Back to Back

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

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

Disney!

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

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

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

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

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

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.

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

IVIG = Success

Today marks a week home from the hospital, and I'm so glad to be back. As anticipated, there has been some degree of a learning curve with the TPN at home, and new medication regimens, but so far, nothing that hasn't been remedied. Having nutrition has made a huge difference in post-hospital recovery time. There are some hiccups with transitioning off student health insurance, but we are trying to do what we can to make it as smooth as possible (after some panic). My new home health nurse is great, and is willing to deal with my twenty million questions and insistence upon sterility. I was able to go into work for part of a day so I could avoid taking a medical leave. This was a big relief, and also reassurance that I am passionate about what I do. I am incredibly lucky that my supervisor is extremely patient and accommodating. Even though graduate school is on hold for now, I am confident that trying to work a day or two a week (despite the hellish commute) will help keep me stimulated intellectually. It also gives me an additional sense of purpose as I focus more on spending time doing the things that I want to do and with my family. My little brother, Cody, started middle school this week, and it was really cool to be home to hear about his day.

In terms of health updates, I did my first methotrexate injection on Sunday, which is the low dose chemotherapy as an immunosuppressant. I certainly felt the side effects like nausea and some dizziness, and overall feeling crummy for the rest of the day, which I hope will decrease over time. Nevertheless, it was nothing unbearable, and something that I will do weekly. I also had my next round of IVIG outpatient today, which was the treatment that seemed to have caused the reaction that hospitalized me at the end of July. Thankfully, the small reactions I had were controllable, and I'm back at home resting.

Follow ups with my primary care physician and nephrology earlier in the week were also both largely uneventful. We are working out the logistics of the TPN with my primary care, and the nephrologist just confirmed what we already knew, which was that the autoimmune process is affecting my kidneys, and thus my electrolytes. We hope that the methotrexate, IVIG, and possibly another immune-modulating agent (to attempt once things stabilize more) can maybe help slow things down. I have follow ups next week in San Diego with allergy/immunology and cardiology. I also am supposed to get my new wheelchair tomorrow, which I think will be helpful particularly for work and times that I'm out and about. Still no news on when and if my port swap will be occurring, but the PICC line is driving me nuts based on where they placed it. It's right by the crease of my elbow, so when I bend my arm, the tubing rubs on the inside of my arm. and it pulls on the skin. Although its annoying, I'm lucky that it is fully functional, and as I have said before, the technology and science to be able to receive these treatments in an outpatient setting.

In addition to the two day San Diego trip for follow ups, I am hoping to get away for one night over Labor Day weekend. Going away for any reason ends up becoming quite the orchestration, and often rather stressful, but I think a change of scenery even just for 24 hours is worth a shot.

Tomorrow marks my grandmother Shelley's birthday, who passed away in April. I think it will be tough for all of us, but I know how much she loved all of the family, and would want us to celebrate her. If I'm up to it, maybe I'll try to hunt down one of her favorite desserts, lemon meringue pie :)

I hope that things will continue to be relatively uneventful, but will continue to past updates as they come.

Escape Plan Initiated

Finally I get to report that I should be on my way home this evening! Thank you again for all of you who have reached out, come to visit, dropped a note, it means a lot to me!

Part of the "weight gain" care package from my friend Molly (and this wasn't even half!).

 We were able to lower the IV steroids a hair more last night without any major episodes, and most of today was spent going back and forth between the pharmacy, home health/nursing agency, regular outpatient pharmacy, and my outpatient team to orchestrate everything. My situation is far from typical in terms of complexity and discharge, but we all can agree that virtually living in the hospital is not a very solid quality of life, nor would really change much long term. I'm incredibly thankful and lucky to have a support system at home to help me through this.

My cousin Maya drew this page free-handed for me to color!

Assuming everything falls into place, the pharmacy is delivering the TPN (IV nutrition) and other medications to my house later this evening, which my step mom will bring to the hospital, connect me to, and then head home. The new home health agency is supposed to come tomorrow to do a dressing change on my PICC (IV line), and make sure that I'm confident in preparing medications. including administering and preparing the Methotrexate injections. I have labs and follow ups with my primary care physician on Monday, and I also need to follow up to see if switching my port is still an option, since ports typically have a lower infection risk than the PICC line (and mine already seems to have some issues with blood return). I have follow ups in San Diego at the end of the month with allergy/immunology and cardiology. The hospital dietician also came by last night to confirm that the current plan is to still get as much of my nutrition as humanly possible by mouth, and also keep me on the TPN, most likely for a couple of months, until my body weight is up. After that, we work on weening off of it, or at least reducing the frequency. The cocktail that constitutes the TPN is regularly adjusted by my doctor and the pharmacist based on my blood tests.

(Warning- paragraph only for those of you interested in the nitty-gritty logistics, otherwise, jump to next paragraph). The Benadryl is the same as it has been before-- I change the bag of medication and tubing that is connected to me daily, and that is attached 24/7, shower, sleeping and all. For Pepcid and the IV steroids, I draw them up into syringes ahead of time, and they have to stay refrigerated-- the Pepcid being twice a day, steroids three times a day (while also titrating the dose down and keeping track of those numbers). The TPN runs for 14/24 hours of the day. Before I can start that, I have to add vitamins and folic acid to the mixture, which ends up being a pretty massive bag. While the TPN is running, I'm carrying the TPN itself, a pump, plus the Benadryl and another pump (which ends up being a solid portion of my entire body weight).

Very excited to ditch this view and the never ending wire tangle.

When I initially started the continuous diphenhydramine/Benadryl infusion (CDI) last May, I wrote a lot about a new normal, adjusting to being connected to the pump constantly, and so many new (and scary) considerations. I certainly am feeling that way-- it is pretty overwhelming and scary to have to track and care for everything. I know I'll get through it, just as I did before, but at the moment, I'll let myself go through the anxiety-- and being at HOME will help with that too!

Well, that was fast--Tough Decisions.

Usually I can come up with some clever introduction to my blog posts, but for this one, I'm kind of at a loss. After my last blog post, I was discharged Saturday afternoon with the IV steroids to be able to continue the steroid taper from the comfort of home, and eventually gradually switch to oral/liquid steroids while also trying to get my weight back up to a healthy level.

Yesterday I was going to a follow up appointment with my primary care doctor, and prior to leaving, I started experiencing the same weird feeling in my chest/arrythmia that I have been feeling intermittently. It came and went, but it was enough to make more really dizzy, nauseous, and short of breath. I got to my doctor's office, and at that point, it seemed to fuel some sort of mast cell reaction. They called 911, and I was back off to the hospital.

When I got here, they gave me the usual anaphylaxis protocol, and also took blood work. Those labs indicated what we already knew and had been seeing in my labs for weeks, severe malnourishment and failure to absorb medications and nutrients. They admitted me back to the ICU Stepdown Unit/PCU Unit that I had been on for the latter part of my last admission, and gave me IV electrolytes overnight.

After speaking with the team here and nutritionists, they decided to start me on TPN, or Total Parenteral Nutrition (LINK). TPN is a cocktail of vitamins, minerals, lipids, fats, etc. that provide complete nutrition via a central line, like the PICC that was placed last week or my port. It takes the GI tract out of the equation in terms of what is getting absorbed, since it bypasses the GI tract entirely, which is why they elected for TPN instead of a nasal or surgical feeding tube. It runs for 12-14 hours a day and provides an entire day's worth of calories and nutrition. While inpatient, they take labs daily, and build a "cocktail" based upon whichever nutritional deficits they are seeing. On an outpatient basis, these labs are usually once a week.

In theory and in the short term, TPN is literally life saving. However, the long term risk profile, risk of complications, and impact on quality of life can be challenging. The current plan is to use TPN for a week + to get my weight up out of the danger zone, and provide a buffer. We suspect that at this point, the autoimmune process that has been ravaging my system is partially fueled by the fact that my body is trying to attack tissues for energy sources. Once we have gotten me out of the danger zone, then we will work on getting me off TPN as soon as humanly possible.

In the mean time, I am supposed to eat as much and as frequently as possible to make sure that my GI tract doesn't take an even more extended hiatus than it already has. I am trying to nibble throughout the day, use supplements like Ensure, etc, again to try to avoid TPN complications and hopefully need it as minimally as possible.

That being said, I was supposed to move into graduate housing at the end of the month to begin my MSW program. In light of recent events, the shift in what our plan going forward is, and the timing of everything, I had to make the extraordinarily difficult decision to defer enrollment in my Master's program for a year.

To be entirely honest, I'm pretty heartbroken. I know grad school will wait for me, and that I need to be focusing on my health, but as I mentioned in my last post, I'm naturally frustrated. I am confident in my team's ability to manage things and to anything and everything necessary to get me back up to speed as soon as possible. I'm also scared of TPN complications, but I know that at this point, I don't have any other choices. While I'm here, we also are going to continue to ween down on the steroids whenever possible while trying to also avoid any more major mast cell episodes.

Once I'm discharged, depending on where we are at with TPN, I"ll either get my port swapped to a double lumen (2 part), or a line called a Hickman, which is similar to the PICC line that they placed in my arm, but in my chest. For TPN, it sometimes is less of an infection risk to use a PICC or Hickman in comparison to a port. My educated guess is that I'll be here at least a few days to a week, but it seems to depend on whether my mast cells cooperate with the agenda. In the mean time, I'm working on snacking, tutoring, coloring, and whatever else is distracting and minimally stressful. b

I also feel the need to apologize for the "Debby Downer" kind of post, but for right now, I think writing everything out is helpful for processing. I hope to have a more positive update soon after they start the TPN overnight.

Puppies do Make Everything Better

Well, maybe not everything, but they certainly are a fun change of pace! I had a really rough morning with some weird cardiac symptoms, but I saw three therapy dogs pass my room and it totally cheered me up (not that human visitors are not awesome too, shout out thanks for the visits). The highlight-- the dog in this picture''s name is Epi, after epinephrine. The volunteer asked if I knew what that was- I responded with, "that's why I'm here!" Too perfect of a coincidence.

A health update from my last post-- I was moved from the ICU to the Progressive Care Unit (PCU/ICU stepdown) Tuesday evening. It's nice to finally have a room with an actual toilet, shower, and slightly more room, etc., especially since this admission has unfortunately been longer than anticipated. It can be a little logistically challenging because when I need emergency medications, I need them then and there, but we seem to have refined it to a system.

Despite the IV steroids, the addition of another oral immunosuppressant, and additional Benadryl, I am still having episodes of angioedema (swelling) around my lips, cheeks, and tongue. Thankfully I have only had one episode since moving out of the ICU that has required an epi pen, but nevertheless, I'm frustrated that it has been more than a week and things still aren't fully resolving.

I guess I should know by now that my body doesn't play by the rules, but its still disappointing. In light of the difficulty getting off IV steroids, continued issues of malabsorption of both food and medication,  maximize comfort, and decrease infection risk, the team is ok with sending me home with IV steroids and the intention of gradually lowering those and transitioning to oral steroids, but liquid instead of pills. The IV immune-modulating medication will be added once things are more stable a month or two down the line.

There was one more inpatient dose decrease in steroids this morning. If things go ok this evening and through tomorrow, I should be able to go home tomorrow. The pharmacy has been great (as always) and is supposed to meet me at home with my supplies. We had a hiccup this afternoon with home health nursing. I have not always seen eye to eye with the nursing agency since I moved back home, but today, where they were argumentative, asking for cash pay despite insurance coverage, and overall creating additional unnecessary hurtles. This agency is totally independent of the pharmacy, and the pharmacy has their own nursing in addition to contracted agencies, so it shouldn't be any issue, but nevertheless seems like a pointless speed bump.

Once I get out of here, I'll be able to have a better gage of my plans for the near future. In the very immediate future, the plan is to continue the medications at home, the new oral medication, the new IV medication (Rituxan) down the road, another attempt at IVIG in a week, and getting my (currently de-accessed) single port switched to a double lumen so the PICC can be removed ASAP.  Current plan is move into graduate housing the last week of August if things stay stable. My new wheelchair should come in about 2 weeks.

In the mean time, I'm occupying myself with online tutoring and orange is the new black, and surrounded by copious amounts of snacks in the attempt to get my weight back up to normal. I haven't been outside in a week now, so fresh air is going to feel amazing. I'm hoping to be able to binge watch Sacha Baron Cohen's new show over the weekend because I heard some positive reviews :P Plus, the ice cream at home is a lot taster than these damn Ensures!


PS-- If you haven't already, please donate to the Denim Dash for Rare Diseases! It is a walk/roll/stroll 5K (or however much you can) for rare diseases such as those that obviously dramatically impact my life. You do NOT have to participate in the Dash to help out. DONATE HERE!

Well, I’m a College Grad! (But Mast Cells, Autoimmune Stuff and Life can Still Rain on the Parade, Stuck in San Diego)

It’s official, I now hold a Bachelor of the Arts degree in Psychology and Sociology from Pitzer College! I still can’t believe I’m done. I have to admit I have been neglecting the blog a bit, but things have been quite the whirlwind since April when I began working at Children’s Hospital Los Angeles (CHLA), which has been amazing so far! Between final papers, orientation, and wrapping up my college career, time has flown by. Nevertheless, I managed to graduate with a 3.83 GPA (technically a semester early), which (selfishly) I am proud of. I also made my decision for graduate school…… I will be attending UCLA starting at the beginning of September to pursue my Master’s in Social Work (MSW).

(Photo Creds to Andrea)

I wish I could say that everything has been rainbows and butterflies, but I think there is a consensus that those sorts of expectations are unrealistic. In the early hours April 21^st, my grandmother, Shelley Balonick Davis, passed away after a health decline that ended up taking a more rapid turn for the worst. This was a devastating loss for the entire family and quite frankly, most people who knew her. I thankfully was able to spend the last couple of hours with her in the Bay Area. While we were in the Bay Area, a few of her nature pals were hanging out around their house. Probably a coincidence, but I’ll take it as a sign that she was checking in on us. Despite this loss, and certainly far from ideal timing, I know that my grandmother would have been so proud of me for my academic, career and personal endeavors, and was at my graduation (and all of our recent happenings) in spirit.

At the funeral, I got a voicemail that I was able to get an appointment with a physician at Mayo Clinic in Arizona that specializes in using IVIG for dysautonomia (POTS/Postural Orthostatic Tachycardia), Mast Cell, etc. I had been waiting for 2 years to get in, so I can’t help but think that maybe there was a little message from Grandma hidden in there. The appointment went really well, and he had some suggestions on how to better fine tune my medication regimen. He also asked me to repeat all of my autoimmune antibody panels. These have been repeated a few times, but he said he wouldn’t be surprised if they started showing up positive at some point.

Met Erica of Celiac and the Beast at the airport waiting for my flight that was 4 hours delayed to Arizona!

Sure enough, this round of labs now showed autoimmune antibodies for Sjogren’s Syndrome and that I appear to be developing Hashimoto’s Disease. These are both autoimmune conditions which can explain why my POTS and Mast Cell Activation Syndrome (MCAS), particularly when combined with Ehler Danlos Syndrome. I never thought it would be a good thing to add yet another term to the ongoing diagnosis list, but it does help explain my symptoms and why they respond to the meds they do. It doesn’t change treatment much either, but again, can maybe shed some light on new developments in the future.

Graduation was shortly thereafter, and also a really positive and celebratory experience. I was a little nervous about the logistics of standing and waiting in line, etc, but I am very appreciative that Pitzer staff as well as friends and family made things work. I was also really appreciative for everyone who came out to support me from near and far. It meant a lot to me, and I was so happy to be able to catch up!

Celebrating Mother's Day....

Celebrating Serena's birthday..

Reuniting from near and far!

Thankful for the love and support for these awesome deans!

On Tuesday, I drove down to San Diego with two friends from school and part of my POTS “crew” for outpatient follow up appointments. We went to dinner when we got into town, and I seemed to have a reaction to a muffin—despite the fact that it at least was not supposed to have anything I can’t eat in it. I used my EpiPen and other emergency meds, but I felt a fluttering in my chest that I have had before, but typically isn’t a super fantastic thing. That component resolved pretty quickly, but I was still reacting, so my friends took me to the ER. I absolutely avoid the ER/hospital at any and all costs, so I refused to get out of the car for about 40 minutes. Eventually I reluctantly agreed to go in, but only for a dose of steroids, then go home.

At least Molly and Serena were having fun in the ER?

Unfortunately, that didn’t go as planned either. By the time I went in, it was kind of to the “point of no return.” I was admitted to the ICU for observation. I began to improve, so I was transferred to the Telemetry/ICU stepdown floor. When I got there, there was a series of unfortunate miscommunications and challenges. I wasn’t able to get my emergency medications as quickly as I needed them. When I need the meds, I need them almost immediately before things begin to spiral. There were also 2 vital sign checks missed, a breathing treatment, and 1-2 doses of my immunosuppressant. 

I can’t even begin to say how fortunate and lucky that two of my friends, Savannah and Molly, were with me in that room. They noticed all afternoon that things were declining, and asked for a re-evaluation, but they were told that someone would be coming soon, or it was the middle of shift change. 

By the time the appropriate team did arrive, I desperately needed epinephrine. My memory from there is more foggy, but from all of the accounts, the doctor asked for Epi. We had already used the EpiPen next to my bed, so the nurse tried to call down to the pharmacy to get another. The nurse stated that she didn’t know that the crash cart down the hall contained epinephrine, which is a MAJOR issue, because that is considered to be standard knowledge in any healthcare environment. I was turning blue at this point, so the doctor, Molly and Savannah used Molly and Savannah’s EpiPens. Those thankfully worked, and I was transferred back to ICU, but most certainly in worse shape than when I arrived.

I was told that if Molly and Savannah had not been there or had their EpiPens, I literally would have died, because there was no crash cart in the room. Even if there was, I didn’t have enough of an airway, which would have meant they would have to attempt a surgical one (but that equipment wasn’t there either). Molly, Savannah and the rapid response physician undoubtedly saved my life.

To avoid these incidents in the future, the hospital is conducting a thorough investigation, including re-training, documentation, stocking more epinephrine autoinjectors, etc. Many managers, supervisors, etc. have come by to debrief. I am at the hospital where my specialty care team is in La Jolla, which has helped facilitate communication as well. 

My dad has customized the info board in my room to be jailbreak themed.

Delicious looking dinner, am I right?

Since then, things have been improving, but I have now been in the ICU for more than a week. The physicians and staff have all been fantastic, with the exception of that one floor. We are making progress- I got an extra dose of IVIG, we doubled my immunosuppressants, and increased the rate of my continuous infusion. We are hoping to get out of here tomorrow, but it may have to be Saturday. I have been blessed to have had lots of awesome visitors while in San Diego (shoutouts to Kara, Gabriela, Julie, Savannah, Madison, Molly, Serena, Willa, Aviva) and my dad has been bedside since the incident last week. Nevertheless, it is still frustrating, and disappointing. I am so thankful for the progress that I have made over the last year—I finally have my life back. And this one hospitalization does NOT jeopardize this progress by any means. I just have to remind myself that slow and steady wins the race. All that is left is the final push to oral steroids (instead of IV).  I am also VERY excited to have non-hospital kibble (the food being nasty is a dramatic understatement).

I’ll keep posting any updates as I have them but hope to jailbreak soon!

Last, but finally not least, I will be fundraising for the Denim Dash this summer, which is a virtual 5K for rare disease awareness. It allows me to fundraise for a cause that is near and dear to my heart (and I can walk/roll, bowl/whatever): https://www.crowdrise.com/o/en/campaign/the-mast-cell-crew

Decisions Decisions....

I can't believe how quickly the end of my last semester of my undergraduate career is approaching! I also have lots of good news along with it. I am excited to announce that I will be working at Children's Hospital Los Angeles starting at the end of April part time as a Peer Health Coach, where I was a former patient! It is really cool to be able to go back NOT as a patient, to be able to help other patients and make a difference.

I also have heard back from half of the graduate programs I have applied to-- and I got into both! I'll keep the universities I have heard from nameless for now, but I'll give you a hint. They are two major universities in Southern California with a long standing rivalry. I am still waiting to hear back from the last two before I make my final decision, but I think I have my decision made. The two programs that I did hear back from are the most competitive out of the bunch, so it is very encouraging that I got into them both. I am glad that I will be able to stay semi close to home, and I can't believe the progress that I have made from almost a year ago, where I spent most of my time in bed or in the hospital, too sick to do much.

Health wise, things have been going really well. The rest of this post may not seem like it, but I promise, I am really feeling a LOT better. I had another three appointments in San Diego, one about 2 weeks ago, and two at the end of last week. I met with my POTS doctor, a gastroenterologist, and my allergist/immunologist. All of them told me how I look like a different person from a few weeks ago. In my last post, I also mentioned there were some difficulties with my GI system and nutrition. It's still been pretty finicky, but I met with a new GI, who started me on a new medication this week. So far, it seems to be helping, so fingers crossed!

"Moon face" is almost gone!

The IVIG has still been very helpful. As I said to my doctor, "I feel like a new person." I have been having some trouble with having reactions to the infusions themselves, and their side effects, so we are working on trying to get the brand switched. Yes, the brand-- although IVIG is a human blood product, there are multiple "brands," which have different filtration and constitution methods (go donate blood people!). I believe I mentioned it in a previous post, but my body does not make igA, which is the antibodies that line mucosal tracts throughout the body, which contributes to my not-so-fantastic immune system. I have been on a low igA brand, but since I have been having a hard time with it, we are going to try to get my insurance to approve a no-igA formulation, and also add in a small dose of IV steroids before the infusion. Regardless, the IVIG has helped enough so although I have had more reactions and episodes, I still have not been hospitalized, and I am WAY down on my steroid dose. No more moon face! We still have a few more milligrams to go, but we're close. It seems to be relatively agreed upon that I won't ever be able to get completely off steroids, but if we can keep them at a super low dose, that is really great.

Steroids have icky side effects in addition to the "moon face," which is why we have been trying so hard to get me off of them. I had a DEXA (bone density) scan over Spring Break, which did show that I have developed osteoporosis in my spine. Luckily, my hips look good for now, but it is something that will be monitored. I'll be meeting with my endocrinologist again in a little over a week to decide if we want to try any medication for it. I have been having some trouble with dizziness, and "greying out," but only if I am standing up (if I am sitting, it's not an issue). Unfortunately, this is very common with POTS, and luckily, doesn't affect any activities that don't require standing. However, in conjunction with the osteoporosis diagnosis, and preparing for my job/grad school, I am using a wheelchair part time to help increase my stamina in terms of being able to be out and about for longer, and actually allow me to do the strength and physical therapy work. Being able to use it just on bad days or for an hour here and there allows me to save my energy for when I really need it, and to be able to do more than I would if I had to sit down every few minutes. I bought a used chair to use temporarily, but I have evaluations this week for a chair that actually fits me, and is super lightweight to make it easier to get around. We tried taking me off one of my POTS medications, to see if it was making my blood pressure go too low, which totally backfired-- so for now, we're keeping things the same there. I'm not going to lie, I have been very hesitant to proceed with the wheelchair, because of how people respond/react to it, and associated stigma. However, being able to use it when I need it has showed me that it's worth it to prevent any complications, and that those who truly matter see through it.

My trips to San Diego have also been fun because I have gotten to spend time with so many amazing people that I have met online who have similar health situations to me. Although we may have met from having the same or similar diagnoses, we all have so much in common beyond that, and I truly appreciate their friendship and support (and letting me crash with them when I'm in town).

Just a part of the San Diego "crew"!

Checking out the Abilities Expo!

Another fun part of Spring Break was that I drove up to the Bay Area for a last minute trip to surprise my mom, see my old neighbors, and a quick lunch with my grandparents. I caught surprising my mom on video:

Also incredibly thankful to my old neighbors for helping me make that happen! I miss them dearly and love any opportunity to spend time with them. And, being able to see my other cousins when I got back to Claremont was an awesome surprise too:


Once I finalize my grad school decision, I will post it here as well :) I am looking forward to my next set of adventures!

A group of us with POTS at the Claremont Colleges meet for lunch once a week. We Skype in whoever can't come in person, and today's set up was pretty comical :P

83 Days and Counting!

I can't believe how quickly this semester is going by so far! It feels like I just got back on campus a few days ago, but we are already approaching the middle of the semester, which means... 83 days till graduation! It is starting to become more real, but it still is crazy to think that I have been in college for four years, and that I'm almost done. It is also very bittersweet-- I am "ready" to be done with college, but it is also hard to look back on the last four years and see everything that has happened. Although I am a hell of a lot more confident in my self and my abilities, it is also sad to think that I wasn't "sick" four years ago, and how dramatically my life has changed as a result of that.

Pitzer Class of 2018! (I'm on the right, second row from the bottom, photo credits to PZ)

IVIG is still a godsend-- with the exception of one ER trip back in mid January rom the needle going into my port cracking which caused a reaction because my continuous infusion was stopped for those couple of hours, and one other incident, I have not needed to use the EpiPen, and my steroid dose is the lowest it has been in years. The moon face is finally going away! I actually feel like a normal, functional human being-- sure, I don't have much stamina, and I still have a lot of symptoms, but I'm functional, and respond to treatment when I need it, which wasn't the case before. The IVIG days in itself still sucks. The past few times, I have gotten really severe migraines, body aches, dizziness, some nausea, and a low grade fever with my infusions, but I also know that I will feel better 3-4 days later. I can also tell when it is starting to wear off, as I get more fatigued, and I know that I am "due." My next round of IVIG is at the beginning of March.

This month, February, is also rare disease awareness month! I have included some infographics made from the Mastocytosis Society below. Not only are they fun and colorful,  but I think they provide some quick insight into the basics of mast cell disease, and how there is so much variation even amongst individuals with the same diagnosis in terms of triggers, tolerances, response to treatment, etc. I am working on planning an event at school for the first week of March to close of Rare Disease Awareness month, so stay tuned!

A reminder/PSA-- IVIG, which I get once a month, is made from blood donations. So if you can, please please donate blood! Not only is it important in the wake of recent tragedies across the country (and globally), but there is also a need for it day to day for a variety of diagnoses and treatments.

This weekend I have been working on a bunch of readings and essays to get ahead for this upcoming week. My core group of grad school apps are submitted, and now I just apply to jobs, and wait to hear back. I head back to San Diego on Friday for my cardiology follow up. My most recent labs were a little wonky, so we're making sure that I am still getting enough nutrition. I also am really excited because we are doing a get together for other young adults with POTS, MCAS, and other related conditions this weekend in San Diego, which I think is going to be a lot of fun! I have made a lot of really great friends online who "get it," so getting to see everyone in person in special. I am actually doing research for one of my courses this semester with one of those friends, which should be cool as well (but if I talked about it, then it would eliminate you from the potential participant pool :P).

So, long story short, things are pretty status quo, which is a very good thing (and a nice change)! I'll post updates as I figure out more of my new adventures over the next few months.

A Sweet End to 2017!

As I was trying to come up with a clever title for this post (which as you can see, did NOT end up happening), I was thinking about the different happenings of the past couple of weeks, and honestly, I am thrilled to report that things have been going really well! Lots of positive things to report, academically, interpersonally, and health wise.
I changed up the formatting a little for this post so you can skip/scroll down to what you are interested in (basically, more fun stuff at the top and bottom, health updates in the middle).
Finishing Finals
In my last post, I was finishing up finals-- I had two, and although they were both tough, I got one grade back, and I aced it! It's official, graduation date is May 12th! I am going to miss everyone in Claremont and at Pitzer, but I also feel like I'm ready to move on (Pitzer pals I promise you aren't chopped liver!). After my second final, I was so ready to go back home, and it was great to be able to be back in time for the first night of Hanukkah. My little brother, Cody, still had a few days of school left before break. My dad and step mom truly went above and beyond this year and got me some great stuff to make me more comfy in the new house. We went to an event in the shopping center located in our new development where they carved a menorah out of ice, which was pretty cool to see! We are certainly getting some smoke from the wildfires, but thankfully, we are far enough from the largest fire currently winding down (the Thomas Fire in SB/Northern Ventura County).

First Round of Outpatient IVIG/Two Patients in the House
A day later, I had my first outpatient round of IVIG (also discussed in last week's post). I anticipated that it would be rough, because the first round that I completed inpatient was rough and had the antibodies removed that I didn't have. This round was pretty tough, more so than the inpatient one. It was run over 2 days for 6 hours a day. It can't go through the port in my chest, because it is chemically incompatible with the Benadryl, so it has to go through a normal IV in my arms. Most of my veins are either scarred or don't cooperate from my EDS, so I went through 6-7 IV attempts, and 3 actual IVs over the course of the 3 days. Side effects were also brutal-- dizziness, nausea, fatigue, migraines, and overall pain/weakness. I was given premeds and fluids, which is standard for anybody undergoing IVIG, but it still was no fun. Luckily, the side effects subsided after a couple of days, and I tried to distract myself in the mean time with studying for the GRE. My stepmom and aunt also came sat with me for a few hours each day, also nice to break up the time.

Even though the IVIG itself sucked, it seems to be doing it's job-- I am now 4 and a half weeks epi free! This is my longest streak since the summer. Like I said in the last post, I'm still pretty symptomatic, but the fact that the symptoms haven't escalated is awesome. Although it is a little early to tell if it is a fluke or the IVIG starting to work, I'll take it either way. I will have to continue to do the IVIG every four weeks (the same 2 days, 6 hours a day), so my next round will be the second week of January.

That weekend was a little crazy due to family gatherings, me feeling crummy, and on top of that, my dad needed to have surgery on the second day of my IVIG, as he fell off a ladder while trying to land Hanukkah lights (he jokes that God was smiting him for being Jewish and still putting up Christmas/Hanukkah lights, but at least these lights are blue and white). Luckily, he didn't hit is head or do anything too awful, but he did tear his bicep off the bone and break his toe. That sounds SUPER painful, but we have all been really impressed that he has been a trooper throughout the whole experience. He is on the road to recovery and working on PT, but will take a while to fully recover.

Updated Beads of Courage

The GRE!
The following Monday, I took the GRE-- although I am not sure if I will be going to grad school right away or attempting to enter the work force, one of the programs I am applying to required the GRE (of course, only one). I was pretty nervous to take it because I had been working through a practice book which was pretty tricky, but I was pleasantly surprised to see that I had done much better than I anticipated, which was also a moment of pride!

Follow Ups in San Diego/Current Game Plan
The next day, I went for follow ups in San Diego with the doctor who manages my mast cell care and a new doctor who specializes in POTS, the autonomic disorder I have that accompanies the Mast Cell. We discussed where I am at, and what the future game plan is-- the IVIG is supposed to take a little longer to work, so it is agreed that it could potentially be a fluke that things have been doing better, but nevertheless, forward progress is great, and if it is from the IVIG, I could continue to have more positive effects, since it can take up to a year to gain the full effects from it. The cardiologist/electrophysiologist agreed that the IVIG is a good idea-- although it is not a "traditional" treatment for mast cell, my situation has been far from "typical" or "traditional," and it has shown promise in other patients, and also is supported by the most recent research that shows that most cases of Mast Cell Activation and POTS are caused by some autoimmune source, although that exact cause remains unknown.. The way it was described to me was the idea of which is the chicken vs. the egg- the POTS causing mast cell symptoms or vice versa (explained really well in this webinar). I was told that the side effects from the IVIG are very standard, so if it continues to be really uncomfortable, the doctor may adjust the premedication regimen, or slow down the infusion. They also said that it does get easier over time, likely from a combination of the body habituating to it/getting better at and refining the premedication regimen.

With the IVIG, the plan is to try to continue to reduce, and (hopefully) discontinue steroids, which will ultimately depend on whether or not my body is able to produce cortisol on its own. This will take in itself at least 6 months to a year, assuming there are no major set backs, and if my body is still able to produce cortisol. I'm really hopeful that the IVIG will help. After that, the plan is to try to reduce some of the other medications that suppress my immune system, since they are risky medications in terms of long term effects, and because it becomes quite dangerous if I do get even a simple virus (so love me from a distance if you have cooties). From there, we work on the Benadryl pump, probably a year or two down the line. Although I want to get off all of this stuff sooner, my body has proven time and time again that it can't be rushed, or I will just end up right back where I started. So, the current plan is to try to very slowly reduce the steroids, and maintain status quo otherwise.

After a long afternoon of appointments, I met up with two other young women who see the same POTS specialist at a local restaurant/brewery. It was great to meet them both in person, and we had a lot of fun, even if it was only for an hour or so! I'm looking forward to seeing them again on my next trip. I am really grateful to have made some amazing friends-- although we all wish we were meeting/talking under different circumstances, it is amazing to have a support network who "gets it." (shoutout to MC who made me a new pump bag/her Etsy store, Molly for the "hot mess express" keychain, spoonie alliance group texts and the lovely notes/holiday cards from the Mast Cell Crew!).

Other Winter Break Fun
As you can see, I have been pretty busy since my Winter Break has started, but I should hopefully have some more down time over the next couple of weeks. Although hectic, there have been other positive/fun developments too! My lease was almost up on my car-- although I have been financing my lease since I got my first car at 16, this was the first time that I entirely financed/negotiated/arranged for the car, and I LOVE it (well like 80%)! I was a little worried about the color, but it looks perfect, and I love the way that it drives. It is also a little higher up, which makes it a lot easier to get in and out of, and also helps with these crazy LA drivers. I am continuing to submit grad school apps, and taking a peek at what job opportunities are out there. It is a little tricky because the opportunities that are listed now are different from what will be available when I graduate in May, but it at least gives me some ideas on where to look.

My mom drove all the way down from Northern California to spend a few days with me, which has been awesome, and today on Christmas Morning, we had some other Jewish friends over for bagels & lox and movies (we tried the Chinese food thing, but it was a 3 hour wait and they would no longer take any orders). I baked some cookies this evening to nosh on too (I probably overdid it today, but it kept me busy). We saw Cavalia, which is similar to Cirque de Soleil, with one of Cody's friend's family on Friday evening.  I went with my new next door neighbor to see Pitch Perfect 3 as well (plot line was predictable, but the music was good).

My little brother is doing well, he wrote an amazing short story about me and is kicking butt academically! He also is enjoying the extra time off and reduced electronic restrictions over the break. I have another 3 weeks until the next semester begins, so I hope to come back to Northern California for a few days at some point over the break as well.

A part of the Jewish Christmas Gang 

My mom and I are big fans of Halo Top, a protein ice cream-- we checked out their new Scoop Shop in the mall, this was her concoction which looked amazing! 

The (Metaphorical) Roller Coaster Ride & IVIG

Good afternoon everyone! I'm sorry that it has been quite a while since my last post, things have been busy! Since being discharged a few weeks ago from CHLA, I was working really hard to get things back on track physically, and to regain my strength and stamina. Any time that I am hospitalized, it is a major setback. I have to "start everything over" to a certain extent.

One of the hardest aspects of recovery after a hospitalization for me is weening off of steroids. The steroids make me feel ok and able to function physically, but long term, the effects of toxicity are not pretty. This is why I have been working so hard with my team to find other "steroid sparing" medications-- this is things like the immunosuppressant (CellCept), oral chemotherapy (Gleevec, which I am non longer on), antihistamines including the IV Benadryl pump, etc. Since starting the CellCept, I generally have been doing better, and after the hospitalization about a month ago, I was able to reduce the steroid dose faster than I am typically able to do. Regardless, each time the steroid dose is dramatically increased while in the hospital, it is rough to decrease the dose-- I get swelling, nausea, fatigue, episodes of anaphylaxis, wheezing, etc. It also makes my face swell with chipmunk cheeks, which I am really self conscious of. They are a mixed bag to say the least.

On the "Calm Down" Mom Panel

Last weekend, I went with Molly, the friend who was at CHLA with me, to the FARE (Food Allergy Research & Education) Teen Summit in Newport Beach. Although we felt like we were a little old for some of the programming and a little out of place, it was great to network, get off campus, and take some excursions in Orange County. We went to Downtown Disney (which was NOT a pleasant experience-- we were told that 'people with disabilities stand in line all day'), got to find fun food allergy safe foods at Whole Foods, found some safe chocolate at a local candy shop (putting some links at the bottom for anybody interested in our favorite finds!).

Sunday afternoon *before* things started not going super fantastic. For individuals with POTS like Molly and I, we are supposed to eat a high salt diet, hence the double shaker action.

I think both of us may have slightly overestimated our stamina going into the weekend-- by Sunday, we both felt pretty awful. All day Sunday, I was feeling "off" from my latest steroid reduction. I tried everything I could to avoid the hospital-- breathing treatments, running extra IV fluids, taking extra medication when appropriate, etc. By the evening, I started running out of options-- I had already exhausted all of my at home treatments, and still was showing signs of minimal improvement. I called the clinic, and was told that because it was impacting my airway, I needed to go in immediately.

Molly was also not doing well as the result of complications from their POTS and Ehler-Danlos and associated complications, and my team was reluctant to send me to the hospital closest to school because of how poorly they had treated my case in the past. After speaking with both Molly and myself, and because Molly is trained in using EpiPens, we ultimately decided that it would actually be safer to go back to CHLA, to ensure that they would be equipped to handle my care. Molly was going to need to return there in the morning anyways, so the doctor asked us to go out to CHLA ASAP, and that they would call ahead to be anticipating my case. Although this may sound unusual, given the situation, I was better off getting to a place that was equipped to handle my care.

We loaded into an Uber right away with 9 EpiPens-- we felt horrible for that Uber driver! Luckily we only had to use 2, but it was quite a relief when we actually made it to the hospital. In the ER there, the team also tried everything to be able to send me home, but ultimately, it was back to the PICU. They restarted the epinephrine drip and cranked the steroids back up. Molly was also readmitted, but to a general medical/surgical floor. It was weird both being back there again, but I think that it helped both of us stay positive/optimistic to have each other there, particularly for the experiences that were painful both literally and figuratively. Given how crazy it was that we were trying to make it to CHLA, we have officially (jokingly) named ourselves the "hot mess express" (which Molly modified a graphic novel to document).

The "hot mess express" book

Throughout this week, I have been kind of MIA on social media/blogging just because, as I am sure you can imagine, this is very frustrating. Even when I try all of the right things/try to be proactive, it is not always enough. In order to help channel my energy/focus, I have been working really hard all week on Graduate School Applications and school work. I am still not 100% sure what I want to do after graduation, but I figure that I should at least apply to some different opportunities and programs to keep my options open. I am caught up, but I want to make sure that it stays that way. I think Thanksgiving will also be a good break to make sure that I can get even further ahead. I am all registered for my final semester of college!

I was on the epinephrine drip until Wednesday, so a total of about 3 days. On Tuesday night, the team at CHLA decided to give IVIG a try (part of the reason why it was important to go to a specialty hospital, to be able to try new treatments). IVIG, or intravenous immunoglobulin, is a blood based product made from blood donors, that is a concentrated version of antibodies (this link describes a little more about it). It is typically used for autoimmune disorders or immunodeficiencies. I have an immunodeficiency, but it is not one that IVIG is typically used for. We also suspect that my mast cell may be caused by something autoimmune, but there has not been any confirmation of that yet. While I was inpatient, they decided to give it a shot. Because of my immunodeficiency, I have to receive a special, more highly purified version of IVIG. Since it is a blood product, it is certainly not the most comfortable procedure--- they gave me some premedication ahead of time, like Tylenol, Extra Benadryl, and steroids, but I still had some discomfort. It ran for a little bit over 6 hours.  I am appreciative of the CHLA team for taking me on, even though I technically am a little old for the place, until I get a more solid team established closer to my parents house. There were some rough moments this week, and their staff was extremely supportive, and willing to work with my primary mast cell doctor (who also has gone above and beyond).

It will take a few IVIG treatments to determine if it will have a positive effect, but my mast cell doctor collaborated with the CHLA team, and was really happy that they got this started. The fact that I didn't have a negative reaction is at least encouraging that it did not make anything worse. The next hurdle is insurance-- because this is somewhat of an "off-label use," and because IVIG is so expensive, I may not be able to get it covered. We are trying to get it covered for a treatment every few weeks. Each dose of IVIG requires pulling components of donating blood from a few thousand donors, so if you can donate blood, do it!! It is not any sort of directed donation, especially since it is processed significantly to make the IVIG itself, but having a healthy supply of donor blood helps make the treatment more accessible.

On Thursday night, the primary team that was caring for Molly (GI), ultimately decided that it was best for Molly to head back home to DC and be admitted to the Children's Hospital there first thing Friday morning upon landing. Molly is planning on returning in the Spring for the next semester. Of course, I am bummed that Molly had to head back, but I am glad that they have their home team/family/support network there as well. These experiences have been "unique" to say the least, but I am incredibly thankful for their support and friendship.

I was discharged last night, and I am SO thankful that my family now lives closer by. Beth, my step mom, was able to visit me once earlier in the week and pick me up last night. I am not going to lie, I'm feeling pretty crummy today, but it is good to be home.  It will also be great to see my dad later this afternoon, who should be landing (any minute) from a business trip to India. Cody wrote a story about me for school, which I absolutely LOVED, and he certainly has a talent for writing! I won't post it here since it is his masterpiece, but you should ask him about it if you're curious....

I am planning on staying here for another night, and hopefully heading back to school tomorrow for classes Monday and Tuesday, and then returning for Thanksgiving. If I don't "bounce back" by tomorrow, then worst comes to worse, I go to school Monday for my home health appointment, and then drive back that evening. Thanksgiving weekend should at least give me a good enough break to be back on my feet a little bit more the following week.
For the rest of the weekend, I am planning on continuing to relax/work/write/etc. I have a bit of recovery ahead of me, but I am trying to remain as optimistic as possible that I can bounce back quickly from this episode as well.

On a happier note, if anybody is curious about some of the fun recommendations from the Food Allergy Conference (not anything promotional, just some favorite finds).....


Cookie Dough Do Bites- these were probably our favorite find, chocolate chip cookie dough that you are SUPPOSED to eat raw, and they are top 8 allergen free! They do have one product with coconut in it, but they take precautions to prevent cross contact.

Sugar Rush Sweet Shoppe- cute family owned candy shop in Irvine. They carry Vermont Nut Free, which makes AMAZING skippers (M&Ms that are nut free) and fudge! You can also order directly from Vermont Nut Free, but since I am on the west coast, it is pretty pricey to ship.

Enjoy Life Foods Chocolate Chip Crunchy Mini Cookies- I have always been a fan of Enjoy Life's mini cookies and chocolate chips especially, but I hadn't had these ones before the conference, and they were pretty delicious!

Spokin- a great app for anybody with food allergies/restrictions/intolerances/etc. You are able to review and read reviews on food products, restaurants, bakeries, doctor's offices, etc.