Jacob Stiles – Heart Failure

Learn more about Jacob's experience with Heart Failure at Children's Hospital.

It's Complicated

No one could pinpoint a reason for Jacob Stiles' persistent stomach aches, which started when he was six and dogged him for the next nine years. Eventually acid reflux, extreme fatigue, muscle and joint aches, fevers, and rashes were added to symptoms that a string of pediatricians and specialists near his home in central Pennsylvania tried without success to resolve. The last place anyone looked, was his heart, and by then it was almost too late.

In December of 2012, the lanky 15-year-old was rushed more than 200 miles via ambulance to Children’s Hospital of Pittsburgh of UPMC after being diagnosed with liver failure. Children’s pediatric intensivists quickly discovered that his heart was vastly enlarged and failing – it was actually the cause of the failing liver and kidneys. Survival prospects seemed grim, but the Heart Institute’s Heart Failure and Recovery team placed Stiles on mechanical respiratory and circulatory support, enabling his heart to rest and providing needed blood flow to his other vital organs.

Replenished with oxygen and nutrients, his other organs were restored to normal functional levels within days. Unfortunately, it was too late for Jacob’s heart, which was so badly damaged that a transplant was required. However, with his body stronger, thanks to the mechanical circulatory support, he was able to handle transplantation, and about four months after being admitted to Children’s he was discharged. The stomach aches and other symptoms that troubled him since elementary school have not returned, suggesting that his heart may have been the root cause, all along.

Perplexing Problems

Jacob’s issues were perpetual concerns for his parents, who routinely sought medical attention for their only son and kept him under their wing, even home-schooling him from fourth through seventh grade. Jacob also has high-functioning Asperger syndrome, a form of autism, which led to social problems – teasing and bullying – when he was in school. He frequently complained that he didn’t feel good, according to his mother, Rebecca. “We thought that maybe a lot of his belly issues came from stress.”

Over the years, the parents had Jacob examined by his pediatrician, and then a series of specialists, who variously tested for food allergies, genetic issues, intestinal disorders, and more, but always came up short. “He had a nuclear scan, several CT scans, endoscopies, all ending the same – they couldn’t find anything,” Mrs. Stiles said.

Jacob played football with the elementary and middle school teams. “Every year he would get slower,” Mrs. Stiles said. “He got benched a lot because he couldn’t keep up. It seemed like his leg hurt, or his belly hurt. When he would run, he would cough until he threw up, which they said was exercise-induced asthma. An inhaler was prescribed for that.”

“I never had a reason to doubt what any of the doctors told me,” Mrs. Stiles said.

In the fall of 2012, Jacob had just turned 15 and started playing soccer, but continued to suffer from stomach aches and fatigue.

“We would go shopping and he would have to sit down,” Mrs. Stiles explained. “People would say, ‘oh he’s tired, or lazy, or just a typical teenager.’ And I would say, ‘no he’s not. My son is always on the go.’ Normally, he doesn’t sit still.”

Mixed Signals and a Rapid Decline

“In 2012, from September through December, we were at a doctor’s office or the ER at least once a week,” Mrs. Stiles said.

Another trip to a gastroenterologist for stomach pain resulted in bloodwork, an EKG and a colonoscopy. The colonoscopy showed no problems, and the blood test showed slightly elevated liver enzymes. The gastroenterologist recommended Jacob follow up with a cardiologist because of some rhythm irregularities, although nothing in the EKG suggested what was actually happening in Jacob’s chest. His heart was enlarged to four times normal size.

Nothing could not account for the series of events that occurred in November and December as Jacob became more lethargic, stopped eating due to loss of appetite, and suffered intestinal spasms. Another trip to the emergency room resulted in tests for suspected gall bladder and appendix issues, but the results were negative.

On a mid-December Sunday, Jacob was resting on the couch in his home. His mother noticed that his lips and fingertips were blue. Another trip to the local emergency room ensued. His blood pressure was low, his liver enzymes were very high, liver failure was suspected and he was rushed via ambulance to a hospital in a nearby town. There, doctors diagnosed him as being in severe acute liver and kidney failure and, anticipating that a liver transplant would be required, the hospital dispatched him to Children’s Hospital of Pittsburgh of UPMC. By the time he arrived, he was gasping for breath. An echocardiogram revealed his enlarged heart and the family was advised that Jacob was in heart failure.

To the Edge and Back

With the situation so dire, Jacob was intubated and put on life support in an induced coma state. On Dec. 22nd Peter Wearden, MD, PhD and his surgical team put Jacob on ECMO to mechanically assist with his circulatory and breathing functions. His body immediately responded. By Dec. 27th his liver functions had significantly improved, and he was taken off ECMO and put on a left ventricular assist device, or LVAD. He was up and in a chair two days later.

In early January, Jacob was moved out of Children's Cardiac Intensive Care Unit and feeling so much better that he half-jokingly asked Dr. Wearden if he might be able to go snowmobiling while on the LVAD. Although his liver and kidneys had completely recovered, it was clear that his heart would not, and he was added to the heart transplant list. Victor O. Morell, MD performed the heart transplant on Feb. 8th, and the Stiles family returned home a month later.

Whether a virus at an early age infected and weakened his heart or a congenital defect caused his heart to fail, the exact cause of Jacob’s heart failure will likely never be known. What is certain is that his recovery has been phenomenal. “From the moment you saw him with his new heart, he looked completely different,” Mrs. Stiles said. Jacob’s recovering body even grew taller while in the hospital on LVAD, being discharged at six-foot-two, a good three inches taller than when he went in. “Since he’s had his new heart, he’s not had any stomach issues at all. He’s not tired anymore. Nothing,” said Mrs. Stiles.

“I can’t say enough good about that hospital and the compassion and the drive that the doctors and nurses have,” she said. “Dr. Wearden and Dr. Morell are outstanding. They send out a vibe of just caring and sacrifice of their own time to just see somebody smile and to do better.”

And not only is Jacob better, but he has a tentative snowmobiling date with Dr. Wearden in the winter ahead, Mrs. Stiles said. “He’s looking forward to it. What a glorious day that will be.”

For first-time parents Rebekah and Ernest Carper of Spencer, W.Va., there was no greater joy than the birth of their daughter. When Baylee was six months old, the couple received frightening news. Doctors diagnosed her with a potentially fatal heart disease — dilated cardiomyopathy. That's when the Carpers turned to Children's — the only pediatric heart transplant program in the region.
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