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.


Sunday, February 8, 2015


On Tuesday, we celebrated Max's first birthday!

To a certain extent, the day felt a little surreal. When I was pregnant and we got Max's HLHS diagnosis, we were warned that Max might not survive the pregnancy. Then we were made aware of the reality that Max might not survive to transplant or through it--crazy things happen during minor surgery and they were replacing his heart. But Max earned his nickname of Mighty Max and to me, he makes it look effortless. The kid is a rockstar.

We weren't able to do as much to celebrate Max's birthday as we wanted because we're still in the midst of his first cold and flu season. But we decorated the kitchen and I baked some cupcakes for Max. He wasn't very interested in eating his cupcake but he seemed to enjoy smushing the frosting around his high chair tray.

The frosting is obviously the best part.

After we finished playing with our food, Jon and I helped Max open his presents. He had a lot of fun! Sure, the wrapping paper was the best part, but he's been having a lot of fun playing with his new toys this week.

Since we weren't able to celebrate Max's first birthday with everyone the way we wanted to, we're planning on doing a barbecue at the park this summer so everyone can come (as long as everyone's healthy)!

Today is another celebration for our family because one year ago today, Max received his new heart. To date, that was the scariest and hardest day of my life but I am so grateful. I'm grateful for my fighter of a son, I'm grateful for all the love and support of our family and friends, I'm grateful for the medical team who saved our son, and most of all, I'm so thankful to Max's donor family. In a time of grief, they made an incredibly generous decision that gave us our son. I have so much love and respect for them and there is no way I could ever even begin to thank them for what they have given us.

This week is so special for us and I want to thank everyone who has stuck by us and supports us. I wish I could say something more eloquent but this week I feel like I'm at a loss for words. I'm just so in awe of my son, my superhero, my Mighty Max. Being his mother is such a gift and I am so appreciative of every day we have with him.

Thursday, January 29, 2015

Biopsies, Scans, and Max. Oh, my!

Things have been a bit hectic for the last couple weeks. We finally found an antibiotic that Max's system could tolerate without throwing up, which luckily cleared up his lingering sinus infection and flu-like symptoms that we have been dealing with for months. This meant that Max was finally healthy enough to... go to the hospital three times!


We are still dealing with that lesion in his mouth-it is much less of a lesion now and more just gum and tonsil swelling-so it was time to do some more tests. We started with a head and neck CT scan to try and get a better picture of what was going on and see if this thing extended up or down from his throat. Since Max is so little, a CT scan involves anesthesia and therefore he can't eat for up to 12 hours, which is a very long time for our little skinny guy to go. However, like always, he was a trooper and held strong even though the radiology department was backed up that day. Things went as smoothly as they could have, and although it was a very long day, radiology had us in and out as quickly as they could. Everyone we dealt with was fantastic and we couldn't be happier with them.

The results were inconclusive, but they didn't see anything concerning, which is great. Nothing is growing or moving towards his brain, and that lesion is very localized. This means that whatever it is, it hasn't really spread away from his mouth. Since the CT didn't show us anything too concerning (yay!), everyone decided it was time to do another mouth biopsy and scope. We were not to thrilled at the idea of Max having two procedures within a week, especially since he just started to feel better and gain weight again, but we really want this whole mouth issue to be resolved.

We ended up scheduling the biopsy a couple days later in which we had the first appointment of the day. This is great because it means that we are in and out rather quickly, the hospital isn't as busy, and Max doesn't have to go without food for as long. The downside is that we have to be to the hospital by 5:00am, which is always fun, but we are almost use to it at this point. Sleep is for the weak, anyway.

No sleep 'till, NEVER!

The procedure itself went very smoothly. Max's ENT is very professional, no-nonsense, and very good. He doesn't sugarcoat anything and is straightforward with us, which is something we really appreciate. He said Max's mouth looked better than in November and the scope down his throat showed nothing to be concerned about. Max had a couple little cysts along his esophagus, but that is fairly common and can be easily removed if need be. He took three biopsies this round, which is really tough on Max, but that hopefully means that we won't have to do this again. The ENT was a little concerned that the growth/lesion/swelling in Max's mouth looked a little reminiscent of HPV-yes, that HPV, there are over 200 strains of the virus and about 20 of them center on the mouth. It is a virus of the DNA, and is in the same family as HPV the STI. Out of the 20 or so oral strains of the virus, 9 of them cause cancer.

We haven't received conclusive results of the biopsy yet, but we are hoping and keeping our fingers crossed that this thing isn't something that serious. We knew that Max is at a highly increased risk for cancer because of the intense medications his transplant required, but we didn't think it was something we might have to deal with this soon. The good news is that either way, it appears to be localized, so if it turns out to be malignant, it should be treatable. And if we have to, we will get over that hurdle as well.

We were able to take Max home later that day because the ENT really didn't want us to spend any more time than we had to in the hospital, which is good seeing as how measles is now apparently in Phoenix (don't even get me started on that). We are trying to keep Max healthy and the biopsy was to help ensure of that, so no one wants him to get sick as a result. He has had a slight fever since the biopsy, but that is to be expected, especially since one of the samples came from his tonsil. Like always, Max is taking things in stride and continuing to do well despite everything that gets constantly thrown at him.

Pictured: Max. 

Since Max has been dealing with a lot lately, our clinic visits have been a little more frequent. We had a quick check-up appointment on Monday, and honestly, it was one of the best appointments we have had in months. Max is gaining weight at an almost typical rate-somehting that has NEVER happened- he is growing, and everyone was happy with his progress (this is such a 180 degree shift from the last few months and a much-needed surprise). Max is actually in a height/weight percentile again, which is incredible. This is all happening after being so sick, having two procedures and not being able to eat for almost 24 hours last week. Even with that, he gained almost half a pound in the last couple weeks, which is a HUGE gain for him. The antibiotic seemed to really help, so he obviously had something bacterial going on that was eating up a lot of his calories. If he was able to gain weight during a week with two hospitalizations and low food intake, we obviously were able to fix something that was going on.

So the last few weeks have been a bit of a doozy, but we are still chugging along. Max turns one on Tuesday, which is a milestone that gets me a little choked up just thinking about. It seems like yesterday that we were told that Max might not make it to birth, and now we are going to be celebrating his first birthday in a couple days. We have come a long way, Max has come a long way, and I couldn't be happier. Sure, things are constantly stressful and extremely difficult, but when I take a step back and look at the looming accomplishment coming next week, I can't help but smile.

Friday, January 9, 2015

11 Months, Chatty Cathy, and Health Updates

This is waaaaaay overdue and we have a lot of updates so hang tight--we're just going to dive right in! Let's start with the fun stuff.

We got to celebrate Max's first Christmas with him at home! The poor guy was sick (more on that later) but he still seemed to enjoy opening presents. Of course, being only 10 months old at the time, I'm pretty sure he didn't understand why we put up a tree with lights in the living room or why we suddenly had a new stack of toys, but the whole holiday was still pretty amazing. At one point, Jon and I weren't sure we'd ever get to celebrate a first Christmas with Max, let alone one at home in Flagstaff (although we almost had to be in the hospital--more on that in a minute). Max made out like a bandit and has been having a great time playing with his new toys. One of his favorites, a drum, gets double duty with the drumsticks; Jon and I are huge nerds and have waved the drumsticks around and said, "Wingardium Leviosa!" which Max thinks is hilarious.

We're so excited to teach him all about Harry Potter.

Physical Therapy Improvements
As regular readers of this blog know, Max is behind in his gross motor skills due to his transplant (although he's either on par or ahead with fine motor and mental development milestones). We realized that now, part of Max's problem isn't that he can't do things like sit up on his own or get up on his hands and knees--he's kind of lazy and just didn't want to. However, with his new drum from Christmas, he has more motivation to sit up and he's starting to do it for longer and longer stretches of time. The last time we took him to physical therapy, he also stopped screaming (he hates PT) long enough to do about 15 minutes of actual therapy.

Pictured: Max's physical therapist.

Chatty Cathy
The biggest development we've had is that at 10 months old, Max started talking! His first word was "bop" which is our word for pacifier. The first time he said it, he was holding his bop and staring at it very intently. Jon and I were floored! A couple days later, he said "poop" much to the amusement of his dad. As of right now, here is a running list of all the words Max has said:

  • bop
  • poop
  • again
  • more
  • eat
  • uh-uh
  • bot (for bottle)
  • no (shouted only once at a nurse who had to swab his nose--it was both sad and hilarious)
  • Da-ee/Dag/Gad (for Dad--all said while looking directly at Jon)
  • "It good" (first baby sentence!)
  • Mama (my personal favorite)
He's trying to say more and more words every day so I'm sure this list is only going to get longer. We're so excited that he's starting to communicate verbally!

11 Months
Max is now officially 11 months old and 11 months post-transplant!

Now the not so good updates:

A couple days before Christmas, Max came down with his first serious cold. He's been somewhat sick since October but this one was really bad. He got really high fevers (up to 102) and it was difficult for him to keep food down because the excess phlegm in his throat was making his gag reflex really sensitive. We had to give him his bottles very carefully and only let him take a couple swallows at a time time keep him from throwing up. This basically equates to spending every waking moment feeding him. However, we managed to get him to take in a high enough volume of food and he kept gaining weight and kept his medications down so we could avoid having to put an NG tube back in. Last week he seemed much better for about five days and then all of a sudden, a few days ago he just tanked again (high fevers, sensitive stomach, and behaving like he just doesn't feel well). We took him back to the pediatrician and she thought he might have developed something . . .

. . . like a sinus infection. After consulting with our transplant coordinator, we've started him on an antibiotic. We saw a noticeable improvement within just a couple doses but the amoxicillin seems to be making his stomach sensitive again. He's still getting the occasional fever but to us, he seems to be making very slow progress in the right direction.

It's all about the baby steps.
The Spot in His Mouth
The positive side is that it looks like it's slowly getting better. The downside is that since his doctors don't really know what it is, they want to go back in and biopsy it. We're refusing to do that until Max's Ear, Nose, and Throat (ENT) doctor looks at it again since no one from PCH has seen it in a month. We also might get a second opinion before we consent to a biopsy because it looks to us like it's healing, albeit very slowly. In normal people with functioning immune systems, a spot like this can take 15 weeks to heal. In a baby with a suppressed immune system, you can assume it's about double that (but of course there's no data on transplant babies with this because Max always has to be Patient Zero). We're not doctors but we're also not sure another biopsy is necessary and we want someone to at least look at it again since it's changed so much since a doctor looked at it.

Additionally, the concern is that the spot is an indicator of something else going on in his body that we just can't see. His transplant teams wants to do PET scan which we agree needs to happen. This is another source of anxiety though because I really want to do the scan but I'm kind of afraid of the possible results. As with anything involving Max, the spot could either be totally benign and just an angry canker sore or it could be an indicator of something much more serious. I'm trying not to dwell on that until we actually know something concrete but I can't help but worry--it's what I do.

My life in a nutshell.

When Max was in the hospital for his mouth biopsy at the beginning of November, we took him in to do a one bang MRI. He didn't have to be sedated and it was really quick. I even got to be in the room with him to help comfort him while it was happening. When we got the results back, we were told Max had a narrow channel in his brain for fluid. We were told that it could be nothing and he'll just grow out of it but then someone else mentioned (in passing, of course, because that's the best way to hear scary news) that it could be an indicator of something more serious. We felt comforted by the fact that neurology didn't feel the need to follow up with us while Max was still in the hospital but we were supposed to see them after his was discharged.

Trying to get an appointment with neurology, however, is nearly impossible. I left message after message and couldn't get through. When I finally did, they told me that they'd never heard of Max's neurologist (she's not a new doctor). Then when I finally talked to someone who knew who the neurologist was, she was out of the country. I tried calling when she returned and couldn't get through. Finally, I asked someone in cardio to help us and boom, just like that Max had an appointment.


We saw the neurologist on Monday who gave us . . . interesting news. Basically, Max has some extra fluid in his brain. When babies are born, they have a lot of extra fluid in their heads but as they get older, their brains typically close some of the open areas and fill in the area with gray matter. Max's brain, however, has not done this and he still has the pockets of fluid which could be caused by a variety of sources. For example, he was born with a janky, half of a heart that didn't pump his blood very well and it was replaced with a slightly older, stronger heart that pumps his blood very well. He's always had high blood pressure which is something we're now really trying to manage. Additionally, as I mentioned earlier, one of the canals in his brain is kind of narrow.

The good news is that Max still has fluid around his brain between his brain and his skull so it hasn't progressed to something called hydrocephalus. He also has the added bonus of the soft spots on his skull which act like pressure valves to relieve some of the extra pressure on his brain. As of right now, none of this seems to be causing him any problems per se. The neurologist told us that he's surpassing all of the mental and fine motor skill milestones so he's ahead at the moment.

Einstein ain't got nothin' on Max.

The narrowed fluid canal in his brain could either be an indicator of  a problem later or it could be nothing and he'll grow out of it. For now, we have to monitor him though because the extra pressures in his brain could cause issues so we have to keep an eye on him for changes in mental abilities, speaking, fine motor, and a severe increase in lethargy. If we see any of these red flags, we'll have to have a neurosurgeon put a shunt in that narrowed canal to allow for some relief of that extra pressure.

The joys of transplant and its ripple effect--it could either be nothing or it could be a huge surgery. The neurologist wants Max to come back in for an MRI in the next couple weeks to check the fluid levels in his brain to compare it with the one bang MRI he got when he was in the hospital in November. And then . . . I guess we'll just go from there. More than likely, it'll just get added to the list of things we're monitoring.

Makin' a list,
checkin' it twice . . .

So that's where we are! Those are all the major updates on Max from the last several weeks. We have a clinic appointment next week and we should have more updates soon on his sinus infection, the spot in his mouth, and the fluid in his brain. Until then . . . we're going to go play drums and Harry Potter with Max.