Leaning Hard

The first week home has been so emotional...joy that's hard to explain from having our baby right here with us to love on anytime we want, fear and stress from the usual worries of being a parent all over again, that doubled a few times over, learning the extras of gtube care, dressing changes and pump feedings, oxygen tanks, heart and apnea monitors, etc etc, trying to navigate through the new world of back and forth to his doctors (specialists in opthamalogy, cardiology, neurology, neurosurgery, genetics, urology, surgery, GI (or not - nobody knows who exactly is in charge of helping us with his Gtube and diet changes, pediatrics, plus setting up early intervention programs for occupational and physical therapy for feeding issues and developmental issues, vision therapy, on it goes. It is a lot to swallow ... and a new challenge or adventure every night. Here are some first-week "initations" we've experienced so far:

Tuesday: after carefully mixing up 24 hours worth of the hospital dietician's formula recipe (30 oz of water to 18 scoops of powder to make it 24 calorie/oz instead of the usual 20), and storing it in labeled bottles so we knew he was getting the right amount at the right time, we got ready for our first night home together. It didn't take more than one occasion of forgetting to pinch the Gtube to remember it's a messy mistake. Then it was bedtime. We were amazed at how well Mason slept all through the night, never cried once! But he grunted quite a bit in his sleep, obviously gassy from the inability to burp. We tried venting his tube and that didn't help, but other than not sleeping much from that, the night went well - we thought. Then the next morning we realized that although the feeding pump said it had delivered all 350 mLs of formula (which it was supposed to do), there was at least 85 of that 350 STILL in the pump. So what did we do wrong?? Did we miscount and put too much in the overnight bottles? Or did the pump misread and just not give him all he was supposed to have?? We had no idea whether to try to give him the extra in case he was 3 oz behind or if it would be worse to overfeed or underfeed him?? The home health nurse who came the next day told us to let it slide for the day...if it happened again we would need to see about a pump replacement.

Wednesday: well the formula amount left over was correct this time. And we vented him using his "chimney" syringe all night, so he slept a lot more soundly, less gas. But to our horror when we woke up the next morning, the chimney had come untaped from his gtube, despite 20 minutes worth of "safeproofing" it, and it spilled formula and stomach contents all over the bed. Uggg... what next??

Thursday: We decided not to vent the Gtube tonight since we decided it wasn't worth crying over spilled milk all over again. We ordered some Farrell Valves which some other moms said were "magic wonders" to vent reliably...they cost a ridiculous amount for a month supply and none of our docs have heard of them to let us know if insurance will cover them, so if these work we'll just buy a few and reuse them I guess. Surprise of tonight? At midnight the food pump alarm goes off with an error message. Funny thing to find out in the middle of the night is, there's no manual. If we have a question we are to call the help line and wake someone up to help us at that hour. Um. Really? We opted to dig up the info online from the user manual. Just as we were getting that sorted out, the apnea monitor went off. At 85 decibals. I hope no one else in the neighborhood was trying to sleep. Thankfully, Mason hadn't had apnea. It was just another error message we had to figure out. "Lead loose." Ahh.  Of course.

Friday and Saturday same story...lots of gas, not much sleep.

Sunday we went to church and Mason had his first seizure since coming home, during the middle of the worship service. He'd gone about a week since the last one, in the hospital. We tried to do what the docs had told us - give him oxygen if he turns blue (he did but the oxygen tank got set up wrong so pretty much a big ugly FAIL there and thankfully it was less than 2 minutes or so and he came out ok on his own), call neurology and tell them so they can adjust his seizure med dose (we did, and now he is on MORE than the max allowed dose of Keppra for babies). Any more seizures means another trip to the neurologist and a new med added or total change of approach, meaning another wean. This I DREAD. Sunday night due to the increased Keppra Mason was fitful and insomnia boy all night, and groaned from feeling sick all over. His big brother woke up screaming after going to bed with an ear ache.

Monday we woke up and Micah had a full-blown cold, along with his ear ache. Mason was sick to his stomach from the Keppra increase, and kept trying to vomit but of course could not. We already had an appointment for Mason, to his first follow-up doc visit to meet his pediatrician. He told us Mason's ear had fluid in it too. Sigh. Looks like a week of sickness on horizon. I guess we'll be back to the ped. tomorrow to talk about Micah. Oh, how I love cold weather. :(

Really, I have no idea what tomorrow holds or how we will get through these long days and never ending doctor visits that don't seem to yield much helpful information, but I imagine it will involve a lot of surprises too. I just hope there are more of the happy ones -- like Mason smiling when I put him down on his tummy for the first time since surgery today, or playing with his musical toys all by himself in the video below :) I will just have to remember a poem my grandma sent me a long time ago by Octavius Winslow ...if ever I needed to "lean hard," it's now...

"Child of My Love! Lean hard! Let Me feel the
pressure of your care. I know your burden, child!
...For even as I laid it on, I said I shall be near, and
while she leans on Me, this burden shall be Mine,
not hers. So shall I keep My child within the circling
arms of My own love. Here lay it down! Do not
fear to impose it on a shoulder which upholds the
government of worlds! Yet closer come! You are
not near enough! ... You love Me! I know it.

Doubt not, then. But, loving me, lean hard!"

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