This Girl is on Fire (not literally)

Hello everybody! I hope you are having a great week, the weather has been absolutely beautiful! I would have like to have spent more time outside this week, but I have been able to enjoy it in small spurts. Not much to update on this week. I am STILL in a flare from my routine TDAP shot 3 weeks ago. I contacted my mast cell specialist, and they told me that it will just take time for my immune system to calm down-- I am not a patient person in this regard, but I know that I need to be.

He also said it would just get better with time, but unfortunately, the past couple of days have been worse. I think that this is because of all of the pollens in the air (I can see a coat of it on the car), in addition to being due for Xolair next week, and the reduction in my steroid dosing. I know I try to remain positive on here, but I am not gonna lie, this week has been pretty uncomfortable. The mast cells cause my skin to flush from head to toe, in addition to itching. If I scratch the itch, it makes it worse and starts to burn. The flushing also makes my skin burn, hence this week's blog post title. The best way I can describe it is a sunburn.

Luckily, I only have had to use the EpiPen once, today, for red pepper flakes on a chicken sandwich that I have had before (they marinated it differently this time). Although I have been uncomfortable this week, this is still a LOT better than before the Gleevec. I haven't had to even think about going to the hospital, and I am actually able to control my symptoms with oral medications. My breathing /airway was only affected in the chicken sandwich incident. Before, I probably would have gone through 3+ epi pens, and not always responded to them. My fingers are crossed that it remains this way throughout the weekend, and my body gets a chance to calm down. In the mean time, I am taking it easy, trying to Benadryl "blast" my system and trying to let my body catch up. The benadryl makes me feel kinda crummy, but it is better than the alternative, hospitalization and steroids.

On a more positive note, my Beads of Courage arrived today! I was super excited for these. Each bead on the string matches a part of my battle, and is a symbol of courage. My bead strand as of today:

And the meaning of each of the beads:
Black- needle stick/port access since 3/1
Magenta- infusion since 3/1
Larger blue beads: each year of treatment (2)
Fish- travel for medical treatment
Bumpy- ankle boot
Rainbow- Physical or other types of therapeutic services
Blue- clinic list since 3/1
Brown- hair thinning

Although adding beads isn't something to celebrate per say, I am proud of my beads, and the fight that they represent.

Last thing for this week, another reminder to sign up for my team for the Superhero Showdown Virtual 5K! It is a benefit for a foundation which helps support patients with MCAS like myself. Here is a registration link:
https://runsignup.com/Race/Register/RaceGroup-355043?raceId=30904
The price goes up tomorrow, 3/31, so sign up now while you can! For those of you in the Bay Area, I am going to try to host a group of walkers and some snacks after. Here is the Facebook event link for that, and please make sure that you register for the 5K AND RSVP on Facebook:
https://www.facebook.com/events/283415038730540/
Since it is a virtual 5K, you do NOT have to be present at the race in Pennsylvania or at the event I am hosting. You walk for 30 minutes, or a 5K, and send documentation to the race organizers (screenshot of a fitbit, treadmill, etc.), and get a medal and a t-shirt. Let me know if you have any questions as far as that goes.

Have a great week everyone!

Attempting to Keep Up the Forward Momentum

Hello again! I hope you all have had a fantastic week so far, we're more than halfway there! I apologize for not posting an update since my San Diego trip. I had two friends visit me on their Spring Break, and got the opportunity to grab lunch with another friend, which was awesome! I loved getting to see and spend time with everybody.

Last Wednesday, I went to my doctor's office to receive a routine vaccination, the TDAP (Tetanus, Diphtheria, Pertussis) vaccine. I anticipated that I would be a little sore afterwards, since the vaccine is notorious for causing that, but I was not anticipating that I would have an anaphylactic reaction to it. Luckily, I was in the right place at the right time, and my doctor was right up the hallway. It was my first time using an EpiPen (injectable Epinephrine/Adrenaline that is used in severe allergic reactions) in a while, and it resolved the symptoms enough that I could go home.

I hoped that using the EpiPen would be the end of that fun, but unfortunately, my immune system wasn't too fond of the idea to calm down. I am not going to lie, this past weekend was pretty miserable-- I went through another 2 doses of epinephrine, and a bottle of liquid Benadryl. I used the new Auvi-Q for the first time, which was SO much less painful than the traditional EpiPen (thanks Kaleo for covering out of pocket expenses for commercial patients). There were many close calls of debating whether to go to the Emergency Room or stay home. Most people are instructed to go to the emergency room after just one dose of epinephrine is administered. However, because I know my body and the way it tolerates the drug well, I am allowed to "wait it out" at home after one dose. In all honesty, I probably should have gone to the emergency room, but I am glad that I was able to wait it out at home. We are trying really hard to ween me off of Prednisone, which is a steroid that I discussed in my post last week, because of all of the negative side effects and potential long term consequences. Going to the ER would have made that difficult, if not impossible.

I got my Xolair dose on Monday, which helped things calm down significantly. I am still pretty reactive, but nothing that isn't responding substantially to Benadryl. Again, thank goodness for antihistamines, Xolair and Gleevec. It is easy to see that I am reactive now and was to the TDAP, and start to think that the Gleevec isn't working, but I know that there is absolutely no way that I would have been able to stay out of the ICU this past weekend had I not been on the Gleevec. It still seems to be making a positive difference, and my Potassium and RBC finally seem to be settling down as well.

With my new "Care About Rare" Mast Cell Disorder awareness shirt.

For those of you interested in learning more about Mast Cell Activation Syndrome (MCAS), my primary diagnosis, this video (click me) is an amazing video put together by BBC, who features another person with the same diagnoses as me, Brynn. She is on a CDI, or continuous diphenhydramine (Benadryl drip), through her port. I have a port as well, but luckily we have been able to avoid having to do a CDI since the Gleevec has been helping me. It is an intense video, but informative and important to share.

Also on Monday, I started physical therapy on my ankle and for my POTS. We are going to work on learning ways to exercise without triggering POTS or my mast cells, positions that are beneficial to POTS, etc. I am looking forward to continuing my work there. Also, sometime in the next couple of weeks, my Beads of Courage should be coming! I will post more about that when they come, but they essentially allow me to document my experiences through a tangible method, where I get a bead for each triumph I encounter (needle poke, anaphylactic episode, etc). Thank you so much to my grandparents for their donation to BOC so I could get off the waitlist before I age out of the program.

Lastly, another reminder to sign up for the Superhero Showdown Virtual 5K! To join my team, click the "join" button next to my team name, Arielle's Mast Cell Milieu. You do NOT have to walk an actual 5K, nor be physically present at any race! All you have to do is walk for 30 minutes or a 5K, and send the race organizers documentation to get your medal and T-shirt. If enough people sign up, I may organize an informal walk in the park so we can do it all together. This raises money for Super T's Mast Cell Destruction Foundation, which helps people like me who are experiencing financial difficulties as a result of complications from Mast Cell Disease.

I am confident that over the next couple of days, my body will continue to calm down and get back to more of a baseline. It's frustrating to have these hiccups, but I have to keep reminding myself that it is still an improvement, and take things one day at a time. I hope you all have a fantastic week!

A Successful Trip to San Diego

Hi everybody! Sorry I haven't posted an update for a while, I figured that I would hold off until the end of the week when I could do one big update. Today, I woke up bright and early to fly down to San Diego, where my Mast Cell Specialist is (shoutout to my step mom, Beth, for also getting up early to take me to the airport). I generally fly Southwest, which is technically challenging, as I have an airborne sensitivity to peanuts and peanut dust. Southwest serves peanuts on all of their flights. In order to minimize my exposure to peanuts, and because of my immunodeficiency, I pre board in order to wipe off my set before sitting down. They also provide me with a peanut dust allergy slip which I hand to the flight attendant, which signals to them to not serve peanuts on my flight.

My flight there was uneventful, and I got a little cat nap in on the short hour long flight. When I got there, I was a little red and flushed, but I figured I was going to the right place. Another young adult with MCAS, Savannah, was kind enough to help me out today by picking me up from the airport, taking me to the appointment, and hanging out with me all day. I am incredibly appreciative to Savannah for making a physically exhausting day just a little bit easier! It was also awesome to get to meet another person with MCAS in person, I never have had the opportunity to do so. We had an awesome time together (at least I did), and I look forward to meeting up with her again (hopefully soon!).

Savannah and I at the end of our day!

Now, for the actual appointment, it also was a success! The Gleevec appears to be helping. I have only had to use one EpiPen in the three weeks that I have been soaking the coating off the medication, which for me, is a dramatic improvement. Also, other than 2 small flares, I have dramatically backed off of my Benadryl use. So, I got permission to start reducing my Prednisone (steroid) dose once I calm down from this mini flare. This made me a very happy camper, as I am NOT a fan of Prednisone. Not only does it have unpleasant long term effects, but short term, it makes me bloated, gives me cravings, gives me "moon face," and makes my hair fall out. I will have to reduce my dose very very slowly, but any reduction is a step in the right direction. In the mean time, I will rely on more Benadryl to reduce the dose of the Prednisone as necessary.

If things continue to go well, I will continue to back off the Prednisone, and a few months down the line, hopefully some other meds as well. We are hoping the Gleevec will continue to provide more widespread coverage. In the mean time, I will continue with Benadryl and Epi as needed, but hopefully not often. Additionally, we will continue to monitor my red blood cell count and my potassium, as both seem to have been affected by the Gleevec and the Prednisone. Nothing alarming, just something to monitor. The flight back home was also uneventful. I premeditated with Benadryl which reduced the "tomato" look after landing, but I still had some flushing. I was a little later for the flight than I would have liked due to me being a poor navigator, but I made it in perfect timing.

For this upcoming week, I have a friend from school coming into town, which is exciting, and otherwise, things are business as usual. I am hoping to maybe begin volunteering across the street at the hospital where I have been hospitalized in the past, in the Sean Parker Center for Allergy Research, which is conducting groundbreaking research on peanut allergies, environmental allergies, and much more. It would be nice to be there not as a patient! I don't qualify for their studies since my allergy is not a "classic" igE mediated allergy, but it still would be cool to be involved there.

One other thing-- I am participating in a Virtual 5k (where you walk for 30 minutes or a 5K at home and email a photo as proof) in June, and I would love it if you would join me! It is called "The Superhero Showdown," and is raising money for Super T's Mast Cell Foundation, which raises money to help patients like me with MCAS with medical and living expenses. Here is a link to join my team, Arielle's Mast Cell Milieu: https://runsignup.com/Race/Register/RaceGroup-355043?raceId=30904#.WMIjrldVqYg.facebook

Have a great week!
Arielle