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.

When it rains, it pours-- back at UCLA .

I apologize for being MIA for a little while-- the past few weeks have been a challenge, with some "speed bumps" to say the least. I've been a little discouraged from blogging because everything has been so up in the air. I feel like I've been regressing to the point where I'm afraid to make too many commitments or plans, because I don't know how my health will be at any point in time.

So far, the new Hickman catheter has overall been working out well-- I have been able to continue the IV nutrition (TPN), the continuous Benadryl infusion, and my other medications pretty easily. It healed a lot quicker than the PICC line that was poorly placed ever did. The TPN is still overall going fine. I am about 10 pounds from the minimum goal weight, but right now we are more focused on maintaining nutrition and electrolytes overall.

Since I left the hospital this most recent stay, I've still been having difficulties keeping my mast cell symptoms, particularly the angioedema (swelling) down over time. This swelling is problematic when it is in my tongue or throat because it can impact my breathing and airway. I have continued to slowly decrease my IV steroids, which we hoped would be a little easier with the addition of the methotrexate (low dose chemo injections). I still seem to be way more sensitive than I already was, and I'm not so sure that the methotrexate is doing anything other than giving me additional side effects. 

I had a follow up with my endocrinologist since my blood tests positive for Hashimoto's, which is an autoimmune condition that targets the thyroid gland. Thankfully, although I have the autoantibodies, it hadn't really impacted my thyroid function as of yet, so I get regular blood work with the knowledge that at some point soon(ish) it will be impacted. My autoantibodies increased, and my thyroid function slowed slight impairment, so when I received a call from the doctor's office saying that I needed to come in to review my test results, I was assuming that it was about my thyroid.

When I got into the exam room, the nurse handed me a glucometer. I asked her why she was giving it to me since I'm not diabetic-- in fact, my A1C and blood glucose have been consistently beautiful, despite the fact that I've been on high dose steroids long term, and that TPN is primarily a giant bag of dextrose (sugar). The doctor came in shortly thereafter, and said that yes, the thyroid looks like it probably will be treated in the near future, but that she called me in because of my pancreas. 

I was pretty surprised to hear that my pancreas was involved since it has never come up before. She said that one auto-antibody in particular shot through the roof, which was concerning since I'm on immunosuppressants and IVIG. She said that this particular test indicates that there are auto antibodies being produced by my own body that are attacking my pancreas, which would make it so that my body could no longer produce its own insulin-- thus, giving me Type 1 Autoimmune Diabetes, and insulin dependent. 

I asked if changes in diet could prevent the diabetes, like it can for Type 2 Diabetes, but I was told that since it is purely autoimmune, that isn't the case.  She said that treating the overall autoimmune disease process could help delay the onset of the diabetes, but not prevent it, and that I'm already on many of the aggressive treatments. I asked about a timeline for the development of the diabetes, and she said the longest she had seen was three years, but their autoantibodies weren't as high as min. Since my A1C and sugars have been so great, she said that I should just check my blood sugar if I'm feeling really off, but otherwise, nothing else needs to be done yet. (The Mayo doc did later say that its possible that this antibody is indicative of general inflammation as opposed to a targeted attack on my pancreas, but nevertheless is rare problematic).

In the grand scheme of the things that I deal with health wise, Type 1 Diabetes isn't a huge deal. They make so many great gadgets and gizmos for monitoring blood sugar, administering insulin, etc. The part that really upset me with this development was more that I am doing everything I am told to do, doing the aggressive treatments that make me also feel like crap, and yet, nothing is changing-- in fact, it is progressing. 

I emailed my primary mast cell doctor and asked if he had any suggestions. The infusion, Rituxan, that we wanted to try wasn't really much of an option right now because I'm so unstable. He said that he wanted to talk to the physician I saw at the Mayo Clinic back in May, but said that it may be time to do plasmapheresis, at least in the short term. Plasmapheresis is somewhat similar to dialysis, but less intense on the body. it involves being connected to a machine that filters the antibodies out of the blood, three days a week for 5-6 hours total each day. Thus, it would dramatically impact my quality of life and ability to maintain some degree of normalcy. The other issue with plasmapheresis is that there are very few facilities equipped to administer plasmapheresis, and many wouldn't agree to taking me on as a patient given my age and the fact that this treatment is atypical or "off label." 

The physician at the Mayo Clinic and my mast cell doctor spoke on the phone and agreed that it was time to pursues one or both of those treatments. I was trying to figure out a facility where I could do the plasmapheresis, but I woke up Monday morning with TPN on my shirt, irritation around my hickman, and some blood by the dressing-- all HUGE signs of infection. Infection is probably the biggest risk of TPN, since the central line used for TPN (in my case, the Hickman) goes directly to the heart. That means that I unfortunately earned myself a one way emergency room ticket. 

We were debating whether we go to the local community hospital or back to UCLA. I was thinking UCLA would be better, since the local community hospital already told me that I'm too complex for them/they are ill equipped, but the community hospital obviously has the advantage of being close to home. We were almost at the community hospital when I got a call from my doctor's office that they didn't like some of my blood work and were concerned about infection. They suggested that I go to the ER at UCLA, and that they would call ahead.

My stepmom took me to the ER at UCLA. This time, I was taken in immediately since where my Hickman catheter was really didn't look good, and because I'm pretty immune compromised. Although there were no clear triggers other than stress, I still started having an anaphylactic reaction. I was so frustrated that not only was I having to make this unexpected ER trip, but also that it couldn't just be for one issue-- now we were dealing with both anaphylaxis AND a suspected line infection. 

Because I'm immune compromised, I probably wouldn't get a fever even if I was in septic shock. That makes it harder for doctors to tell if I have an infection virus irritation or even an allergic reaction to the line itself. They took some blood to run cultures and additional testing. The cultures take a few days to grow, but the other testing was "borderline." Regardless, they were admitting me at that point for the anaphylaxis anyways. They gave me 3-4 injections of epinephrine, plus steroids, additional Benadryl and other antihistamines, and I still had pretty severe angioedema. They decided that they needed to start an epinephrine drip, which I unfortunately have had many times before, which also means a one way ticket back to the MICU (Medical Intensive Care Unit).

In the ER, they had the rheumatology and allergy/immunology teams come see me, which was great. Since it is a teaching hospital, they were going to collaborate and see if they could come up with some sort of game plan. I was moved up to the MICU, but overnight, my labs started to go way downhill. I didn't have TPN, since they don't run TPN until infections are fully ruled out. My electrolytes tanked, and my blood became acidotic, both of which can be dangerous. They ran electrolyte replacements, but actually overcorrected these abnormalities. As a result, my blood sugar was all over the map, and I felt absolutely awful overnight. I had a throbbing headache and felt weak and tired, and had tingling in my hands and feet. I didn't get to sleep until almost 5 AM because of how bad the pain was, and getting blood pulled from my line every 30 minutes to an hour. They eventually figured out that the reason why my headache wasn't responding to pain medication was because my blood sugar was so low. Once they corrected that, I felt a bit better, but I was still hangry to say the least.

They finally let me start eating again mid day today, but don't want to restart the TPN until they are entirely sure that there is no infection. I'm hoping to go home tomorrow or Thursday at the latest. The preliminary cultures are negative, but they said that there could still be a local infection, and the cultures could still grow something, so I am on 2 IV antibiotics. They said they may want me to continue the IV antibiotics at home to err on the side of caution. Thankfully, today has been more stable mast cell wise, but the hospital is actually running low on the one brand of IV Benadryl I can have (I'm allergic to the preservatives in others) which is a MAJOR issue. They're trying to sort that out, but its pretty scary that even a major medical center is having significant supply issues. The rheumatologists here are repeating all of my autoimmune labs here so they have the tests within their own reference ranges, and can see if anything was missed, or if there are any underlying genetic issues that could help explain why I'm not responding to treatment like I should. These will take a few days, and certainly are far from providing any answers.

I'm hoping that after tomorrow, they will be able to send me home pretty quickly as long as the angioedema is relatively stable. They did not dramatically increase my steroids this admission, which is very different from the way things typically go. Hopefully, that should still be enough to cover me, but may explain why I needed so much epinephrine. I am beyond exhausted both physically and mentally, but trying to maintain a positive attitude.  Thankfully, my boss has been very accommodating, and has been giving me a few hours of work remotely a week to help keep me busy in addition to tutoring.

This cascade that seems to be happening is why I titled the post "when it rains, it pours." For some reason, maybe related to the change of seasons, most people I know with my condition or similar ones are really struggling health wise. It's hard to not only experience this myself but to also see others struggle, without many answers or explanations. Research is happening, but it certainly doesn't seem to be fast enough.

I'll continue to post any updates as I have them. 

Hickman is In

Good evening! Quick update-- I had the Hickman catheter placement procedure this morning under general anesthesia to manage any complications in case they arose. Thankfully, no complications arose during the procedure, and they successfully placed the Hickman in my left upper chest! It's a little hard to describe, so I stole this fancy graphic from Google for a visual.

Coming out of the procedure, I did experience more pain than I had anticipated. My neck and shoulder were extremely sore and stiff both along the incision and up my neck, so as the anesthetics wore off, I was a very unhappy camper. Getting upset and pain both trigger mast cell reactions, so I did have a minor reaction, but thankfully, it responded to Benadryl. They were able to give me Tylenol, heat/ice packs and a one time very very small dose of pain medication, which helped (although I was super frustrated that it took 3 hours for even that to happen). Tylenol and a heat pack seem to be doing the trick for now. 

I am really excited that the plan is to go home tomorrow! I am still a little loopy and weak from today, but I am confident that getting some sleep will help. I was supposed to have my next round of IVIG Thursday, but my outpatient doctor decided that to be safe, we should wait till next week so I have more time to recuperate. I'll post an update from home later this week, and thanks again for everyone's well wishes! <3

No Infection! But Still Stuck

Good news-- there is no infection in my PICC line! This means that a) IV antibiotics were able to be stopped b) no concerns of sepsis and c) they were able to resume my TPN (IV nutrition). Although the PICC is operational and useable, now that we know that there is no infection, it still is really poorly located, and still has not fully healed. As a result, we are planning on proceeding as planned to place a Hickman line in my chest, and remove the PICC.

Originally, we planned for that procedure to happen today. However, I have continued to have swelling, and one particularly bad episode, so the team decide that they want to use general anesthesia (knock me out) instead of local anesthesia like they usually do for the procedure. This gives them control over my airway in case I react to anything during surgery. That is scheduled for 8 AM tomorrow morning, so fingers crossed that it will be smooth sailing, and then I would go home mid week.

The other part of the procedure plan that was slightly changed was that they are no longer planning on removing my port tomorrow. Since the port is fully operational, gives blood back, etc, they decided that they would keep it, but de-access it (no needle), as I have been doing since I got the PICC. This means that tomorrow's procedure will be slightly less invasive than planned, and provides access in the event of an emergency where the Hickman is not working, or if infection concerns arise. Once I am able to get off TPN, they can remove the Hickman, and I still have the port without having to go through another surgery.  I am still a little uneasy about having 2 central lines, even though one is not accessed, but having the port resting under the skin as backup only isn't a terrible idea since it is functional.

Yesterday, we did the weekly Methotrexate (low dose chemo) shot, and started TPN late last night. For today, we are waiting on the dietician to refine the TPN regimen. Since I have been here, I am continuing to have issues with losing electrolytes, so we may need to tweak the "cocktail" that goes into my TPN. I'm glad that we were able to resume it, because I really noticed a difference not being on it (which I honestly didn't anticipate). We are also keeping anybody wearing fragrances far away from my room to avoid another mishap :P I'll post an update when I have one!