Thump, thump, thump….Thump, thump, thump, thump.
Jake Hafer, a freshman at La Salle University, picks up speed as he runs late to class. His heart begins to beat faster, but then slows as he breaks into a walk just the same as other students. But one thing isn’t the same.
Jake’s heart is not his.
Born with a congenital heart defect, Jake, now almost 20, got a heart transplant at Philadelphia’s St. Christopher’s Hospital for Children when he was just seven and a half months old. His was one of the first pediatric heart transplants in the Philadelphia area and one of only a smattering across the country. That he survived the surgery and has lived this long has helped to establish that even infants with faulty hearts can do well with transplants.
“You couldn’t tell”
“Unless you know he has a transplant, you couldn’t tell,” said Dr. Pieranotnio Russo, the former director of cardiac surgery at St. Chris, who performed the delicate and risky operation on Jake. “I am very proud when these children develop to be normal adults and contribute to society without being disabled.”
Jake was born to Kathy and Tom Hafer, of Reading, Pa., on February 14, 1996. After running the normal newborn tests, doctors found something was not right. Jake was diagnosed with hypoplastic left heart syndrome. The left side of his heart, which pumps oxygen into the aorta, was severely underdeveloped. “This was very rare for this to happen to me, “Jake explained. “It was a really scary time for my family.”
The U.S. Centers for Disease Control and Prevention estimates that each year about 960 babies in the United States are born with hypoplastic left heart syndrome. That amounts to about one out of every 4,344 newborns.
“We were devastated,” Kathy recalled during a recent interview. “We had a healthy two-year-old boy at the time. How could this be?”
Kathy and Tom faced a difficult dilemma. Doctors could do a series of operations to repair the defect, but that would entail constant surgeries and there was no guarantee they would work. On the other hand, the couple could go for a transplant, but this was a risky operation and there was little precedent for its long-term success in babies less than a year old.
“We wanted him to have the best quality of life we could,” Kathy said.
So, they took the risky route, and in July of 1996, they put Jake on the heart transplant list. At the time, the procedure had only been done for a little more than a decade. The first successful pediatric heart transplant was performed on a 4-year-old boy in 1984. Twelve years later when Jake was to receive his transplant, many improvements were still being made.
“He and his family were very courageous,” said Russo. “The risk factors were many. There weren’t many children with these types of cases done under 12-months-old. His family was initially directed to another hospital where they would have done three to four procedures instead of replacing the heart. But the family said ‘no’ because they wanted to cure him.”
There are many more patients waiting for an organ transplant than there are donor organs, according to Columbia University Medical Center.
Long waiting list
There are over 122,000 people on the organ transplant waiting list, with a new name added every 10 minutes. The transplant list continues to grow, but the rates of donation stay stagnate. An average of 22 Americans a day die waiting for a heart transplant, according to the U.S. Department of Health and Human Services.
It was a normal September night for the young family. Kathy was up late, doing things around the house. Her husband, Tom, and children slept quietly in their beds. It was 12:15 a.m., and then the phone rang.
Tom groggily answered, “Hello?”
On the other end was a nurse of the transplant wing at St. Christopher’s. They found a heart for Jake. It was a perfect match.
The first question that popped into Tom’s mind was, “Who did the heart come from?”
A five-year-old boy, who was killed in a drunk driving accident in Atlanta was Jake’s donor. “It’s a heart so we knew it had to come from a child who passed away,” Kathy said. “I think of them (the family) all the time and I am so grateful for them.”
Kathy wrote to the mother of the other boy a couple of times, but never received a response.
St. Chris a pioneer
At St. Christopher’s, Russo had built a reputation as a leading cardiac surgeon during his nearly four decades of practice. The hospital, on Erie Avenue in North Philadelphia, was one of the few hospitals in the country to perform pediatric heart transplants.
“My team was really a pioneer in transplanting children less than 12-months-old,” Russo explained. It was just four years earlier — in 1992 — that the hospital had done the first successful pediatric heart transplant in Philadelphia.
When the decision was made to transplant Jake, he was so young that doctors feared the heart of a five-year-old might be too big. “The doctors were skeptical because they didn’t know if the five-year-old boy’s heart was going to fit inside me,” Jake said.
Russo noted, however, that a baby born with a congenital heart problem usually has a heart three to four times larger than a normal child. This makes it easier for a transplanted heart to fit the child. Still, doctors had to create more space around Jake’s lungs to get the new heart in.
Jake’s parents left immediately for the hospital and drove the hour from Reading to Philadelphia. When they got to St. Chris and handed Jake over to the nurses, a second question popped into their heads.
“Is this the last time we will see him?” Kathy said.
During the surgery, the nurse came out every two hours to give Jake’s parents updates.
The first two hours went by, and all Tom and Kathy could do was make calls to their relatives. The clock ticked, and each minute, Tom and Kathy were left wondering.
Another two hours went by, the nurse came out. He was ok.
They continued to make calls. Another two hours passed, and Tom and Kathy nervously watched the door, waiting for the nurse to come through with the update.
He was still ok. More calls were made, and all they could do now was put Jake’s fate into God’s hands.
“We were just praying and hoping for the best,” Kathy said.
After an eight-hour surgery, the nurse came through the doors. and gave Jake’s parents the good news: The surgery was a success.
“You are so happy, but you see your baby laying there with tubes coming out of him and he has a big scar on his chest,” Kathy recalled.
While the surgery was a success, the challenge was far from over.
St. Christopher’s transplant wing closed and Jake’s care was switched to Children’s Hospital of Philadelphia until he turned 18.
There he had regular cardiac cauterizations and biopsies. First, doctors gave him “an awful tasting medication I always rebelled at having to take.” The medicine put him to sleep. Doctors then threaded needles through his neck and groin. Once the needles reached his heart, the physicians snipped small tissue samples, which then went to the laboratory for testing.
The procedure, Jake recalled, was always done in June. When he woke up, he was in pain and couldn’t move his head because of all the bandages on his neck.
“It was a stressful day for a kid,” his mother recalled. “Afterward he had to lay flat for six hours and that is hard for a toddler to do. We would bring Gameboy back in those days and videos to keep him busy. We would always treat him with a new toy or treat after.”
Jake’s favorite toy was a Buzz Lightyear action figure, which he got when he was seven.
He went weekly, then monthly, and now he goes for check-ups twice a year. After 10 years of no signs of rejection, Penn Medicine, where he currently receives treatment, says he no longer needs the biopsies. He now completes stress tests to monitor his heart rate.
“Walking away with a clean bill of health is always a good feeling,” Kathy said. “Until that next appointment comes.”
One of the concerns with any transplant is the possibility of rejection by the body’s immune system. So far, there have been no signs of that for Jake. But in many ways, he is entering unknown territory because there are so few pediatric heart transplant patients in Jake’s age cohort.
“We don’t know what happens beyond 20-years-old because we don’t have enough numbers to tell us that,” Russo explain. “But there have been patients that have survived so well that there has been no rejection.”
Survival rates for pediatric heart transplants
However, The Society of Thoracic Surgeons released a study that reviewed medical charts of 337 pediatric heart transplant patients since 1985 and found that 54 percent of those patients survived at least 15 years past surgery.
Research from John Hopkins Medicine examined data from more than 22,000 American adults who got new hearts between 1987 and 1999. The research found that the age of the donor was significant. For every decade younger the donor was, the recipient was 10 percent more likely to survive long term.
To stay on track, Jake takes medicine to suppress his immune system. But the treatment comes at a cost. His immune system is not 100 percent, and he is much more vulnerable to illness.
“My parents really took extra caution for me,” Jake said. “Everywhere I went they made sure I stayed clean. Over time, I stayed healthy, but they really stressed the importance of hygiene. I always carry hand sanitizer everywhere, it’s embarrassing sometimes.”
To keep him safe, Jake’s mother often pulled him out of school if other children were sick. As a result, he had to repeat a year.
“At the beginning of the first year or two, obviously the risk is much, much higher because there are larger doses of immune suppression,” Russo said. “Now it is much less intense and so the risk of infection is much less than in the early days. But, he still must wash his hands more, and be very cautious.”
Survival odds rise at age 20
According to Russo, once a pediatric transplant patient reaches the age of 20, the chances of rejection are greatly diminished because, by that time, the body has begun to recognize the transplanted heart as part of itself.
When Jake was growing up on his parents’ dairy farm in Douglassville, Pa, things were tougher. His mom didn’t want him to help out on the farm for fear he’d get sick from exposure to the dirt. When he turned 12, his mother finally relented and he joined his older brother and a younger sister in helping out with farm chores.
As he continued to grow and started high school at Berks Catholic in Reading, he began thinking about college and stumbled across Jessie’s Day, a scholarship geared toward college-bound transplant patients.
The scholarship was started in the name of Jessica Beth Schwartz, a heart transplant recipient when she was a teenager. She went on to study journalism at Temple Univeristy. Her dedication to earning her degree was cut short when she died at the age of 23. .
The Jessica Beth Schwartz Scholarship Fund was started to give young transplant recipients a chance for a higher education. Since it was established 13 years ago, Jessie’s Day has awarded 45 scholarships worth more than $90,000.
Jake won the scholarship, along with another freshman at La Salle, Kevin Brighter.
Despite the challenges he has faced, Jake leads an active life. When he’s home in Reading, he watches the Reading Phillies, attends events at his Catholic parish, and volunteers at a local historical site. Last spring, as a high school senior, he competed in the Penn Relays, running the 4 x 1.
At La Salle Jake can be spotted crossing the campus in a Berks Catholic pullover. He is a history major and hopes to go on to law school at the University of Pennsylvania.
“I really think it is a normal life for me,” he said.