We left Friday morning at 7 and headed to MUSC for Derrick's pre-op work-up. We learned some things we weren't so excited about today, got an in-depth course of "Derrick's Complex Little Heart 101," and somebody hit a major milestone today! For his big news, you have to wait until the end of this insanely long post (but it will be jam-packed with interesting info...to us at least).
We were at the hospital from nine to FIVE! Needless to say, we're both mentally and physically exhausted, but Derrick was such a big boy. He slept the whole way down and the whole way home, and he only had about a 20 minute nap the entire day. He was a happy guy for the most part...minus the blood draw of course. He was so good, in fact, that his daddy promised him that we'd stop to get him ice cream on the way home.
He liked it!
We started out the day by having a chest x-ray.
He was a little nervous
We thought these were just too cool not to share...
A side view of D's chest
D's chest from the front. Remember, he has Dextrocardia, so the apex (the point) of his heart points down to the right, instead of the left.
Those little safety-pin-looking-things are the wires that were put in to hold D's chest together after surgery. That's NOT what I thought they looked like. I pictured more of a shoelace type thing, especially because of the way his chest feels. Apparently I was wrong.
In anticipation of the echo.
After that he had an EKG, which he thought was fun because he enjoyed trying to pull off all of the stickers they put on his chest. Finally, he had to have his blood drawn for various tests and for a blood transfusion match-up for surgery. He tried hard to be a big boy, but this one hurt! Sorry, no pictures...I prefer NOT to have a picture of me holding my child down while someone is sticking him with a needle! Throughout all of these procedures we were visited by anesthesia, a cardiology fellow from the PCICU, one of the cardiologists, Dr. Hsia (D's surgeon), his PA (who will give us updates during surgery), AND the doctor who did his cath a few weeks ago who gave us the Holter Monitor report. Lots of information, let me tell you!
As far as the Holter monitor goes...While they were monitoring Derrick during his cath a few weeks ago, they noticed that he was having some periods of tachycardia (rapid heart beats). They sent him home with a Holter monitor that he had to wear for 24 hours so they could get a picture of what his heart was doing throughout the day. When looking at the report, he did have a few bouts of tachycardia, but they weren't extremely fast, and his body appears to handle them without a problem. Before being sent home after surgery an electrophysiologist (doctor who studies heart rates and rhythms) will come in and do a test on Derrick's heart to see how easily it goes into tachycardia. Depending on the results of that test, he may or may not be put on medicine to control these rapid beats.
We had to wait a while on Dr. Hsia because he was in a really long surgery all day.
At first we felt a little overwhelmed being stuck waiting for so long,
but we made the best of our time.
Dr. Hsia (pronounced like Asia without the A. Just Sha...just in case you were wondering) was excited to see how well Derrick was doing. He played with him and went on and on about how cute he was. While he talked to us, Derrick listened intently so he could understand exactly what was going on with his heart. Seriously, he was pretty active while we talked to everyone else, but when Dr. Hsia came, he sat on my lap just as still and quiet as he could be. We have such a good boy!
Dr. Hsia said he was much more comfortable with Derrick's pressures in both branches of his pulmonary artery and his heart now than he was after the first cath. While Dr. Hsia was explaining the whole pressure thing, he opened D's chart to show us the difference in the numbers between the first and second caths. In there was a little treasure....a (drawn) picture of D's heart! Whoop-de-do, right?! No. What you may not understand is that Derrick's heart is so unique you have to put many different pictures together to get an idea of what's going on in there. While he was in the PCICU after birth the nurses would have the doctors attempt to draw D's heart because it's so difficult to picture and understand.
Anyway, I couldn't help but ask for a copy of the picture, and Dr. Hsia was happy to give me one! Seriously, you have no idea how exciting this is to me. We re-drew it because all of the picture was not accurately Derrick's heart, they just changed enough of it to mark all of the pressures.
**Disclaimer: This is our interpretation of Derrick's heart to the best of our understanding. It is by no means completely accurate and should not be taken as medical knowledge in any shape or form. It is simply posted here for our future reference and your curiosity.
Normal heart v. D's heart for quick comparison.
Derrick's heart right now (pre-Bidirectional Glenn)
Derrick is missing his left ventricle (the pumping chamber that normally pumps oxygenated blood to the body). He has an extremely small main pulmonary artery, which normally carries unoxygenated blood from the heart to the lungs, but it is too small to do it's job. If you look at the normal heart compared to the D heart, you can see how large it should be. He has a small ASD, which is a hole between the two atria. Normally this is a problem, but for him, it is a necessity because it's the only way the oxygenated blood can communicate with his pumping chamber (ventricle).
During his first surgery (the BT Shunt), his main pulmonary artery was disconnected so it wouldn't allow any blood to go to his lungs, and the shunt would take over it's job. By disconnecting it they could place a more accurately sized shunt because they knew exactly how much blood would be flowing to the lungs. They really weren't sure how much blood could make it through his tiny pulmonary artery, but it wouldn't have been much. The shunt has been working like it's supposed to, but because of some of the unequal pressures, it has caused some stenosis, or narrowing, of one of the branches of the pulmonary artery. We're not completely clear on which side because of the positioning of D's heart.
Right now, Derrick's single ventricle is doing the job of two ventricles. (In the picture of D's heart, you see a lot of purple blood. This is mixed blood, meaning it's not completely oxygenated or unoxygenated. This is why his sats are low, and will not be significantly better until after his third surgery, the Fontan.) Anyway, D's ventricle pumps mixed blood (should be oxygenated) up through the aorta, which sends oxygen-rich blood to the body. Because he doesn't have another ventricle to pump blood to his lungs, his shunt steals some of the blood as it passes through the aorta, and sends it to his lungs to be oxygenated.
Because Derrick has (finally) outgrown his shunt, his body isn't getting enough oxygen and it's time for the Bidirectional Glenn (BDG) surgery. This surgery will alleviate some of the work of the single ventricle so that his heart doesn't have to work so hard. It was described to us that his heart has to work like he's exercising all of the time. That's a lot of work to be done all the time.
During Derrick's surgery Monday, several things will happen. They're numbered, but not necessarily in the order they will be done. I just thought they'd be easier to explain with numbers.
1. Dr. Hsia will enlarge D's ASD. Right now it is large enough for the needed amount of blood to get from his left atrium to his right atrium, but as he grows, it will eventually be too small. This would create higher pressure in his heart which would NOT be a good thing.
2. The extremely small main pulmonary artery will be removed because it is not serving a purpose.
3. Because the shunt and the unequal pressures caused stenosis in one of the branches of the pulmonary artery, it will be stretched. If it can't be stretched enough, it will be cut and a bovine (cow) patch will be placed to allow it to be as large as his heart needs it to be to effectively deliver blood to his lungs.
4. The BT Shunt will be removed.
5. The superior vena cava (SVC) will be disconnected from the right atrium and reconnected directly to the pulmonary artery. The SVC carries blood from the upper part of the body to the heart for it to be sent to the lungs to be oxygenated. Since the surgery will move the SVC to the pulmonary artery, the upper body blood will drain directly to the lungs by gravity, it won't be pumped there. This will take some of the work away from D's heart. I'm sure this isn't really done last, but like I said, the numbers are simply for explanation. I have no idea the order in which this will be completed.
So, what's next? Sometime within the next four years, Derrick will need his third surgery, the Fontan. This is our version of the anticipated Fontan. Of course there could be other things that have to happen, but we hope it's the cut and dry Fontan. We'll see.
During the Fontan, the inferior vena cava (IVC) will be disconnected from the right atrium and connected directly to the pulmonary artery, just like the SVC will be done in the BDG. The IVC brings the blood from the lower body to the heart to go to the lungs to be oxygenated. After the Fontan, the IVC will deliver the unoxygenated blood from the lower body directly to the lungs without being pumped, but by pressure driven from the veins. At this time, Derrick's heart should have only red and blue blood...no purple (mixed) blood. His sats should be in the 90's, but will most likely not ever be at 98-100% like "normal." After the Fontan, Derrick will functionally be working with half of a heart.
I mentioned earlier that we had some not-so-exciting news. That was the ASD (hole between the atria) thing. Really, we didn't know anything about it, although it only makes since that he has to have one for his blood to flow. When the ASD is enlarged, Dr. Hsia will have to cut into his heart. Because of this, Derrick will be on bypass. We knew he would be on bypass for this surgery (he was able to avoid it for the first surgery), but for some reason I was thinking that every time someone was put on bypass that their heart was stopped. I guess this shows my true lack of knowledge for all of this heart stuff, but apparently sometimes you're put on "beating heart" bypass where the heart still beats, but doesn't pump blood through the body. That's taken care of by the machine.
To allow Dr. Hsia to go in and cut out some of the wall between the atria and make that hole bigger, Derrick's heart will have to actually stop. While this freaked me out before, now that I know hearts are not always stopped because of bypass, it freaks me out even MORE. It really does terrify me to know that Derrick will be in the OR with his heart stopped. Of course this being done on top of the Glenn brings added risk. However, if it is not done now, it will be an additional surgery. Of course we don't want Derrick to have an extra surgery if he doesn't have to, so Dr. Hsia will take care of it while he's in there.
We do have some good news from Friday to share. It's really bittersweet to me, but...
Derrick pulled up all by himself for the first time!
How exciting to hit such an important milestone at the hospital, right?! I was really hoping that he'd pull up before surgery. It's sad to me, though, because after surgery he won't be allowed to do any moving around that places pressure on his chest. No crawling, pulling up, nothing. I just know that if we had about three more weeks this kid would be walking. I know he would. Now he'll have to wait a lot longer because he can't practice and I'm sure it will take a while for him to get his strength back. That's ok...I'm not really ready for him to walk, he just wants to so badly. I'm not sure how we're really supposed to keep him down though...this kid is crazy active, and I'm not sure a sore chest is going to keep him down for six weeks! I have a video of him pulling up that I will post soon. I really don't feel like waiting for it to upload...it's 4:30am and I should probably go to bed.
We have to be at the hospital Monday morning at 5:30. Anesthesia will come take Derrick around 6:30, and the actual surgery should start around 7:30. It's anticipated to take 6-8 hours once it actually starts. Because Derrick has had heart surgery before, he has a build up of scar tissue that has to be cut through. As they cut through the scar tissue, they have to be extra careful because sometimes the heart will stick to the scar tissue, and they have to be careful not to cut the heart. Another problem with having had surgery before is that the scar tissue makes it impossible for the surgeon to see the nerves that control the diaphragm. Dr. Hsia will be working at a spot very close to where these nerves are located, and not being able to see them, there is a possibility of them getting injured. If this happens, Derrick's diaphragm could become paralyzed. We wouldn't know if this were the case or not until they try to take him off the ventilator. If he can't come off, then most likely the nerves have been damaged and he will have to have another surgery to tack down the paralyzed diaphragm. Before D left the hospital the first time they thought part of his diaphragm was paralyzed, but it appears to have healed itself. I know it's not uncommon, but I really hope it doesn't happen.
Sometimes they shouldn't tell you so much.
Monday is almost here. The day we've been waiting for for almost six months, yet the day of dread. I know that Derrick is in the best place for his surgery and care afterward, and I know that God will be holding Derrick with one hand while He guides Dr. Hsia and his team with His other hand. Please, please pray for a successful surgery and recovery for Derrick.
Thank you for all of your comments, emails, phone calls, visits, goodies, and PRAYERS. While D was having his last surgery it was feeling prayer from so many that kept us calm, and reading your comments and emails that kept us busy. We couldn't thank you enough for your support.
We love you all, and thank you from the bottom of our hearts for loving our little man.
Derrick, and the man who is going to save his life for the second time, Dr. Hsia.
P.S. We've had several requests about sending donations for the CHD research breakfast. I'm sorry it's taken me so long to get this up, but if you can't come to the breakfast but still want to donate, you can send a check made payable to "MUSC Children's Hospital Fund" with "pediatric cardiology" in the notes field to Shannon Carter, 239 Hillsborough Rd, Columbia, SC 29212. We'll deliver your donation when we take the money from the breakfast down. If you would like to purchase tickets (or sell tickets, or help serve), email me at email@example.com. If you're in the area, PLEASE come join us. It'll be good...promise! Thanks for your support!!!