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