Hickman is In

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

No Infection! But Still Stuck

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!

Well, I'm at UCLA! But NOT in the way I wanted to be.

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.

APP or bust!

Shabbat Shalom!

The calm before the storm!

Follow Ups... and More Follow Ups

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.