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