Thankful and Bye Bye TPN!

Although my last few blog updates have been relatively negative, I wanted to start off this post (which is finally more positive) acknowledging the fact that I have so much to be thankful for. The past few months have been absolutely positively brutal, but the past couple of weeks have served as a reminder to take a breather and count my blessings.
 In my last post, I mentioned the severe anemia and admission to UCLA in Santa Monica for the blood transfusion, iron infusion, and PICC line replacement. That seemed to help with my energy, and the new PICC line they placed seems to be agreeing with me more than the Hickman and my last PICC did. It turns out that what we kept thinking were infections were actually a reaction to the material that the actual line is made of. It is currently being used for the continuous IV Benadryl, fluids, Pepcid, and steroids.As we anticipated, the GI scopes/biopsies and genetic testing didn’t reveal anything helpful, although as more is understood about MCAS and the other conditions that impact me down the road, it could come in handy for me or other patients.
 All of the central line frustration and concerns raised the urgency to get me off TPN even though I am not up to the goal weight yet. My oral food intake has been up, so we decided to cut my TPN in half for a week, and monitor my weight and electrolytes, and then reassess. They both seemed to hold for the week of half TPN, so we cut it entirely. I have now been off TPN for about 2 weeks, and so far, my weight is holding (thing to be thankful for #1). My electrolytes have been borderline a couple of times, but not to the severity they were before starting TPN. I am still supposed to work on gaining more weight, but as long as I’m maintaining or gaining, and not losing, I should be able to stay off of it! It certainly is nice to cut out that prep work from my nightly routine.
 I have been also trying my best to work one day a week for both my sanity and a change of environment. My boss has continued to be extraordinarily supportive, and I love what I do (thing to be thankful for #2). The commute is brutal, as it can take up to 2 hours each direction with traffic, which is absolutely exhausting for me but one day a week is worth it. Having that day where I am NOT the patient is refreshing.

Dogs dressed up for Thanksgiving at work! 

November 8^th was a challenging day for my family and community as a whole. For those of you who don’t know, my dad, step mom, brother, and I live in Thousand Oaks, CA. A little after midnight on 11/8 I was woken up by Facebook emergency alerts stating that a shooting had occurred at Borderline Bar & Grill, a restaurant close by, which actually shared a parking lot with my infusion center. I had not been there before. Information was just coming out since the shooting had only occurred an hour before, but by the time I woke up in the morning, the details of the atrocity began to emerge. It felt surreal to be seeing Thousand Oaks and buildings that I recognized on CNN. I did not know anybody who was injured or lost their life during this incident (thing to be thankful #3), but I know many people who did. One of the umpires for my brother’s baseball league that him and my father recognized was one of the victims.
 That morning already felt like a time warp, and I was scheduled to go to work that day. I left early because part of the freeway was still blocked off, and I would have to pass the shooting scene on the freeway to get to work. When I did drive past on the freeway, it was eerie—the parking lot was still full of cars from the night before, cars that I knew belonged to the victims. My thoughts were with those who had loved ones there, and I could only imagine the pain they must have been experiencing.
 I was at work, and by the early afternoon, I was experiencing symptoms of a mast cell flare up (itching, swelling of my lips and tongue, hives, etc.). I gave myself additional Benadryl, inhaler, etc, but the episode didn’t seem to be fully responding. I was terrified of having an episode in a patient’s room or not being able to make it to a reliable medical facility if I needed emergency medical attention since I am not supposed to use the one at Children’s. I decided it would be best to head home early so I would have access to more of my emergency meds if needed. My step mom texted me to let me know that I should wear a mask when I got home because there was a fire on the other side of the freeway, but I didn’t really think twice about it.
 I was approaching the 101/405 interchange when I got a call from my dad saying that our neighborhood was just placed under mandatory evacuation orders, and that they were packing up the essentials and needed to leave now. The fire jumped the freeway, and we received emergency notifications saying that we needed to immediately evacuate. This was the Hill fire that started while I was at work, the Woolsey fire had not started yet.
 Before I got this call, I was already debating pulling off the freeway to use the EpiPen or go to an ER because I wasn’t feeling well, and I still wasn’t responding to my other emergency medications. I decided to get on the 405 instead because I have family who lives close to UCLA, and I figured I should be close to where I could receive medical care if I needed it. I was on the pass and still couldn’t control my symptoms. I looked to find a place to pull over to use my EpiPen and call 911 if that didn’t work, but I didn’t realize there aren’t many places to pull over on the pass (don’t try this at home kids). There were only 2 exits until the exit for the main UCLA hospital, each a couple of miles apart. If I got off the freeway there, it would have taken longer for EMS to get to me. Traffic was moving on the freeway, so I tried to get as close to the hospital as I could before I pulled over. I got off the freeway, and of course, still no where to pull over. I ended up having to use the EpiPen at a red light. I finally found a place to pull over, but by then, I was 0.25 miles away from the hospital, so it was faster for me to just keep going. I had a friend on the phone who knew my location the entire time in case something happened.
 The entire drive was absolutely terrifying to say the least, but I was so thankful (reason #4) that I got to the hospital safely, and that things didn’t hit the fan until after I got there. I tried to wait a few minutes in my car in the parking lot, trying to convince myself that I didn’t need to go into the ER, but things were worsening, so I went in. I’m glad I eventually did because even though they didn’t even triage me before pushing me back to the trauma bay, my oxygen saturation was in the 80s while my heart rate was around 150. They immediately gave me additional epinephrine, fluids, medications, etc. A cousin came to sit with me which was much appreciated, because it was scary to be going through the ER with the trauma I experienced at that hospital the last time while literally watching my home town burn on live TV.

What I was watching on TV...

This reaction was stubborn—I ended up getting a total of four or five doses of epinephrine, which landed me back in the MICU. They mixed an epinephrine drip to have on standby, but thankfully (reason #5) they didn’t have to use it. This hospitalization was a lot better than my previous ones there. It COULD have been related to the complaint letter that I wrote and received the response/investigation findings from the day before I was re-admitted. Regardless, I was glad that this experience was slightly more positive given how much stress was involved both in the hospital and with the wildfires and the shooting all on the same day.

Meanwhile, my family evacuated, and had to re-evacuate 2 more times, as each place they were evacuating to was evacuated as the mandatory orders spread with the Woolsey fire. They returned home on Saturday evening. Thankfully (reason #6), our house was totally fine, which was a HUGE relief. With the whole idea of giving thanks/thanksgiving, seeing the devastation from the shooting and the fires all around our area has been sobering. The damage everywhere is extensive, and although the fires are gone, the smell of smoke is still strong at times. We are lucky that we have our home to seek refuge in, but hundreds of families weren’t that lucky, made worse by the fact that this all happened right before the holiday season.
 I was discharged late the next Monday evening (11/12), because we had to wait for the reaction to calm down, the air to clear up a bit, and I ended up developing a UTI that was causing some kidney pain while I was there. I was home in ample time for Thanksgiving, and I have been able to return to work twice since that admission (thing to be thankful for #6).
 Despite the immense amount of stress and chaos Thousand Oaks has had over the last couple of weeks, things seem to be returning to status quo. All schools re-open tomorrow for the first time in about 2 and a half weeks (my brother is NOT excited). Roads are re-opened, and most people have returned to work. The signs (picture to the right with mention of the Borderline 12, a reference to those who passed in the shooting, are no longer on all of the major thoroughfares. As I said earlier but will say again because of the gravity of the situation—I am THANKFUL that our family is able to return to our status quo.

 In terms of what I’m up to, things are still pretty up in the air. I had a consultation with a hematologist who specializes in MCAS in Orange County a few days before the chaos. She agreed that I likely have a hereditary angioedema (swelling) or secondary angioedema component to my symptoms. The angioedema is usually what keeps me in the hospital since it has the potential to block my airway. I was started on a medication for hereditary angioedema just to see if it makes a difference, even though we aren’t confident that my angioedema is specifically hereditary. My insurance has not approved the medication, but the manufacturer covers the first month.
 Because of the additional hospitalization, the hematologist and my primary mast cell physician told me that they feel it is now time for me to start plasmapheresis, which is a treatment in which the blood is filtered to remove auto-antibodies. Unfortunately, this would dramatically change my routine, as it starts off at 4-6 hours 5 days a week, and no facilities close to home are properly equipped. I am waiting to hear about what the logistics of this will be, and then I’ll post an update explaining more. In the meantime, I had the first dose of the angioedema medication, and am trying a liquid iron supplement to see if I can absorb it in hopes of avoiding more blood and/or iron transfusions in the future. I also am supposed to go back to Mayo for a follow up in December to see if they have any additional suggestions. I also need to make a decision about graduate school somewhat soon-ish, so I’m working on looking into my options and access to accommodations to see what is feasible. I am back on IV antibiotics now because this UTI doesn't want to go away, and is still causing some kidney pain. This week I also have follow ups in San Diego, which should be relatively uneventful.

 In summary—I have a LOT to be thankful for. In addition to the reasons explained above, I can think of countless more—having a 15 year old lab who made it through the evacuations (heck, the fact that she’s still living it up is a miracle in itself), to have the financial means to access healthcare and live comfortably, a house that is still standing, to have access to food, water, shelter, supportive family, some of the top medical experts in country (with some exceptions :P), etc. The list could go on and on. I have very many long and treacherous journeys ahead, but I knowing that I have a support system means the world to me. Happy belated Thanksgiving everyone!

PS- If you have not already, PLEASE sign this petition here! There is current a national IV Benadryl shortage which not only threatens my access to this life saving medication (I receive a 24/7 infusion of it) and others with MCAS, but also many others using it as a pre med for chemotherapy, treatment for allergic reactions, etc. It takes just a minute, and every response helps! There even is a video about the shortage featuring yours truly :)  Petition Link

 Video about the Dipenhydramine Shortage

Signs for first responders on the entry sign to our neighborhood.

View from our house the morning after I came home.