When it rains, it pours, hails, snows, sleets, etc. -- Failed IVIG, 2 Additional Admissions, Bye Bye Hickman, The Return of the PICC, Frustrations and Got Blood?

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.

When it rains, it pours-- back at UCLA .

I apologize for being MIA for a little while-- the past few weeks have been a challenge, with some "speed bumps" to say the least. I've been a little discouraged from blogging because everything has been so up in the air. I feel like I've been regressing to the point where I'm afraid to make too many commitments or plans, because I don't know how my health will be at any point in time.

So far, the new Hickman catheter has overall been working out well-- I have been able to continue the IV nutrition (TPN), the continuous Benadryl infusion, and my other medications pretty easily. It healed a lot quicker than the PICC line that was poorly placed ever did. The TPN is still overall going fine. I am about 10 pounds from the minimum goal weight, but right now we are more focused on maintaining nutrition and electrolytes overall.

Since I left the hospital this most recent stay, I've still been having difficulties keeping my mast cell symptoms, particularly the angioedema (swelling) down over time. This swelling is problematic when it is in my tongue or throat because it can impact my breathing and airway. I have continued to slowly decrease my IV steroids, which we hoped would be a little easier with the addition of the methotrexate (low dose chemo injections). I still seem to be way more sensitive than I already was, and I'm not so sure that the methotrexate is doing anything other than giving me additional side effects. 

I had a follow up with my endocrinologist since my blood tests positive for Hashimoto's, which is an autoimmune condition that targets the thyroid gland. Thankfully, although I have the autoantibodies, it hadn't really impacted my thyroid function as of yet, so I get regular blood work with the knowledge that at some point soon(ish) it will be impacted. My autoantibodies increased, and my thyroid function slowed slight impairment, so when I received a call from the doctor's office saying that I needed to come in to review my test results, I was assuming that it was about my thyroid.

When I got into the exam room, the nurse handed me a glucometer. I asked her why she was giving it to me since I'm not diabetic-- in fact, my A1C and blood glucose have been consistently beautiful, despite the fact that I've been on high dose steroids long term, and that TPN is primarily a giant bag of dextrose (sugar). The doctor came in shortly thereafter, and said that yes, the thyroid looks like it probably will be treated in the near future, but that she called me in because of my pancreas. 

I was pretty surprised to hear that my pancreas was involved since it has never come up before. She said that one auto-antibody in particular shot through the roof, which was concerning since I'm on immunosuppressants and IVIG. She said that this particular test indicates that there are auto antibodies being produced by my own body that are attacking my pancreas, which would make it so that my body could no longer produce its own insulin-- thus, giving me Type 1 Autoimmune Diabetes, and insulin dependent. 

I asked if changes in diet could prevent the diabetes, like it can for Type 2 Diabetes, but I was told that since it is purely autoimmune, that isn't the case.  She said that treating the overall autoimmune disease process could help delay the onset of the diabetes, but not prevent it, and that I'm already on many of the aggressive treatments. I asked about a timeline for the development of the diabetes, and she said the longest she had seen was three years, but their autoantibodies weren't as high as min. Since my A1C and sugars have been so great, she said that I should just check my blood sugar if I'm feeling really off, but otherwise, nothing else needs to be done yet. (The Mayo doc did later say that its possible that this antibody is indicative of general inflammation as opposed to a targeted attack on my pancreas, but nevertheless is rare problematic).

In the grand scheme of the things that I deal with health wise, Type 1 Diabetes isn't a huge deal. They make so many great gadgets and gizmos for monitoring blood sugar, administering insulin, etc. The part that really upset me with this development was more that I am doing everything I am told to do, doing the aggressive treatments that make me also feel like crap, and yet, nothing is changing-- in fact, it is progressing. 

I emailed my primary mast cell doctor and asked if he had any suggestions. The infusion, Rituxan, that we wanted to try wasn't really much of an option right now because I'm so unstable. He said that he wanted to talk to the physician I saw at the Mayo Clinic back in May, but said that it may be time to do plasmapheresis, at least in the short term. Plasmapheresis is somewhat similar to dialysis, but less intense on the body. it involves being connected to a machine that filters the antibodies out of the blood, three days a week for 5-6 hours total each day. Thus, it would dramatically impact my quality of life and ability to maintain some degree of normalcy. The other issue with plasmapheresis is that there are very few facilities equipped to administer plasmapheresis, and many wouldn't agree to taking me on as a patient given my age and the fact that this treatment is atypical or "off label." 

The physician at the Mayo Clinic and my mast cell doctor spoke on the phone and agreed that it was time to pursues one or both of those treatments. I was trying to figure out a facility where I could do the plasmapheresis, but I woke up Monday morning with TPN on my shirt, irritation around my hickman, and some blood by the dressing-- all HUGE signs of infection. Infection is probably the biggest risk of TPN, since the central line used for TPN (in my case, the Hickman) goes directly to the heart. That means that I unfortunately earned myself a one way emergency room ticket. 

We were debating whether we go to the local community hospital or back to UCLA. I was thinking UCLA would be better, since the local community hospital already told me that I'm too complex for them/they are ill equipped, but the community hospital obviously has the advantage of being close to home. We were almost at the community hospital when I got a call from my doctor's office that they didn't like some of my blood work and were concerned about infection. They suggested that I go to the ER at UCLA, and that they would call ahead.

My stepmom took me to the ER at UCLA. This time, I was taken in immediately since where my Hickman catheter was really didn't look good, and because I'm pretty immune compromised. Although there were no clear triggers other than stress, I still started having an anaphylactic reaction. I was so frustrated that not only was I having to make this unexpected ER trip, but also that it couldn't just be for one issue-- now we were dealing with both anaphylaxis AND a suspected line infection. 

Because I'm immune compromised, I probably wouldn't get a fever even if I was in septic shock. That makes it harder for doctors to tell if I have an infection virus irritation or even an allergic reaction to the line itself. They took some blood to run cultures and additional testing. The cultures take a few days to grow, but the other testing was "borderline." Regardless, they were admitting me at that point for the anaphylaxis anyways. They gave me 3-4 injections of epinephrine, plus steroids, additional Benadryl and other antihistamines, and I still had pretty severe angioedema. They decided that they needed to start an epinephrine drip, which I unfortunately have had many times before, which also means a one way ticket back to the MICU (Medical Intensive Care Unit).

In the ER, they had the rheumatology and allergy/immunology teams come see me, which was great. Since it is a teaching hospital, they were going to collaborate and see if they could come up with some sort of game plan. I was moved up to the MICU, but overnight, my labs started to go way downhill. I didn't have TPN, since they don't run TPN until infections are fully ruled out. My electrolytes tanked, and my blood became acidotic, both of which can be dangerous. They ran electrolyte replacements, but actually overcorrected these abnormalities. As a result, my blood sugar was all over the map, and I felt absolutely awful overnight. I had a throbbing headache and felt weak and tired, and had tingling in my hands and feet. I didn't get to sleep until almost 5 AM because of how bad the pain was, and getting blood pulled from my line every 30 minutes to an hour. They eventually figured out that the reason why my headache wasn't responding to pain medication was because my blood sugar was so low. Once they corrected that, I felt a bit better, but I was still hangry to say the least.

They finally let me start eating again mid day today, but don't want to restart the TPN until they are entirely sure that there is no infection. I'm hoping to go home tomorrow or Thursday at the latest. The preliminary cultures are negative, but they said that there could still be a local infection, and the cultures could still grow something, so I am on 2 IV antibiotics. They said they may want me to continue the IV antibiotics at home to err on the side of caution. Thankfully, today has been more stable mast cell wise, but the hospital is actually running low on the one brand of IV Benadryl I can have (I'm allergic to the preservatives in others) which is a MAJOR issue. They're trying to sort that out, but its pretty scary that even a major medical center is having significant supply issues. The rheumatologists here are repeating all of my autoimmune labs here so they have the tests within their own reference ranges, and can see if anything was missed, or if there are any underlying genetic issues that could help explain why I'm not responding to treatment like I should. These will take a few days, and certainly are far from providing any answers.

I'm hoping that after tomorrow, they will be able to send me home pretty quickly as long as the angioedema is relatively stable. They did not dramatically increase my steroids this admission, which is very different from the way things typically go. Hopefully, that should still be enough to cover me, but may explain why I needed so much epinephrine. I am beyond exhausted both physically and mentally, but trying to maintain a positive attitude.  Thankfully, my boss has been very accommodating, and has been giving me a few hours of work remotely a week to help keep me busy in addition to tutoring.

This cascade that seems to be happening is why I titled the post "when it rains, it pours." For some reason, maybe related to the change of seasons, most people I know with my condition or similar ones are really struggling health wise. It's hard to not only experience this myself but to also see others struggle, without many answers or explanations. Research is happening, but it certainly doesn't seem to be fast enough.

I'll continue to post any updates as I have them.