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.

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. 

Perspective

Hi Friends, many people have been worried about and praying for me for a long time. I know that what's wrong with my heart is very serious but I was thinking last night while looking out my window on the snowy Boston skyline. I'm just learning that, by comparison, maybe this is not so bad. Monday is the 3rd birthday of another little guy who is here from Spanish Fort, AL, next to my home town. He is here in a special room where they are giving him medicines and keeping his heart going until he can find a new heart. I hear he's first on the list so I think he has a really good shot.

Family Week!

Aunt Jen, teaching me how to blog

Hi Friends, thanks for all who have been asking about me. Sorry for not writing sooner, but I've been so busy this week with all my family who have been here to visit and entertain me! My goodness it's been such a fun and exciting week. I'll try to catch you up before I fall asleep again.

First, I'm starting to feel like a prince - there always seems to be someone in line to hold me, feed me, even change my diapers. The other day my Aunt Jen, Aunt Addie and Uncle Jamie all teamed up to change the mess of a diaper I had made just for them, right after Mom and Dad left the room. You should have seen the whole thing. I was laughing so hard at the three of them. Let me tell you, I decided to challenge them. As soon as that diaper came off I stuck both my feet into the mess down below. While Aunt Jen was cleaning my feet off, I decided to potty again, just to see how they'd react. Oh, it was a hoot watching the three of them in action!

All Smiles

Hi, again! Sorry we've been so quiet the past few days. Matthew is still doing well but won't go home this week like we thought he might. This week the doctors have been running different tests on Matthew's heart and lungs to see how they look post surgery.

With all of his family keeping close watch, Matthew shows us more and more of his personality each day. He is a good, happy baby who sleeps a lot and only cries in short bursts if he needs his diaper changed. He has started waking up and getting restless before feeding times and is regularly taking his full bottle. He's now up to 6.1 lbs and growing!

I Will Survive!

Check out Dad here!

Hi Friends, first I have to say thank you to the many, many of you who have been sending lots of love, energy and prayers for me and Mom and Dad the last few days. Your support has been so important and helpful for us during this time.

Wow, where to start! Well, two nights ago I knew something must be going on. Mom and Dad looked a little more concerned than normal. I thought maybe it was just because Dad was still worried about Mom. She got sick that morning and had to go to another hospital to get help. She still looked a little rough when she got here but I was just so happy to see her and Dad. She looks way better today, by the way.

Anyway, it turns out their concern wasn't about Mom but about me, which I realized when people in masks and colorful gowns came and wheeled me away from Mom and Dad. I was really scared at first, but then I saw them putting stuff in those plastic tubes and suddenly I was floating on clouds, seeing gumdrops and lollipops and didn't even realize I wasn't with Mom and Dad anymore.