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.

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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

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! 

Finals Season, 3 Weeks Epi Free & IVIG Update

I can't believe it is already the end of the semester! These past couple of weeks really flew by. But, I am happy to report that I am officially done with classes for my second to last semester of college! I have 2 finals-- one on Monday, one on Tuesday, then I'm done! All of my other final papers and assignments are turned in, with the exception of one that just needs to be fine tuned. I have also been doing my online tutoring when I am able to as well. To be totally honest, I am still pleasantly surprised with how things have worked out academically this semester (but granted, I did put a LOT of effort in too).

I have been having to "hide" at school a lot this week. We are about 30 miles away from the closest fire, but the air quality is awful. Also, everyone seems to be sick, which is really not an option for me. I don't see or smell smoke at school, but I tried to go without wearing my mask, and boy, did I feel it. Yesterday (Friday) and today (Saturday), I tried to go without the mast on my limited excursions outside, but still was having some trouble. My parents' house has been more affected, as they are pretty close to multiple fires, but luckily, far enough away that nothing was at risk. And I'm sure my brother enjoyed the time off school.

My school is on the semester system, so I will have a month off-- a nice break. Nothing too exciting is planned, but I would like to try to make a trip back up to Northern California at some point during the break.

I double checked my calendar, ant it has been three weeks since I last had to use an EpiPen! That may  not sound like a lot, considering most EpiPen users don't have to use them (or have to rarely), but for me, that is the most time I have gone without having to use it in the last 5-6 months or so. That isn't to say that things haven't been rough physically, and I still have reaction symptoms daily, but the fact that it hasn't escalated to that point is great.

Now, for figuring out why my reactions have not escalated, so I can keep the trend going for as long as possible---- could be a fluke, or could potentially be the IVIG that I received at Children's. I thought I remember my doctor saying that the IVIG would take a few treatments to see if it is working, so I don't know if it is even possible that it is the reason why I haven't had to use the EpiPen. But in the mean time, I'll take it and not question what the source may be.

As I discussed in my last post, getting insurance coverage for IVIG, particularly when it is not being used in a "traditional" way, can be a challenge. This is especially true for me, as I have a separate immunodeficiency which can increase the risk of anaphylaxis to blood products (more info about it here). My body doesn't produce the antibody igA. Since IVIG is a blood product, it contains igA. The type that I received in the hospital either had low or no igA to minimize the likelihood of reaction. My insurance approved IVIG, but not the lower/no igA version-- I have to try the other version first.

To minimize the likelihood of a reaction, they are going to run it very slowly over the course of two days, for 6 hours each day, at an infusion center closer to my parent's house. I will also be given some additional medications before and during the treatment. It was not a particularly pleasant experience last time, even with the low/no igA version, so I am not looking forward to it, but I know that it will help in the long run. I have been told that it is pretty typical for IVIG to be somewhat uncomfortable. And, I always remind myself that it could be a LOT worse. I plan to distract myself by cramming for the GRE, which I am taking the following Monday, and maybe catching up a little bit on my favorite fall TV shows. And maybe binge watching some new shows too :)

I have started submitting grad school apps, which also feels so weird! I still don't know if I want to go right away, or work for a bit first, but I want to keep my options open. I'll start looking for job opportunities in the early Spring as well. Now I should probably stop procrastinating and get back to studying....