Wednesday, May 13, 2015

Normality 2.0

In my last post I shared the new normal that my family has found, with Jake being the healthiest he has ever been...then shortly after that my little man had to challenge us once again.  Jake has been waking every morning with puffy eyes, and when this swelling started we contacted Jake's cardiologist who wanted him seen for immediately for an echo and a CT scan because the concern was that the swelling could be caused by a compromise of his Superior Vena Cava, which drains to his lungs because of the connection made during the Glenn open heart surgery.  If there was a blockage, then the pressure would move back upwards and could show in swelling in the face.  We spent 7 hours at the Janeway that day, with Jake fasting for sedation, with an unsedated echo followed by bloodwork, 7 pokes for an IV and then sedation for the CT Scan.  That day passed in a haze - I genuinely felt like I could puke at point.  Jake had a rough go of it, feeling very upset over all the procedures and his fear of hospital workers came back full force. That day it was raining outside, so Jake wore his rubber boots.  And he insisted...demanded...that he be allowed to keep his boots on.  He had his boots on for all procedures except the CT scan :) I believe that was his way of keeping a small measure of control over his life, when everything else was spiraling for him.  Here he is with his boots, and his cars - waiting on the results of his echo.

 When the cardiologist came to us at the end of the day with the results, she told us that we should be saying prayers of thankfulness - there was nothing wrong with Jake's heart.  His heart is holding stable, and the CT showed a great surprise...Jake's severely narrowed left pulmonary artery (which was 4.5 mm in size at the last CT scan) had GROWN.  GROWN! This growth was a huge surprise for everyone - but something to be celebrated.  I don't have the exact measurement yet, but the estimation was that it had gone from 50% the size of the normal pulmonary artery - to around 75% the size of the normal PA.  That is nothing short of miraculous in my mind - Jake has been having monthly echos ever since his Glenn surgery because of this narrowed LPA, and the dangers it presented.  Now those dangers are less.  Some of you may remember that the team at SickKids attempted to repair this narrowing with a patch last year during his Glenn procedure, but the patch failed.  The plan is now to attempt a new fix during his Fontan open heart procedure, likely in the Fall of this year.  With this growth I have hope that maybe a fix won't be necessary, or at least won't be as complicated.  The final stage of Jake's heart repair is complicated enough with any added issues.  Please, if you are someone who regularly prays for Jake - add this to your prayer list - more growth for his LPA.   Thank you Lord.  You have proved your faithfulness to Jake and our family over and over again.  I praise you for that.

Jake is still waking in the morning with puffy eyes, and we have not figured out why.  Allergy meds are not being responded to - so it's unlike to be an allergy but we don't know for sure.  Celiac disease was ruled out in the bloodwork.  We don't have the results of the thyroid bloodwork yet.  But, Jake has been referred back to Gastroenterology to see if they have any answers.  So, we wait...

While we're waiting we have been enjoying more of our normality...and discovered that Jake has a great love for the outdoors.  Some days we spend all afternoon outside, even though it is still super cold here - we bundle up and live outside or on the patio for hours at a time.  


We can't get enough of our wonderful life together.

Today, Jake got rid of the enormous surgical g tube, and now has a Nutriport G Tube.  The difference is mainly one of size, and comfort for Jake.  It also means we don't need to use tape on his sensitive belly skin.  I took a picture before it was placed in Jake.

See that tiny thing in the middle of the picture? Shaped like the letter T? That's a nutriport, and it's now in Jake's belly.  The Doctor took the water out of the balloon that was holding the surgical one in, then removed the old tube.  Then he slipped the Nutriport into the hole with a little bit of difficulty since the hole is so small (and Jake had to be restrained by 3 adults to make this happen, he was so upset).  The nurse then filled the nutriport's balloon up with water to hold it in place and voila! A new, skin level G tube for Jake. 

Here's a file picture of what the tube looks like when the balloon is filled with water.  The flat part is what we see on the outside and the balloon in inside Jake's body.

We are adjusting to it, and learning how to use the attachments that come with the Nutriport.  Next on our list is to find a supplier for this new G Tube because it is not one used at SickKids, where we buy the rest of our supplies.  We're waiting for a prescription, then this Momma will set out on a mission to make that happen.  These are quite expensive, but we need to have a few on hand in case this one is pulled out.  Jake's new higher calorie formula is also significantly more expensive, and since we are not a two income family we have to carefully budget for these expenses.  

We are still struggling with weight gain, in a big way.  There has been no weight gain.  For every gain Jake has made, the following week there has been a loss.  We see the dietitian weekly because of this.  There are several concerns here - Jake is so small (roughly 2-3 on percentile chart), for Jake's open heart surgery in the Fall the recommended weight is 35 pounds which means Jake is 14 pounds too small.  And today the general surgeon added a new concern to our list.   It was something we had never heard of before - in people with very little subcutaneous fat layer (read: people with no fat under their skin), the pacemaker can actually erode the skin.  I literally turned to the doctor and said "what?" when he said that.  Then he reiterated, and I said "you cannot be serious".  Basically what he said is that the pacemaker is protruding so far from Jake's belly now, because he is growing length - wise but not gaining any actual weight while doing so, that it may actually come right through his skin if he doesn't start gaining weight.  

We already started a higher fat diet with Jake, and if the results for this week don't show any improvement when he is weighed on Friday, then another step will need to be taken.  It's a struggle because we can always give him more of his high calorie formula, but that means he will no longer feel hunger and will stop eating.  And we have fought so hard to get him to a point where he will eat well.  

Whew! There's a big update on all that is new with us right now.  We're still taking it one day at a time, and trusting God through it all.  Jake is no longer happy to walk into the Janeway though - and today after his new tube was inserted he fell asleep.  When we got home I laid him down and then went to hook up his pump to his new G Tube.  As soon as I touched the site Jake stirred in his sleep, and said "no!" :( My heart breaks for all the suffering - so much suffering.  Here are some pics from today at the hospital - as you can see, Jake is not his smiley self when he is there.  Please pray for comfort and strength for my little warrior, and for the bruises that my heart gets every time I have to hold him down for something that is hurting him.

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