Caring for a Medically Complex Child

Deciding where to go to college, what to wear to prom, or how to ask out a crush are normal concerns for many teenagers. Wondering how long their new heart will keep them alive is usually not one of them.

But for Sam Allen it is.

At just 16 years old, Sam has already had 8 open heart surgeries – including a heart transplant, which he received last year. He was born with a life-threatening heart condition called hypoplastic left heart syndrome (HLHS). HLHS is a genetic heart defect in which the left side of the heart is underdeveloped. The left side is responsible for pumping oxygen-rich blood throughout the body.

"There are varying degrees of severity," says pediatric cardiologist Kenneth Goertz, MD, at The University of Kansas Health System in Kansas City. "But at some point, if the left-sided structures of the heart are too small, the left ventricle can’t pump blood to the body."

HLHS is noticeable at birth and usually detected soon after the baby is born.

"Sam was 27 hours old when he got sick," says Laura Allen, Sam's mother. "The nurses noticed there was something wrong. Within 6 hours, a pediatric cardiologist had diagnosed him."

After Sam’s health issues were revealed, Laura had a decision to make. She was a single mother at the time, with a young child at home, and had planned to give Sam up for adoption. She'd traveled from her home in Hays, Kansas, to San Antonio to give birth near the adoptive parents. But after receiving Sam's diagnosis, Laura had a change of heart.

"My first thought was, 'He's going to die. And I'm going to give him up.'," Laura says. "He had a lifetime of medical issues in front of him … I decided to keep him."

Laura was then faced with another difficult decision – how to care for Sam. His care team gave her 3 options: Place him on a heart transplant list right away, perform a 3-stage surgical procedure, or take Sam home and let him die. Laura chose the surgeries, but she knew they wouldn't cure him.

"The procedures were just a temporary fix," Laura says. "We always knew he was going to need a heart transplant someday."

Geography complicated Sam's medical issues. The hospital in San Antonio where Laura delivered, and where Sam received all 3 heart surgeries, was a 12-hour drive from her home in Hays. For each procedure, Laura would pack up Sam and her older son and head to Texas for up to 5 weeks.

And because Sam's health issues were complex, he needed special ongoing care. Specifically, he needed to be monitored by a pediatric cardiologist. Yet that level of medical care was only available in larger cities that were hours from Laura's home, or so she thought.

As a single mom of 2 kids, Laura didn't find trips to Kansas City very appealing at the time. "I called around and found out that Dr. Goertz came every other month to Hays." Dr. Goertz monitored Sam’s heart health at outreach clinics in both Hays and Salina. He worked with Sam's local pediatrician in Hays, and the surgical team in San Antonio to coordinate Sam's healthcare. This helped ensure that all of Sam's care providers were informed and up-to-date on his current health issues.

"Having those outreach clinics provides the opportunity for continuity of care for families that wouldn't have access (or as easy of access) as they would otherwise," Dr. Goertz says.

HLHS wasn’t Sam's only medical problem. When he was 5, Laura learned that Sam was missing the left lobe of his liver – a problem that could require a liver transplant down the road. He also had an abnormal heart rhythm and, when he was 10, was diagnosed with a leaky tricuspid valve.

"Each time Sam had an operation, his heart suffered a little and didn't fully recover," Dr. Goertz says. "His tricuspid valve leaked; he had to have a pacemaker. He had fatigue, abdominal pain and persistent enlargement of his liver that were concerning me."

Despite intense medical management to control Sam's pain and symptoms, he wasn't getting better.

Dr. Goertz reached out to Sam's team in San Antonio to express his concerns. "I felt that the impact of his tricuspid valve leak was significant," Dr. Goertz says. "And I felt like I was not getting attention to that issue."

Convinced of the seriousness of Sam's condition, Dr. Goertz contacted a colleague in Michigan, who agreed to perform Sam’s tricuspid valve repair in 2012.

"Dr. Goertz always did a great job of getting us to where Sam needed to be," Laura says.

Within a year, Sam was on oxygen full time and getting sicker. He was in and out of the hospital, and was dizzy and having migraines.

Sam's care team had done as much as they could, but Sam's health continued to decline. That's when Dr. Goertz decided it was time to start thinking about a heart transplant. He sent Sam's medical records to several heart transplant hospitals, and eventually secured Sam an appointment in St. Louis.

Sam was placed on a heart transplant waiting list on Friday, January 13, 2017. By Sunday, Laura got a call that a heart was ready. Sam was taken to the operating room that Monday at 6 p.m. and received a new heart on Tuesday, January 17.

Yet Sam's heart transplant was not without its difficulties. Within 2 months, he had tricuspid valve repair on his new heart, and his heart function was also not as great as was hoped. And although his heart is now much healthier than it was before, Dr. Goertz notes that Sam's health issues are not over.

"I often tell heart transplant patients they will be living with a new disease," Dr. Goertz explains. "We hope it's a better disease than the one they had. But it's a new condition. You're trading 1 condition for another."

Today, Sam is learning to live with his new heart. He's able to enjoy most activities that other healthy teenagers can, and even plays baseball at his high school. He's involved in drama and musical theatre, and one day hopes to become a diocesan priest.

"He's really good at self-limiting, so we've always been pretty okay with him deciding when he needs to stop," Laura says.

Children like Sam who have heart transplants have about a 50% survival curve between 12-15 years. That means half of children who receive a new heart either need another new heart or don't survive 12-15 years after they get the organ. It's a sobering statistic, but one that Laura says Sam takes in stride.

"It could be much worse. I'm not going to say that he never gets down or sleeps more than he should, but some of that is being a 16-year-old boy. In the grand scheme of things, he handles it very well."

Sam continues to see Dr. Goertz and receives follow-up care for his heart transplant at Children's Mercy in Kansas City. He's also now receiving endocrinology care because he was recently diagnosed with diabetes.

"It's never ending," Laura says. "But it's worth it. He's a good kid."

Dr. Goertz has been Sam's cardiologist since he was born, but that will change. In a few years, Sam will transition out of pediatric care. Dr. Goertz is working on a plan over the next 2-3 years to assemble Sam's new care team so everything is in place for his ongoing care. This plan includes a physician who cares for adult pediatric cardiology patients.

This is a critical time for kids like Sam who are born with congenital heart issues because changing care providers can result in delayed or inappropriate care. Fortunately, Sam won't have to go far to find the same compassion and expertise Dr. Goertz has provided over the last 16 years.

"Sam's transition should be pretty seamless," Laura says. "It's such a relief."

Although he understands his cardiologist will change, for Sam, there's one space on his care team that can never be filled.

"Dr. Goertz is a great man and a great doctor. He has done nothing but care for me," Sam says. "He is not just a doctor; he is more than that. He’s even more than a friend."

Patient testimonial: As with all treatments, individual patient results vary. It is important to discuss your treatment options with your healthcare provider.