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-- 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!

Well, I'm at UCLA! But NOT in the way I wanted to be.

Instead of getting to move into grad housing as I initially had planned (although this was deferred a few weeks ago), I've been at Ronald Reagan UCLA Medical Center since Wednesday evening-- again, not the tour I wanted.

For the past few weeks that I have had the PICC (peripherally inserted central catheter) line at home, my nurse and I noticed that there has been some drainage from the insertion site. To some degree, this is normal, but once it was consistent more than 6 weeks after placement, the insertion site had gotten larger, and had some irritation, I was told to go into the ER to get the PICC line pulled and replaced with a double lumen Hickman, which I have mentioned before, but is similar to the PICC line, just placed in the chest. I called my doctor first to see if they could get me on the schedule with Interventional Radiology and the community hospital that placed the line. Because I'm a high risk patient, the vascular surgeon refused to operate on me, out of concern of complications.

This meant that we would have to go to UCLA, since my primary care doctor is part of the UCLA network. It is about an hour/hour and 15 minutes away from home. When we got to the ER, it was packed. I am really immunocompromised, so I was wearing my mask and placed into a little cubicle, but the earliest I was able to get a room was after waiting almost 3 hours. I was better off in the cubicle until I got that room, because it was so crowded that they had gurneys lined up 2 x 2 in all of the ER hallways.

I was wheeled into the room, and even with the mask, it STUNK of bleach- they had just poured it on the floor to clean the room. One of my sensitivities is extremely strong smells, so I began to have a reaction to the bleach. They wheeled me out right away, but even the nurses there commented on how strong the smell was. The reaction progressed really quickly, so they grabbed the doctor, who had the nurse grab the epinephrine from the crash cart, and gave me a dose of IV epinephrine. That put a lot of stress on my heart, so I began writhing in pain in my chest and stomach. I got really dizzy, and later found out that my pulse was pretty thready, so they put me in another room that didn't have the bleach smell in the trauma area.

Although I responded to the medications, I continued to react, and still needed more epinephrine. My visit only for issues with my PICC now was turning into an admission for anaphylaxis. I "earned" myself a one way ticket to the MICU (Medical Intensive Care Unit). I was not a happy camper, but knew realistically that even for the PICC line alone, they would probably have to admit me at least overnight, because anybody who could place a new PICC or Hickman was gone for the day.

They started me on IV antibiotics through the PICC line, and accessed the port that is still located in my chest. I also was given the increased steroids for the anaphylaxis, additional Benadryl, epinephrine, oxygen, etc. I have been in the MICU since then, and unfortunately, have still been really reactive. I have had episodes at 4AM 2 days in a row requiring additional epinephrine.

On the bright side, it does not look like there is an infection in my PICC line. It has been 36 hours since my cultures were pulled, and knock on wood, nothing has grown yet. I have not had any fever, but I was told that because I am so immune compromised, I wouldn't necessarily get one. However, my white blood cell count went through the roof, more so than it usually does for steroids. Because of that, they are continuing the IV antibiotics, and trying to make sure there isn't an infection hiding anywhere else.

We are pretty confident that it is just my body undergoing a lot of stress, between the reaction, and the fact that the TPN (IV nutrition) had to be stopped temporarily. TPN is primarily dextrose, or sugar, which can harbor bacteria. Even though we are pretty confident that there is no infection, it isn't worth the risk of spreading any infection. Because of stopping that, and the additional epinephrine , my electrolytes are all out of whack, and I feel pretty terrible from the lack of nutrition. It really shows how much the TPN has been helping me.

Once the cultures have a little more time to "cook" then they will resume the TPN. The current plan is to place the Hickman Monday, and start the TPN through the PICC if we are fully confident there is no infection, or restart the TPN once the Hickman is placed. Once I get the Hickman, they can remove the PICC and the port.

Basically, we are in a "holding pattern" until Monday. This also means that we have to restart the steroid taper, which as always, is frustrating, and of course, that I am not stable enough to start the Rituxan. At least we are getting the Hickman line taken care of while I am here.

UCLA's Medical Center and staff as a whole have certainly been better equipped to handle my needs. Because it is such a major center, they have been able to have rheumatology and allergy/immunology, see me. Although none of the staff are familiar with my conditions beyond the basics, since it is a teaching hospital, they are very willing and able to learn and adapt, which helps a lot. They are really intrigued by my case, which is not an award I want to win, but also means that they are willing to think outside of the box.

Surprise visit from Sasha

Being farther away from home is a little trickier. I am incredibly thankful that Beth, my step mom, stayed bedside the first night, and has visited daily. I also got to have my cousin Maya and Uncle Marc come last night, and brought me some Ben & Jerry's to make up for the TPN calorie loss, and my friend Sasha surprise me by visiting when I thought that she already flew home from Boston! I'm hoping to get out Tuesday, but needless to say, I'm antsy to get out. They brought by a cute Shabbat kit since it is Friday evening. The food here is also REALLY good- not worth being in here for it for sure, but helpful. For dinner last night I got pasta with sun dried tomatoes, black olives, grilled chicken, and goat cheese (not a fan of the goat cheese, but still yum).

Check out this menu! This is one of ten pages.

Another curveball that we will have to deal with upon discharge is that the is currently a national shortage of injectable/IV Benadryl. For someone like me, who literally depends on this medication to stay a live, this is extremely problematic. It isn't clear when this shortage will be resolved. This situation is even more complicated by the fact that I am allergic to the preservatives in some brands of IV Benadryl. I have had issues with some brands before, but use of another brand the other night confirmed that I still have reactions to the preservatives. They have been able to stock it here in the hospital for me, but my outpatient pharmacy is unsure of how much they can get. I'm hoping this shortage is resolved sooner rather than later, because this adds a lot of stress and means that I could have to be re-hospitalized just to get my medications. I am thinking I might try to write to my congress person and the FDA just to add any additional pressure wherever possible.

APP or bust!

Shabbat Shalom!

The calm before the storm!

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!

The Waiting Game

Although I would LOVE to say that I am back at home catching up on sleep, I am stuck in the ever so familiar holding pattern- the waiting game. Since my last post, I was able to tolerate the final day of of IVIG, which we were hoping would provide enough of a boost to see an improvement in my symptoms, and as always, to decrease the ever-so hated steroids. Each round of IVIG doesn't necessarily make a difference independently, which was true in this scenario. High dose IV steroids have now been building up for almost two weeks, which should mean that we able to decrease the dose slowly- yet, again, my body doesn't seem to be a fan of the agenda.

The IV steroids work. But that's also the problem. Although they help reduce my symptoms, reduce the severity and frequency of reactions, there are countless side and secondary effects that make them dangerous long term. I have already developed osteoporosis from them, and long term use increases the risk for metabolic issues, diabetes, advanced bone degeneration, and a whole host of other issues. 

To try to make up the "gap" that the IV steroids fill, I have been on multiple immunosuppressants for a few years. Now that we know for a fact that my gut absorption is close to nada, we discontinued two of my immunosuppressants. Instead, we have now added Methotrexate, which is another immunosuppressant and chemotherapy medication. Although its technically a chemo, I am on a low dose, and side effects are supposed to be minimal, and primarily related to mouth sores, hair loss, and issues with blood counts. It is an injection that I will receive once a week.

I have received one dose of the new medication, but unfortunately, even if it is helpful (which we have no guarantee of), it can take 3-4 weeks plus to see a difference. In addition, like the other immunosuppressants I have taken, it is "broad spectrum," meaning that it impacts my entire immune system, but may not be specific enough to address the particular autoimmune process that is happening for me. I'm hoping that over time, as that does builds up, and because it is an injection instead of an oral medication, that it can help with the steroid reduction.

So, for now, we wait. I still feel pretty guilty being a "negative Nelly," but reasonably, I'm frustrated. We're all frustrated. The team here has been amazing, and looking into a variety of treatment options, research, etc. We know what's happening, we know the treatment options, but there just isn't a whole lot of targeted therapies that we can try and apply. It is just going to take time for things to mellow out, and to slowly decrease the steroids. Originally, the game plan was to have me out today or tomorrow, but now it is looking like I would be going home later this week if things go ok. I've burned through quite a few coloring and sticker books to avoid going stir crazy from not leaving the unit in more than a week :P There have been some more canine and human visitors which also helps the time go by :)

On the bright side, having nutrition, vitamins and proper electrolyte levels through the TPN has made a major difference, even though I'm not thrilled about going home on it. I am working on trying to eat as much as I can in addition to the TPN to help keep my gut somewhat functional. My electrolytes are finally starting to stabilize, and I'm not having  any more of the weird cardiac episodes. The PICC line in my arm that I am receiving meds through is being finicky, but we are trying to preserve it for as long as possible. I suppose "counting my blessings" is right in this context, that I have the ability to go home with support like TPN so I can avoid living in the hospital. I'm hoping my next post will be one heading home!

IVIG Day #2, TPN Day #4

Thanks again for everyone's well wishes and support over the last couple of days! It certainly has felt like a bit of a time warp, which I guess is kind of to be expected when you're in the same room for a while. Since my last post, I had my first three days of Total Parenteral Nutrition (TPN), and two days of IVIG (intravenous immunoglobulin).

For those of you who have been following my story for a while, I have been receiving IVIG monthly, and then bi-weekly since November. My first admission of the past couple of weeks occurred after an adverse reaction to my standard IVIG infusion. IVIG has helped immensely, so we were not ready to give that up unless there was really persuasive evidence that I could no longer tolerate IVIG. To test that, we have been doing 1/3 of my usual IVIG dose each day, with the third and final day tomorrow. With the high dose IV steroids I have been receiving for almost 2 weeks, these infusions went smoother, which overall is a good sign. I still react to the infusions, but have responded well to the meds to treat the reaction symptoms.

Awesome surprises!

The first 24 hours on the TPN was kind of rough. I felt even more run down/beat up than usual, almost as if I had the flu, and some reaction symptoms. My body seems to have adjusted well to it, and now that I'm actually getting the nutrition that I have been so short on for so long, I am already noticing a difference in my strength, energy, and thinking. When I came into the hospital, I could barely walk because I was so weak, and experiencing arrhythmias from low electrolytes, which have now almost entirely resolved.

We are still working my way up to a "full" daily dose of TPN. Because of how malnourished I was, they have to slowly add in nutrition to avoid what is known as Refeeding Syndrome. Refeeding Syndrome occurs when the body is overwhelmed by nutrients if they are introduced too quickly after extended periods of malnutrition, and can cause issues with electrolytes, metabolism, etc. They watch for it closely through daily labs, and adjust the "cocktail" of vitamins, minerals, calories, and electrolytes in the TPN accordingly.

My labs are still showing significant issues with electrolytes, which again, is absolutely no surprise because of the levels I was starting with and the continued auto-immune process impacting my kidneys and gut. In addition to the TPN, I have been getting additional infusions of potassium and phosphorus.

Yellow= TPN, White= Lipids, Clear = Benadryl

I haven't gained weight (supposed to take time, not overnight), and my electrolytes are improved but certainly not resolved, which means that the plan is to continue TPN after I am discharged. I mentioned this in my last post as well, but usually TPN is used for individuals with little to no ability to consume food or use their GI tract. In my case, it is being used as a supplement to get me closer to a healthy weight, maintain electrolytes, and a minimum of 1,000 calories a day of intake. The hope is that over time, we will wean me off the TPN in a few months. I still have concerns of infection, practicality, and the impact on the rest of my organs related to the TPN, but at this point, it's what we need to sustain me in order to even explore other treatment options down the line.

After the final day of IVIG tomorrow, the plan is to over the weekend to slowly start decreasing the IV steroids. I'm still on a higher dose of steroids than I was re-admitted on, so that also is going to take time and patience. Real conversations of discharge haven't started yet (other than confirming that the TPN is sticking around), so realistically speaking, we are looking at mid next week.

For those of you who are curious what goes into TPN. Lipids are a separate bag.

I'm of course still majorly bummed about graduate school and the overall conversations about treatment options and such, but I'm glad we are at the very least seeing progress. My def The team here that is part of the UCLA network has been amazing, compassionate, patient, and realistic about the situation at hand. They have taken the time to listen to us/our concerns, and helped me continue to maintain confidence when I bring about concerns or questions. I am a little irked that nobody was more aggressive about nutritional support up until this point, but there's not much that can be done about it.
In the mean time, the snacking/Netflix/coloring/sticker book/tutoring saga continues. I am SO appreciative of the visitors, messages, gifts, food deliveries, and support over the past few days. It really does help pass the time and keep me occupied. I'm hoping for a very uneventful weekend with the steroid decrease so I can make my jailbreak (a much longer one) next week!

Another reminder that 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!

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!

PICCing and Choosing My Battles

I am just now realizing it has been 2 months since my last blog update-- whoops. Since my last post, I have been working hard on recovering from my last hospitalization back in May, continuing my work as a Family Resource Coach whenever possible (and loving it), and even got in a VERY much needed vacation!

We left at the end of June for Disney World and a Disney Cruise to celebrate my college graduation and my little brother's fifth grade graduation. My doctor was a little reluctant to let me go because I have been having some electrolyte issues (see boring medical update below if interested), but I was SO thankful that I was able to. I have always loved cruising, particularly Disney, so the opportunity to go on an 11 Day trip on an itinerary that is only offered once a year was too great to pass up. This trip was certainly complicated by more health related logistics from previous trips, like IV medications, pumps, coordinating lab timing, etc., but everything fell into place really well. We flew in two days early to have a "buffer" for shipments and lab work before getting on the ship, and time to adjust to the time change and recuperate from traveling. We got on the ship in Port Canaveral, Florida, spend some time at sea, followed by Aruba (checked out coral reefs in a submarine), Martinique (wheeled around the immediate port area), Barbados (stayed at the in-dock shops), San Juan, Puerto Rico (my personal favorite), and Castaway Cay in the Bahamas (I went to a shore side store then hopped back on the boat), which is Disney's private island.

!

The food on the cruise was absolutely amazing, and I certainly would recommend it to anybody with food allergies. Everyone really went above and beyond to find me safe, delicious food so I could indulge like everyone else! Some of my favorites included the top 8 allergen free molten lava cake, a cookies and cream sundae made with Enjoy Life Cookies, Udi's muffins, and Katz brand donuts. I also had the opportunity to meet another young adult with the same conditions as me, which was shocking since the conditions are so rare. I really enjoyed getting to know and spend time with her, and I was also thankful to meet a new friend who "gets it!"

After coming back from the cruise, I also got an opportunity to meet my friend Brittany for the first time in person. Brittany and I have been talking for a couple of years now about our common academic and social interests, in addition to the fact that she is also impacted by the same conditions. We both weren't feeling 110% physically, but we still had an amazing time, and certainly stayed occupied! We figured out how to get into the pool at least up to our waists with central lines, tie dyed hospital blankets to make them more homey, explored the various FroYo shops with allergen friendly options in the area, crafting, and did some baking. The time really flew by, and we are already working on ideas for another meet up!

Now, the health update. Since my last blog post back in May, health wise, things were improving, but very slowly. I have still been having issues with gut/nutrient absorption, and particularly a loss of electrolytes, which can be a problem because a) it makes me feel crummy and b) electrolytes (or a lack there of) can cause cardiac complications as well. After lots of lab work, it appears that there is an autoimmune process (probably a component of the Sjogren's, Hashimoto's whatever) that is going after my kidneys and/or parathyroid that is throwing things off, but we are still waiting for a full interpretation of those results. My GI track seems to be joining the party as well, as my labs look like I am not absorbing my immunosuppressants, which also impacts nutrition. This obviously poses a major issue and complicates treatment.

This past Friday, I had my regularly scheduled IVIG infusion. I was already having some signs of a flare up, but was hoping it would help. This reaction just didn't respond to meds like it should have, so the infusion center sent me to the ER. There, things kind of went down hill, and I ended up in the ICU on an epinephrine drip. They weren't able to get a vein that would hold the additional IV needed for the drip, so it took a whopping 10 tries (which was absolutely miserable) to get an IV that only lasted for a couple of hours. I am still in the ICU, but supposed to be getting moved to the step down unit this evening. I'm a little concerned about how step down will go based upon my last hospitalization in San Diego, but certainly am excited for more privacy and have to give the team here the benefit of the doubt. This is my first time at this hospital close to my parent's new house, and there have been some issues so far (including being served cake with coconut on it), they are trying and have been responsive.

The next morning, they decided to place a PICC line in addition to my port to provide additional reliable IV access without having to dig for veins. I have had them before-- it is a tunneled IV that is placed in my arm and leads to my heart. The current plan is to keep it in place until my single lumen port can be switched to a double, because IVIG, Epinephrine, and another new infused medication the team is hoping to try are not compatible with the Benadryl that is infusing 24/7 through the port. They de-accessed (removed the needle) from my port in the mean time and are using the PICC line instead to reduce the infection risk that would arise from using both the port and PICC at the same time. If my arms heal before they can get me in to place a double lumen  (lines/separate tubes) port instead of my single lumen, then they will pull the PICC line out early. I'm really uncomfortable with the idea of having more than one central line because of the infection risk, but I also understand why it makes sense to keep it in until we have a better back up plan.

What a double lumen port looks like under the skin. My current port only has one clear bubble.

As all hospitalizations are, this stay has been difficult, but even more so because of the length of stay, and honestly, frustration. We know that everything is this autoimmune sh*tstorm, but there really isn't a way to stop it. I am genuinely concerned about what my future holds, and how this disease progression is going to go. There are a whole lot of unknowns and not a whole lot of answers, even from the "best of the best" in the field. I don't know what long term quality of life holds, nor what is going to happen next. It honestly is a horrible position to be in at my age, but at this point, there's also not a whole lot I can do about it. I am sick and tired of being sick and tired, and also frustrated that even when I do everything I am supposed to, things still progress.

This cascade is making me nervous about graduate school. There have been some snags in the process of getting accommodations and field placements sorted out. I am trying to avoid it, but I may need to defer enrollment for a year to get a better handle on things. I have to ultimately make a decision in the next couple of days, so I'll see how things go here, and I'm continuing to speak with disability services to ensure that everything falls into place. On the bright side, my health insurance finally approved my custom ultralight wheelchair. Although we were not able to secure power assist in time, it will be helpful to at least have the chair.

In terms of my hospital jailbreak, it is yet again boiling down to an inability to come off IV steroids. We tried once already to no avail, so we are going to try again tomorrow morning. I have appreciated having some visitors while I have been here, and food that is more edible than what they serve! :P  One new oral medication was started this morning, although I was told it likely won't have much of an effect, and if it does, will take 3-4 months. They may try one other infusion while I am inpatient, which still takes 3-4 weeks to work, but is better than 3-4 months.
We are hoping for discharge later this week, so keep your fingers crossed!

Operation LA or Bust: Success

I am very happy to announce that "Operation LA or Bust" was a success, and that I am resting back at home! I was discharged yesterday late afternoon, and my dad and I got home around dinner time. I'm so relieved to be back in my own house and bed, but each time when I do get discharged, it is unfortunately a reminder of how much strength or energy I've lost during a hospitalization. It's really frustrating to feel that I'm doing so well, and have made so much progress health wise, yet can't get around even in my own house. It almost feels like I've been in some sort of time warp for the past couple of weeks. I have my 22nd birthday to look forward to in a little over a week, although my brain isn't quite there yet.

I'll get my my strength back, the never ending steroid taper will resume, and the moon face will come and go again. But in the mean time, any progress is progress, and I just have to continue to remind myself (again) that slow and steady wins the race.  I've lost a lot of body mass, including muscle, which will take time, physical therapy, and lots of super yummy nutritional shakes to build up :P I'm hoping to try to work for a day or two this week to help get "back in the groove."

Between now and July, I am participating in the virtual "Denim Dash," a 5K for rare disease awareness, something that is obviously quite near and dear to my heart. This race is something I can complete from home-- walking, wheeling, pedaling, whatever. If you are interested in donating or joining the team, sign up here: https://www.crowdrise.com/o/en/campaign/the-mast-cell-crew?utm_campaign=oc&utm_medium=facebook&utm_source=crowdrise

I get food cravings on steroids, but once they're actually in front of me, I'm uninterested. This was an ongoing point of light hearted conversation between my dad and I and the medical team-- "Cookie Mountain." They kept bringing them to me and I decided to try to have some fun with it. 

Good bye and good riddance!

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