MRI ... check; stress test ... canceled; catheterization ... SOON

We were supposed to have Luke's exercise test on Monday but he decided to not only get a cold a week before (which would totally skew the results since being sick impacts his exercise tolerance), but also to start throwing up Saturday night. I'm okay with putting that test on hold. We have his MRI results and after his catheterization on May 27, we can send all of that information across the country to the Children's Hospital of Philadelphia. We are anxious to hear what they have to say.

This has been such an interesting process the past few months, really diving in and figuring out if continuing to delay Luke's Fontan is the right thing to do.What's become clear is that the answer, depending on who you talk to, is not at all clear. That said, I do feel like each baby step we've taken in this process has confirmed that for Luke, waiting is where we're supposed to be. And we definitely feel peaceful about taking a summer 2014 Fontan off the table.

I'll back up a little to a little website called Facebook. I'll be the first to say our Facebook culture has it's flaws, but one thing it does really well is connect a group of people (in my case, heart moms) who would be much less powerful on their own. There is great power in sharing knowledge, sharing tears (happy and sad), and building on each other's passion. I don't know all the specifics, but after one family's local hospital refused to operate on a little boy with Hypoplastic Left Heart Syndrome, and after the family's insurance refused to pay for treatment at a hospital that WOULD attempt an operation, this family didn't give up. The mom turned to social media for help and within days, Medicaid approved a life flight to Boston Children's Hospital and the little boy now has a chance to live. That is powerful.

My Facebook story isn't quite as dramatic, but for my family, still profound. It was through a Facebook connection that I got to talk on the phone with Dr. McConnell, from the Sibley Heart Center in Atlanta. Like Dr. Stefanelli, he feels like delaying the Fontan — for the right cases — is the right path. He believes that every year he can buy these kids is a year closer to a better solution. This is a hotly debated topic among pediatric cardiologists and I don't want this post to be about one position being right and the other wrong, but Dr. McConnell's rationale sits well with me, for Luke. We are talking a major (MAJOR!) open-heart surgery for a young child. We are talking about a new physiology, while certainly beneficial in some ways, also starts a ticking clock on the health of that child's liver. Not to mention other potential nasty conditions that only happen with the new Fontan physiology. When you hold those factors in one hand and see Luke running off to recess with his first grade buddies in the other, waiting just makes sense for our family.

But here's where we want to be smart. We don't want to delay this third stage surgery just because WE want to delay this third stage surgery. We want to be smart and do our due diligence in this process.

Luke, happy as a little clam with the help of a little Versed pre-MRI.

Which is where all this testing comes in. Luke had his cardiac MRI back in late March and we got some good information from that, confirming Luke's excellent heart function (his ejection fraction is within "NORMAL" range. As in, normal normal. Wow.) They were able to get good measurements and map his vascular system. They did see what is most likely a collateral vessel on his right side which is the main reason we are moving forward with also doing a catheterization. In the cath lab, they can test and see if Luke is actually using that vessel (if it is "diffuse") and if so, they will coil that inefficient vessel. The metal coil in the vessel causes a blood clot to form and close the vessel. Over time, tissue grows around the coil, forming a permanent seal.

I know, right?

So we'll be heading to the cath lab this Tuesday morning to see what we can see. At the minimum, they will place a catheter both through his femoral artery (to access his left ventricle) and also the internal jugular vein in his neck to test his pulmonary vascular resistance. This data determines whether Luke is a good candidate for the Fontan. They will also measure the pressures in his ventricle.

If all they decide to do is collect data, Luke should be out in recovery within an hour or hour and a half, and we will get to go home after his 4 hour recovery (they need the kids to lie as still as possible for 4 hours to prevent clotting issues in the access sites. Yuck. Borrowing an iPad for this portion of the day.)

If they decide to coil any collaterals (there could be multiple, we just don't know), we will need to stay overnight and Luke will receive three mega doses of IV antibiotics before discharge. They will also do a chest x-ray before discharge to make sure the coil is where it should be.

Finally, Dr. B. will measure the gradient pressures above and below Luke's narrowing in his aorta. Luke had this narrowing ballooned back in 2010 and it has stayed pretty open since. If there is moderate narrowing, Dr. B. will do another ballooning. If it is significantly narrowed (very unlikely), he will place a stent to hold it open.

So a lot of possible outcomes on Tuesday. We are prayerful and hopeful that no interventions will be needed and we will all sleep in our own beds that night.

Thankfully, with the anticipation of getting a post-cath pet turtle (the things you do, right?!), Luke's spirits have been really good going into next week. I am so thankful for that. He doesn't know he may have to spend the night yet, since we don't want him stewing on something that may not even happen.

If you'd like to pray for our family, here are some specific requests:

1.  Luke's nose is a little stuffy right now. We need wisdom on whether or not to postpone in the next couple of days. Dr. B. is comfortable moving forward with an upper respiratory bug, but since this is an elective procedure, we would love it if Luke were 100% healthy. We don't want any unnecessary risks!
2. Please pray for the skill and steadiness of Dr. B.'s hands. This is a fairly straight-forward procedure, but goodness, it is a catheter entering Luke's heart! Please pray that Dr. B. would have clarity on what, if any, interventions are needed.
3. Pray for mom and dad's anxiety to be calmed as the long weekend is ahead of us. It helps so much to have Luke not too stressed about the whole thing. For my little worry-wart, this is a huge blessing.

Phew. That's a lot to digest. Thank you for being a part of Luke's "team". He is one lucky boy.

It's really not my job, as much as I think I want it

It's become clear to me that jumping rope is pretty popular in the first grade. It's happening at recess, in P.E., and a jump rope club is starting up on Thursday mornings.

Last weekend, my son, never one to be left out of social goings-on, decided we needed to buy a small rope so he could practice at home. My husband, ever the willing, found a short rope in our garage and spent 20 or 30 minutes with Luke, showing him the ropes (yes, intended "pun"ishment).

I adore my son's determination. It can be such a thorn in our sides sometimes, this strong will and opinion, but in reflection, it has served him so well. This kid who has to battle a bit more than most, to fight against his physiology, he continues to amaze us in his tenacity to keep up with his friends and do what he decides he wants to do.

Where was I during this half an hour? Not in the same room. I had to admit to myself (and now to blogland) that it is hard for me to watch Luke battle physically. To see his blue fingertips, his breathing heavier than the norm, to watch him battle what doesn't come easily to him.

Many, many heart kids have to battle gross or fine motor delays simply because of the amount of time spent in a hospital crib. I have read that one day in the hospital equals three developmental days. In that equation, Luke has lost 198 development days, over half a year. We noticed it in the fact that he didn't crawl until 10 months, or walk until 17 months. He didn't walk up the stairs one foot per step until he was five.

Now, I am not bemoaning our experience. Luke has done exceptionally well and never even qualified for OT or PT. I am thankful his body has adapted to his heart defect as well as it has.

But, he does have to work a little harder to learn large motor skills. Pedaling, climbing, monkey bars, skipping, and now jumping rope.

He worked hard last Saturday. He can get one rotation, sometimes two rotations in a row. Roger pumped up his tenacity and desire, even if the skill isn't quite there yet.

After their practice session, I asked Rog how it went. He said something that will hopefully change my attitude for a long, long time.

I shared with him my little pity party I was throwing, telling him that it's hard for me to watch Luke battle and that I wish he didn't have to.

You know what he said? "Jess, in my mind, the fact that he has to battle is such a good thing. I'm thankful he has to battle."

Well.

That is one way to look at it.

I wish that was my natural bent. But a friend reminded me, "Jesse, you are his mom. It's natural for you to want to rescue him."

She nailed it. I want to rescue my son. From discomfort. From embarrassment. From discouragement. And I can't.

And if I did all I could to rescue him, I wouldn't be a very good mom. When it comes right down to it, that's not my job. I don't want Luke or Laney to grow up looking to me for rescuing.

I want them on their knees, looking up.

I do think it's natural for us to resist trials. Naturally, we all want those sweet seasons where we're not being tested, when life is smooth sailing. But if I say I want my children (and myself!) to be dependent on a trustworthy, ever-present, always-good God, then I need to let go of trying to be their savior. Only One can save. And I do not want to stand in the way of His work.

Step by baby step

Okay.

We had a cardiology appointment a couple of weeks ago and subsequently a few more are now penciled in on the calendar.

His appointment was on a Wednesday, and later that evening we had a few minutes until we had to leave for Woodland's Math & Science night. Luke really wanted to go, but I'm still not convinced it was math and science drawing him. I'm thinking the free pizza and chance to hang out with buddies was his motivation. In those short minutes, I laid down on the couch with Laney, feeling just wiped. I am always surprised (you'd think I'd have learned by now) with how much cardiology appointments take out of me. The countdown leading up, the restless sleep the night before, the anxiety during each test, the emotional let down after (even if the news isn't bad). I honestly felt like I should be presented with mom-of-the-year award when I dragged myself off the couch and out into the pouring rain to take Luke to school. I was wiped.

But the news was good and the conversation continues fruitfully on. I feel like we have some next steps in place to help us make this ever-looming decision of when (if?) the Fontan should happen. It is a recurring battle in my brain, this weighing of risk to benefit of this next surgery. I am prayerful and confident that God will lead us to make the best decision for Luke.

That said, we are scheduling a few tests in what I'm calling this "research-gathering" season we're living in.

Although a little on the young side, Dr. Kim would like to try and run a stress test on Luke. This could tell us more accurately how well his body is tolerating exercise, his VO2 output, how low his sats get when exercising, and how fast he recovers.

The second test happened this morning. We had an ultrasound to look more closely at Luke's diaphragm function. Most likely during his first surgery at five weeks old, his phrenic nerve was nicked, paralyzing his left diaphragm. The phrenic nerve originates in the neck and passes down between the lung and heart to reach the diaphragm. There are two, a right and a left, and Luke's left was damaged. When we discovered the paralysis, we were at Seattle Children's, post-Glenn, and Luke was having trouble getting his sats up into the 80's where they expected them. He was running low to mid-70's. They could have left the paralyzed diaphragm alone, but because of the damage, that diaphragm was actually working paradoxically, or opposite of how it should have been. So instead of contracting during inhalation, it was expanding and vice versa. This was negatively affecting Luke's lung capacity and oxygenation.

Because a non-functioning diaphragm is not ideal for Luke's next surgery, the Fontan, Dr. Kim wanted to see what that left side was up to, if any movement had been recovered.

The radiologist couldn't tell us a whole lot, but there was definite movement happening today. Not as strong as the right side, but movement nonetheless. We'll see how Dr. Kim interprets this info, and what this could mean for Luke, if anything. I'm pretty sure they don't do plication reversals, but maybe they do? Or maybe a kind-of functioning diaphragm is better than nothing?

Thirdly, we are doing a cardiac MRI to get a deeper understanding of Luke's heart anatomy and function. An MRI is a non-invasive way to get more information than an echocardiogram can give. The MRI will create three-dimensional images of complex heart defects and measure how well the heart is pumping blood. The only bad part will be the anesthesia. This, along with the results of the other tests, his latest echo, and his surgical notes will all be sent to the Children's Hospital of Philadelphia. We are hopeful they will weigh in on Luke's unique case and give us good wisdom in our decision process.

Writing all of this post makes me realize how thankful I am that we have so many doctors who care about not just Luke, but all medically-fragile kids. When I talked to a cardiac nurse at CHoP, I was blown away by her receptivity to looking at Luke's case and her compassion for a family she's never met, living across the country.

And how about this for a date on our calendar? MAUI for Spring Break!! We got to go two Christmas's ago, but this little lady is itching to get back there. And huge blessing, we'll get to see our beloved Dr. Stefanelli while we're there. Poor guy has to do bi-monthly outreach clinics on Maui, so he scheduled his for the week we're there. CANNOT wait. We feel so blessed that he's stayed a part of Luke's care and we can't wait to see him and for him to see Luke in person.

Now we just need to get Laney to practice wearing a swimsuit, since I'm pretty sure my loves-to-be-nakey and hates-anything-restrictive girl won't be allowed on the beaches of Maui sans clothes.

Theoretics

The Fontan conversation picked up again these past couple of weeks. Between our old cardiologist and us; between us as Luke's mom and dad; between us and God.

The last we talked with Dr. S, back in September after Luke's cardiology appointment, the "plan" was just to continue to hold off on this third stage surgery indefinitely. To continue to watch and wait for Luke to determine the timing. It was agreed that the risks didn't outweigh the benefits at this time.

There are benefits to finishing the Fontan circulation. No pediatric cardiologist would argue with the potential benefits. You are taking volume load off the heart, you are improving the child's cyanosis, often giving the patient near-normal oxygen saturations, and in most cases you are increasing the child's exercise tolerance. No one would argue with the fact that there are risks to this new circulation as well: The Fontan could fail, a patient could develop long-term complications such as Protein-Losing Enteropy (a nasty, nasty condition) or heart arrythmias, and most talked about right now, the potential damage to the liver with this new circulation. There are single ventricle patients in their 20's and 30's that have such severe cirrhosis of their liver that they require a heart and liver transplant.

So here's our impossible situation: The theoretical benefits to Luke, if all goes well, would be great. I'd love to see his exercise tolerance improve, and would love to not have to worry so much about his sats getting too low, especially when he's sick. The theoretical risks to Luke, if things don't go well and his body doesn't like his new circulation, are really really scary to a mama's heart.

That's a lot of theoretics. Dr. S. will be the first one to tell you: There is no easy answer. Luke's case is so tricky in the fact that he is seven, that his sats have stayed in the mid-80's, and his heart function and energy have stayed really good.

But you want to know what's not theoretical?

God's great love and good plan for our son.

In as much as we've been going around and around on this topic the last three weeks (which is good, by the way, and a necessary part of this process), we've had to stop and remind ourselves what God says, "If you need wisdom — (yes! over here!) — ask our generous God, and he will give it to you. He will not rebuke you for asking." —James 1:5

Or how about this promise? "Ask and it will be given to you ..."

God is not about making our path muddy, or our decision impossible. And He definitely doesn't want us to make this decision in our own limited knowledge. Yes, he has given us smart people to weigh in, and we're in the process of adding another smart brain from the Children's Hospital of Philadelphia to help us make this decision. But even Dr. Rychik's vast knowledge of all things Fontan is nothing compared to the wisdom of God. Nothing.

That is what we cling to as we walk forward through this situation that feels like has no right answer. It's scary because we want the best for Luke, but I tell myself a million times a day that God does too. Even more than me. Because His best is better than my best, any day of the week.

Another biggie conversation

Thursdays are the craziest day of the week for our family. At the same time Luke and Roger are heading off to school, I take Laney down to her sitter's house so I can go into the World Vision office for the day. After school, my friend Erica picks up Luke and he hangs out with his buddy Marcus until the hubby picks him up around 4:45. At the same time, I'm picking up Laney and then finally we all converge at home for the night. Phew.

As soon as Laney and I walked in the door a few Thursdays ago, Rog told me Luke got a pass to spend his second recess in dad's classroom. The story unfolded that Luke told the recess duty that he just couldn't keep up with his friends today while they were all playing football. Mr. Chris, the duty, asked him if he wanted to go hang out with his dad for the remainder of the recess.

It was time for another sit-down conversation with Luke about his miracle heart.

The conversation went a little like this:

Luke: "I don't understand why I can't run as fast as my friends when we play football."

Dad: "Do you remember when we talked about how God made your heart? And how it's different than a lot of other kids' hearts?"

Mom: "Do you remember what a heart looks like? How it has four different sections that have different jobs?"

Luke, blowing us away: "Yeah. I know that I have this one (pointing to top right) and this one (pointing to top left) but I think I'm missing this one down here."

Mom: "Exactly. Those sections are called chambers. Most people have four chambers in their heart but you only have three. You are missing your right bottom chamber."

Luke: "But why does that make me not run as fast?"

We explained a little about oxygen levels, how your muscles need a lot of oxygen to move, and how his one ventricle has to work harder to pump because it's doing two jobs instead of one.

Dad: "Do you realize, Luke, that the fact that you are playing football at all at recess is a big deal? Your heart is superman strong for only having three chambers. Do you feel like you aren't keeping up during other times, like playing with your friends at home, or P.E.?"

This is where you can relax knowing his confidence is intact ...

Luke: "Oh, I totally keep up in P.E."

We explained that playing football at recess is harder because the field is so big and that you have to run a lot farther when you play outside. We told him he could find a different activity at recess, but he didn't love that idea since "all his friends play football". So we told him he'll just have to figure out a way he can play even if he's feeling like he's not running as fast or far. Or if he's not getting the ball. But ultimately, he's going to have to decide. As much as we'd like to, as much as we'd do anything we could to, we can't fix this problem for him and we definitely can't make it go away.

He asked, "Is it okay that I told the duty?"

Dad: "Yep. Mr. Chris already knows about your heart. All the adults at your school know about you."

"All of them? Does Miss Culver know? Mrs. Brumbaugh? Mrs. Miller?"

Dad: "Yep, they all know because when you were born and had your surgeries, Daddy had to take a lot of time off and so they knew about your heart. A lot of them even prayed for you."

Luke: "Do my friends know?"

Mom: "Not unless you tell them. That's going to be up to you."

Mom: "Do you know what jealousy means, Luke?"

Luke: "Yeah, like when you are sad because your friend has something and you want it too."

Mom: "There might be times when you feel a little jealous of your friends because they can run faster, but your job is to remember that God gave each of us different gifts. Not everyone can read as well as you, so maybe they feel a little jealous about that."

Dad: "I have three boys in my class, that just today, they struggled with an art project. Now they can catch a football really well, but art is really hard for them. You might feel bummed from time to time that your heart is different, but faith is trusting that God gave you this heart because he's got a big plan for you. You have to remember how many people have been blessed by your life already. And you're six!"

Luke: "Did Miss Culver come to the hospital to visit me? Mom, can I have more pizza?"

And that was pretty much the extent of the conversation. It was really good. I thank the Lord that He gave Luke a confident spirit so that he can see how many gifts he has been given. I pray that stays with him. I also pray so hard that he would never get to a point where discouragement causes him to stop trying.

Since then, we haven't heard any more rumblings about being discouraged at recess, and just yesterday, he came home filthy because he was playing football. So we keep pressing on, conversing with him as he leads.

Part of the above conversation also included talk about how karate might be a really good sport for Luke since it's not as much running as football. Luke has been begging to take karate for months, and this Tuesday was his first class:

I think he got a kick out of it.

So sorry :)

I hope this ends up being a good fit for him. He's a little bummed he's not playing basketball this year like he has the last two years. Maybe after the his next surgery we can revisit basketball, I don't know. For now, he's excited about karate so we'll see where that takes us. And we'll wait to see the unfolding of God's plan for our sweet boy.