It's a big zoo

Two weeks after a pretty hefty heart procedure, Luke hopped on a bus with the rest of the first graders and headed to Pt. Defiance Zoo for his end-of-the-year field trip.

I knew about this field trip from the beginning of school and off and on throughout the year, I caught myself wondering, "How is this going to work? Can he walk the entire zoo? What if he can't?" In all honesty, I didn't want to be a volunteer for this trip because my experience as a parent of a heart kid can be very different from others'. It's not as easy for me to just tag along, enjoying the moment. I get caught up in thinking and analyzing and overthinking and then analyzing from a different angle.

And all of that did happen, but you know what? There were also a lot of moments I did enjoy. A lot of moments where Luke was just one of the kids and except for the trek up about fifty steps back to the bus at the end of the trip, Luke made it. He walked the entire zoo with his buddies. I could tell at one point, during the long walk to the polar bears, he was petering out a little bit. I offered him a piggy back and he looked at me like I was nuts. "None of my other friends are getting carried!" Okay then.

So when he saw all those steps back up to the bus, he looked at me and said, "Okay. I'll have that piggy back now." Knows his limits, that one. The great thing was, no one even really noticed the piggy back and definitely no one said anything. It made my mama heart feel even better when I heard a couple of the girls behind us say, "When I get home I am going to do NOTHING but sit on my couch! My legs are tiiired!"

Part of this crazy decision making process to determine when Luke's next surgery should be is a continual check-in of the question, "How is Luke's quality of life? How much does his three chambered heart impair him?"

On a day like this:

Can you find Luke?

We can rest assured that this kid is living life. And in the areas where his one ventricle doesn't allow him as much stamina as his friends, we have seen over and over his capability to adapt and compensate really, really well.

All of Luke's data, from his recent cath and MRI, his latest echo, clinical notes and surgical notes, has all been sent over to CHOP by our cardiologist. I am anxiously waiting to hear their thoughts on our boy. I can't imagine anything they say or see will cause us to move forward with the Fontan this summer, but I guess one thing you learn in this journey is that the unexpected should be expected.

While we wait, we will enjoy two and half months of SUMMER. School let out last Thursday and we have days of swimming at the lake, hanging out at daddy's camp, S'mores, neighborhood water fights and theater camp to look forward to.

I was definitely the sad one to see school end. I realize how ridiculously spoiled I was this year to have such a dear family friend teach Luke. It was a year where I never once had to worry about how well Luke was being cared for. And he knew it, too. He blossomed this year, as a student and as a leader in the classroom. The foundation Tam laid for him, a foundation of confidence, is priceless to this mama. He loves school and loves being at Dad's school. Best decision we ever made.

Second grade, here he comes!

Cath results

It's a day like Tuesday, a day where prayer was so specifically answered, that cements for me this: The God of the Bible is a God that cares about the details of my life.

... He keeps a record of my tears {Psalm 56:6}.

... He numbers every hair on my head {Luke 12:7}

... He goes before me and he follows me {Psalm 139:5}

I know I should just jump right to the details of the cath, but I couldn't not share my heart too. God was good to us on Tuesday. He is good today and He is still good if Tuesday's news would have been different.

Tuesday will be a day Roger and I can point each other to — and hopefully point others to — when doubts and fears try to steal what we know to be true. It's so simple, but it changes everything: God cares!

We have spent the last several months praying that God would be clear as we move through these medical tests with Luke ... that HE would help us make good decisions and that HE would direct our paths.

The results of Tuesday's catheterization is one more piece confirming our peace to not move forward with Luke's next open-heart surgery right now.

Ready for some answered prayer?

• Luke's cold was completely gone by Monday.
• There was so little anxiety (for Luke I should clarify) leading up to the big day. He actually said on Sunday, "I wish it were Monday because then I would be closer to my cath." Granted, his logic included his post-cath pet, but still. What a huge blessing to this mama's heart.
• Luke was safe and protected in the cath lab. Anesthesia went beautifully with the amazing Dr. Lord and Dr. Belotti got easy access through the femoral artery and jugular vein.
• The numbers were AMAZING! His Glenn pressures were 11-12 which makes him an excellent candidate for the Fontan when we decide to move forward. His filling pressure was beautiful, the squeeze and relax of his heart measured healthy. His pulmonary arteries are a nice, big size and aortic arch gradient was 10, meaning no need to balloon or stent Luke's coarctation.
• Dr. Belotti did find a pretty big collateral vessel coming off his aorta and twisting and turning a while until dumping back into his pulmonary arteries (basically a totally useless circuit). He placed two coils in the vessel, which blocks all blood flow. This went well and his late afternoon chest x-ray showed the placement to be just where Dr. B. expected.
•  NO OTHER SIGNIFICANT COLLATERALS. If Luke had been developing AVMs (arteriovenous malformations), this would most likely move us forward to the Fontan. This is actually pretty astounding and unexpected that Luke has not developed these. He had a few tiny spidery collaterals coming off his aorta, but nothing needing intervention.
• So, his recovery. A lot of prayer was said for these four to six hours post-cath. I had one friend text me that she was praying that Luke would come out of anesthesia completely differently than he ever had. It was so clearly God because our experience on Tuesday was so radically different. For the first time ever, Luke slept for two hours after his procedure. He slept through that cranky, irrational, agitated stage. He was able to nod or shake his head and take a sip of apple juice, but then he would go right back to a deep sleep. About two hours into his recovery, he started opening his eyes and asking for apple juice. No thrashing, no crying, no us having to hold his legs down to protect his groin site. It's been a few days and I am still a little stunned with how calm he was. Luke's first grade teacher (and our dear, dear friend) came by to bring us snacks and treats and Luke two new DVDs since we planned on staying the night. He loved having her there, talking about school friends and reading group and the upcoming Flag Day concert (BIG deal around these parts ... Luke is the Bald Eagle in the play). Before we knew it, he was able to raise his bed a little more and he ordered a lunch of chicken nuggets. He played a little on the iPad and we laughed through some Mad Libs.
• After Dr. Belotti saw his chest X-ray, he told us he was comfortable letting us go home if we were. I'm pretty sure our anesthesiologist championed this idea for us. This guy is awesome. He even called later that night to check on Luke and to tell us again that he was proud of us for seeking second opinions in Luke's case. So after a second dose of IV antibiotics and the removal of all the leads, wound tape, and IV, we were on our way home by 6pm! Obviously, we would have survived spending one night in the hospital, but to not have to was a huge gift. I'll take my bed any day.

It's Sunday morning now and Luke says he's 98% back to normal :) I'd say. He played outside with the neighbor kids from noon until bedtime last night, coming in only for pizza. He's back to school and except for a little post-cath heartburn, his recovery was as smooth as can be.

We are beyond thankful. Thank you for praying for us, for checking in on us, for your texts and well-wishes. Beyond thankful.

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.