Hope for Max and Alex

Things are looking up

Well, Max's work of breathing is definitely much better. We have moved the yuckies around so that his lungs are much better on the right side, but not super great on the left. This is to be expected. The pulmonary doctor has also increased his Bi-Pap pressures which seems to be helping considerably. His fever has been down for a full 48 hours, so the plan is to switch him to oral antibiotics (off of IV) and make sure he goes a full 24 hours with no fever. Then we can officially say the scales have been tipped, which means keeping him at the hospital is risking exposing him to more infection, so it may do more harm than good. Since we don't have our inverter in our van yet, Aaron is off to purchase a cargo carrier to attach to the trailer hitch on the back of the van so that we can run the generator in route to power his Bi-Pap. If all goes well we can expect to be sent home Monday. That is good, because while Max is getting stronger and wanting to play and interact more Mommy and Daddy are still going at the same speed and we are pretty exhausted. He is watching his Thomas movies right now and singing along and asking me to dance for him. This is defintely not the same kid from a few days ago.

Here is a picture of Max in his nasal prong mask. He isn't so fond of it, but it's nice to give the skin on his face a break from the face mask. We convinced him to wear it by pointing out how much easier it is to watch tv. It's so good to see that smile! This is daddy after being up all night doing respiratory treatments, a little rough around the edges. He is heading home for some sleep. I have the day shift today.

Sorry for the long post, it's been a work in progress

Friday:

So it is 4:30am and we are doing Max’s second respiratory treatment of the night. I am definitely closing in on a look that could only be described as “hospital exhaustion chic” complete with greasy hair and super awesome sweat suit. I am literally counting down until the cafeteria opens for coffee and possibly a calorie splurge of a donut. ***This just in: Found out that while there are some good things about being in a smaller facility (e.g. x-ray doesn’t come at the ungodly hour of 3am), there is one major flaw, no cafeteria or Starbacks until after 6:30am, ACK! The PICU is locked down from 6:30-8am for shift change report, so I can’t leave or come back between those hours! Help, a hungry pregnant lady is trapped in the PICU (fyi – I added this at 7am). Okay, Aaron did bring me crackers and power bars, but doesn’t coffee and donuts sound SO MUCH BETTER.

Words/phrases we have become very adept at using:

Moving good air (our personal favorite)

Crackles (possible fluid in the lungs)

Infiltration (aspiration of foreign material in the lungs that has decided to set up residence)

Rubbing

Wheezing

Diminished (when the air isn’t moving well in a particular area)

Atelectasis (collapse of an area of lung)

We are currently dealing with infiltration, probably the aspiration, that turned into crackles, that then became rubbing/wheezing, giving us diminished lung sounds and are now trying to prevent atelectasis. The infiltration is in the right upper lobe. How do we get rid of it? Well, we have to stop the bacteria growth with antibiotics and then just think of the Mucinex commercials. We have to get those guys out. Here are the kickers. Max likes to lay on his right side, because it is easier to breathe, go figure, those lungs are open. Also, when you turn him on his left side his oxygen levels decrease because the air isn’t moving as well. They continue to drop during the treatment because we are really aggravating the “yuckies” and moving them around. Now to add to the fun that is all this crazy we switched out his g-tube button a few days ago in hopes that some of the problems might be related to needing a new button (which happens occasionally). Well, when we change the button out we have to be careful for the next few days that what little irritation we caused his g-tube site can heal properly. Unfortunately, his increased treatments aren’t helping that process + the Bi-Pap, which puts some air in his belly = leaking food when he is on his left side. So here we are trying to run that fine balance of nutrition, rest, and treatments and we end up with his food (and don’t forget stomach contents leaking all over the poor kid. Yuck and crap.

Now this evening we finally got to meet Max’s new pulmonary doctor. We REALLY LIKE HER! She has lots of experience with SMA (she came from a much larger facility). I think we may even be able to learn a thing or two. The first thing she is gonna set us up with is antibiotics to have on hand for any time there is a respiratory issue. She feels we could have prevented this had we only had better communication (on the part of those within her office). We talked for quite a while about our protocol, her background, and our thoughts of the respiratory therapists here (while they are super nice, they aren’t very aggressive with treatments, but in their defense maybe they are deferring to us more than they might usually). We did have one slightly disturbing incident today where Aaron asked a therapist to do a deep suction (which is a catheter that actually goes into the lungs) on Max and she seemed uncomfortable and then wanted to do it without a sterile kit (just an open catheter suction). Aaron stopped her because that is clearly unsanitary. We brought this up with the therapist this evening and she was even shocked, so maybe it was a miscommunication. I think we were a little spoiled by our “think outside of the box” group at MUSC. To make a comparison, the skill level we have seen here seems more like what you would see on a regular unit floor RT vs. ICU.

Well, as you all know talking (and blogging) a lot may be a habit of mine, but it is one that I think comes in handy sometimes. I find that if I tell doctors a lot of info generally we hit something of true value that solves a problem. For instance, as Dr. Stewart was leaving tonight I happened to mention Max’s tooth and how one of our concerns on Sunday was that he has actually aspirated on it. We said how relieved we were that the x-rays didn’t show the tooth. She said that a baby tooth may not show well on the x-ray and she really wished she knew this (fyi – I have told every single medical professional I have spoken with about this). If Max doesn’t show much improvement tomorrow she is ordering a CT of the chest. Just when we thought we had passed the major hurdles, something else to worry about. I don’t know who I will be more mad at if this tooth is the problem, myself or the ER staff for assuring us that it would have showed. As a side note, I have to give Aaron props, in addition to his usual awesomeness (they guy did bring me Chick-fil-a for breakfast) he has never been convinced that the tooth wasn’t the issue. He looked up a lot of x-rays of swallowed teeth and they weren’t as evident as we were told they were. Now he and I are both hoping that this is not the case, because then they would have to go into his lungs to retrieve it.  

Saturday:

During the night we rolled with treatments every 3 hours.  At 3am we noticed blood on the bed. Max’s IV was messed up and we had to pull it. I asked about waiting until daylight to replace it, since he is only getting the antibiotics through it twice a day. Well, his next dose was due at 5am, so we had to go ahead and get a new line. That is all fine and good, except he is a really hard stick and she wasn’t having much luck in the way of locating a good vein. She was able to get one and a half hour later we were getting back to sleep. Well, when I went to get him ready for his next treatment I turned him only to find his fluffy friend “Jack the Chameleon” was soaking wet. Apparently his IV meds leaked all over instead of going into Max’s line, so they ended up pulling that IV out and are now going to have to try and find another one. The poor kid is exhausted with the increased treatments and now we have to wait again after treatment for a new IV. This is why at 3 am I was like, can’t we just wait until the morning for this. I’m not really fond of a middle of the night IVs that aren’t emergent. It took two additional nurses before we finally got a working IV this afternoon. We have definitely seen some improvement today. We have been able to decrease his oxygen to 1 liter vs. 5 from Thursday. He still freaks out if you try to take his air (aka Bi-Pap) off of him. I think the increased treatments and deep suctioning are paying off. Poor kid is now on three different nebulizers, one of which will have to last for 28 days (it’s an inhaled antibiotic). Dr. Stewart seemed pleased with his progress today, but I don’t think we are going anywhere until Monday at the earliest. On top of everything else I feel a cold coming on, so Aaron went home for a couple of hours this evening and I will go home to sleep for the night.  I hope they have a much less eventful night than we had last night.

A few thank yous: Of course we appreciate all the prayers.  In addition Max’s nurse Robin stayed with us for the ambulance ride and until we got settled in the PICU on Thursday, which is a really long day. She returned on Friday loaded down with fruit, cookies, protein bars and drinks for us. My cousin Lisa brought us dinner on Thursday night and my brother Travis brought us some dinner tonight. I will say it is nice that this PICU allows food in the rooms.

A new PICU, a new crew

I am not going to get into all the particulars (because I could rant and rave for a while), but Max has not been improving at home. His fever continues and does not let up even with medication. We finally convinced the pulmonary that he needed an antibiotic, but it was this morning and it was too little too late. With a fever that wouldn't seem to quit and worsening lung sounds we knew we were at decision time. His breathing respirations were consistently in the 50s vs. his typical 20s and he was just having to work so darn hard. Why are respirations important, well, given his short shallow fast breaths there was concern for gas exchange, we didn't want him exhaling off too much CO2, there was also the fear that he would simply wear out and not be able to breathe even with the Bi-Paps assistance. The LAST thing we want is an emergent intubation. We were so concerned that we actually had him transported via ambulance this afternoon. For those keeping track this is our 3rd ambulance ride in less than a year...not a trend I want to continue.I was a little alarmed when the paramedic with Max told the driver to roll "10-50"...for those in the know that means full lights and sirens, so sad Max couldn't appreciate this. I gotta say, it is a lot more unerving rolling through an intersection when that light is red and you are praying the other drivers are paying attention and did I mention rain and tornado warnings, sometimes I do feel like our lives are an episode of Grey's Anatomy.

Well, after spending all afternoon in the ER we were finally admitted to the PICU...they were seriously busy today. Max's chest x-ray does show "infiltration," but pneumonia results take at least 24 hours. They are treating him like he has...wait for it....wait for it...aspiration pneumonia! Hmmmm, sounds so familiar, oh I know, it's what I've been trying to explain to all of his docs for the last four days. Wow, it's almost like I've seen this before or heaven forbid can use my amazing mommy powers of deduction or that Aaron and I may not know crap about all of medicine, but we know EVERYTHING about Max. I just want to point out that I don't think we have avoided the hospital for 2 years on luck. I also think that the staff finds me, Aaron and Max overwhelming...how many type As can you put in one family. Just so you know Max also still has his wits about him, he is introducing himself as "DJ Max E Max" and then proceeds to do a little "beat box" for them. The staff has even commented on how polite he is, he even thanks Aaron after each cough treatment. There is nothing sweeter and more heartbreaking then hearing Max say, "Thank you Daddy" after Aaron does a cough machine cycle on him. I have to say Max has been much more in tune to the benefits of the cough machine this time, even asking to be coughed and directing Aaron to turn up the pressure settings, wise beyond his years. These are good signs. He is verbal and he is digesting food, we would be much more worried if these two things weren't happening.

So that is where we are at: Max's CO2 levels are normal and we are on some broad spectrum antibiotics. Our hope would be that we would see a turn around in the next 24 hours. A turn around would be lower temps and less work of breathing, also, getting off the supplemental oxygen (right now he is on 4 liters). I would love to make a prediction for this, but for my faithful readers you know as well as I do that Max drives this profile. Thanks for all the prayers.  We will update again soon.

I was really hoping for a kinder, gentler 2012...

Well, we officially have a big boy in our house, but not without the typical drama associated with all of Max’s milestones. Max lost his first tooth. It was slightly overshadowed by the fact that he also swallowed his first tooth. I get a call from the nurse when I was on my way home from the gym and I can tell she is on edge. She tells me that Max’s tooth has come out and she can’t find it. Apparently, after the tooth came out he clamped his mouth shut. Thankfully, I was almost home. We looked all in his mouth, really wishing the whole time that he could open his mouth a bit wider. Our worst fear was that he could have possibly inhaled it, but he wasn’t having any breathing issues and he said that nothing hurt in his throat. Once we quit messing with him he calmed down quite a bit. I have to say it was a bit frustrating. Now, to be honest, I have never looked forward to this phase. I hated pulling my teeth when I was a kid. It totally freaks me out and that is without all the obvious problems that could arise in our particular situation. Teeth have really been nothing but a problem since they came in. Teething was a nightmare for Max and with all that drooling we were always concerned about aspiration and pneumonia. It was such a relief when that was over and now we have a whole new set of worries.  Well, the remainder of the week passed without any tooth sightings, if you catch my drift and frankly I do have a line and searching through poo is definitely it. Sorry, if that makes me a bad mommy.

Okay, so you can't see the missing tooth in this one, but he is so darn cute.

So here we are one week later and we have a very sick big boy on our hands. On Saturday, he began running a fever. By Sunday, he was having problems breathing with oxygen saturation in the low 90s, still fever, and complaining of chest pain. Aaron and I decided to take Max into the ER for a chest x-ray, as Aaron has still not been able to locate a home x-ray machine. Our biggest concern was that instead of swallowing the tooth, he has inhaled it and it was causing problems in his lungs. Well, we braved the chaos of a Sunday afternoon at the pediatric ER and his lungs were clear (yea!) and all the viral tests (RSV, flu) were normal, so we headed home. He really doesn’t seem to be getting much better yet. He is requiring his Bi-Pap for breath support and some oxygen to give him a break. Of course, my biggest complaint is that his pulmonary doctor has relocated and we were completely unaware until I tried to page him this weekend, so now we have a new pulmonary with no idea what our protocol is. Even better, is that we couldn’t get in for a follow-up until May…are you freaking kidding me? Anyone looking to go into the medical field, we are in DESPERATE need of pediatric pulmonary physicians. It’s not glamorous, and sure there aren’t many cures out there for kids with pulmonary issues (e.g, Cystic Fibrosis, neuromuscular diseases with respiratory complications, asthma), but I haven’t heard of a hospital that wasn’t a 6 month wait for a pulmonary appointment. Now the good news is that they are supplementing with physician assistants and nurse practitioners, who we have always had good luck with, so after much prodding and basically telling the nurse how angry I was (in not the nicest words) that we weren’t aware there was a change in doctors we have an appointment on Monday with the NP.  It is times like this that I miss our Charleston team so much. As always I have already learned some valuable lessons from this experience. I will NEVER let a doctor convince me that we should only be in touch when Max is sick. If I had only kept our standing six month appointment, then we wouldn’t be in this position right now. The doctor’s reasoning made sense at the time. He didn’t want to expose Max unnecessarily to sick kiddos, but now that he is sick we are in a bad position. Of course I should also thank Tricare for cutting nursing hours. Guess what two parents were up all night with Max because we don’t have enough hours…yep, us. Since Max isn’t in school this week I don’t have enough hours to cover daytime help like I use to either.  Don’t get me wrong, I get it. Money is an issue and cutbacks have to be made somewhere (and we are far more fortunate than many families who lost care completely), and typically nursing coverage isn’t a problem for us, but when he is sick we definitely feel the strain.   I think if we could just get Max comfortable enough to sleep we would all feel better. We are walking that fine balance of rest, food, and treatments. Thankfully, we have only heard a few crackles (suggests the presence of fluid, not mucus) in his lungs and we are running treatments every four hours to clear them out. I do think today is our best day as far as fevers go, meds have barely touched them in the past few days, but today seems much better and controlled with the meds.

Here’s hoping to some sleep tonight for all of us (well, except the night nurse she better stay awake).  Poor Max whimpered all night and wanted to be turned every 5 minutes…I really wish I was exaggerating. While I did get a lot of cleaning done throughout the night…I’m now only one week behind in the DVR and Aaron was able to work on our taxes, I much prefer sweet dreams for all of us.

I think that is enough ramblings from the sleep deprived, nauseated, oh and did I mention pregnant (so I’m not quite as quick on my feet as I use to be) mom for right now. I guess I should have labeled this post with an appropriate warning.

What more is there to say?

Mary said, "Behold, I am the handmaid of the Lord.
May it be done to me according to your word."
Then the angel departed from her.

These were the last words from the gospel reading on December 20, 2011. This was also the day we had the CVS test performed. I took great comfort in those words, especially on that day. I was really nervous about the test (not really one for pain) and was also nervous about the risks involved and then the results themselves, well that goes without saying.  After reading those words, I was very much at peace. Not only the obvious, the words themselves, but I have always used that particular scripture as my own “pray without ceasing” prayer.

Well, almost three weeks later and here we are. Aaron and I received the call from the genetics counselor. Our little Alexander Dominic is SMA positive. As I am writing this the shock is still there. I guess shock isn’t the best word, as we knew the risks involved. We took those “risks” welcoming whatever child came our way, SMA or SMA free.  It still doesn’t make the news any easier.

Once again, it is a time for Aaron and I to let go. We must let go of expectations, plans for the future, and really any control we mistakenly thought we had over our own lives. We prayed for “normal.” We prayed for just a small slice of what other parents get every day. Aaron keeps reminding me that normal is relative and if I’m being totally honest if normal is what society tells us that it is, well, it’s almost downright disturbing.

We understand that you have no words of comfort and those that think they do…well, to be frank, they’re probably not very helpful. We don’t believe God only gives you what you can handle or that God wouldn’t do this to us if we weren’t strong enough to handle it. I don’t think and truly anyone with a stellar grasp of theology would agree that God doesn’t specifically punish people with some calamity to prove a point. Please save the clichés for someone else.

Here is what we know. God only creates things that are True, Good, and Beautiful. Whether or not that falls into what the world considers such is really not our problem.  We have no idea what the future holds for us. We can generally assume that Alex will present with the same type that Max is, but there are really no guarantees and to try and make plans seems like an effort in futility. We only know that it is a blip on the radar compared to eternity. We only hope that we can provide for our children and give them as many opportunities as possible, really all any parent can hope for. Please don’t assume this doesn’t make our current pain any less real. I sometimes think I know exactly how Mary felt when Simeon said to her, “-and you yourself a sword will pierce-“ (Luke 2:35). We experience that pain over and over anytime we see Max stifled by his physical limitations. I’m sure it will be no different for us with Alex. Thankfully, we will be better prepared to provide what Alex needs because we have walked this path before. I guess if the truth be told, we know this path better than raising a mobile child. In some ways having found out that Alex was SMA free would have provided its own set of challenges. How would we explain to Max the lottery that is genetics? We knew it would be painful to have one child “running free” while the other could only watch. Instead, perhaps we will have two beautiful boys who will completely understand each other.  

So what is helpful? Just treat us normally. Our lives have not changed, yet. We are still struggling with disappointment and I would be lying if I said we weren’t angry. Give us some space. Being around parents with small children is difficult for us. It is a painful reminder of our own family’s limitations. Continue to pray. Max has been such an incredible blessing and little Alex will be a powerhouse in his own right to be sure.

Peace.