JJ reached for a block, grasped it and placed it on top of the tower.
With a flourish, he knocked down the stack, grinning as the tower tumbled.
Such a victory for this little boy, a survivor of an extraordinarily complex heart defect.
At one point, his parents were told there were no more options, other than to make him comfortable for his last days.
But after consulting with the specialists at the Congenital Heart Center of Spectrum Health Helen DeVos Children’s Hospital, they moved to Michigan for a chance to fix his heart.
And now Jeffrey Smith—nicknamed JJ—is crashing through milestones like a toddler knocking down a stack of blocks.
“He’s a trouper. He ain’t going nowhere,” said his mother, Tonya Goudie. “He’s Mama’s miracle baby.”
Born in Indianapolis in May 2015, JJ didn’t show signs of his heart defect in his first hours. He weighed 6 pounds, 10 ounces, and had a full head of curly blond hair.
He was “gorgeous,” said his mom. “Perfect. I wanted to cry. I cuddled him and didn’t want to put him down.”
Twenty-four hours later, he turned blue. A medical team worked to revive him. And he transferred to another hospital, where his heart ailments were diagnosed.
(We are guided by) a willingness to try anything possible to see if we can help the child.
For the next two and a half years, JJ underwent 10 operations on his heart, as well as several on his stomach and knees. But the next surgery JJ needed was considered too risky. His heart was not accessible for further cardiac catheterization assessments due to blocked central veins. Eventually, doctors in Indiana sent him home with the promise of palliative care to make his last days comfortable.
His parents, Goudie and Kyle Smith, began investigating other options.
“I was thinking, I cannot lose my baby,” Goudie said. “He is who I live for day to day. He fights for me. I’m going to fight for him.”
JJ’s grandparents knew someone whose child had been been treated by the congenital heart experts at Helen DeVos Children’s Hospital. They suggested consulting the doctors there, to see if they could help.
Joseph Vettukattil, MD, a pediatric interventional cardiologist and the Congenital Heart Center’s co-director, told Goudie and Smith he would to look into JJ’s case, but made no promises anything could be done.
His approach is guided by “a willingness to try anything possible to see if we can help the child,” he said. And yet he also takes care not to encourage unrealistic expectations.
“That can be damaging to the patient, as well,” he said. “It’s a very narrow path to negotiate. But I think if you are truthful and honest and professional about it, you can do it right.”
A 3D heart model
First, Dr. Vettukattil needed to perform a detailed three-dimensional imaging of JJ’s heart with an echocardiogram. When the results showed promise, the next step was to perform a cardiac catheterization to get a better understanding of the physiology of the little boy’s heart.
That was considered impossible at another major center and proved challenging because of blockages in his heart arteries and veins. But Dr. Vettukattil managed a complex procedure allowing him access through a small vein in the boy’s neck.
Using images from scans, the Congenital Heart Center printed a 3D plastic model of JJ’s heart, which the doctors used to discuss possible treatment options.
“He had almost every diagnosis you can have,” said Marcus Haw, MD, a pediatric heart surgeon and co-director of the Congenital Heart Center.
JJ was born with such large holes in his heart that, instead of four chambers, his heart essentially had one large chamber. It formed on the wrong side of his chest and faced the opposite direction. The veins from the right side of his lung to his heart were absent, and the two major veins from his brain to both sides were blocked.
He also had transposition of the great vessels, which involves an abnormal arrangement of the vessels that bring blood to and from his heart. He had one functioning lung and there were serious issues with veins and arteries that connect his heart to his lungs.
I couldn’t ask for a better life as long as I have my son.
The 3D model helped the physicians to decide surgery was possible—and that it could improve JJ’s heart function.
Without the 3D model, “I wouldn’t risk it,” Dr. Haw said. “The message for me is that 3D printing is a fantastic way to assess the operability of these borderline hearts.
“We as a center feel we can offer corrective surgery to many children who have been given what they call a palliative operation.”
To be close to JJ’s medical team, Goudie and Smith moved to an apartment in Grand Rapids, Michigan, with financial help from Smith’s parents. It was hard to leave their home and large family in Indiana, Goudie said. But they didn’t hesitate to make the move.
“Whatever it takes, you do it to take care of your baby,” she said. “I couldn’t ask for a better life as long as I have my son.”
On Aug. 27, 2018, Dr. Haw performed surgery on JJ’s heart. He created patches to divide the chambers of JJ’s heart and created tubes to correct the blood flow—improving the flow of unoxygenated blood to his lungs and oxygenated blood to his body.
Of all the surgeries Dr. Haw has performed, JJ’s “was one of the most difficult,” he said.
As JJ recovered from the operation, a hole opened in the wall that had been created to separate the lower chambers of his heart.
Dr. Vettukattil, who was visiting his mother in India, cut his vacation short to return to help. Because JJ could not undergo another open-heart surgery at that point, he and Dr. Haw performed a “keyhole” surgery. Through a small incision, Dr. Vettukattil threaded a catheter through which he placed a vascular plug to close the hole.
“I would say this is akin to precision treatments for advanced cancer,” Dr. Haw said. “This is precision treatment for congenital heart disease that has been deemed inoperable.
“I think we have the ability to reassess patients who in the past have been told there is no treatment available.”
Bigger and stronger
JJ still faces challenges. He has only one lung and he has impaired vision and other issues. But he has a heart that can take him into the future.
Before the surgery, he likely faced a short lifespan.
“If we didn’t do it, he would have died a natural death,” Dr. Vettukattil said. “Either he would have become more and more blue and have no oxygen to the brain, or he could have had a bleed into his lungs.”
JJ’s parents marvel at the changes since the operation. The oxygen level in his blood, which once hovered in the 40-percent range, now is in the 80s. His fingers and toes no longer are blue and he is growing bigger and stronger.
“He has definitely gained a lot of weight,” his dad said. “We struggled for years to get him to gain any weight at all. His heart was working so hard.”
With physical, occupational and speech therapy, JJ is making strides, learning to play, walk, use sign language and feed himself.
On a recent afternoon, he stood at the coffee table in the living room of his family’s apartment, reaching for a toy. On every wall hung photos documenting his life, from pink-cheeked newborn to fragile infant to tiny toddler.
He cruised along the table in his jeans and hoodie sweatshirt, an active little boy radiating energy and enthusiasm.
Speech therapist Jaclyn Suchecki blew bubbles in the air and JJ smiled and swatted at the bubbles.
“His eye contact with those bubbles was amazing,” Suchecki said, as they moved on to the next activity.
She sat on the floor with JJ and they looked at a book filled with animal pictures.
“Show me the doggy,” she said.
He pointed to the page and tried to make the sound of a panting dog. And then he turned the page to find the elephant, patting the picture in the book.
“Aw. You love that elephant so much,” Suchecki said.
The session over, JJ lay back against a pillow, eyes closing. His dad spread a fleece blanket over him and tucked his teddy bear beside him.
“I just want other parents out there to know if you have a kiddo in this situation, don’t give up on them,” Goudie said. “We went from no hope to the best hope you can have. It’s worth it.”