/tutoring saga continues. I am SO appreciative of the visitors, messages, gifts, food deliveries, and support over the past few days. It really does help pass the time and keep me occupied. I'm hoping for a very uneventful weekend with the steroid decrease so I can make my jailbreak (a much longer one) next week!

Another reminder that if you haven't already, please donate to the Denim Dash for Rare Diseases! It is a walk/roll/stroll 5K (or however much you can) for rare diseases such as those that obviously dramatically impact my life. You do NOT have to participate in the Dash to help out. DONATE HERE!