Scary Setbacks

I've been kind of been procrastinating on writing this blog post a) because I haven't been feeling fantabulous and b) I don't exactly know were to start without coming cross as whiney or dramatic. So-- I guess I'll start off from my last blog post.

After the SCIG fiasco mellowed out, and I was discharged from the hospital, things were going pretty well-- back down at a lower steroid dose, increasing my work hours, working on the two online classes that I'm currently enrolled in, I was home alone with the dog from few days without anything catastrophic going down, etc. As the week progressed, I wasn't feeling fantastic, but I initially assumed that it was from my steroid dose slowly decreasing. However, I get weekly bloodwork, and my white blood count doubled since discharge, which shouldn't happen when decreasing steroids unless there's an infection or inflammation brewing somewhere. My home health nurse and I separately called my doctor's office twice to ask them to check urine and blood cultures because of my weakened immune system and how many UTIs I have had this year. My doctor was out of town most of the week, but the covering provider and other staff assured us that cultures weren't necessary unless I was clearly symptomatic.

Last Friday morning, the 19th, I woke up feeling unwell, but I had a meeting scheduled, so I decided to go into work, and then was going to leave early once my meeting had concluded. On my way in, I called the doctor's office one last time, and told them that I didn't feel right, and thought that I should stress dose my steroids (add an additional dose to avoid a crisis), and that I thought cultures were needed at this point. They told me to not stress dose, and that they would call to "check in" on me in 3 hours.

Well, unfortunately, when they called back 3 hours later, I literally was in the OR being sedated to be intubated. I went into work, and went to my meeting, but my boss immediately noticed my facial droop (when my angioedema/swelling starts, it typically looks like the right half of my face is drooping, but it is actually because fluid fills in the bottom of my cheek), and that my voice was hoarse. At this point, I had already administered a few of my rescue medications, angioedema meds, epi pens, etc, but was continuing to progress. When she saw that nothing I was trying was working, she quickly put me in my wheelchair and took me running to the ER.

They called a Code Blue once I got there, which summons fellows (training docs) and attending physicians (in charge) from Critical Care Medicine, Anesthesiology, and Ear Nose and Throat, respiratory therapists, social work, as well as additional ER nurses and physicians. They tried all of the usual "tricks" that have allowed me to narrowly avoid intubation many times in the past-- an epinephrine (adrenaline) IV drip in addition to epi shots, continuous nebulized (inhaled) epinephrine and albuterol, high flow oxygen, which pushes large volumes of air into the nose to try to open up the airway, and heliox, a helium/oxygen combination with a similar mechanism-- all to no avail.

They were attempting combinations of all of these interventions for around 2 hours, and nothing brought full relief. Any positive response was temporary and partial. At this point, anesthesiology told me that it was a very realistic possibility that they were going to have to take me to the OR to intubate, which is when there is a tube placed into one's airway, and is connected to a ventilator, and takes over breathing for them-- life support. I have been given this warning many times before, but usually something starts helping.

Those two hours were awful. I was absolutely exhausted literally just trying to breathe, not even able to swallow my own spit at that point, unable to breathe through my nose or mouth from the swelling in my mouth and face. Initially, my blood gasses looked ok, but they quickly started to decline as my lactic acid shot up, so they decided it was time to run to the OR. They had already been prepping the OR given the urgency of the situation, so all of the surgeons and staff were present, which of course didn't particularly help an already petrifying situation, but I knew that it was necessary.  There were at least 8-10 doctors in there from the different specialties that responded to the initial code. They told me that they would try to insert the tube through my nose first, which is under partial sedation, and if that failed, that they would go through my mouth, and if that failed, that they would have to put an incision in my throat to do a temporary tracheotomy.

They tried to get the nasal airway twice, but hit swelling both times, so they proceeded with full sedation. I was terrified that I was going to wake up with a trach. Thankfully, once I was fully sedated, they were able to use a small, flexible camera to place the breathing tube through my mouth, and put me on the ventilator.

From there, I was taken to the Pediatric ICU that I work in. I was on the ventilator for about 12 hours, and then remained sedated for an additional 12ish. I woke up Saturday, but I have little to no recollection of Saturday or Sunday other than wanting to try some chicken broth late Saturday (yes, I woke up hangry, which is pretty typical for me). Supposedly I stirred a bit during the time on the ventilator, but like I said, I have zero recollection (which is a good thing, I don't want to have any memory of it).

While I was sedated, they collected blood and urine cultures. Shocker-- my urine cultures were blatantly positive for a UTI, a different organism than I had before. This confirmed my home health nurse and I's suspicions, and in conjunction with a lower steroid dose, can explain the severity of the episode. They were able to ween me off the epinephrine drip, and move me out of the PICU to the step down hematology/oncology floor on Monday. IV antibiotics were started Wednesday. They also re-checked my igG, which is a key part of the immune system that has been low (why we had tried the SCIG). It has gone down further, which was expected, but with the infection in conjunction with the low igG levels and how dangerous infections are for me, they decided to restart IVIG inpatient. CHLA has a small supply of the brand that I can tolerate, so every night, they have been giving me a small amount to fit. Even with pre-medications, these infusions aren't fun-- my body still likes to throw a temper tantrum every step of the way, but at this point, its a necessary evil.

I have the last night of IVIG tonight, and they are continuing to try to ween down my steroids. The hope is to get me home early this week, but they are waiting on urine cultures collected today to see if the antibiotics are working. This whole experience was humbling to say the least-- this is the closest to death I have ever gotten, and I've started processing it emotionally, but I have a long ways to go. I know that it is something that will take time for both myself and my family. I have lost a lot of trust in my primary care doctor's office/academic affiliation, which was already minimal. I'm also naturally concerned that this will happen again, because being intubated for these types of attacks once increases the likelihood that I will require it in the future. We have now had to restart my steroid taper, but thankfully, the only remaining issue I have from the time intubated is a bit of a dry cough primarily from my throat being itchy.

I am very thankful that I was in the right place in the right time, for my boss' response and willing ness to accommodate, my family, co workers, you name it. The staff and physicians here have been compassionate, respectful, and supportive. I asked if there was anything I could have done to avoid this, and other than administering the extra steroids that I was told not to administer, I did everything else "right."

Welcoming Shabbat and celebrating Grandma Shelley!

James!

Tonight is my last night of IVIG for now, and then we continue antibiotics as we wait for the cultures. I was really bummed to have missed my grandmother's unveiling ceremony last Sunday. However, I know she would have understood, and I've been lucky to have familial support. Last night, on her first yartzheit (one year in the Jewish calendar since someone has passed) my cousin Maya came to eat dinner with me, light the Shabbat candles, and chat, which was great. Today I have felt really awful after a 4 AM reaction to the IVIG, but hoping maybe I'll get some sleep tonight. I've been working on some coursework as both a distraction and to avoid falling behind. I just got a furry friend visitor who was kind enough to come on a Saturday as well, so I'll end on a happy note with some pitbull cuddles.

Doggy carpet!

Trials and Errors- Rituxan and SCIG

I can't believe that it's April already! The past few weeks have totally flown by.

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!

Playing tourist in LA with a friend from the Bay Area on one of my good days!