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.