Monday, August 17, 2015

Spongebob is Evil and Nothing Makes Sense

Poor Max has had a rough weekend. On top of being sick and having to deal with fevers (thankfully not high enough to warrant going to the hospital), poor Max has been dealing with a diaper rash which has made it incredibly painful for him to pee--he screams and writhes in pain every time he urinates. We've started applying some Neosporin which is definitely helping but the area appears to have a way to go before it stops hurting so much. Overnight, Max woke up about every 30-40 minutes screaming in pain and we took turns walking with him as the pain subsided before he fell back asleep.

This is normally the part of my post where I'd insert an amusing picture with a witty caption but there's nothing even vaguely funny about having your baby scream and cry until he's hoarse because it hurt so much and there's nothing we could do except hold him as he rode it out. 

This morning, we loaded Max into the car and took him down to Phoenix to get his blood draws at Phoenix Children's. He managed to nap for about 20 minutes or so in the car but it was really rough for him considering all of his diaper issues. Then, when we got there, we took Max in for his labs and I have to say, I hate taking him into the lab draw station. The staff is really nice and they're excellent at always remembering to wear masks around Max,but none of that is enough to balance out how miserable Max is as soon as we walk in. I'm usually carrying him when we go in and when he recognizes where we are, he clings tighter and tighter to me and sometimes starts to cry even before I put him down on the table for the blood draw. Thankfully, the nurses are quick and so sweet to him although I'm pretty sure the Spongebob Squarepants wall decals in the draw station are going to give him some weird PTSD when he's older.

Like this kid doesn't have enough to deal with.

After the lab draw, we put Max back in the car and drove him back to Flagstaff. We had the option to have him seen in the emergency department at PCH but after our last traumatic experience there in February, we decided to see his pediatrician in Flagstaff instead with the approval of our transplant coordinator. While we were with Max's doctor, she did a urine test to check for a UTI in addition to looking him over. After the results came back, the pediatrician called our transplant coordinator to not only fill in the team but also to check on Max's blood work results. They're not all back, but here's what we know:

1. Max's urine sample is clean and he doesn't have a UTI--the pain seems to be a result of the diaper rash.

2. Over the last week, Max's neutrophil levels (bacterial infection fighting white blood cells) have jumped from 0.1 to 0.6.


My jaw nearly hit the floor. We even increased his dose of anti-rejection med over the last week (a possible cause of the decreased neutrophils that we discussed with the hematologist) and his neutrophils still jumped up.

As usual, Max continue to defy medical logic like the snowflake he is.

We're still waiting on several tests, including one to check for a developed autoimmune disorder that means Max is producing antibodies to his own neutrophils as well as cultures on his blood and urine, but for the time being Max is at home with us and we've started a seven day run of antibiotics. Despite all the vomiting he's been doing over the weekend, the doctor said he looks well hydrated and he's only thrown up twice today (it's all about the small victories). Depending on what the other lab work says and what his temperature does over the next couple days, we might still have to check him into the hospital but at the moment, he's sleeping and we're really thankful he's able to finally get some rest.

We should probably get some sleep too.

Just to top it off, our dog was sprayed by a skunk in the face for the third time since we moved back up here in October because of course she did.

This is not the face of a dog who learns from her mistakes.

Saturday, August 15, 2015

Please, Please, Please Don't Make Us Go to the Hospital

To start, I want to say that we don't have to go to the hospital--yet. Hopefully not at all, but we'll have to see how things go.

For the first time in six weeks, our Mighty Max is sick again. We suspect he might have picked something up at his clinic appointment on Monday or his physical therapy appointment on Wednesday but honestly, it's hard to say. In our last post, I talked about how Max's neutrophil (white blood cell that fights bacterial infections) counts are almost nonexistent, leaving him wide open for bacterial infections. We'd been pretty lucky thus far but our luck might be wearing thin.

Max started throwing up a couple days ago but it would only be once and it seemed like maybe he just overate a little each time and it was no big deal. He's also had a dry cough all week and hasn't been sleeping well but again, we thought they were unrelated. Today, however, Max started getting a fever. He got up to 100 but dropped on his own and is currently hanging out around 99.1, which is fine. Max wants to eat but he can't keep down his bottles very well and can't seem to get comfortable enough to sleep for any significant amount of time (needless to say, it was a long night for everyone).

Motherhood has really changed the way I look.

I've already touched base with the transplant team just to give them a heads up and we're all keeping an eye on him. We're hoping he'll be able to keep some more of his bottles down so that he doesn't get dehydrated and we're also hoping that his temperature stays down because if it hits 101.5, we have to check him into the hospital for at least 48 hours to get a blast of antibiotics as a stand-in for his missing neutrophils. Hopefully it won't come to that but with Max, it's hard to say anything with any kind of certainty.

If anyone has a minute to offer up some prayers, happy thoughts, and good vibes for Max, we'd really appreciate it. He's trying to hang in there but the poor guy is just so miserable, it breaks my heart to see him feeling so awful.

Monday, August 10, 2015

Who Needs Neutrophils?

Today we took Max down to Phoenix for his heart clinic appointment. We got some good news and some not so great news:

The Good News
His heart function not only looks great, it's actually improving. We've dealt with a little muscle stiffness in the past but that seems to be gone. The transplant director said that Max's heart function looks totally normal--yay!

Max has also gained some weight and he's up to 8.95kg (approx. 19.7 lbs). Just to give him an extra boost, we're going to try and get him some higher calorie formula so we can hopefully continue to fatten him up.

Max's blood pressure appears to have stabilized which means we can stop his blood pressure medication! That means we're down to just five daily medications (reminder: Max was on sixteen when he was first released from the hospital).

Max hasn't had a cold in almost a month despite the fact that we've been able to go out and do more things like normal-ish people!

The Not So Great News
The fact that Max hasn't gotten sick is somewhat of a miracle because not only are Max's white blood counts down from 5,500 to 4,600 (most people have an average of 7,000), his neutrophils (type of white blood cell that fights off bacterial infections) have dropped from the very critical level of 0.4 down to an almost non-existent 0.1. Both the transplant team and the hematologist said that's probably because we're so proactive about hand washing and sanitizing.

But seriously, this is why our kid isn't currently in the hospital.

So why are his neutrophils dropping? There are two likely causes we're investigating:

1. The increased dosage of his anti-rejection medication. We've had to bump up his dosage (boo!) because he's gaining weight (yay!) But this is kind of the balance of transplant--he needs enough of the med to keep the body from rejecting the new organ but not so much that he'll get sick if someone two towns over sneezes.

2. Max's body might have developed an autoimmune disorder and started producing antibodies to his neutrophils, essentially attacking itself. This can happen as a result of the anti-rejection med. We won't know for sure if this is what's happening until we go in next week for a blood test and then we'll have a better picture of what's going on.

What's Next?
For the time being, we're just kind of waiting. Because Max hasn't been getting sick despite the fact that his immune system has essentially peaced out, the hematologist isn't really anxious to make any big changes and he wants to see what Max's system does on its own.

Pictured: Max's immune system

Depending on what the antibody test says, there are a couple different medications we can try ranging from an oral medication to a shot we'd administer at home. On the bright side, the hematologist didn't seem overly concerned. He said they've had great success in boosting neutrophils in their cancer patients (who have their neutrophils wiped out by chemotherapy).

For the time being, we basically have to keep doing what we're doing in terms of our sanitation precautions. We already track his stats daily at home (heart rate, blood oxygen saturations, temperature, etc.) and we need to keep an extra close eye on his temperature. If his temperature gets to 101.5 or higher, Max will have to be hospitalized to get a blast of antibiotics to essentially replace his absent neutrophils.

Like this, but with less Keanu Reeves.
I mean, there will be some Keanu Reeves. Just less.

In the meantime, Max is just his usually happy self. He's making big improvements after only a couple weeks of restarting physical therapy so hopefully we'll have some great news to report soon. But in the meantime . . . please don't touch him. We don't miss living in the CVICU.

We prefer to love you from afar, PCH.