Wednesday, February 27, 2013

Last Visit Before Transplant

We had another very long, but very productive day at CHOP last week and we're on track for transplant on April 11th!

We started off our visit with Dr. Baluarte, the transplant nephrologist and Stephen pulled out all the bells and whistles to prove to him that he's big and ready for the surgery! I'm actually not kidding. My kid acted like a complete angel, walking all around the office in his diaper and "spanx" showing off, and let Dr. Baluarte lay him down on the table for a full exam. Which never happens!! He was adorable, talkative, and kept himself occupied with an Elmo video, his trains, and some cereal.

After that, we headed over to the Wood Center to meet with the infectious disease doctor, Dr. Kim. We met with Dr. Kim for a quite away and Dr. Baluarte eventually joined in on the discussion as well. The main objectives of meeting with Dr. Kim were to get his opinion on the cdiff and his immunology status (i.e., IVIG). Dr. Kim actually has a different opinion on Stephen's immunology status than the doctor we saw here in Virginia. This is actually a first for us that two of Stephen's doctor's disagree on his plan of care. Dr. Kim feels as though Stephen has responded well to his vaccines and is making antibodies, and therefore, does not need to receive IVIG. And feels as though the risks of IVIG outweigh any potential benefit. Whereas, Dr. Alpan (the immunologist in VA), believes that Stephen needs the IVIG because his IGG is low. And Dr. Kim feels as though Stephen does not need to be re-tested for cdiff ever again (he was very adamant about this) unless he is having very very specific cdiff symptoms.

Dr. Kim did request some additional bloodwork to be done to double check his B cells and his immunization responses, and since last week, everything has come back normal. So Bill and I are inclined to agree with Dr. Kim, but we're still waiting on his doctor's here in Virginia to conference on these latest results and come back with their opinions.

After that, we then met with the other transplant surgeon, Dr. Abt, who we had not yet met with. He was very nice and obviously brilliant, and we feel even more confident about the surgeon's skills and knowledge. I haven't been doubting our choice to travel to CHOP for this, but have just been questioning whether or not going the whole way to Philadelphia was the right thing to do. But after this trip, and discussing it more with Bill on the way up to Philly, I can now say that there's no doubt in my mind that this is the right decision for Stephen, and for Kristin too. I know that not only my child will be in amazing hands in the operating room, but so will his donor.

And last but not least, we had to update our "profile" with the social worker. Because dialysis and transplant centers are overseen by Medicare in some ways, there's all this stuff we have to do that the government requires. But I think the social workers at CHOP are going to come in handy after transplant trying to coordinate insurance and medications, which I'm looking forward to. Up until now, I've navigated this on my own, but am looking forward to having someone help me a bit with all of this new "post-transplant" world.

All in all, we were at CHOP from 11:30 to 4PM. It's a very long day for a two year old who didn't nap, but again, helpful in the long run.

And before we headed out of town, we went for a tour of an apartment building; The Franklin Residences. I'll share more about our housing plans in a separate post, but I'm happy to say, the housing decision is behind us and I couldn't be happier with our final decision! Thank you very very much to everyone who offered advice, help, and suggestions for housing in Philadelphia, and to those who offered their homes to us as well. The outpouring of support was amazing and I truly appreciate it!! Once we solidify everything, I will post where we plan to be and when and how you can come see Stephen if you want! Our doors (and the hospital doors) will be open for visitors at anytime!!!

Monday, February 18, 2013

Weekend wrap-up

Even though Stephen was sick on and off this past weekend and I think his new nephrologist (there's a new neph in the practice) would have liked us to take him to the emergency room on Friday night, we managed to do some fun, but totally normal, stuff this weekend. Between the continued diarrhea and Stephen throwing up pretty much everything, he became really dehydrated which caused his blood pressure to drop really below normal levels (he had BPs in the 50s-70s instead of around 110...yikes!). That's what prompted his doctor to want us to take him to the ER. But since we really hate the ER and they always seem to keep Stephen overnight, we decided we would manage the dehydration at home and give it a day or two to see how things went. And by this morning, the puking had subsided and we're working on getting back on track with his eating, weight, and hydration. Thank you to pedialyte!

But in the midst of that, we took Stephen for a haircut, a new pair of sneakers, to the pet store to see all of the animals, and to the bookstore for some new books. I'm embarrassed to say that I hadn't been in a Barnes & Noble in years (sorry, Anne!), and was completely shocked at how amazing their children's section is! They have an incredible section of books, board books, toys, puzzles, and games. And there were kids everywhere reading and playing. It was awesome! And Stephen had a blast looking at the books and climbing up on the stage and stools and interacting with all of the kids. There were a lot of "hi's" to all of the other kids. We had so much fun that I think Barnes & Noble will be a frequent trip for us; not to mention their weekly story times!

I actually used to take Stephen to Petco during the Summer when it was really hot to see the turtles and the fish. Kind of like a poor man's zoo :) We actaully hadn't been in awhile and I need to get a new collar tag for Mabel, so off the to "the zoo" we went. This time we didn't see any turtles, but Stephen fell in the love with the cats that were up for adoption and the dogs who were getting groomed. It's so cute seeing him "talk" to the dogs and cats and he really loves animals!

We also took him to the mall for a trim. He was in desperate need of a haircut and at the suggestion of a reader, we took him to a kids haircut place this time. Stephen did much better this time being able to watch Cars and all of the other kids and loved the lollipop he got at the end. And then Billy picked out a cool new pair of bright orange sneakers for Stephen. I probably would have never picked them out myself, but they look so cool on Stephen! And of course no trip to the mall is ever complete without a stop at Nordstrom's for a cup of coffee for Mom and Dad and some good ice for Stephen!

Here is a good before and after shot of the hair. You can see someone's mop was cute, but pretty unruly! It's a bit short, but still so cute and his hair seems to grow like a weed so I'm sure it will be back to unruly in no time!

Friday, February 15, 2013

False Alarm

Good news! The repeat lab work we did the other day to confirm the dangerously low white blood cell count came back normal! Hallelujah! There must have been some sort of error in the way the original labs were drawn or how they were handled or within the lab to get such a crazy result. But it doesn't really after. All that matters is that the labs turned out to be fine!

We're still waiting on the repeat PD fluid culture from yesterday to confirm whether or not Stephen has peritonitis, but the doctor didn't seem too worries, so I'm not getting worried about it. I am however a bit concerned about the crazy diarrhea Stephen still has and the super low blood pressure he's had for the past 24 hours. Plus, he just seemed off today and was a bit lethargic. I'm just hoping that maybe he's got a little stomach bug and this will pass by the end of the weekend. So we're staying at home this weekend and taking it easy. Maybe a little haircut for Master Stephen and a ride on the choo choo at the mall. Can't wait to see his face when he sees a real live choo choo train!!

Wednesday, February 13, 2013

Clinic Visit

It's only Wednesday at 2PM and already it's been one hell of a C.R.A.Z.Y week!!

We were missing in action last week because Bill and I actually snuck away for a few days (didn't want to share this news with everyone and jinx ourselves) to enjoy some sun and sand in Ft. Lauderdale.  Bill received the Leader's Club award from Wells Fargo and the sales conference was in FL so we took the opportunity to get away for a few days.  It was wonderful being away and I was truly able to relax and forget about things for a bit, but life certainly hit us like a freight train the minute we returned!!  I shall explain.

The day we left, Stephen's vancomyacin taper ended and he began a two week stint on a new antibiotic that would hopefully kick the cdiff out once and for all. But Sunday afternoon as we were picking Stephen up from Bill's parent's house, his diarrhea returned with a vengeance. A very bad sign that the cdiff was no where near running it's course. Then Monday morning as I was cleaning Stephen up from blowing out his diaper in bed overnight and tending to his super raw tushy, fluid started squirting out of a hole in his PD catheter! It was just unbelievable!! It's like we were being punished for enjoying a few days of vacation! I just couldn't believe what was happening!

Thankfully after spending three hours at the nephrologist's office taking blood work, PD cultures and fixing his catheter, they were able to repair the catheter without having to go to surgery to replace it. The hole was very close to his transfer set, so the dialysis nurse was able to cut it off just above the hole and put on a new transfer set. This actually isn't the first time this has happened and you'd think that the catheter manufacturer could make the tubing a bit more durable to prevent this from happening. There's basically a pressure point at this one particular place unless you let your catheter dangle straight down all day long. And that's a big no-no, so you have to coil up and around your body in some way. But anyway...they gave Stephen a loading dose a two antibiotics (just what he needed while already having diarrhea and battling cdiff) and sent us on our way.

Later that day I spoke with his infectious disease doctor who shared the same fears as I did and asked that I take a stool culture the next morning to they lab to see if he was in fact still positive for cdiff. So thats what we did and in the meantime, I've been bleaching every piece of clothing, blanket, and sheet in sight. And pouting. It's been a real kick in the pants to say the least and I was completely convinced that his transplant would be postponed indefinitely to the point of not thinking it would happen at all this year. I felt totally and utterly defeated Monday night.

But I am so glad I didn't announce that on the blog, because his culture came back negative for cdiff this morning!!! Amazing, fabulous, stupendous news! And the doctors actually don't think that this is a false negative this time (we've had those over the past 5 months) because they ran a very sophisticated test. So we actually got great news first this morning.

But then of course with the good news came the bad news; as always. Stephen's blood work should a very significant drop in his white blood cell count. So much so that the on call doctor received a page from the lab. The nephrologist believes that this may be a sign that Stephen's liver and spleen aren't functioning properly to the point where blood isn't flowing from the liver to the spleen properly causing the spleen to become very enlarged (which we already knew was the case) resulting in portal hypertension. We knew this was always a possibility, but up until now, all the tests have shown that Stephen's liver is functioning just fine; that it was just enlarged. But now they're (and we're) not so sure. There are ways to fix this including placing a shunt in the liver via Stephen's neck and possibly a liver transplant, but we're going to have to go see some more specialists (yippee) to figure this out before transplant.

And of course as we're pulling into our neighborhood from the nephrologist's office today, I get a call from the dialysis nurse, that the preliminary results of Stephen's PD fluid culture came back with a growth of gram positive cocci. In layman's terms that means he's got some type of bacteria growing in his PD fluid from the hole we found. Just fabulous!! But the nephrologist thinks it could be a false positive because his white blood cell count in the fluid was normal. So for tonight, no antibiotics at home and then back again tomorrow for another culture and a loading dose of antibiotics. It looks like we just might be at nephrology every day this week!

And let's not forget about our follow-up appointment with immunology tomorrow and cardiology for an ECHO on Friday.

So in essence (I apologize for the boring details), today was not unlike most days; BIG highs and SUPER low lows. One big gigantic roller coaster ride. Gotta just focus on that negative cdiff culture results, because otherwise, I just might lose my marbles this week! Or maybe just hitch a flight back to sunny Florida :)