Valentine surprise marks anniversary of liver transplant for 3-year-old congenital heart patient

While watching the TV cartoon “Bluey” in a waiting room, 3-year-old Karl Hadley reluctantly walked away Wednesday for yet another of his frequent visits with a Spokane cardiologist.

But a surprise awaited him in the exam room: Balloons and a toy, the Bluey’s Family Home playset, complete with its dog figurine. Dr. Carl Garabedian watched along with Karl’s parents, Randy and Brenda Hadley of Post Falls, as the boy played briefly until his routine checkup began.

The Providence Health gift marked a milestone from three years ago, when Karl had a liver transplant on Valentine’s Day 2021 at Seattle Children’s Hospital. A viral infection had caused his liver to fail.

Karl still has ongoing health issues from an unrelated congenital heart defect, called pulmonary vein stenosis, which means progressive narrowing of veins. Since his birth on Jan. 5, 2021, in Coeur d’Alene, he has spent most of life in hospitals or visiting them for procedures because of the liver failure and his heart condition.

“He spent most of his first 13 months at Seattle Children’s Hospital,” Brenda Hadley said. “He’s had 17 heart catheter procedures.”

Garabedian, a Spokane pediatric and adult congenital interventional cardiologist, took over Karl’s care after the initial Seattle treatments. He is a specialist at the Providence Sacred Heart Children’s Hospital center for congenital heart disease.

Karl’s heart condition means that the veins bringing blood back from his lungs to his heart become narrowed over time, Garabedian said.

The heart defect was diagnosed shortly after the liver transplant. Seattle Children’s specialists at that point completed one open heart surgery in April 2021 to correct some of those heart vein issues, but Karl’s defect still progressed more so than in most cases, Garabedian said.

“The narrowing required a surgical procedure to repair the pulmonary vein narrowing, but unfortunately, the disease process was more complex, and he continued to develop progressive narrowing of all of his pulmonary veins,” Garabedian added.

“Since that time, he has required multiple catheterization interventions on his pulmonary veins to balloon-dilate them, place stents in them … all these kinds of fancy balloon-type of techniques to keep his pulmonary veins open, and despite this, they continued to progressively decline or to worsen.”

The heart-catheter procedures came at about one- to three-month intervals. Karl is now on two primary medications, including one that is a chemotherapy-like drug, which seem to be slowing progression of the veins’ narrowing, Garabedian said.

He said the catheterization procedures are expected to continue, but now at decreasing intervals of perhaps once every three to four months.

A main complication is that the boy’s pulmonary veins are so tiny. His growth over time is expected to make a difference.

“If you take a 2 year-old’s pulmonary vein that is 3 to 4 millimeters (diameter) in size, any narrowing to those is going to clog or totally block them off,” Garabedian said.

“Take someone bigger like me, who has a 12- to 13-millimeter pulmonary vein and maybe a little bit of narrowing. It’s not as significant, so again, just from a size perspective, I expect it to get better over time.”

Most young children with pulmonary vein stenosis require a “one and done” surgery, he said, but Karl is on the other side of that spectrum, requiring the regular interventions to keep his pulmonary veins open.

“When pulmonary veins narrow, you have breathing difficulties,” Garabedian added. “Blood can’t get out of your lungs. Children, as you can imagine, get colds all the time. So between his multiple illnesses as well as his pulmonary vein problems, he’s had multiple hospitalizations throughout his lifetime.”

Brenda Hadley said Karl’s medical issues took the family by surprise after he was born five weeks early at Kootenai Medical Center.

At 11 days old, Karl went with his dad on a plane to get to Seattle Children’s, while she recovered in the hospital.

Karl is an easygoing child but still argues at home with his siblings like any other kid, Randy Hadley said. He credits Karl’s caregivers, including Coeur d’Alene specialists who fought for him.

“Despite all of this, he is a happy kid,” Garabedian said. “A lot of kids who undergo these kinds of procedures over and over, they have medical fear; they don’t want to see you. With Karl’s family and the beauty of our child life services, what they’ve done for this kiddo to keep him understanding what we’re doing and not be fearful, that has really made his quality of life wonderful despite all this.”