First of two parts: A 2-year-old Richland boy comes off the transplant list thanks to a still-experimental device
February 1, 2009 10:00 AM
Cindy Gradowski with her son, Ethan, 2, at home in Richland. Once in line for a priority heart transplant, Ethan's time on the Berlin pump allowed his heart to heal.
Cindy and Ethan Gradowski in the cardiac Intensive Care Unit at Children's Hospital in December.
By Mark Roth Pittsburgh Post-Gazette
If Peter Wearden ever needed a poster child for the value of pediatric heart pumps, Ethan Gradowski could be it.
In November, the 2-year-old Richland boy was being kept alive on an experimental device called the Berlin pump, with four tubes snaking in and out of his tiny chest, and he had been put on the heart transplant waiting list.
By last week, he was at home, off the pump and off the transplant list, and fascinated with pushing the buttons on the camera that a photographer had brought to take pictures of him.
Ethan's case isn't typical, but it shows the value of heart assist devices in helping children get better or make it alive to a heart transplant.
Dr. Wearden, a heart surgeon at Children's Hospital of Pittsburgh, is heading up a five-year-old research project to develop a miniaturized heart assist device for children that could be put inside their bodies.
The need for such devices is great, he said.
Last year, more than a fifth of the children waiting nationally for a heart transplant -- 54 of 240 -- died before they got one, according to the United Network for Organ Sharing, which oversees organ transplants. And nearly 1,800 infants in the United States die each year from congenital heart defects, a 2006 report in the journal Circulation said.
Older children can be kept alive with the help of adult heart assist devices that are already available, but the federal Food and Drug Administration has not yet approved any device for routine use in younger children.
The Berlin pump, made in Germany, comes in sizes small enough for young children's bodies, and is undergoing an FDA evaluation trial now at 15 hospitals.
In most cases, though, the pump -- known officially as the Berlin Heart EXCOR Pediatric -- has to be approved in the United States on a case-by-case "compassionate use" basis.
Each time a doctor gets permission to use the Berlin pump, the device must be shipped to the hospital handling that case. Dr. Wearden has used it that way nine times.
Cindy and Joel Gradowski brought Ethan to Children's on Nov. 7 for what they thought would be a routine repair of an atrial septal defect -- a hole in the wall that divides the heart's two upper chambers. Dr. Wearden performed the surgery.
"Dr. Wearden came out and said, 'Ethan did very well, he asked for mommy, and give us 30 minutes to get him situated in his bed, and then you can see him,' and we said OK," Mrs. Gradowski recalled.
But then, disaster struck.
Ethan's lungs filled up with fluid and he suffered a heart attack. With the doctors and nurses administering chest compressions, he was taken to the catheterization lab, where he suffered another heart attack. The dye test showed that his coronary arteries were extraordinarily narrow.
Doctors assumed he had been born that way, but the problem hadn't shown up until he underwent the stress of surgery. They immediately put him on a pump called ECMO, for extracorporeal membrane oxygenation, a device that takes over for a patient's heart and lungs and is used during bypass surgery.
And they placed Ethan on the transplant list, saying it was his only hope.
Within five days of his crisis, Dr. Wearden had switched Ethan from ECMO to the Berlin pump. He did that partly because Ethan's lungs could function on their own, but also because ECMO is not intended as a long-term device.
Among other problems, it can cause severe inflammation in the body's organs because of the way the blood reacts when it flows past the machine's plastic membranes.
But the Berlin pump -- Ethan actually had two implanted, one for each side of his heart -- is not benign either. The valves and pumping action can cause clots that lead to strokes.
Ethan did in fact suffer a stroke, which doctors at first feared might leave him blind, but Dr. Wearden said it was probably caused by his initial heart attack or the ECMO device.
The Berlin pump also has tubes entering and exiting the body, a possible route for infections, although Dr. Wearden said children seem less prone to those than adults.
As the Gradowski family headed toward the holidays, keeping hopes up and fingers crossed, Ethan's body and heart continued to improve.
That's when Dr. Wearden approached them and suggested performing one more catheterization before any transplant took place, just to see if there had been any change.
"In the back of all of our minds," he said, "we were suspicious about those narrow arteries, and wanted to take another look."
Because Ethan had suffered a heart attack during his previous catheterization, the Gradowskis were worried about the procedure, but they reluctantly consented. On Dec. 17, Joel Gradowski carried Ethan into the testing room.
An hour and a half later, his cardiologist, Dr. Jacqueline Kreutzer, came out, Mrs. Gradowski said, "and she had tears in her eyes, and she said it looked like a different child; the arteries were normal. I just fell to my seat and I was bawling and Joel was bawling. It was a Christmas miracle."
The Gradowskis barely had time to absorb the news when Dr. Wearden said that in view of the results, Ethan should be weaned off his heart pump.
"When?" Mrs. Gradowski asked.
"Tomorrow," he said.
So the next day, as his parents watched, the doctors slowly turned down the pump, waiting to see if Ethan's heart would take over on its own.
"Finally Dr. Wearden said 'OK, stop,' and he said, 'His heart function looks great.' "
Today, Dr. Wearden said his two main theories on why Ethan's arteries were so narrow after his first surgery are that he experienced some kind of acute inflammation or had a reaction to his medications.
In either case, he said, Ethan needed the heart pump to support him until his coronary arteries returned to their normal diameter.
And while no child in the United States is put on a heart pump today unless it is to support him until a transplant, about 5 to 10 percent of children recover and can be taken off the transplant list.
At Children's Hospital here, about four or five children have regained normal heart function while on heart pumps over the last four years, Dr. Wearden said.
Even if children still need a transplant, newer heart assist devices will allow more of them to make it to that point, particularly as the technology becomes smaller and more reliable, Dr. Wearden said.
At Children's, doctors perform roughly 20 heart transplants a year, and have been using assist devices beforehand in five to six of them.
"The more comfortable we become with it," he said, "the indications for its use will broaden."
Ethan Gradowski is not out of the woods yet, but has strong prospects for being able to live a normal life, his mother said.
His heart so far shows good function, she said, although it is not back to 100 percent pumping efficiency. He has recovered the speaking ability he lost during his hospitalization. He must go to physical therapy for now to overcome muscle damage he suffered during his first catheterization, and he is on medications to guard against allergic reactions and to help his heart pump.
When she looks at her laughing, chatty boy today, Mrs. Gradowski remembers what she heard during the tough weeks in the hospital, when all he could do was whimper.
"A couple of the nurses said to me, 'Look, if he were an adult, I wouldn't have a lot of hope, but don't lose faith, because these kids are unbelievable.' "