The Bravest Little Boy I Know

Since he was old enough to notice it, my grandson has pointed to the “zipper” on his chest with pride. That’s what he calls the scar that runs the length of his sternum. He remembers nothing, of course, about his first open-heart surgery as a newborn; but he knows that beneath the zipper is where the doctors fixed his special heart. Like the scar, his understanding is only skin deep. He is too young to fully comprehend the intricacies of the special heart that beats behind the scar. In fact I’m not sure that I do. 

Every time I read about Hypoplastic Left Heart Syndrome (HLHS), I'm amazed by how the heart is designed to work in concert with the rest of the body, and I'm reminded of  Psalm 139:14: "I praise you because I am fearfully and wonderfully made. Marvelous are your works."  So the fact that HLHS surgeries basically reconfigure half-a-heart to function as if it were whole is, in some ways, equally marvelous.  

Like the psalmist, most of us tend to take the wonders of God’s creation for granted until, for reasons we may never understand, there is a deviation in its beauty. It may be an earthquake, a tornado, a tsunami, a flood, or a "funky heart" (as the HLHS community calls such conditions). It's then that the shared root of the words awful and awesome reflect both the fear and wonder of creation when something it is out of rhythm. I wrote of this years ago in the poem below: 

When it comes to the human body, it is deviations from the norm that explain how we are both “fearfully” and “wonderfully” made. Thanks to our autonomic nervous system, the vital systems of our bodies maintain pressures and temperatures and rhythms without our effort or care.  It is only when a deviation occurs (and the fear it may bring) that we are reminded of the wonder set in motion before we were even born.

In my grandson’s case the deviation is an incomplete heart. He has no left ventricle. This was discovered during an ultrasound months before he was born. That was the first time my daughter and son-in-law heard the term: Hypoplastic Left Heart Syndrome (HLHS). It is common enough to have a name yet rare enough that most people know nothing about it. 

That is why I’m writing this post as I pass the hours in a waiting room of a renown Children’s hospital in West Michigan. Writing is cathartic for me--even if no one reads my thoughts. 

I'm not alone here in the waiting room. In the chairs beside me are the three other grandparents of the bravest little boy I know. The four of us were also here together when he was born and a few days later when the first incision made “the zipper.”

We are also not alone in a different sense. Each year, more than 1,000 babies are born with HLHS in the United States (roughly 1 out of 4.000 births). This statistic does not include numerous other types of congenital heart defects.  Until a few decades ago, the series of operations that increase the odds of bringing HLHS babies home from the hospital did not exist. 

Someday my grandson will know all of this, but for now he knows basically two things: 

First of all, he knows he tires more quickly than his sister, cousins, and playmates at church and preschool. (This is because he has no left ventricle. The surgery makes it possible for his right ventricle to do double duty—pushing blood to his lungs for oxygenation AND THEN pushing that oxygenated blood from his right aorta to the rest of his body. Normally, the right ventricle pushes the blood to the lungs, and the larger, stronger left ventricle performs the big push to the extremities.

For five-and-a-half years, this brave little heart has worked twice as hard to bring the color to his cheeks and oxygen to his organs and muscles. Even so, "twice as hard" is not quite enough to sustain normal oxygen levels as the blood courses the long paths to his hands and feet where his nails are sometimes slightly blue. This is why—even on warm days—his little hand in mine is sometimes cold. It’s why when walking from a sandy beach back to the car, he smiles up and asks, “Papa, can you carry me?” True, my whole-hearted grandkids sometimes ask for such a lift, but with HLHS kids the earnest plea is because their half-a-heart is working twice as hard. . . every minute of every day. 

This is his “normal,” and for the most part, he lives each day like a normal little boy. Most people who meet him when his zipper is covered by a shirt, know only that he is a funny, imaginative, adorable kid who gets along especially well with his doting older sister. 

The other thing this brave little boy knows is that there is a reason his scar a "zipper": The opening and closing of this sternum incision is a recurring reality all HLHS face from birth to age four or five, and today it will be opened again. 

The surgery performed at birth is called the NORWOOD PROCEDURE. Norwood Procedure: Details, Recovery & Outlook The second “open heart” surgery takes place at four months. This is called the GLEN procedure. The Glenn Procedure | Nemours Kids Health And at age four or five the GLEN modification is outgrown and other blood vessels must be rerouted in what is called the FONTAN procedure. Fontan procedure. These names are known by all HLHS parents because instead of having mixed emotions about the first day of kindergarten, they know they will walk again through the Valley of the Shadow made hopeful only through faith and small miracles performed by surgeons who understand more than most of us that we are “fearfully and wonderfully made.” 

I've never actually asked my daughter this, but I think the biggest non-medical challenge for parents awaiting their child’s of FONTAN compared to the first two surgeries is the fact that their baby has grown up; four “Happy Birthdays” have been sung; four Christmases have come and gone; four Halloween costumes have walked down the driveway into the neighborhood beyond.  The Fontan patient is now a thinking, talking child being asked to demonstrate a level of bravery that most adults have never faced. 

 This shared challenge forms a bond of Biblical proportion between parent and child because it is drenched in the anguish of walking an unfamiliar path between faith and fear: the faith part is anchored in HOPE (without an “s”) which fills their night-time prayers, but the fear part wakes the parent in the night because earthly HOPES (with an "s") have no guarantees. This, of course, has been true for all parents since the beginning of time, but those who live with a child who lives with physical deviations from the norm know well the path between "fearfully and wonderfully" made each day. 

Parents who step each day onto the HLHS stage learn not to squint in the harsh lights; they know how to block out the background and not to overthink the unscripted lines of people new to the play. They learn to enjoy the rehearsals as they learn from each review. Through the process, they become empathetic observers of the stories around them. Their eyes may water when commercials for St. Jude's or Shriners Hospital flash unexpectedly on TV. HLHS parents have a deeper understanding of sermons about Mount Moriah, or King David’s lament, or the healing of Jarius’s daughter… and indeed, of Calvary itself.

Today my grandson’s sternum zipper will open again. 

A network of friends and family have anticipated it for years. It's been scheduled for months. Multiple pre-operative visits have let my grandson know it’s coming, and he has been remarkably brave each time. . . as have been his parents (who in some ways carry a heavier burden). On my phone, I have a picture taken one week ago today when the bravest little boy I know met the surgeon who would do first-hand all that I've discussed above. Even then, there was a peace in his demeanor that passes all of my understanding. Both the foctor and child look calm and thoughtful. Perhaps someday, with the parent’s permission, I'll add the photo to this post.

 

For now… imagine with me a similar scene from moments ago: Imagine thst same surgeon stepping into pre-op for one final conversation with the parents and patient. He speaks with the same calm voice he had the week before. He uses the same sterile terms and cadence that only parents understand…and all the while they nod and try to mystically transmit courage into their child’s widening eyes. Then the surgeon inhales optimistically and asks if there are any questions.  Both parents shake their heads no. 

Then he turns to the little boy and says, “How ‘bout you, young man. Do you have any questions?” 

I’m not sure the surgeon was expecting a reply, but he got one.  All the other conversations were about someday in the future, but it is now apparent to this four-year-old that there is no longer a buffer of time. My daughter told me he just looked up with those big blue eyes and pleaded sincerely, “Do we have to do this surgery now?” 

When my daughter told me this account a few hours ago, my eyes welled up and my thoughts turned to Gethsemane where Jesus knew what was coming, He knew why it was coming. He had many “pre-operative" conversations with his loved ones—repeatedly telling them that it was coming, and yet when the buffer of time had vanished…alone there in the garden… he pleaded with His father: “Lord, is there any other way? Can we let this cup pass from me?” I’ve heard my grandson’s voice when he bravely asks hard questions, and to me it was as if he was asking the doctor a Gethsemane-like question: “Do we have to do this surgery now?” While his parents tried to think of what to say, the doctor said something sincere, but the little boy could read the body language. The calm of his parents’ faces was upstaged by the their tearful eyes. The bed began to roll… 

“So, it’s time?” Their little boy asked. My daughter did her best to fill the silence with reassurance as his bed rolled down the corridor and the large doors closed between them.

I wrote the above account in August but did not post it until today, November 24. My grandson’s surgery was a huge success. He is enjoying kindergarten—learning to read and write—but his conversational skills are downright precocious and funny as the following will confirm:

This past week, I underwent a “nuclear stress test” that has prompted a heart-cath scheduled for Tuesday morning… with the possibility of coming home before before Thanksgiving Day. There are other scenarios that involve a longer stay, we’ll know by noon.

When my daughter told my grandson about the possibility of me having surgery rather than a stent or two, he said, "Hooray! Papa and I might be ‘zipper buddies.'" 

I’ll know weather or not I get a “zipper”  after tomorrow’s heart cath, but I took great comfort in having a new level of camaraderie with the bravest little boy I know.