Good News/Bad News Update

A bit of fun before procedures today.

It’s been a one step forward, two steps back kind of week. Or maybe it’s been a one step forward, one step back kind of week. Essentially, all good news seems to be tempered with bad news on the rare days we get good news.

Here’s the high level: We discovered several days ago that Matthew had a large pericardial effusion, which means he had fluid around his heart. This was keeping the fluid output from the lungs high and is why we had the chest tubes far longer than we wanted. Draining the effusion effectively dried out the lungs so we could remove the chest tubes. Those came out two days ago.

Draining the effusion, however, also meant we had to put in a new tube, to drain and measure additional fluid building around the heart.

Earlier today Matthew had a sedated echo to get a good look at his heart. The result was positive in that they pulled the drain and, for the first time since surgery, he is tube-free. We were high-fiving and celebrating for about 30 seconds until we learned that, during the echo, they were unable to get a clear view of his left pulmonary artery (PA). 

If you recall from an earlier post, they found that the left PA was "tortuous" or a bit obstructed during surgery and they did some work to enlarge it. The Fontan (the procedure Matthew has had) does best when there is unobstructed blood flow to the lungs so we don't want to take a chance on this.

Because Matthew has already been without food or drink all day (yet AGAIN this week) they have taken him straight to the cath lab to get a better look at the left PA and decide if it needs intervention (i.e.: a stent). 

We are now awaiting word from the cath lab on what they find and will let you know. We're all so ready for this kid to catch a break and be able to come home again!

A Good News Update!

Matt Matt & Mimi find the play room

In case you missed it in some of my previous rants (Matt Matt's too), Matthew still has his chest tubes, which are pulling fluids from his lungs post-op. Additionally, it's not a good thing that he still has them a week out. The chest tubes are horribly uncomfortable and lots of times they're downright painful. They keep him from being able to move around, which blessedly he has wanted to do more and more in the last few days.

Finally this evening Dr. Breitbart said we are pulling the tubes in the morning! That collective sigh of relief you heard this evening was from an east wing at Boston Children's Hospital. This feels like a champagne toasting event. We won't be drinking of course, but one of you guys enjoy a Mimosa in the morning and raise a toast in Matthew's direction. I wish I could convey to him how much better he will feel immediately after, but I guess we'll settle for counting down the hours and consoling him in the meantime.

So what does this mean for the the rest of his stay? Well we are definitely not coming home Wednesday as originally planned. However, after the tubes are out, Matthew will stay two more days and then have a chest x-ray. If all looks good (no air or fluid pockets visible) then he'll be discharged. So Thursday at the very earliest.

It will be early afternoon before we can probably update again tomorrow but we'll post as soon as we can. Thanks again for all the prayers and love. They are felt and appreciated so very much.

Quick Status Update

Matthew was moved out of CICU and into a room last night. His oxygen canula was removed and his sats have been pretty good overall. He has received the "blow by" oxygen a couple of times though, when they dropped.

He continues to have too much fluid draining off his lungs so the chest tubes are still in, which, in my opinion, is one of his bigger issues right now from a pain standpoint. The tubes are really large and reportedly the big kids say the day the tubes come out is the day things change for them in terms of feeling better.

Sometimes he seems to feel like getting up and about but as he moves he has pain from the tubes then changes his mind. Ideally he shouldn't still have this much drainage a week out and if it continues we may be heading back to the cath lab to investigate.

He's irritated and vacillating between whimpering and crying for the last couple of hours and is clearly just plain miserable. He went outside this morning and hopefully we'll get him back outside again this afternoon for a bit.

Matthew's No Spin Zone

Hi Friends,

I hear lots of you have been asking about me, praying for me, sending me love and energy for swift healing and I thank you for it. They told me about some of the updates you've been getting and I'm here to tell you that you can ignore all that sugar-coated crap that's been coming your
way.

Dad says people want to know how I'm doing so I'm going to tell you. Jen Jen says she's unwilling to transcribe swear words for me because I'm too young to say them but take every one you've ever heard, put them all together and then scream them at the top of your lungs while you're completely constipated, have daggers in your chest, tubes from every extremity and oh yeah, a gaping wound in your sternum. Okay, maybe it's not gaping. In short friends, I feel like hell. I kind of feel like I just had open heart surgery. What's that? Oh, I'm being told I did. Well, there ya go. That's how I feel.

Honestly, I appreciate everyone trying to help me feel better but I am just sick to death of everyone touching me (except my family of course), poking me, prodding me, sticking me, measuring me, poking me again, forcing me to take meds, holding me while I puke them back up. I've taken to letting out a warning whimper every time a medical person walks in, just for good measure, even if it's just the janitor here to change the trash. Better to make it clear he shouldn't even think of coming near.

We finally got to go outside today, which was nice but I wish I could enjoy it more. Sometimes I want comfort. After outside Mommy held me in her lap until I fell asleep. I feel better when she or Daddy or Mimi or Jen Jen are nearby and holding me or my hand.

Other times I'm just ready to get on with things. I tried to stand up earlier. I had help from Mommy and Daddy and held on to Mommy's legs but I guess what they say is true... use it or lose it! Time to hit the gym again apparently. Most times though I just feel too darned crappy to move so I just lie here and wait for it to pass... including my bowels... I hope they pass very soon!!!

Ohhhhhh.... well I just got a shot of something magical in my IV line and I'm starting to see things again that people around me can't. Better sign off rather than risk saying something goofy. :)

Thanks for all the love and well wishes. I'm trying to get through this... whatever this is. I thought we came to Boston to have fun! The airplane, the ball game, the museum, the harbor cruise... guess we weren't here for a fun vacation after all. Off to sleep and let the body heal.

BCH Has Gone To The Dogs

Matthew has had a rough couple of days. He's had a lot of fluid continuing to drain from his lungs and basically just still feels pretty rotten. He's still in CICU despite the ongoing discussion of moving him to the floor. This morning there was a dog show here at the hospital and the day nurse, Jess, agreed to load him up and help us take him to see the pooches. Matt Matt just observed quietly. Then we got a huge bonus - we were able to take Matthew out into the beautiful healing gardens here. I read about the garden here a couple years ago in an issue of Sci Am Mind, which featured studies being done on the healing powers of outdoor green spaces on recovering patients. As we've talked about before, "outside" is in the top 5 if not top 3 all time favorite words for Matt Matt so we have been anxious to start bringing him out here. Once he's out of CICU we'll be able to bring him out more and more. The fluid is reducing today and there is still a possibility he'll get out to the floor today. Here's hoping!

Post–Op: Day 4

 

They say day 3 after surgery is always the worst and there was no exception for Matthew. He had a couple of bumps the road but had a good night and seems to be doing much better today. Jen Jen is on her way! :-)

 

Man, I have been looking all over the place for this thing!!! It's great to see you smile again, son!

Post-Op: Day 3

Matthew's common atrial line (an IV that went directly into his heart) was removed today which allowed us to take a short walk. Earlier today, the plan was to transfer Matthew out of the CICU and out to the floor, but due to a shortage of beds on the floor, Matthew gets to enjoy being the "wellest" baby in the CICU. We've had a few issues this afternoon with urinary retention as well as continued output from his chest tubes. These problems will eventually take care of themselves with continued treatment. All in all a good day. It only gets easier from here!

Post-Op: Day 1

Matt Matt and Jen Jen, pre-surgery. Happy baby!

EVENING UPDATE: Matthew has been extubated and is doing very well so far. His pressures look good. O2 sat is great. Plan to start pulling central and arterial lines tomorrow.

AFTERNOON UPDATE: Things are sounding more positive for Matthew. They have removed one of three chest tubes. He is responding well to diuresis. ABG's look good as well. Planning to extubate him around 7:00 eastern today.

MIDDAY UPDATE: All is progressing with Matthew. It's a hard recovery process (at this point harder for the family members sitting and watching him than for Matthew) but everything seems to be going as expected, which is a good thing. Mimi says he will occasionally raise his arm (though he is not conscious) or move it, which the nurses say is a good thing for him to do. Matt Matt probably knows this instinctively. ;)

MORNING UPDATE: The overall news remains positive for Matt Matt. He had a rough night in terms of bleeding and fluid loss. He received a lot of replacement in terms of packed red blood cells, platelets (and other medical terms Will and Nikki rattled off ;).

His sinus rhythm is normal and has remained so through the night. There was some concern as they were warming him after surgery because he was a "junctional" rhythm (an abnormal heart rhythm that results from impulses coming from a locus of tissue in the area of the atrioventricular node, the "junction" between the atria and ventricles). This corrected on its own however and the continued normal rhythm is positive.

Of course we all want to race ahead to the point where Matthew is up and playing in the outdoor garden space at Boston Children's and walking around in his new light up shoes from Mimi, but it's a tremendous relief to have a successful surgery behind us and now it is one step at a time. Hopefully the breathing tube comes out later today.

Thought I'd share one of my favorite pics with Matthew this morning... just because it makes me smile.

SURGERY UPDATES

First, many thanks to all the amazing people who care so much about Matthew and are eager to hear how he is doing, as well as for all the love, thoughts, and prayer throughout this process.

I will update THIS POST throughout the day as we have updates from the OR. Please check back here for the latest on Matthew's progress. All times below are Eastern.

2:43 - Incision has been made. Surgery is underway.

4:15 - Matthew is now on heart/lung bypass. 

6:00 - No significant update. Matthew still on bypass. Surgery is progressing well. 

6:45 - During the bypass process, they cool patients down to slow the cellular metabolic rate down. The liaison nurse just told us that the operation is essentially complete, and they have just started warming him up to take him off of bypass. We have not heard from surgery for a full report of the operation yet. 

7:23 - Officially off of bypass. It will be another 45 min to 1 hour before we see him or hear any more. 

8:20 - Matthew is back in the CICU (Cardiac Intensive Care Unit). Still haven't seen him or the surgeon. 

10:20 - Will and Nikki are with Matthew now. Dr. Baird said that everything looks really good. They put a graft into his left pulmonary artery (PA) to expand it. For those of you who missed the earlier post - during the heart cath last week they said Matthews left PA (supplies blood from the heart to the left lung) was "tortuous" or in lay terms... had some obstruction. Dr. Baird said they will watch the PA to see if it needs any more attention in the future, however, any intervention could be done in the cath lab. Just got one more quick update from Mimi, who just saw Matthew and announced that he looks "beautiful." :)

So many thanks to those of you who have remembered Matthew and family today in thought, prayer, or an uplifting word.  Our gratitude is hard to measure. 

In Surgery

Thomas saves the day!

Matthew was taken back about half an hour ago. Here are a couple pics from pre-op. Looks like the hospital staff is smart enough to know that having plenty of Thomas the train characters on hand will go a long way in distracting a hungry/thirsty 2 year old.

More Thomas!

Happy Boy

My favorite new way to travel!

Lately I can't walk a real long way without having to rest and catch my breath, which my surgery today will help correct. Dada says we walked about 8 miles around Boston on Saturday though and I didn't feel tired AT ALL (Dada didn't seem to share my energy level at the end of the day though)!! What a DAY!!

Big Day!

Does my butt make this chair look big?

Hi Friends,

Matt Matt here. I've been in Boston more than a week now and mostly we've just been having lots of fun! There was one day they took me to Boston Children's and back to the cat lab, but again, I saw zero cats in there (I'm thinking they need a better name for that place). I did go sound asleep again but this time my visions were lots more exciting. Probably because I have seen and done so many things now.

I was really sleepy for the rest of the day but also I was really sick of people telling me to lie still and flat on my back. Don't they know how many things there are to do in this city?? I was ready to jump up and get OUTSIDE but nope, uh uh. Mama was able to lie down in the bed with me though and at least that made it all a little nicer.

The next day things took a big time turn for the better. Mama, Dada, Mimi and I all went to a big museum just for kids!! It was so cool even the adults in my family were acting like kids and playing with me. There's a group here made up of men that are blue (kind of weird but whatever) and they play these funny drums made out of white tubes. They had a set at the museum and we all got to play even though we aren't blue! I'm glad they made that exception.

Just when I thought things couldn't get better, someone gave us tickets to see the Red Sox play!! I haven't been to a baseball game before and Dada said I was very very lucky to see my first game at Fenway Stadium. Everyone seemed a bit more relaxed and Mimi told me it's because I'm here to have a big surgery but it got moved to Monday (today) so we had some time to relax a bit.

The weekend was totally awesome as well. After Grandfather got here we all got on a big boat in the harbor and went around to see all the cool things about Boston by water. I dubbed myself captain at one point and took the helm.

We went to a bar that apparently has a long history of knowing everyone's name but that bar did not live up to that reputation. That's okay though because I already know exactly where everyone knows my name, and that's at Boston Children's Hospital.

Speaking of, I have to go take my shower and get ready for the talented surgeons here in Boston to open my chest up once more and complete the process of replumbing my heart so that I can breathe easier and so things will run a little more smoothly for me going forward. It's a complicated procedure and I think Jen Jen will tell you a bit more throughout the day.

But once again, I'll be sleeping soundly, flying with birds and superheros, lifted up by angel wings, and missing out on all the action here. I asked Jen Jen to send you pictures of some of our cool adventures while I'm sleeping though so I hope you guys enjoy them. She will also be telling everyone how I'm doing throughout the day.

For now, much love and appreciation for all of your thoughts, prayers and focused, positive energy for me and my family today.

XOXOXO!
Matt Matt

Surgery Date Moved

Couple quick updates.

1. During the heart cath the doctors found Matthew's left pulmonary artery (PA), which supplies blood from the heart to the left lung, was "tortuous." This is a medical term which basically means coarse which causes some obstruction of blood flow. This is not the kind of thing they would intervene on during a heart cath and they may or may not elect to do so during surgery.
2. Otherwise, the doctors were pleased with everything else they found during the cath.
3. Finally, the surgery has been bumped to Monday from this Friday due to an influx of emergent open heart surgeries at Children's Hospital. 
4. Matthew and family are having a day at the Children's Museum in Boston, thanks to some generous donors at Boston Children's Hospital who make tickets to all kinds of cool events available to families in town for medical care.

Heart Cath Complete

Matthew is in recovery now. Will and Nikki will go back in the next 30 - 45 minutes to see him. He will be required to lie flat for 6 hours and let me just say, I have NO idea how they'll pull that off! This is one of the busiest kids I know.

Good news is Matthew will be able to leave the hospital this evening (there was fear they would have to stay overnight after this procedure) so they will enjoy one more free day tomorrow in Boston before the surgery.

No interventions were done as far as balloon dilation or otherwise. We will know more once they get back there and can speak with the doctor directly and I'll update again then.

I'm guessing Matt Matt is going to have a few words of his own later today!

Heart Cath In Progress

Matt Matt is sleepy this morning (all that fun in the park yesterday must have worn him out!) and as I type now his heart cath is underway. He went back just a few minutes ago. This procedure is critical to give the doctors an understanding of Matthew's current heart condition (pressure, volume, etc.) in advance of Friday's operation.

Thief of Hearts

Matt Matt and Mimi

We are now just 3 weeks away from Matthew's third open heart surgery, the Fontan Completion. In a couple weeks he will make his way, once again, to Boston Children's Hospital where the pre-operative care and testing will begin.

I have been fortunate to spend a lot of time with "Matt Matt" (as he calls himself - and now so do we) over the last several months. He is a great talker with a huge vocabulary and excellent enunciation (better than many adults I know). He really seems to enjoy words and you can watch the process as he learns a new word - he stops and considers the word first, then begins to utter each syllable, one at a time, before stringing the word together. He will continue to work on the word until he is comfortable that he's saying it like he hears it.

Matt Matt has also been going on the potty for the better part of the last year. Not exclusively of course but he's very good about it every time he wakes up and randomly throughout the day. He's truly an exceptional kid. He's a fan of redundant syllables (Mama, Dada, Mimi, Papa) and so I have become "Jen Jen." And let me tell you - Jen Jen loves her Matt Matt!

In many ways I feel this will be the hardest surgery. Not due to the level of difficulty of the surgery itself (though it will be highly complex like the others), but because Matthew is now a little person - the best little person we know. We loved him during his first two surgeries, when he was 6 days and then 6 months old, but he was still a little blob who made strange noises we couldn't quite identify. Now he talks to us, he interacts with us, he makes us laugh (a LOT). He has his own personality and his own distinct way of interacting with the world around him. The very peaceful and loving presence his mother exudes is evident in Matthew's sweet and goodhearted nature.

Everywhere he goes people fall in love with him. I asked my brother one day, HLHS aside, if he knew how lucky he was to have a two year old who is so pleasant and agreeable nearly all the time. He did. We took Matt Matt to a local alpaca farm a few weeks ago where we picked up some locally grown, organic vegetables and eggs. Matthew breezed through there like a cool wind on a warm day that draws your attention upward and brings a smile to your face. I watched him effortlessly steal hearts from every person he encountered. If he'd had a bag to put them all in it would have been overflowing before we left.

I marveled how a child, whose own heart is so badly broken, became such a master of stealing the best of what others hold in their hearts. Someone told me recently that I couldn't understand a particular sentiment because I didn't have children. I replied: I assure you that, while I haven't given birth, I'd give half my own heart to make this child whole again if I could. This is the effect Matthew has on people. This is the Matthew we are taking to Boston this time around. And this is the precious child we look forward to watching walk down the sidewalk without losing his breath when we come back.

Hope is the thing with feathers that perches in the soul.

Matthew's birthday. Such a treasure this child is. Two years today and he's already lived a lifetime of pain and trauma. Yet he is the happiest baby, blissfully unaware of what's in store for him in 5 short months.

Open heart surgery is not the sort of thing babies should have to anticipate, much less endure. Children should not have to face the idea of death before they've had the chance to fully embrace life.

But we know it happens every day. Sometimes unexpected illness takes a life. Sometimes the inexplicable act of a single human who has lost its grip on reality senselessly takes 20 little ones from their families just before Christmas.

It always seems like it will, but our pain does not stop the Earth's rotation. The sun does not go dark and the moon does not lose its luster. How can that be? These events do not define us, do not steal our ability to be happy, joyful people and to go on loving, even when it seems that it should.

Surgery Updates

Will & Matthew, pre-op

Hi All,

Aunt Jen here. Mimi is in Boston with Will, Nikki and Matthew, so I will plan to update this post as I receive news. All times EDT:

Friday, May 13
2:30: Matthew is in pre-op. Will and Nikki are still with him at this time
4:30: Matthew's surgery begins
5:45: Matthew is on bypass
8:00: Surgery went well. Surgeons are wrapping up (closing, etc.), then Matthew will be moved out of OR and will have chest x-ray and other tests necessary after the procedure. Probably will be another hour or so before Will and Nikki can visit with him.
11:00: According to Dr. Baird, Matthew's left ventricle was better developed than they anticipated. They proceeded with the Glenn operation, but said there is hope for a future biventricular repair, which is outstanding news.

Matthew is recovering.

Saturday, May 14
9:00am: Matthew is doing pretty well. He is still intubated this morning, but has started breathing over the vent. The weaning process has begun and hopefully he will be extubated by this evening.
6:00pm: Matthew has been extubated and is doing well. He woke up this afternoon for the first time since surgery and he apparently protested rather loudly. The team gave him some meds to calm him and he's resting comfortably now. Now that he's waking up I have a feeling he'll have an update for himself soon... :)

Matthew's Update From Boston

Hi Friends!

I don't think I've had a chance to talk to you all directly for a couple of months, but I've been spending a lot of time with Mimi and she told me she's been updating everyone. As you know, I'm back in Boston with Mom and Dad, getting ready for my second heart surgery, the first step of the Fontan Procedure. Below is an overview of the issue and the operation, for you really smart folks that like the details. I had the Norwood procedure back in December and now I'm going to have the Bidirectional Glenn procedure this time.

Do I have a story for you!

I was scheduled to have surgery on Monday, but after they made me go to sleep so they could keep me still and get a clear picture of my heart earlier this week, I woke up and have wanted to keep the oxygen ever since. For this reason Doctors Baird and Brietbart decided to move my surgery up to tomorrow.

I heard Dad saying something about Friday the 13th and Mom said that must mean really good luck for me! Plus Aunt Jen told me she and Uncle Ryan intentionally got married on Friday the 13th because they thought it was such a lucky day, so I think this is a great sign.

Dr. Baird told Dad today that I'm in really good shape for this next surgery and they are really excited for a good outcome. Dad told me it's because I've been doing my arm and leg aerobics so much and getting my heart ready!

My Grandpa Hall was planning to be here with us, but Mom's grandma passed away yesterday so he has to be back home with the family. I just found out Mimi has decided to hop on a plane and join us up here for all the festivities. She'll be here tonight and she's going to stay at the hospital with me. It will be just like back home when I get to spend the night with Mimi on the nights Mom and Dad are both working at the hospital!

I think I've told you guys most of the important stuff. You can read more about what the doctors are doing to my heart and why in the information below. Thanks for all your love, thoughts and prayers. I know Aunt Jen and Mimi will keep you updated and I'll write again as soon as I can! XOXOXO!!

Matthew

Children with hypoplastic left heart syndrome have a single effective ventricle supplying blood to the lungs and the body (either from birth or after an initial surgery e.g. Norwood procedure). They are delicately balanced between inadequate blood supply to the lungs (causing cyanosis) and oversupply to the lungs (causing heart failure). In addition, the single ventricle is doing nearly twice the expected amount of work (because it has to pump blood for both lungs and body).

The Fontan is usually done as a two staged repair.
The first stage, also called a Bidirectional Glenn procedure or Hemi-Fontan (see also Kawashima procedure), involves redirecting oxygen-poor blood from the top of the body to the lungs. That is, the pulmonary arteries are disconnected from their existing blood supply (e.g. a shunt created during a Norwood procedure, a patent ductus arteriosus, etc). The superior vena cava (SVC), which carries blood returning from the upper body, is disconnected from the heart and instead redirected into the pulmonary arteries. The inferior vena cava (IVC), which carries blood returning from the lower body, continues to connect to the heart.
At this point, patients are no longer in that delicate balance, and the single ventricle is doing much less work. They usually can grow adequately, and are less fragile. However, they still have marked hypoxia (because of the IVC blood that is not fed into the lungs to be oxygenated). Therefore most patients are referred for another surgery.
The second stage, also called Fontan completion, involves redirecting the blood from the IVC to the lungs as well. At this point, the oxygen-poor blood from upper and lower body flows through the lungs without being pumped (driven only by the pressure that builds up in the veins). This corrects the hypoxia, and leaves the single ventricle responsible only for supplying blood to the body. 

Fat and Happy at Mardi Gras!

Mimi pimped my crib for Mardi Gras

Hi Friends! I'm writing less often because... well who wants to keep hearing about how blissfully happy I am these days! I keep getting fatter and happier.

I was starting to worry about my weight and, since I know Mom would never tell me the truth about it (she thinks I'm perfect), I asked Mimi when I stayed with her last week. I asked her if she thought I should go on a diet. Mimi said this is the only time in my life when chubbiness will be "adorable" and I should really embrace it while I can.