A blog by Arielle, a twenty two year old with MCAS (Mast Cell Activation Syndrome), Autoimmune Angioedema, POTS (Postural Orthostatic Tachycardia Syndrome), Sjogren's Syndrome, immune deficiency, osteoporosis, and EDS (Ehlers Danlos Syndrome). Thoughts, feelings, academic endeavors, daily adventures (and misadventures).
Pitzer College Class of 2018 (Psychology/Sociology) ----
UCLA Extension Patient Advocacy Certificate Program Winter 2019 ------
Family Resource Coach
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 8th 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.
As you might be able to tell from the title of this blog post, a LOT has happened since my last update. I haven't been the best about updating this over the last couple of weeks, nor updating too many people over text or phone either, mostly because of the sheer frustration of having more questions than answers at this point, and quite a few traumatic experiences.
Over the course of my second admission, there were a few "snags" that we hit. No hospitalization ever is completely problem free, but there certainly seemed to be a shift in providers' behaviors that indicated that they were kind of "over" me/my situation. Hence, the hot potato begins-- everyone is intrigued because my conditions and presentation are so rare, but when they realize there are no real answers to be found, they were more than happy to rush us out the door, even though it was a bit premature.
My Hickman line ended up thankfully not being infected-- we suspect that I was reacting to the material in the line itself, as it was the same material as the previous PICC line that I also had issues with. Nevertheless, I came home on IV antibiotics to play it safe. Once I got home, settled, and showered, I was eating dinner when I saw that there was some blood leaking again from the Hickman catheter. That automatically warrants a trip back to the emergency room. I was obviously reluctant to return, so I called the floor that I was on, the MICU, and spoke with one of the residents. This resident didn't seem to fond of me during the stay, and insisted that because I can and do eat, I don't need TPN, even though I have obvious malabsorption and electrolyte issues. He told me that it was fine, to just keep an eye on it. I asked if I should run TPN that evening, since if there were any concerns of leaks or infections, the dextrose (sugar) in the TPN could worsen it. He said that I could skip it for a few nights. I expressed my concerns about electrolytes, but he insisted that because I had some dinner, it would be fine.
The next morning, I went (or at least attempted) to go to IVIG. I was feeling pretty crummy after skipping the TPN, and I suspected that my electrolytes were playing a role. The infusion center staff also felt the same way, but agreed to give it a shot. Sure enough, I was more reactive than usual, and not particularly responsive to medications. They tried giving me additional steroids, fluids, called my doctor, etc., and nothing seemed to be working. I was stable-- not improving, but not really getting worse too quickly either. When I was discharged the previous time, the allergist mentioned that in my next flare, we could potentially try a medication for a condition called hereditary angioedema, which is a condition that can cause swelling, or plasmapheresis. I don't have hereditary angioedema, but we were wondering if giving the med a try was worthwhile. Thus, we loaded up and headed back to UCLA in hopes of giving it a try. We called on the way to give them a heads up to get the allergy team. They told us that they would meet us in the ER, but never showed. Since I was having a reaction, I unfortunately earned yet another one way ticket to the MICU.
At this point, it was very very clear that the doctors were frustrated with my lack of response to medications, and readmissions. Of course I shared the same frustrations, but that doesn't mean that my medical care should be compromised. This admission was the one that was particularly traumatic. I had one nurse say that the angioedema in my tongue was "subjective," and that I should just stick my tongue back in my mouth. One physician loudly and sarcastically announced during rounds, "Look who's back! Ms. Davis!" I was told that my outpatient physician had revoked my diagnosis, and that the rheumatology team said there was absolutely nothing wrong with me-- both of these statements were immediately cleared up by their respective parties-- neither was true in the slightest. I was told that you can't have MCAS and dysautonomia, which is INCREDIBLY untrue-- most people have both. The rheumatologist asked the MICU team to increase my low dose chemo slightly, and the MICU team refused to give it to me all together. Yet, they were the ones that called in the rheumatology team to consult in the first place. My hemoglobin, red blood cell count, and iron also got extremely low. I asked them to try an iron infusion while I was there inpatient, since iron infusions are notorious for causing allergic reactions, even in "normal" people. They insisted that my primary care would handle it outpatient. On top of it all, they didn't have anybody look at the potentially leaky Hickman until the day I was discharged. They removed the stitch holding it in place since it appeared irritated. I suggested that they re-suture it because my skin is so fragile and doesn't heal well, but they insisted that it would be fine.
Unfortunately, it was not fine. In regard to the hemoglobin, iron, and overall anemia, my primary care doctor was not able to help set up iron infusions, and said only a hematologist could, which I couldn't get into for weeks. My energy upon discharge was worse than it was before, likely because I was so anemic. After insisting I see a doctor that could help with the anemia ASAP, I got into a hematologist/oncologist this past Monday. She saw my labs and how severe my bruising ones and said that she was going to direct admit me to the ICU at UCLA in Santa Monica for a blood transfusion and iron later in the week after obtaining a pre authorization from my insurance. She also promised to help coordinate my care long term, which was encouraging.
Back to the Hickman, Monday night/Tuesday early morning, I woke up around 3 AM to use the restroom. On my way back to bed, my TPN tubing got stuck on the corner of my bed, and my Hickman literally fell out since they had removed the sutures. It didn't hurt at all, nor bleed, which attests to the fact that it was not healed like I had suspected. This also warrants an automatic ER trip. I stopped the TPN, accessed my port, and moved my continuous Benadryl to the port. I packed a bag and it was back to the ER.
RIP Hickman
Since my last UCLA experience was so awful, my step mom and I agreed that it was worth a try to see if the local community hospital could help , knowing that it was likely that they would still have to transfer me. Sure enough, they said that I needed a higher level of care. We called the hematologist, and since she was going to direct admit me a couple of days later anyways, she helped facilitate my transfer to UCLA Santa Monica by a critical care transport ambulance.
Once I got to the UCLA in Santa Monica, they already had a nurse bedside with the equipment to place a new PICC line. So far, this one is looking and feeling a lot better. It is a different material than my Hickman and more recent PICC were, but the same material as the ones I had a few years ago. It is much smaller and light weight, and placed in a better spot. They ordered the blood for my transfusion, but because my igA deficiency is so severe that it is undetectable, I required"washed" red blood cells, which basically means that they remove the antibodies from the blood. Of course, given the fantastic series of events that were already occurring, there were strikes at all UC Health facilities, which meant it took 9 hours to get the blood! Thank G-d I was not bleeding out any time in the immediate future.
New PICC Line
Got blood? (It's in the doughnut from the antibody filtering)
I had a small reaction when the transfusion started, but it responded to benadryl and was fine afterwards. The hematologist thought that I had a GI bleed somewhere, so they insisted that I got a endoscopy and colonoscopy, which I was NOT a happy camper about. I was already NPO (no food or water) because of the airway concerns and the potential for a GI scope, but with the timing of the tests, I was not allowed to eat or drink for nearly 2 days. This felt like literal torture. I got TPN the second night, but TPN does not do anything to impact hunger. In addition, because my airway is so finicky, I had to be put under full anesthesia for both procedures, which meant that we had to wait longer to have an anesthesiology team. After 24 packets of Miralax (I never, ever want to see that stuff again), the scopes were done on Wednesday, and came back clean. No anesthesia hiccups. There was some friability on my upper GI system, but they said it was likely steroid related/not clinically significant. They took 7 biopsies to do some stains to see if there was anything on the microscopic level, but they didn't seem to think there would be anything useful. I didn't know that there is a bit of discomfort after the procedure as well, so I felt pretty crummy, and my GI tract still seems to be recovering.
Yesterday, the hematologist called me, and said that she wanted them to do an iron transfusion before I left, but was really only interested in the anemia alone. Thus, hot potato-- bouncing around again to other team members, not because nobody wants to help, but because I'm not a clear cut case, its frustrating to them. Communication from everyone is lacking, and its incredibly disappointing and frustrating. I was just thankful that I was able to go home last night. At this point, we still don't know if I'm losing blood somewhere else or if I was just insanely iron deficient. I apologize to those of you who I have not been giving updates too-- as I said, it's been so frustrating having more questions than answers that I haven't really been particularly social. I am hoping to try to work a day this week. I have some follow ups and appointments with new specialists in the next couple of weeks, so I'm going to attempt to be more diligent about my updates.
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.
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
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!
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.
Over the last two weeks, I feel like a lot of what I have been doing is... you guessed it... follow up appointments. I had 2 scheduled follow ups, as I discussed in my last post- one with my allergist/immunologist, and one with cardiology. My step mom, Beth, came with me to keep me company, make sure I didn't over-do it (I unfortunately have a history of doing so), which made for a really nice mini-trip despite all of the appointments!
My Beads of Courage-- they're a little hard to see in this photo, but each bead represents some sort of medical experience/triumph- every black bead, a needle stick, white beads with red hearts, an ICU admission, yellow, a night in the hospital, etc. Unfortunately, they're getting rather long, but a good reminder to find inner strength!
As anticipated, both appointments were pretty uneventful. Both doctors were really happy that I am on the TPN (IV Nutrition), and agreed with the current plan moving forward- to continue keeping my medications primarily IV, including steroids and the TPN, continue IVIG, the methotrexate (low dose chemotherapy injections once a week), and to try Rituxan, which is another immune-modulating drug. This one in particular is considered to be one of the "bigger guns," so I will have the first dose in the hospital, hopefully this Friday. My IV steroid dose is still very slowly being decreased, evidenced by my lovely moon face at the moment! My allergist also assured me that he isn't giving up on me. Although never said he would, probably one of my biggest fears is that the doctors will get frustrated with my situation, so it was nice to have that reassurance. The other suggestion from cardiology was to consider an external vagal nerve stimulator to see if it helps balance out my parasympathetic nervous system. Both appointments were overall encouraging, as there are still things to experiment with.
I also had an appointment with the Voice Clinic at UC San Diego. Although Vocal Cord Dysfunction has been ruled out multiple times, my doctor still wanted me to check it out to see if they had any additional suggestions. They agreed that I don't have vocal cord dysfunction, but nevertheless were able to show me some tricks to help when I have swelling in my throat to force the vocal cords open. Back home, I also had follow ups with rheumatology, who is going to "run the show" for the Rituxan, and endocrinology, who is monitoring my labs closely. I have the antibodies for Hashimoto's Disease, which means that we are basically waiting to see if/when the autoimmune process starts impacting my thyroid. Catching that early on is best so the thyroid hormones don't get too out of whack.
Since coming home on the TPN, I have gained about 4-5 pounds, which is a good start. I still have a ways to go until their goal weight, but slow and steady weight gain (1-2 lbs a week) is best on the body as it re-adjusts to having nutrition. It also would probably be really physically uncomfortable to gain weight much faster than that. I'm hoping I won't have to be on the TPN for long, but the general consensus amongst my doctors seems to be that they're in no rush to stop it, as it still appears that I'm not absorbing much through my gut, and my electrolytes wouldn't hold without it.
We're still trying to figure out a long term central line solution-- UCLA isn't able to do the double lumen port as originally planned, so their interventional radiology department (IR) suggested having a double Hickman placed where my single port currently is, which is similar to the PICC line I currently have but placed in the chest. The PICC would be removed at that point too, since the Hickman could be used right away. They initially wanted to wait until there was an issue with my PICC, but the PICC is not healing as quickly as it should, which increases the risk for infection. So, at some point over the next couple of weeks, I'll probably have to go through that procedure.
The goal for this week is for family time early in the week with my dad's birthday and Rosh Hashanah, the Jewish New Year, working two partial days this week, a follow up with my primary care doctor, and on Friday, to do the Rituxan infusion. I am hoping that it is something that I am able to tolerate, and that it is is complication free! And for those of you who observe Rosh Hashanah, Shanah Tovah!
My new wheelchair came in! It fits me a lot better than my old one, and is far more comfortable. I use it primarily at work and for long distances, and it does the job well.
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.
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!
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!
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!
Usually I can come up with some clever introduction to my blog posts, but for this one, I'm kind of at a loss. After my last blog post, I was discharged Saturday afternoon with the IV steroids to be able to continue the steroid taper from the comfort of home, and eventually gradually switch to oral/liquid steroids while also trying to get my weight back up to a healthy level.
Yesterday I was going to a follow up appointment with my primary care doctor, and prior to leaving, I started experiencing the same weird feeling in my chest/arrythmia that I have been feeling intermittently. It came and went, but it was enough to make more really dizzy, nauseous, and short of breath. I got to my doctor's office, and at that point, it seemed to fuel some sort of mast cell reaction. They called 911, and I was back off to the hospital.
When I got here, they gave me the usual anaphylaxis protocol, and also took blood work. Those labs indicated what we already knew and had been seeing in my labs for weeks, severe malnourishment and failure to absorb medications and nutrients. They admitted me back to the ICU Stepdown Unit/PCU Unit that I had been on for the latter part of my last admission, and gave me IV electrolytes overnight.
After speaking with the team here and nutritionists, they decided to start me on TPN, or Total Parenteral Nutrition (LINK). TPN is a cocktail of vitamins, minerals, lipids, fats, etc. that provide complete nutrition via a central line, like the PICC that was placed last week or my port. It takes the GI tract out of the equation in terms of what is getting absorbed, since it bypasses the GI tract entirely, which is why they elected for TPN instead of a nasal or surgical feeding tube. It runs for 12-14 hours a day and provides an entire day's worth of calories and nutrition. While inpatient, they take labs daily, and build a "cocktail" based upon whichever nutritional deficits they are seeing. On an outpatient basis, these labs are usually once a week.
In theory and in the short term, TPN is literally life saving. However, the long term risk profile, risk of complications, and impact on quality of life can be challenging. The current plan is to use TPN for a week + to get my weight up out of the danger zone, and provide a buffer. We suspect that at this point, the autoimmune process that has been ravaging my system is partially fueled by the fact that my body is trying to attack tissues for energy sources. Once we have gotten me out of the danger zone, then we will work on getting me off TPN as soon as humanly possible.
In the mean time, I am supposed to eat as much and as frequently as possible to make sure that my GI tract doesn't take an even more extended hiatus than it already has. I am trying to nibble throughout the day, use supplements like Ensure, etc, again to try to avoid TPN complications and hopefully need it as minimally as possible.
That being said, I was supposed to move into graduate housing at the end of the month to begin my MSW program. In light of recent events, the shift in what our plan going forward is, and the timing of everything, I had to make the extraordinarily difficult decision to defer enrollment in my Master's program for a year.
To be entirely honest, I'm pretty heartbroken. I know grad school will wait for me, and that I need to be focusing on my health, but as I mentioned in my last post, I'm naturally frustrated. I am confident in my team's ability to manage things and to anything and everything necessary to get me back up to speed as soon as possible. I'm also scared of TPN complications, but I know that at this point, I don't have any other choices. While I'm here, we also are going to continue to ween down on the steroids whenever possible while trying to also avoid any more major mast cell episodes.
Once I'm discharged, depending on where we are at with TPN, I"ll either get my port swapped to a double lumen (2 part), or a line called a Hickman, which is similar to the PICC line that they placed in my arm, but in my chest. For TPN, it sometimes is less of an infection risk to use a PICC or Hickman in comparison to a port. My educated guess is that I'll be here at least a few days to a week, but it seems to depend on whether my mast cells cooperate with the agenda. In the mean time, I'm working on snacking, tutoring, coloring, and whatever else is distracting and minimally stressful. b
I also feel the need to apologize for the "Debby Downer" kind of post, but for right now, I think writing everything out is helpful for processing. I hope to have a more positive update soon after they start the TPN overnight.
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!
