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.

Thursday, July 16, 2015

The Problem with Neutrophils (a.k.a. ALL THE UPDATES)

I apologize for the lack of updates, things have been really busy for us so this post might be kind of sizable while we get everyone caught up!

Outside the Bubble
Since our last post, we've been able to venture out into the real world with Max and behave like a normal family! Well, a normal family with an overabundance of Clorox wipes and hand sanitizer, but we got to leave the house so we're calling it a win. Actually, the first day we got to go out was on Mother's Day which was an awesome present. We've been able to go out a few times and meet up with friends who finally got to meet the infamous Mighty Max and it feels awesome to get to see some friends again.

Back in the Bubble
As was to be expected, when we started going out, Max would get sick a few days later nearly every time. We were as careful as we could be: we cleaned our hands a lot, we wiped off whatever table we sat at with Clorox wipes in case Max touched it, and our friends (very wonderfully and respectfully) gave Max a little space. However, we can't control everything and you can't expect to go from no exposure to lots of exposure without Max picking up something. Since he's had to be under house arrest for so long, he doesn't have the antibodies everybody else does. So we've just had to deal with each bug as it comes and when he's better, we do our best to resume normal life (well, normal for us).

Max Likes to Move It, Move It
I can't remember if I talked about this in the last post but Max is definitely mobile now. He's not crawling, but it's not because he can't--he'll push up into four point (on hands and knees) but then realize it's way easier to just roll where he wants to go. And he is fast. He's keeping us on our toes and it's fun to see him getting more adventurous. We're hoping to start physical therapy again soon so we can work with Max and help support him as he gets bigger.

So, Teething Blisters are a Thing
About a month ago, Max got super sick. He was throwing up a lot and had a blister on his gums, which concerned us that we were looking at another issue like what we had back in November with the atypical histiocytic granulomas (in regular people language, the angry canker sore in his throat that hospitalized him in in the fall).  We took him into the pediatrician and it turns out that while Max had a bug, he was also teething and she suspected that the blister on his gums wasn't another granuloma but said it was an eruption blister. Our pediatrician said those can be common and it's basically a blister over a new tooth coming in which equals a lot of pain for poor Max. We monitored in closely and sure enough, within a couple days the blister went down and in its place was one of Max's front teeth poking through his gums. This poor kid has been through enough--why does he also have to have a wicked awful time teething?


Shots, Shots, Shots, Shots
A couple weeks ago, Max was finally able to get his next round of shots--yay! He got a whole bunch including DTaP, polio, and Hep B (plus a couple others). We're so excited to be able to start moving forward to get him caught up on the shots he can have. The downside is that Max had a rough couple days after the shots. The injection sites seemed to be sore and Max doesn't have a whole lot of fat on his skinny legs to help cushion the shot. He had a rough couple days so we just gave him Tylenol around the clock and kept in contact with the transplant team and then he came out on the third day back to his happy, smiley self.

The Problem with Neutrophils
Our biggest issue at the moment is with Max's neutrophils. For those of you who don't spend all their time reading medical literature, neutrophils are a type of white blood cell that make up about 40-75% of all the white blood cells and are an essential part of the immune system. Max is critically neutropenic, which means his neutrophil levels are extremely low. To give you an idea of what this means, Max's last level was 0.4. If he had cancer and need chemotherapy, the cutoff to receive that treatment is 0.5 which means Max is very susceptible to bacterial infections at the moment. We got another lab draw this last Monday so hopefully we'll hear some results tomorrow to see where his level is currently at.

So how do we fix this? There are a couple options in terms of medication but what it might come to is me having to give him a daily shot at home. I'm not excited by this idea but it can't be worse than when Max first came home from the hospital with a double lumen RA line straight into his heart that I had to flush out every day ("If you get an air bubble in there, he'll die. No pressure, new mom!") Hopefully we can manage this with medication but if this continues to be a problem, worst case scenario is that Max could end up needing a bone marrow transplant. But that's not even being discussed at the moment--we've only begun to start looking at fixing this neutropenia. All the same, it's something Jon and I would rather be aware of now instead of potentially being surprised by it later.

Ugh, can we not?

Overall, Max is doing well and aside from when he was really sick with that eruption blister, he's been slowly gaining weight. He loves dancing, playing with us, and anything involving Elmo (or "Eldo" as he's started calling him). He's just the best kid and we feel so lucky to have so many people love and support us.

Saturday, May 9, 2015

Guess Who Has Two Thumbs and is Bad at Updates?

It's us. We're bad at updates.

We're sorry about the lack of updates! We 've been bad because things have gotten busier for us. Jon and I both found work from home jobs (yay!) so we've been working pretty much all the time. But we wanted to post an update about how things have been with Max!

Overall he's been okay. We haven't had to check him into the hospital since February which is great but we've had a few close calls. Most notably, in March Max got a fever of 103.0 degrees but after an afternoon at our pediatrician's office and a series of tests at Flag Medical Center, we got to stay home and were able to get his symptoms under control. Fevers are always scary for babies but it's always in the back of our heads that a sudden fever like that could be a sign of acute organ rejection. Luckily everything looked okay and through it all, his heart function is still good. The general consensus seems to be that he just caught a random virus. The worst part though is that the high fever caused him to get a few fever blisters on his lips which are just now FINALLY starting to heal. We would be out in the world doing more stuff but with such a big entry point for infection, we've had to lay low until very recently.

Max has also needed oxygen a few times overnight but we got an O2 concentrator so we don't have to worry about running out/dealing with an empty O2 tank. He doesn't need it often but whenever he gets a stuffy nose or has a change in altitude (like going to Phoenix and back), he'll usually need it for a night but he hasn't needed it during the day which is great.

Physical Therapy
We're working on getting Max back into appointments with a physical therapist but in the meantime, he's made awesome progress. He's sitting up really well and can get himself up to sitting from a prone position. He's not crawling yet . . . but we have a suspicion it's because he's either lazy or too smart for his own good. If he wants a toy that's out of his reach, instead of crawling he does one of four things:

1. Tries really hard to reach it from where he's sitting without moving.
2. Shouts at one of us to get it for him.
3. Flops down and rolls across the bed/floor/whatever to get it.
4. Decides it's not worth it and plays with something closer.

He's a bit of a stinker.

He's still super chatty and he's started to get more conversational (answer questions, responding to what we say, etc.) which has been awesome. However, he is very shy and doesn't really like to talk in front of other people. When we were at Max's last heart clinic appointment, he wouldn't talk to anyone when they were in the room but as soon as someone left and closed the door, he'd whisper, "Bye bye!"

". . . but only after you leave."

The Outside World
With cold and flu season FINALLY over (despite the sudden May snowstorm in Flagstaff because WTF), we can finally start integrating into society and stop being shut-ins all the time. We're slowly starting to venture in the world which is both terrifying and amazing.

"Okay, we're ready!"

This is very much a condensed version but that's what's been going on with Mighty Max! Hopefully we'll be able to start seeing more of you so people can actually meet Max instead of just taking our word about our hypothetical baby no one has seen.

"Suuuuuure you had a baby . . ."

Friday, February 27, 2015

The Altitude Test

Yesterday was a pretty good day for Max. He seemed more like himself and spent the bulk of the day just playing and hanging out. His favorite game in the hospital is to see how many lead he can pull off to make the monitor beep so we played that. A lot.


His nurse found out Max loves balloons so she got him a heart shaped balloon to make up for the fact that she had to draw blood from him in the morning. Max was thrilled with the balloon and he forgave the nurse . . . for now. 

Yesterday's big event was the altitude test. In order to simulate Max's body's reaction to the thinner air at a higher elevation, they put him in a plastic box and pump in the modified air. Initially, when a respiratory therapist stopped by to talk to us, she told us that the test would take six hours.

Have you ever seen a baby sit quietly still for six hours without needing food or a diaper change? Because I sure haven't. They said they'd try to do it at night while Max was sleeping which would help but we also weren't excited about the idea of Max falling asleep cuddling up to one of us and then waking up alone in a weird plastic box.

Fast forward to about midnight. The night shift respiratory tech came by to start the altitude test. Beccause he'd only taken about a half hour nap in the afternoon, Max was very deeply asleep which made it easier to slide him into the box. When I initially heard about the test, I thought the sides would be a harder plastic but they're not, they're more like the thick, clear plastic packaging that bedsheets come in. Or, as I first thought, it's made out of the same stuff as all the packing materials that are usually stamped with warnings that say, "Don't let your baby play with this!"

Max made it through about two and a half hours of his test before he woke up and started to get really fussy but at that point, the doctors said that was good enough and they felt like they had a good enough idea of how he'd react at altitude. Max passed the test jut fine and in their assessment, respiratory said that he should be fine but if he needs it, we have a tank of oxygen and we can put him on 0.1L but they don't expect him to need it. Jon and I feel much better having the tank just in case something unexpected happens again.


At the moment, Max is kicking his feet and playing with his balloon while we wait for rounds. Once they come by, we'll have a better idea about whether or not we're actually getting discharged today but so far, it's looking good. Of course, Flag is supposed to be hit with epic snowstorms this weekend so if we're discharged too late in the day, we might try to stay down here and then drive up in the morning. But we'll see how it goes. As of yesterday, everyone seemed motivated to get Max out of the hospital so fingers crossed that we're heading home!

Wednesday, February 25, 2015

Blood Oxygen, Crazy Coughs, and No Sleep Till Ever

Just as I finished typing a huge blog post, my iPad decided it would be best if it deleted it and made me start over from scratch.

Stupid technology.

Take two . . . as I mentioned, I'm writing this on my iPad so if there are any wonky formatting issues, that's why.

Since the last post, Max has been a busy little bee. Right after we celebrated being one year post-transplant, Max got sick (of course). He seemed to have caught a bug of some kind plus the extra iron he was on was irritating his stomach. He needed to be on such a high dose of iron to help combat his severe anemia but it was only supposed to be temporary. We pulled him off the extra iron and after a few days of adjusting, he seemed to feel better. He also started to gain more weight and we're now up over 7kg! That's still pretty skinny for his age/height, but he's definitely heading in the right direction.


Now that he's finally doing well, let's ruin it by going to the hospital!

Yesterday was Max's one year post-transplant heart catheter and biopsy. His procedure was scheduled for 7:30am which meant we had to check in at 5:30am which meant we had to leave Flagstaff around 3am. Could we have stayed down here the night before? Sure, but we were worried about exposing him to any kind of sickness right before his biopsy (this will be funny later--not funny ha-ha, more like funny FML).

 The procedure went well except our cardiologist told us that Max's blood oxygen levels had been a little low during the procedure, around the mid-80s, whereas he's usually 98-100% in Phoenix (92-96% in Flagstaff at altitude). But after the procedure, his blood oxygen level was at 98% and after the requisite six hours of waiting, a chest x-ray, and the one bang MRI neurology had requested, we were given the green light to go back to Flagstaff.

Once we got home, we used our home machine to check his blood oxygen levels and were surprised to find them ata 85%. We called the transplant team and they said we were fine to stay in Flag as long as Max's numbers didn't go any lower. Within 30 minutes, his numbers had dropped to the mid-70s and the team told us to drive down to PCH. We didn't feel good about driving down to PCH instead of going to Flagstaff Medical Center to get him oxygen but they insisted so we did as we were told. When we started driving, Max's numbers were in the low 60s. They quickly dropped to 55% and I had Jon pull over so I could get Max out of his car seat. The anesthesia from his procedure made him throw up and although we didn't think he had, it's possible Max could have aspirated some of the vomit. I thumped him on the back a few times and he coughed wetly and heavily. Immediately after that, his numbers jumped up to the mid-80s for the rest of the drive.

Since it was 3am when we got to PCH (that's 24 hours without sleep for those of you keeping track), we had to go through the Emergency Department (ED). They were full so we ended up having to share a room with a child whom I believe to be patient zero for a new super strain of tuberculosis or the plague. Luckily, Max was on board with wearing a mask the whole time we had to be in the ED which helped. He's never done that before but this was an excellent time to start.

What he wasn't on board with, however, was everything else. He kept shouting, "This is bad! This is bad! I'm sad!" which was compeltely devastating. The only thing sadder was the way he kept asking, "Bye-bye? Bye-bye?" because he wanted to go home.

                                                                        I can't even.

When we were finally transferred up to the CVICU around 5am (26 hours awake), we were assigned the same nurse we had the night of Max's transplant. I can't tell you what a huge relief it was to see a familiar, friendly face. Max was also quite popular amongst some of the other nurses who all wanted to stop by and say hi. None of them saw him when Max was inpatient in November so this was the first time they'd seen him in a year. Needless to say, he looks a little different now. Around 6am, we were finally able to lie down and try to get some rest. Max, however, decided that sleep is for the weak after about 30 minutes so we had to turn to coffee for comfort.


During our brief nap, Max's blood oxygen levels dropped to the 80s so he was put on 0.5L of oxygen. He's already been weaned down to 0.1L by tonight with a blood oxygen level of 96% so hopefully we'll get him back on room air tomorrow.

Thankfully, Max decided to take a huge nap this afternoon which meant Jon and I got to finally take naps, too. We're nowhere near caught up to the amount of sleep we need, but at least we slept enough to stop hallucinating from exhaustion.

                                                      It's been a weird couple of days.

As for what's wrong with Max . . . we don't know yet. On the more benign end, this could be a combination of anesthesia aftereffects mixed with a cold. On the bad end, it's the beginning of pneumonia coupled with organ rejection which is problematic because we can manage the rejection by upping his immune suppressor medication but that would make it more difficult for him to fight off an infection. Hopeefully it's the former and it's something that's easy to work out.

                                    Please, please, please, please, please, please, please . . .

Right now, Max is on a 48 hour run of antibiotics so we'll be here until at least Friday, barring any bad test results. We're still waiting for the results of his biopsy; we were supposed to hear back by noon today but in hospital time, "noon" is anytime between actual noon and Christmas of next year.