THERE'S NO PLACE LIKE HOME!! :D

Did somebody say I can go home soon? For REAL??

All dressed up for his big debut outside NICU room 1848, after living here basically his whole life...just shy of 12 weeks. HOORAY FOR HOME TIME!!!

Ready to ROLL!!

I love my big brother, my basket, and my OWN bed :)

Playtime isn't bad either! :)

It's been one amazing week...details will come in the next blog :) Thanks for all your prayers to get us this far!! God is so good :D

Getting closer to going home! :D

Look Mom, no more tape on my cheeks! Bye, bye, NG tube!! :D

So....the doctors are starting to use the "H" word, along with actual target days now! The plan is, if Mason continues to improve, they are shooting for Tues or Wed. to go HOME!! :D The level of excitement around our house is climbing...we just want it to be for real!!

His follow-up apptmts are mostly all set up, except for his pediatrician and genetics (that doctor doesn't want to see him for 6 mths so it'll be a while until we have a "next step" there).

Neurology said they don't want us to have emergency seizure meds at home but they DO want us to have oxygen to be delivered by cannula if he is blue (no matter how long he is blue). They are not sending us home w a pulse ox monitor, but with a heart and resp monitor to be worn at night because "the fact that his heart drops with seizures is worrisome." and the pulse ox is not very reliable - as we know it doesn't pick up well sometimes and the doc thinks it will keep us up all night with false alarms. (well, if you ask me, if a heart monitor is gonna alarm off every time he bradies....which is when his heart rate drops, which happens A LOT when he is sleeping and doesn't have anything to do with a seizure most times, well sheesh. No sleep that way either. Anyway we'll have to figure that out. I guess we can buy our own pulse ox if we need one and use that if it's more helpful (they aren't too expensive).

The doc is bumping up his feeds again today, not by amount, but going to 110 mls in only 30 minutes (instead of an hour) "because life will be a lot easier for all of us that way" ... according to the doc. I told him that might be too fast since Mason had trouble before surgery with only 90 ml in an hour, and now his stomach is half that size due to surgery...but he said "the stomach is highly stretchable, he should be fine if given a little time. consider Thanksgiving." Anyway...so PRAY for him please because all of them agreed he should move to 30 minutes. They said if he is having issues they will leave it at an hour but they want to at least try. Because if he can't do it in 30 minutes then there's no point in bottle feeding because that will happen over an even shorter period. If it goes well, he can start trying to eat from a bottle again tomorrow. We'll see if his throat has healed enough from the reflux and surgery to go for it. If not I may wait until after he gets his Mickey button (7 weeks from now) to try again, just so he doesn't associate eating with pain. Here will be the schedule at home if he can do it:

On a "chimney feed" to vent (burp) his food through the G-tube. Starting today he is off the chimney and can just vent for a few minutes in the middle of a feed if he needs it, just like a regular burp time.

9 am-9:30 eat

12 pm eat

3 pm eat

6 pm eat

9 pm start continuous feeds through 6 am

They are going to teach me how to draw up his meds before we go. Today the nurse is trying to get his hearing screen set up for this evening. He has to have a car seat test before home which the dr said he imagines he will pass "with flying colors". Also, they are setting up a cardio consult for after home and repeat echo because there was another finding on all his heart tests we haven't heard yet - some kind of "prominent ventricular muscle bundle" to follow, but the doc told his team "well it hasn't caused heart failure yet so I think it's probably not too significant." They just said it was a consistent finding with each of the tests and his PDA is still there a tiny but so we'll see. The opthamology resident is coming by to check his eye pressures to see if he can stay off the glaucoma meds or if he has to go back on.

That's the update for now, he's sleeping well and has had physical therapy this morning, much to his disdain.

 

 Thought for today:

"We stopped relying on ourselves and learned to rely only on God, who raises the dead. And he did rescue us from mortal danger, and he will rescue us again. We have placed our confidence in him, and he will continue to rescue us.  And you are helping us by praying for us. Then many people will give thanks because God has graciously answered so many prayers..."

2 Cor. 1:9-11 NLT

G-Tube/Fundo Surgery Update

A little TV goes a long way during recovery...

We sure have been appreciating all the prayers coming our way ... Mason has had a kind of rough recovery, not so much from pain (they are giving him Fentanyl really often, every 1-3 hrs as needed), just because his intubation was so hard (they had to try 3 times and kind of traumatic on his throat/lungs to do that...so he is real swollen in there and has quite a case of stridor from it (sounds wheezy/barky/loud every time he breathes, kind of similar to croup). He's had 2 treatments for it but so far they haven't done much except prevent him from sleeping (they are adrenaline aerosol "vapo" treatments). He had a very hard time sleeping afterward the entire 24 hours, even when his pain was controlled the first night--they had to keep suctioning his vent because he had a really huge and unexpected amount of secretions/saliva down in it. Of course the constant suctioning drove him nuts. He didn't sleep hardly any from when he woke up from surgery Wednesday (around 2 pm) until after I left (around 2 pm Thursday) so I was really relieved to hear he zonked out after my mom got there and slept well most of the afternoon since. He has been wearing some kind of humidity helmet/hood for the stridor but they took it off this morning b/c he wasn't a fan. Pray his stridor gets better on its own (today so far is going MUCH better both pain-wise and stridor-wise)...and that he can slowly wean off the Fentanyl without a lot of pain or withdrawal. Also, pray LOTS of protection over his IV...it is looking like it might be giving out and if it does he has to get a new one for Fentanyl and fluids since he's not up to full feeds (just on a slow drip of Pedialyte through his G-tube, but that will change to formula this evening, Lord willing). He's already had some dirty diapers so that part is great :) But if his IV dies that will be bad because he is SUCH a hard stick and there won't be much of a way to sedate him for that if his IV gives out. Thank you for praying that it holds out as long as they need it!! :)

Curveball

 So...as if this situation hasn't been confusing enough, it just got even more so.

It turns out that at least one other person in the known universe DOES have Mason's exact deletion of 14 genes. We were kind of hoping that if someone else had ever had it, it would give us some insight to how to treat Mason better.

BUT. Not exactly the scenario we were looking for has transpired. It turns out that this one other person (at least that we know of right now) is...you guessed it. 

ME. Myself. And I. 

That's right. His own mommy has it. The FISH blood work we had showed the least likely scenario of all--the one the geneticist said they totally did not expect at all. But of course if we've learned anything on this journey so far, it's to expect the unexpected. The geneticist expected the test to show that this deletion was brand new in Mason, in which case it was probably the reason for all his issues. Or, he expected it to show that Brian or I had all the genes Mason is missing, just possibly rearranged (called a balanced translocation - anyone can have translocations and not show symptoms or a syndrome of any type, but when they pass it on to a baby it can become a deletion). Well the test showed that I am missing the very same chunk of genes he is. ??? is all we can think. I always knew he was a mama's boy. But ... a little more of mama than is good for him I guess. But what does it all mean?

We have no idea. The geneticist's best guess is that, since I have the deletion and have no huge health issues like Mason, then another mutation somewhere (another un-tested-for-yet syndrome) is behind Mason's issues. Obviously this brings up a lot of questions we will have to talk through with the doctor before we have any idea what the next step is. For example, he's a boy...I'm not. Things may play out differently based on that fact with the very same deletion. But right now it basically means we are back to square one, trying to find what is actually causing all these problems. It may be a really long time until we have answers, if ever. Obviously if we have Micah tested and he has this deletion too and he is "generally okay" (although we know Micah has never been exactly the picture of health) then it means that probably this deletion causes SOME problems but by itself shouldn't cause the degree of problems Mason is having and they will need to test for other things as he has other symptoms growing up and a clearer "syndrome" emerges. (He hasn't had any genes for specific syndromes tested at all yet--he's only had a test looking for missing chunks of genes, which they found. But for example if they think he has a particular known syndrome instead, something known to be caused by a change on a certain gene, they can look at that gene. Right now they have no clue where to begin looking at the genes since his set of symptoms is so unique. But we do have some theories and ideas for them on where to start and will be talking to them more about this later in the week we hope.

In the meantime, the ball is rolling to seeing the light at the end of the NICU tunnel. Wednesday is the day scheduled for Mason's G-tube surgery, with Nissen wrap (see previous post for the why's and what's of this). It's scheduled for 10:30 am. Please pray with us that they are able to control his pain much better this time than after his malrotation surgery and that he doesn't have any withdrawal issues or more seizures and fevers in the recovery period (which will be longer and more painful than if he was getting the G-tube alone).

Today he had a repeat heart echo to be sure the final heart defect, a VSD, is gone (they have a hard time believing it would close up on its own in only 10 days but we have a big God so I don't doubt it!!). He also had a repeat kidney study today to make sure his swelling is gone that he had when he was born. He has a few other consults and tests to be done before he can go home ... a skeletal survey x-ray, developmental pediatrician, another MRI probably, etc. But if his recovery goes well that's the plan...to get us trained in all his home care needs and to go home!! He's still growing like a weed...check out the meat on his bones!! Last night he crossed the 12 pound mark! :)

 

It's Monday again...so here's what I've been trying to tell myself to get through another long week:

I gotta keep my eyes on Jesus
Through the weak days;
In a world where we really don't belong,
I've discovered if I keep my eyes on Jesus
Through the weak days,
Then even on the weak days
He'll make me strong.

- Steven Curtis Chapman

Pressing On...

Well, it's been a long time since an update...I keep trying to wait to update until I have actual new information, but right now it's still a waiting game everyday. Here is where things stand with Mason right now.

1. He was seizure free since the 12th of Oct. on his increased dose of Keppra, and no phenobarbital. Last night he had another seizure. He's had some slight seizure activity during the day today too. No med changes yet, unless the seizures continue. If that's the case, my preference will be to make a SLOW increase in his meds over the course of a few weeks so that hopefully the side effects will be less severe. (His main side effects so far seem to be insomnia in the daytime and increase in reflux/vomiting).

2. We have made a decision to go forward on the G-tube for now because he is so up and down on his ability to eat. We had hoped to be able to do a simpler procedure called PEG (basically just like ear tube surgery -where you're just sedated with gas and they poke the tube in and you're done, hardly any recovery to it other than maybe a little Tylenol.) But due to his intestinal malrotation and previous scar the surgeon says it's too risky - they might accidentally puncture his colon that way and can't take that chance. This means he'll have to have an open incision gastronomy put in, which is a much bigger deal and much more post op pain and therefore the need for narcotics. I was really disappointed about this since we're not sure how he'll handle more pain med withdrawal; I asked if they could do the On-Q style anesthesia (where they leave a novacaine pump in the incision to avoid the need for narcotics) but sadly they said this is supposedly only for adults and they don't offer it at our hospital. So I need to talk to anesthesia about which drug we should use. I did talk to the lady who did the mouse research on beta-arrestin2 knockout mice (see previous post). She said that morphine may be a better choice for him than Fentanyl, because at least in mice, they had less severe withdrawal from morphine than from Fentanyl and needed less of it for effectiveness, but it's hard to say what's going to happen with Mason. She offered to talk through it with his docs, and she wanted more details about the other genes missing to get a better picture of what was going on. Some of the genes he is missing are things for like glycolipid transport and if this affects the metabolism of certain substances, it could cause an underlying nervous system/neurological disorder hasn't been identified yet, which could have a lot of the same symptoms as withdrawal on the surface, but not actually BE withdrawal; or he could have both - a more severe withdrawal from certain drugs due to missing proteins, and/or also a brain disease that has symptoms very similar to drug withdrawal in general. Whatever the case, it's definitely confusing.

3. Another factor involved in the G-tube surgery is whether it will be JUST a g-tube surgery, or if it will include a procedure to help treat his reflux/vomiting issues. He had an upper GI barium swallow study today to determine if he's having reflux and how much, but we don't have the results yet. Basically, if the results show a significant amount of reflux or some structural problem that isn't going away, Mason will need what is called a Nissen wrap or fundoplication procedure, where they wrap part of his stomach around his throat to keep him from being physically able to throw up. If he clearly needs this, we'd rather do it now with his G-tube surgery than have to subject him to more surgery/pain meds later. But we need prayers for clear direction one way or the other as neither option sounds all that great to us. If he DOESN'T get the "fundo" surgery now, the surgeon is afraid his reflux will get worse from the G-tube, because they have to "tent up" his stomach for the G-tube, making the angle more likely for the stomach to reflux; ongoing reflux can damage his insides, make him hate eating, and cause issues with the g-tube. If he DOES get the wrap, it brings more possible complications - the size of his stomach will be reduced by about 50%, meaning he'll have to eat tiny meals all through the day, most likely, and for a while after the surgery, will need to be suctioned often because he may not be able to swallow his own saliva. Plus, it could make it hard for him to eat solids later if he is able to by mouth, and also, the sensation of having his stomach wrapped around his throat will be more distressing to him than the reflux he had before (according to his surgeon), since he's used to the feeling of reflux, so they really really don't want him to have it unless he really needs it. And we really really only want to have to do one surgery, not 2 ... so please pray for extremely clear direction on this decision, which I suppose we'll need to make tomorrow after the surgeon gives us his recommendation after seeing the upper GI results.

3. Mason's left eye had glaucoma when he was born - they have gotten that under control with eye drops, and now the drops have been stopped to see if it stays under control by itself. If not he will go back on the drops as needed.

4. Our parental FISH test results (the ones telling us whether this deletion in Mason was inherited, or a one-time random event) are still not back.

5. If we can get Mason through his surgery (hopefully next week), and we have a good plan for seizure control, hopefully within the next couple weeks we can start thinking about home!!

And since I need it, here is another song for today (No Matter What, by Kerrie Roberts):

http://www.youtube.com/watch_popup?v=OA3MSqufJP4&vq=medium#t=13