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!

Back to Back

Forgive the title of the blog post-- I'm running out of cheesy back related puns :P

Since my last blog post, I had my Disneyland 24 hour mini vacation, which was amazing! It was a welcome break from my back to back appointments, treatments, and such that seem to go on and on. I wasn't able to go on some of my favorite rides, which was disappointing, but I was really pleasantly surprised that there were rides I could safely go on and enjoy. I used my own wheelchair in Downtown Disney, but had a hard time propelling myself, so I rented a scooter to go into California Adventure. It was great to spend time with my friend Brittany and her mom, who live in Seattle, that I rarely get to see, and to hunt down all my favorite Disney food finds. I may or may not have brought home a giant Jack Jack Cookie Nom Nom, on top of a safe Mickey Ice Cream bar, a nut free pasta dish... Disney certainly knows how to do allergy friendly food right.

Disney!

After the fun in Disney, I was quickly back to my back to back appointments and not-so fun stuff routine. I was nervous for my two appointment with the orthopedists because I have been getting some scary muscle spasms in my right leg, plus the back pain, and the finding of the compression fractures in my MRI that I mentioned in my last post. The first appointment did not go well. It seemed like the doctor was trying to be nice, and felt bad that he couldn't explain my pain, but just said that from the one view of my MRI that there were no explanation for my pain, no compression fractures, just some degeneration from my osteoporosis. That would be absolutely lovely if that was the case, but I found it very hard to believe that 2 radiologists, a hospitalists, my primary care doctor, and a neurosurgeon were all wrong about the fractures in my thoracic spine at T11 and T12. I left the appointment upset, not because he told me that there were no fractures, but because I felt completely confused and defeated. I was really upset leaving the appointment, but taking a glass staining class that I received for Hanukkah from my dad and step mom was a good distraction until the appointment the next day.

I actually almost canceled my second opinion appointment because I was so frustrated from the first one. I'm glad I didn't (ha ha get it-- another back to back day of appointments). This doctor took 3-4x the amount of time with us in comparison to the other one. He went through my MRIs vertebrae by vertebrae, and from multiple angles. The doctor the day before only looked at one angle, and only looked at the vertebrae in question. The second doctor pulled up tons of dimensions of images form the MRI and immediately identified and showed us what the fractures at T11 and T12 looked like, and in addition, found a third fracture at T6. The fracture at T6 is considered to be an "acute" fracture, as it still has fluid around the fracture. Neither radiologist caught it, but even my dad and I were able to very clearly see it once the doctor scrolled through the MRI.

It may seem silly to be happy that there is a third compression fracture, and a more noticeable one, but in my mind, I felt so relieved that there was now a very clear explanation for my pain. It explains why my pain is so severe (because it's a newer fracture), where my pain is, and also gives us hope that once I am out of the "acute" phase, the pain will dissipate, which should take about 8-12 weeks. In the mean time, I am keeping up with heat, IV tylenol, a numbing gel, and I was able to start physical therapy today. He was hopeful that the new injected osteoporosis medication I am on should help prevent my osteoporosis from progressing, and that he has seen really fantastic results from this particular medication in other patients. For now, I have enough room between the fractures and my spinal cord. However, I have to be VERY careful to avoid future injury. He also believes that the scary leg spasms are electrolyte related, so hopefully supplementing those will prevent any future spasms.

Having a third fracture also adds more evidence to our existing concern that the steroids are absolutely destroying my body. My steroid dose has been decreasing over time, which the Rituxan has helped with, but they don't feel like it is decreasing fast enough. In fact, the Rituxan has been making a HUGE difference-- I have been having a lot fewer angioedema episodes, and my steroids are the lowest they have been in a while. However, to continue to decrease the steroids (and to decrease them faster), they decided to switch my Rituxan to a more "nuclear" one, which is more similar to the protocol they use to treat cancer. This means that I am going to have 2 8 hour infusions a week apart to completely nuke my immune system. The first one is this upcoming Friday. I'm a little nervous for the infusion, because of the side effects, risk of infusion reaction, and the consequences of being very immune compromised, but I know these are all risk that have to be taken if I want to avoid further rapid progression. Thankfully, CHLA was able to make an exception to allow me to do these infusions there in case there are adverse reactions or if the speed of the infusion needs to be slowed down.

Until Friday, I have been working on starting up a local chapter of Sick Chicks, which is a non-profit that is focused on empowerment for young ill and/or disabled women (Sick Chicks Website), tutoring, and baking. My mom also was in town for 24 hours, which was a blast-- we were able to go to some of my favorite restaurants, shop in a warehouse of a designer I like, and hang out. I'm looking forward to seeing the final result from the glass class I took last week. I'll make sure to post a picture once it has been fired in the kiln!

Some Back-Breaking Stuff (Literally) and Rituxan Round 2

As promised, here is a blog post that is not the length of a novel, nor overdue!

Some of you know from Facebook or chatting with me that I was re-admitted to the hospital the day after Valentine's Day. I called my doctor because my bloodwork was showing that my white blood cell count increased, which is typically indicative of infection, and I felt that my UTI had come back yet again. My pain near my kidneys was back, I felt crummy overall, etc. My doctor didn't have enough time to call me back (yes, I am still bitter about that), but their secretary got me in with a general family medicine doctor. My step mom took me to that appointment, and unsurprisingly, the doctor took one look at me, my records, and my labs, ran a repeat urine test showing protein and blood, and sent me to the ER.

We were in the elevator to head to the hospital, and I passed out twice. I have passed out many times before for standing in one place for too long, but this one was different. I felt like the elevator had moved, even though it hadn't, and I wasn't particularly dizzy before I fell. I came back "with it" pretty quickly, but then I had an angioedema (swelling) attack of my lips, face, throat, etc, so I used my EpiPen and 911 was called. When I got to the ER, my lactic acid was sky high (another indicator of infection), my white blood cell count was still elevated, my urine was positive for blood and bacteria, and the started me on an epinephrine drip to because the EpiPens and shots of epinephrine were not enough. My step mom brought the fast acting angioedema med from home, which they also administered.

They presumed I was septic because of the lab tests, but turns out, my cultures (both blood and urine), which is the true indicator of whether or not there was an infection, were totally negative. A nephrologist came and saw me because I have always been suspected of having renal tubular acidosis, which is caused by autoimmune disease and leads to electrolyte imbalances. He confirmed that because my electrolytes were a hair off, that it was likely I have it, but it wasn't acting up horrendously at the time, and also would not explain my pain.

I was still in miserable amounts of pain, so because I have pretty significant osteoporosis because of being on chronic steroids, the doctor ordered x-rays of my spine. Here comes the "back breaking stuff" reference in the title-- turns out that they found a compression fracture of my T12 vertebrae.

That prompted a lumbar MRI, which confirmed that fracture, but thankfully, that it was minimal and an older fracture, so surgical intervention wouldn't help. This kind of explained the pain that I have been in/am in, but not entirely. A neurosurgeon came and saw me, and when I pointed out where the pain was, was concerned that I could have additional fractures or spine issues. He ordered another MRI, a thoracic MRI, which showed that T11 is also fractured, and I have three mild bulging disks from T4-T5, T5-T6, and T6-T7. This combination of the 2 fractured vertebrae (T11 and T12) plus 3 bulging disks certainly could explain my pain.

Because the fractures are related to steroid induced osteoporosis, they were able to justify giving me my next Rituxan infusion which I was already overdue for. My last and first infusion was at the end of December, and was extremely helpful. It is a powerful immunosuppressant typically used for lymphoma, but has recently been identified as useful for autoimmune disease and dysautonomia (both of which I have). It is hard to get approval for it because it is a risky drug, wipes out a good chunk of the immune system, and in my case, is off label. However, since I was inpatient, and had the evidence of the fractures, they were able to infuse the Rituxan because it ultimately should help reduce my steroid dose.

I haven't seen the neurosurgeon since the second fracture revealing the more extensive damage in my thoracic spine because the neurosurgeon is out of network for my insurance. I am thankful that it doesn't appear that surgical intervention will be necessary, and that they are currently mild, but I am terrified of the long term implications of this, because I know that it is likely to worsen over time, and in the few people I know who have had similar issues, it hasn't ended well. The disks are bulging towards my spinal cord, and don't appear to be affecting it yet, but it concerns me that they could.

The pain is also a challenge to deal with, which also adds to my fear of things worsening. Things are generally controllable at the moment with IV Tylenol, but I want to see what can be done now to prevent things from worsening, if there is anything. At the hospital, they just said to strengthen my core, but now with the bulging disks on top of the fractures, I am wondering if any sort of bracing, use of something like a TENS unit, physical therapy, or other interventions could help slow progression of things. It's challenging in my situation because there are so many complexities.

I've already been on osteoporosis medication for a year, but I'm clearly not absorbing it (which I told them was a concern when they put me on an oral osteoporosis drug with known absorption issues), so I am supposed to switch to a daily subcutaneous (under the skin) injection. The Rituxan infusions were approved by my insurance (finally) to continue outpatient, but I thankfully shouldn't need another for a couple of months. I have 3 different consultations with different spine specialists over the next two weeks to ensure that we explore all options possible.

I also should be starting subcutaneous immune globulin, which is a form of immune replacement, in the next week or so. This is similar to the IVIG I used to receive, but just under the skin, which is supposed to help make it less likely to cause a reaction. From the nephrology/electrolyte standpoint, my blood work is just being very closely monitored and replaced as necessary.

As with any hospitalization, this admission wasn't without its challenges. There were some communication challenges at first where changes were attempted made to my regimen without discussion, as they didn't quite get the nuances of MCAS (ex. putting me on a different multivitamin even though I brought in the only brand that I have been able to safely tolerate). These were sorted out once they were explained, but nevertheless, annoying.

The worst incident that was pretty re-traumatizing for me was that after I was moved to the Oncology floor for the Rituxan, I was told that I was being taken for a chest CT scan. I didn't know why, because I had already had the MRIs, and the doctor hadn't mentioned it to me, but I said that it was ok as long as there was no contrast (I'm severely allergic). I was assured that it was contrast free. I get to the scanner, and they are preparing and getting ready to hang a medication on my IV pole. I asked what it was-- contrast. I immediately told them to stop, that I was allergic, and that they needed to look at my chart. Once they look at it, they saw the allergy, and didn't actually see anywhere in the notes why the scan was being ordered in the first place.

I start having a full blown panic attack because this totally triggered my PTSD related to medical trauma, since they almost gave me a medication that I was allergic too (and a documented allergy!). They had no benadryl with them, and I'm sure there is probably stock epinephrine somewhere in the room, but nobody present would have been qualified to administer it. I took a picture of the scanner and how close the power injector was to my IV pole-- I was already in the scanner! They called the doctor to see if they wanted a scan without contrast. They came back in, and told me that we were going back upstairs. Ready for what the doctor said?..........THEY ORDERED THE CT SCAN WITH CONTRAST ON THE WRONG PATIENT. And that patient happened to be one who is deathly allergic to contrast.

The photo I took while I was laying on the scanner bed with the power injector in the corner. The info on the screen with my name, birthday, medical record #, etc were correct-- it was the CT Chest with Contrast that should have been ordered for a different patient.

I still was freaking out, my dad was furious, and so were the nurses-- this mistake was so blatantly reckless, and we are all SO lucky that I caught it prior to the injection. They would not have been prepared to handle the consequences of this mistake, and it was a completely unnecessary test in the first place.

When the doctor came in the next day, we asked what happened. She apologized and admitted to ordering the scan on the wrong patient. She said she had both charts open, and was on the phone with a specialist about the other patient who needed this chest CT with contrast. Instead of entering the orders into their chart, she entered it into mine. I still can't believe this happened, and again, I'm so glad I caught it. She said she was glad too, but needless to say, the little trust I had was pretty much shattered from there.

To help buffer and lighten the mood after the CT fiasco, my dad and I ordered a good pizza for dinner because my hospital-provided pizza was inedible. It was rock hard, and going in the trash, so after we finished the yummy pizza delivery, my dad initiated a game of hospital pizza bocce ball (see video below), and "Where's Waldo, The Pizza Edition." If you look carefully in the photo, maybe you can find the inedible pizza :P

Where's Waldo? The Pizza Edition #1

Where's Waldo? The Pizza Edition #2

I got home last Friday night after a week long admission. I'm so glad to be home, and this week is primarily filled with follow ups, calling doctor's offices and the pharmacy, etc. I am going back to work again later this week. I'm annoyed to not really have many answers on the situation with my spine, but I'm hoping the appointments next week and the following week will shed some light on a potential course of action/expected progression.

In terms of actual fun stuff-- I am looking forward to returning to work, and seeing a friend from out of town at Disneyland Monday! I think my Mickey break will be a welcome distraction from everything else going on :) We also went to Malibu for lunch Sunday for a little mini day-cation, and ran into Gordon Ramsey at Starbucks when I went on a restroom break-- Cody was brave enough to go up and ask for a photo! I didn't have the guts to, but now regret not doing so...

Cody and Gordon Ramsay

A New Form of Patient Insight and Fighting Against a Broken Immune System

Oh boy-- the daunting task of attempting to describe was has transpired over the last few months....  A good way to sum it up is two step forwards, one step back, a lot of frustration, and navigating a broken healthcare system. A few people have reached out to ask why I haven't written a post in a while, and to be honest, I haven't simply because there are so many questions, and frustrations that accompany a lack of answers. That does not mean, however, that things have been all bad-- we have made some progress, and I'm hopeful that we are starting to go on the right track.

 For those of you who are friends with me on Facebook, you may have seen that I was hospitalized in the Pediatric Intensive Care Unit (PICU) at CHLA not once, but twice. And for how I ended up there-- I'm too old to be a CHLA patient, but I do work there one day a week at a Family Resource Coach (actually in the PICU too). The UTI that I mentioned in November that I had 2 recurrences of came back the first week of December, only a few days after finishing the second course of IV antibiotics. I had been in a flare for a few days, which I later found out was because of another recurrence of the infection, and had a few really frightening swelling attacks that was not responding to medication. I was at our department Holiday Lunch, and started swelling (no, it was not from the food, my body was just already in "fight mode." I told my boss that I may need to call into a meeting we had scheduled later that day, and when she saw the swelling in my face, she immediately took me to the ER.

Even though it was just down the hall, by the time I got there, I was a mess. I received upwards of 5-6 doses of epinephrine, starting an epi drip, steroids, additional benadryl, and breathing treatments, and was still in respiratory distress. Because CHLA has inpatient/in house allergy, an allergist was at the ER within 5-10 minutes of my arrival, which was really really helpful. She was able to see exactly what happens during an attack, and my medication response. My specialists outpatient have seen pictures, reports, talked to ER doctors, etc, but never have seen my worst attacks. The swelling in my tongue was severe, and the ER doctor didn't think that she could intubate me because of it (placing a tube in the throat to take over breathing), so she called anesthesiology and ENT. They told me that they were prepping a OR because they were thinking they were going to have to intubate, and if they couldn't get the intubation, to place a tracheostomy. They had me sign consent and all-- it was petrifying. The ENT placed a scope through my nose to look in my throat to see if there was an obstruction in my throat or if it was primarily in my upper airway. Thankfully, the swelling was all in my mouth and upper airway, so they did not have to take me to the OR, nor intubate me. The combination of the epi drip and epinephrine shots started to kick in, so they moved me to the PICU.

The allergist who was in the ER with me was fantastic, and was able to take care of me throughout my stay, and consulted with my outpatient team since she did witness everything. There was a lot of difficulty at first weening me off the epinephrine drip. After I was weened, they kept me on the step down unit for a while to reduce my steroid dose as fast as possible, because I am having so many complications from the steroids (osteoporosis, scoliosis, cataracts, you name it).  I also completed an additional antibiotic dose (and meet Carly Rae Jepsen at the same time too :P) I spent almost all of Hanukkah in the hospital, but had my parents bring in an electric menorah, and I converted a plastic Christmas tree that a non profit organization distributed into a "Hanukkah bush." .Click here for a cool video of what the LAPD did for the Holidays.

The Hanukkah Bush

When I got home, I was ok for a few days, but still struggling. I started having daily attacks again that weren't responding well to meds. We called the on call CHLA allergist, who told me to come back into the ER (an age exception was made because I was just discharged a few days prior). We did a mad dash to the CHLA ER, which is an hour and a half away, and made it just in time. By the time we got there, I was in full blown anaphylaxis again, with severe angioedema (swelling). My blood pressure was something like 86/65, and I was placed back on an epinephrine drip.

Sure enough, the infection was back, again.  I was moved from the PICU to a stepdown unit after a few days. They started more aggressive antibiotics, and I completed another course in the hospital. We began discussing what treatment options are still available because I've exhausted so many, and obviously what we were doing wasn't working. The allergy team at CHLA spoke with my outpatient team, and were deciding between plasmaphrereis, a more invasive procedure similar to dialysis which I mentioned in my last post, and Rituxan, which is a pretty nuclear immunosuppressant that is used to treat certain cancers and severe cases of autoimmune disease. They ultimately decided on Rituxan because of the invasive nature of plasmapheresis with minimal data to support it, and the hope to preserve my quality of life as much as possible.

What happens when you spend Christmas in a Children's Hospital...

Once the antibiotics were done, they moved me to the Bone Marrow Transplant unit to receive my Rituxan infusion, because the PICU was full, and the BMT unit is the unit that uses it the most. Rituxan is notorious for causing infusion reactions in a lot of people, even those who are not prone to reactions in any way. Infusion reaction and over "nuking" my immune system were the biggest risks/concerns. Its an incredibly fragile balance between immune suppression to prevent the autoimmune issues from progressing and avoiding infections. I received the dose on the BMT unit, and thankfully, had no major issues.

On the day of discharge, New Year's Eve, the UTI symptoms returned again. I asked them to do a repeat culture, and then they could call me if it was positive. They reluctantly agreed. 3 days later, on 1/2, I got the call that those cultures were positive-- again. Now the concern was escalated because I had just received a really potent immunosuppressant drug, in addition to the fact that I was already pretty immune compromised.

I started another two week course of IV antibiotics at home. I was still having pretty severe kidney pain, and we didn't know why the infection keeps coming back. My primary care doctor had me get a renal ultrasound. During the ultrasound, the tech was like, "well, I see why you're in pain." The pain from them pushing the ultrasound probe was so bad I literally had to use an EpiPen mid ultrasound because I was reacting to the pain. She said that I had 6 kidney stones, including one I was currently passing. I recieved a call from my doctor the next day confirming this. I was having pain, but not what I would expect with kidney stones.

They referred me to Urology, who said I would need to have surgery to both blast and physically remove the stones, because one was larger than 8 mm, in addition to one or two stones that were around 5 mm. She had me get a CT to confirm stone location prior to surgery. I got the CT, and got an email the next morning-- no stones. Half of the doctors I have spoken to who have seen the ultrasound photos have said that I absolutely had stones, and passed them, while the other half said that what they measured on the imaging was shadows. Regardless, there are no stones there now, but I am retaining some fluid in my kidneys, which could be causing some of my discomfort.

I was so confused and frustrated. Of course, I was thrilled to not have kidney stones nor need surgery, but at least having kidney stones would explain both my pain and why the infection kept coming back. I was told my repeat urine culture was clean, so I stopped the oral antibiotics the urologist gave me to take for a week to ensure the infection was gone. The urologist never personally called me to explain why the ultrasound was misread, or how the stones passed with pain, but not excruitiating pain. Thus, I never was told to continue the antibiotics.

After only 2 days off antibiotics, the infection was back, again. I was so frustrated that nobody from the urologist's office had followed through. My doctor even called their office, to see what they suggested in regard to the infection coming back again, and they didn't even respond to my doctor. The oral antibiotics were not absorbing, which I told them was a concern, but nobody listened. My doctor was waiting to start IV antibiotics until they heard from Urology.

I called my doctor because by day 3, I felt awful. I was weak, nauseous, had an insanely painful headache, and literally crying in a ball on the couch in pain. I don't know how to describe it beyond that, other than the fact I literally felt like I was dying (I know it sounds melodramatic, but that's how bad I felt). He said to go to the local ER for blood cultures, and that he would call ahead so they didn't panic.

I got to the ER, and per protocol, they called "Code Sepsis," which basically means that you are seen immediately, get an immediate chest x ray, EKG, and blood cultures. They were expecting me already. The doctor at the local ER was fantastic. Unfortunately, the local hospital is not able to handle my allergic issues/angioedema, simply because they aren't adequately equipped, but they were able to treat an infection.

The doctor came in and said that my lactic acid was really high, causing acidosis, where the blood literally turns acidic, and is one of the most telltale indicators of sepsis. That explained why I felt so sick. I was admitted to the Cardiac ICU (where they had a bed) to start aggressive antibiotics and fluids. Initially, I was told that it was urosepsis, but later, different doctors said different things on whether this episode was actually sepsis, or just a severe kidney infection and UTI. Regardless, I was on antibiotics yet again. In the hospital, they gave me a small dose of IVIG (Intravenous Immune Globulin) divided over three days to try to give me some immune system to fight the infection on top of antibiotics. I used to be on IVIG for about a year, which was helpful-- it has the antibodies from thousands of blood donors in one dose to help build an immune system. I had to stop it because it was causing swelling and reactions. This admission, by dividing it up over three days, giving me a ton of pre-medication, and insanely slowly, they were able to give me one dose. However, it wouldn't be sustainable to do that regimen outpatient.

I'll finish up that round of antibiotics in 2 days. I already did a culture Friday to see if the infection is still there. Because of the infection, I haven't been able to receive the next dose of Rituxan although I'm long overdue. I also saw an angioedema specialist since my previous 2 admissions, who said that my bloodwork is indicative that I have autoimmune acquired angioedema, which is extremely extremely rare (even more so Than the other rare conditions I have). There also are little to no cases documented of individuals who have both MCAS and the autoimmune acquired angioedema as I do. I was placed on two new angioedema meds, which I don't think are really doing anything, but at least aren't hurting.

Because of this cluster, CHLA allergy can make an age exception to continue to see and follow me under the premise of a rare condition. They are working on spearheading the Rituxan, and starting SCIG, which is a different form of IVIG under the skin that they hope that I'll react to less. However, they are limited because I'm too old for any of the other specialities or auxiliary services there, including being transferred there in an episode. The local hospital isn't well equipped to handle angioedema, and usually transfers me out, but UCLA, the other option, has been an absolutely nightmare. That basically means we have to mad dash to CHLA when Im in a swelling episode.

I have had follow ups since these admissions back at the Mayo Clinic in Arizona, and San Diego. Everyone is very much in support of continuing the Rituxan once the infection is gone, and staying SCIG. Since the infection seems to have been fueling the angioedema, we hope that once the infection is gone and stay away, that the angioedema may calm down. We still aren't sure why the infection keeps returning other than the fact that I have little to no immune system right now.

So, as you can see, there has been a LOT of back and forth, which has required extensive navigation efforts, and caused an immense amount of frustration. I've been trying really hard to keep a positive attitude about it, but it is really frustrating when I have been telling providers that there's an issue, or that there's a potential issue, and I'm blown off, or told to "watch and wait." Clearly, the "watch and wait" has been costly. They literally have been waiting till I'm in really critical condition before someone steps in, which has been traumatic not only for myself, but also for my family.

I thankfully was able to work one day last week, and it was great to be back on the "other side" of things. I have continued my online tutoring, and I've also been doing some online health literacy material review for CHLA, since that is a specialty of mine, to keep busy. Having these experiences as a patient at CHLA have provided me with new ideas and insights into programming. I've also been doing some "diamond art" to keep myself busy. If the cultures from last week come back clean, I should be able to get the next dose of Rituxan soon if insurance approval works out.

I've also been able to have some non health related fun, which has been a very very welcome break. A couple of weeks ago, I got to go to a Kelly Clarkson concert with my step mom, which was absolutely amazing. This past weekend, I stayed with a friend who also has MCAS/POTS/etc, and we went to a Gluten Free/Allergy Friendly Food Expo, which we both really enjoyed. I got to spend some quality time with my aunt and grandmother while I was in Arizona for my appointments, and met someone in person with similar struggles who I have been talking to online for a while, but never met face to face.

Over the next few weeks, I'm hoping to see continued progress, improvement, hopefully clean cultures, and getting back into the groove at work. I'll work on making my next post not as long and not as overdue!

Meeting up with online friends!

Checking out the Gluten Free/Allergy Friendly Expo

Thankful and Bye Bye TPN!

Although my last few blog updates have been relatively negative, I wanted to start off this post (which is finally more positive) acknowledging the fact that I have so much to be thankful for. The past few months have been absolutely positively brutal, but the past couple of weeks have served as a reminder to take a breather and count my blessings.
 In my last post, I mentioned the severe anemia and admission to UCLA in Santa Monica for the blood transfusion, iron infusion, and PICC line replacement. That seemed to help with my energy, and the new PICC line they placed seems to be agreeing with me more than the Hickman and my last PICC did. It turns out that what we kept thinking were infections were actually a reaction to the material that the actual line is made of. It is currently being used for the continuous IV Benadryl, fluids, Pepcid, and steroids.As we anticipated, the GI scopes/biopsies and genetic testing didn’t reveal anything helpful, although as more is understood about MCAS and the other conditions that impact me down the road, it could come in handy for me or other patients.
 All of the central line frustration and concerns raised the urgency to get me off TPN even though I am not up to the goal weight yet. My oral food intake has been up, so we decided to cut my TPN in half for a week, and monitor my weight and electrolytes, and then reassess. They both seemed to hold for the week of half TPN, so we cut it entirely. I have now been off TPN for about 2 weeks, and so far, my weight is holding (thing to be thankful for #1). My electrolytes have been borderline a couple of times, but not to the severity they were before starting TPN. I am still supposed to work on gaining more weight, but as long as I’m maintaining or gaining, and not losing, I should be able to stay off of it! It certainly is nice to cut out that prep work from my nightly routine.
 I have been also trying my best to work one day a week for both my sanity and a change of environment. My boss has continued to be extraordinarily supportive, and I love what I do (thing to be thankful for #2). The commute is brutal, as it can take up to 2 hours each direction with traffic, which is absolutely exhausting for me but one day a week is worth it. Having that day where I am NOT the patient is refreshing.

Dogs dressed up for Thanksgiving at work! 

November 8^th was a challenging day for my family and community as a whole. For those of you who don’t know, my dad, step mom, brother, and I live in Thousand Oaks, CA. A little after midnight on 11/8 I was woken up by Facebook emergency alerts stating that a shooting had occurred at Borderline Bar & Grill, a restaurant close by, which actually shared a parking lot with my infusion center. I had not been there before. Information was just coming out since the shooting had only occurred an hour before, but by the time I woke up in the morning, the details of the atrocity began to emerge. It felt surreal to be seeing Thousand Oaks and buildings that I recognized on CNN. I did not know anybody who was injured or lost their life during this incident (thing to be thankful #3), but I know many people who did. One of the umpires for my brother’s baseball league that him and my father recognized was one of the victims.
 That morning already felt like a time warp, and I was scheduled to go to work that day. I left early because part of the freeway was still blocked off, and I would have to pass the shooting scene on the freeway to get to work. When I did drive past on the freeway, it was eerie—the parking lot was still full of cars from the night before, cars that I knew belonged to the victims. My thoughts were with those who had loved ones there, and I could only imagine the pain they must have been experiencing.
 I was at work, and by the early afternoon, I was experiencing symptoms of a mast cell flare up (itching, swelling of my lips and tongue, hives, etc.). I gave myself additional Benadryl, inhaler, etc, but the episode didn’t seem to be fully responding. I was terrified of having an episode in a patient’s room or not being able to make it to a reliable medical facility if I needed emergency medical attention since I am not supposed to use the one at Children’s. I decided it would be best to head home early so I would have access to more of my emergency meds if needed. My step mom texted me to let me know that I should wear a mask when I got home because there was a fire on the other side of the freeway, but I didn’t really think twice about it.
 I was approaching the 101/405 interchange when I got a call from my dad saying that our neighborhood was just placed under mandatory evacuation orders, and that they were packing up the essentials and needed to leave now. The fire jumped the freeway, and we received emergency notifications saying that we needed to immediately evacuate. This was the Hill fire that started while I was at work, the Woolsey fire had not started yet.
 Before I got this call, I was already debating pulling off the freeway to use the EpiPen or go to an ER because I wasn’t feeling well, and I still wasn’t responding to my other emergency medications. I decided to get on the 405 instead because I have family who lives close to UCLA, and I figured I should be close to where I could receive medical care if I needed it. I was on the pass and still couldn’t control my symptoms. I looked to find a place to pull over to use my EpiPen and call 911 if that didn’t work, but I didn’t realize there aren’t many places to pull over on the pass (don’t try this at home kids). There were only 2 exits until the exit for the main UCLA hospital, each a couple of miles apart. If I got off the freeway there, it would have taken longer for EMS to get to me. Traffic was moving on the freeway, so I tried to get as close to the hospital as I could before I pulled over. I got off the freeway, and of course, still no where to pull over. I ended up having to use the EpiPen at a red light. I finally found a place to pull over, but by then, I was 0.25 miles away from the hospital, so it was faster for me to just keep going. I had a friend on the phone who knew my location the entire time in case something happened.
 The entire drive was absolutely terrifying to say the least, but I was so thankful (reason #4) that I got to the hospital safely, and that things didn’t hit the fan until after I got there. I tried to wait a few minutes in my car in the parking lot, trying to convince myself that I didn’t need to go into the ER, but things were worsening, so I went in. I’m glad I eventually did because even though they didn’t even triage me before pushing me back to the trauma bay, my oxygen saturation was in the 80s while my heart rate was around 150. They immediately gave me additional epinephrine, fluids, medications, etc. A cousin came to sit with me which was much appreciated, because it was scary to be going through the ER with the trauma I experienced at that hospital the last time while literally watching my home town burn on live TV.

What I was watching on TV...

This reaction was stubborn—I ended up getting a total of four or five doses of epinephrine, which landed me back in the MICU. They mixed an epinephrine drip to have on standby, but thankfully (reason #5) they didn’t have to use it. This hospitalization was a lot better than my previous ones there. It COULD have been related to the complaint letter that I wrote and received the response/investigation findings from the day before I was re-admitted. Regardless, I was glad that this experience was slightly more positive given how much stress was involved both in the hospital and with the wildfires and the shooting all on the same day.

Meanwhile, my family evacuated, and had to re-evacuate 2 more times, as each place they were evacuating to was evacuated as the mandatory orders spread with the Woolsey fire. They returned home on Saturday evening. Thankfully (reason #6), our house was totally fine, which was a HUGE relief. With the whole idea of giving thanks/thanksgiving, seeing the devastation from the shooting and the fires all around our area has been sobering. The damage everywhere is extensive, and although the fires are gone, the smell of smoke is still strong at times. We are lucky that we have our home to seek refuge in, but hundreds of families weren’t that lucky, made worse by the fact that this all happened right before the holiday season.
 I was discharged late the next Monday evening (11/12), because we had to wait for the reaction to calm down, the air to clear up a bit, and I ended up developing a UTI that was causing some kidney pain while I was there. I was home in ample time for Thanksgiving, and I have been able to return to work twice since that admission (thing to be thankful for #6).
 Despite the immense amount of stress and chaos Thousand Oaks has had over the last couple of weeks, things seem to be returning to status quo. All schools re-open tomorrow for the first time in about 2 and a half weeks (my brother is NOT excited). Roads are re-opened, and most people have returned to work. The signs (picture to the right with mention of the Borderline 12, a reference to those who passed in the shooting, are no longer on all of the major thoroughfares. As I said earlier but will say again because of the gravity of the situation—I am THANKFUL that our family is able to return to our status quo.

 In terms of what I’m up to, things are still pretty up in the air. I had a consultation with a hematologist who specializes in MCAS in Orange County a few days before the chaos. She agreed that I likely have a hereditary angioedema (swelling) or secondary angioedema component to my symptoms. The angioedema is usually what keeps me in the hospital since it has the potential to block my airway. I was started on a medication for hereditary angioedema just to see if it makes a difference, even though we aren’t confident that my angioedema is specifically hereditary. My insurance has not approved the medication, but the manufacturer covers the first month.
 Because of the additional hospitalization, the hematologist and my primary mast cell physician told me that they feel it is now time for me to start plasmapheresis, which is a treatment in which the blood is filtered to remove auto-antibodies. Unfortunately, this would dramatically change my routine, as it starts off at 4-6 hours 5 days a week, and no facilities close to home are properly equipped. I am waiting to hear about what the logistics of this will be, and then I’ll post an update explaining more. In the meantime, I had the first dose of the angioedema medication, and am trying a liquid iron supplement to see if I can absorb it in hopes of avoiding more blood and/or iron transfusions in the future. I also am supposed to go back to Mayo for a follow up in December to see if they have any additional suggestions. I also need to make a decision about graduate school somewhat soon-ish, so I’m working on looking into my options and access to accommodations to see what is feasible. I am back on IV antibiotics now because this UTI doesn't want to go away, and is still causing some kidney pain. This week I also have follow ups in San Diego, which should be relatively uneventful.

 In summary—I have a LOT to be thankful for. In addition to the reasons explained above, I can think of countless more—having a 15 year old lab who made it through the evacuations (heck, the fact that she’s still living it up is a miracle in itself), to have the financial means to access healthcare and live comfortably, a house that is still standing, to have access to food, water, shelter, supportive family, some of the top medical experts in country (with some exceptions :P), etc. The list could go on and on. I have very many long and treacherous journeys ahead, but I knowing that I have a support system means the world to me. Happy belated Thanksgiving everyone!

PS- If you have not already, PLEASE sign this petition here! There is current a national IV Benadryl shortage which not only threatens my access to this life saving medication (I receive a 24/7 infusion of it) and others with MCAS, but also many others using it as a pre med for chemotherapy, treatment for allergic reactions, etc. It takes just a minute, and every response helps! There even is a video about the shortage featuring yours truly :)  Petition Link

 Video about the Dipenhydramine Shortage

Signs for first responders on the entry sign to our neighborhood.

View from our house the morning after I came home.

When it rains, it pours, hails, snows, sleets, etc. -- Failed IVIG, 2 Additional Admissions, Bye Bye Hickman, The Return of the PICC, Frustrations and Got Blood?

As you might be able to tell from the title of this blog post, a LOT has happened since my last update. I haven't been the best about updating this over the last couple of weeks, nor updating too many people over text or phone either, mostly because of the sheer frustration of having more questions than answers at this point, and quite a few traumatic experiences.

Over the course of my second admission, there were a few "snags" that we hit. No hospitalization ever is completely problem free, but there certainly seemed to be a shift in providers' behaviors that indicated that they were kind of "over" me/my situation. Hence, the hot potato begins-- everyone is intrigued because my conditions and presentation are so rare, but when they realize there are no real answers to be found, they were more than happy to rush us out the door, even though it was a bit premature.

My Hickman line ended up thankfully not being infected-- we suspect that I was reacting to the material in the line itself, as it was the same material as the previous PICC line that I also had issues with. Nevertheless, I came home on IV antibiotics to play it safe. Once I got home, settled, and showered, I was eating dinner when I saw that there was some blood leaking again from the Hickman catheter. That automatically warrants a trip back to the emergency room. I was obviously reluctant to return, so I called the floor that I was on, the MICU, and spoke with one of the residents. This resident didn't seem to fond of me during the stay, and insisted that because I can and do eat, I don't need TPN, even though I have obvious malabsorption and electrolyte issues. He told me that it was fine, to just keep an eye on it. I asked if I should run TPN that evening, since if there were any concerns of leaks or infections, the dextrose (sugar) in the TPN could worsen it. He said that I could skip it for a few nights. I expressed my concerns about electrolytes, but he insisted that because I had some dinner, it would be fine.

The next morning, I went (or at least attempted) to go to IVIG. I was feeling pretty crummy after skipping the TPN, and I suspected that my electrolytes were playing a role. The infusion center staff also felt the same way, but agreed to give it a shot. Sure enough, I was more reactive than usual, and not particularly responsive to medications. They tried giving me additional steroids, fluids, called my doctor, etc., and nothing seemed to be working. I was stable-- not improving, but not really getting worse too quickly either. When I was discharged the previous time, the allergist mentioned that in my next flare, we could potentially try a medication for a condition called hereditary angioedema, which is a condition that can cause swelling, or plasmapheresis. I don't have hereditary angioedema, but we were wondering if giving the med a try was worthwhile. Thus, we loaded up and headed back to UCLA in hopes of giving it a try. We called on the way to give them a heads up to get the allergy team. They told us that they would meet us in the ER, but never showed. Since I was having a reaction, I unfortunately earned yet another one way ticket to the MICU.

At this point, it was very very clear that the doctors were frustrated with my lack of response to medications, and readmissions. Of course I shared the same frustrations, but that doesn't mean that my medical care should be compromised. This admission was the one that was particularly traumatic. I had one nurse say that the angioedema in my tongue was "subjective," and that I should just stick my tongue back in my mouth. One physician loudly and sarcastically announced during rounds, "Look who's back! Ms. Davis!" I was told that my outpatient physician had revoked my diagnosis, and that the rheumatology team said there was absolutely nothing wrong with me-- both of these statements were immediately cleared up by their respective parties-- neither was true in the slightest. I was told that you can't have MCAS and dysautonomia, which is INCREDIBLY untrue-- most people have both. The rheumatologist asked the MICU team to increase my low dose chemo slightly, and the MICU team refused to give it to me all together. Yet, they were the ones that called in the rheumatology team to consult in the first place. My hemoglobin, red blood cell count, and iron also got extremely low. I asked them to try an iron infusion while I was there inpatient, since iron infusions are notorious for causing allergic reactions, even in "normal" people. They insisted that my primary care would handle it outpatient. On top of it all, they didn't have anybody look at the potentially leaky Hickman until the day I was discharged. They removed the stitch holding it in place since it appeared irritated. I suggested that they re-suture it because my skin is so fragile and doesn't heal well, but they insisted that it would be fine.

Unfortunately, it was not fine. In regard to the hemoglobin, iron, and overall anemia, my primary care doctor was not able to help set up iron infusions, and said only a hematologist could, which I couldn't get into for weeks. My energy upon discharge was worse than it was before, likely because I was so  anemic. After insisting I see a doctor that could help with the anemia ASAP, I got into a hematologist/oncologist this past Monday. She saw my labs and how severe my bruising ones and said that she was going to direct admit me to the ICU at UCLA in Santa Monica for a blood transfusion and iron later in the week after obtaining a pre authorization from my insurance. She also promised to help coordinate my care long term, which was encouraging.

Back to the Hickman, Monday night/Tuesday early morning, I woke up around 3 AM to use the restroom. On my way back to bed, my TPN tubing got stuck on the corner of my bed, and my Hickman literally fell out since they had removed the sutures.  It didn't hurt at all, nor bleed, which attests to the fact that it was not healed like I had suspected. This also warrants an automatic ER trip. I stopped the TPN, accessed my port, and moved my continuous Benadryl to the port. I packed a bag and it was back to the ER.

RIP Hickman

Since my last UCLA experience was so awful, my step mom and I agreed that it was worth a try to see if the local community hospital could help , knowing that it was likely that they would still have to transfer me. Sure enough, they said that I needed a higher level of care. We called the hematologist, and since she was going to direct admit me a couple of days later anyways, she helped facilitate my transfer to UCLA Santa Monica by a critical care transport ambulance.

Once I got to the UCLA in Santa Monica, they already had a nurse bedside with the equipment to place a new PICC line. So far, this one is looking and feeling a lot better. It is a different material than my Hickman and more recent PICC were, but the same material as the ones I had a few years ago. It is much smaller and light weight, and placed in a better spot. They ordered the blood for my transfusion, but because my igA deficiency is so severe that it is undetectable, I required"washed" red blood cells, which basically means that they remove the antibodies from the blood. Of course, given the fantastic series of events that were already occurring, there were strikes at all UC Health facilities, which meant it took 9 hours to get the blood! Thank G-d I was not bleeding out any time in the immediate future.

New PICC Line

Got blood? (It's in the doughnut from the antibody filtering)

I had a small reaction when the transfusion started, but it responded to benadryl and was fine afterwards. The hematologist thought that I had a GI bleed somewhere, so they insisted that I got a endoscopy and colonoscopy, which I was NOT a happy camper about. I was already NPO (no food or water) because of the airway concerns and the potential for a GI scope, but with the timing of the tests, I was not allowed to eat or drink for nearly 2 days. This felt like literal torture. I got TPN the second night, but TPN does not do anything to impact hunger. In addition, because my airway is so finicky, I had to be put under full anesthesia for both procedures, which meant that we had to wait longer to have an anesthesiology team. After 24 packets of Miralax (I never, ever want to see that stuff again), the scopes were done on Wednesday, and came back clean. No anesthesia hiccups. There was some friability on my upper GI system, but they said it was likely steroid related/not clinically significant. They took 7 biopsies to do some stains to see if there was anything on the microscopic level, but they didn't seem to think there would be anything useful. I didn't know that there is a bit of discomfort after the procedure as well, so I felt pretty crummy, and my GI tract still seems to be recovering.

Yesterday, the hematologist called me, and said that she wanted them to do an iron transfusion before I left, but was really only interested in the anemia alone. Thus, hot potato-- bouncing around again to other team members, not because nobody wants to help, but because I'm not a clear cut case, its frustrating to them. Communication from everyone is lacking, and its incredibly disappointing and frustrating. I was just thankful that I was able to go home last night. At this point, we still don't know if I'm losing blood somewhere else or if I was just insanely iron deficient. I apologize to those of you who I have not been giving updates too-- as I said, it's been so frustrating having more questions than answers that I haven't really been particularly social.  I am hoping to try to work a day this week. I have some follow ups and appointments with new specialists in the next couple of weeks, so I'm going to attempt to be more diligent about my updates.