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Miraculous Mending
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Worcester Telegram & Gazette
April 12, 2004
Section: LOCAL NEWS

Nathaniel Murphy had one question when he woke up from surgery.

"Do I have one kidney or two?'' he asked his mother, Jenifer Mahitka. Good question.

The 11-year-old boy from Brimfield had just become one of only a handful of people - few of them children -to have one of their kidneys removed, repaired and returned to their bodies.

So the answer was yes, a simple way to sum up a complicated story.

"It's not unheard of, but it's what we call heroic surgery,'' said Dr. William A. Primack.

Young Nate had developed a renal artery stenosis, which is a narrowing of the blood vessels leading to the kidney. That caused an aneurysm, or pouching out, of the artery under pressure. The problem affects an estimated 0.1 percent of the population, mostly adults. Few children have the condition to begin with, and an even smaller number of them have their aneurysm located in the smaller branch arteries going to the kidney, making surgery to correct it more difficult.

Seeing the sixth-grader buzzing around his house on a recent Friday afternoon, it was hard to imagine him needing the help of two transplant surgeons and a vascular surgeon on Feb. 5. Nate munched on cookies his 16-year-old brother, Ryan, had just taken out of the oven. He flitted around the house until he could race away with a friend on his "heelies," a hybrid of sneakers and in-line skates. Mugging for the camera, he hugged the Christmas snowboard he never got to use this past winter.

Flashing a dimpled grin, he wrapped his arms around his mother, who rolled her eyes at his antics but loved seeing his infectious energy.

Nate had been healthy before August, when during his annual physical examination, his blood pressure was unusually high. His mother could pinpoint the start of his problems to that month,

because during a trip to the emergency room in July after he'd gotten stuck by a fishhook, his blood pressure had been normal.

Repeat checks told the same story. The top range of acceptable blood pressure in an 88-pound 11-year-old boy like Nate is 123 over 81. Nate's topped out at 160 over 120. After a urine test implicated his kidneys, his primary care physician referred Nate to Dr. Primack, who is a pediatric nephrologist practicing at the Fallon Clinic in Auburn.

High blood pressure in a child is different in two ways from high blood pressure in an adult, he explained, making physicians pursue its cause more aggressively.

"If you diagnose high blood pressure in a 10- or 11-year-old like Nate, your likelihood of finding something wrong is much higher and the likelihood that you can do something about it is much higher than if I diagnose it in a 50-year-old,'' he said. "The younger the child, the more likely you're going to find a reason for it that we can fix.''

As Nate's evaluation proceeded, it became clear that something was wrong with the blood flow to his right kidney. Kidneys normally demand up to a quarter of the body's blood supply to perform their two functions of cleaning the blood and producing urine. If a kidney is not getting the amount of blood it needs, it sends a hormonal message to the body to get more blood. The way the body responds is to raise the blood pressure.

"This left us with a difficult situation,'' Dr. Primack said. "It explained the high blood pressure but it left us with a decision as to what to do.''

Just as in adults, high blood pressure in children can lead to heart disease or stroke. One solution is to give medications to lower blood pressure, but that is less than ideal for a child. There is very little data on what a lifetime of blood-pressure medications might mean for children. In the short term, the drugs made Nate feel tired and nauseous for the few months he needed them.

Dr. Primack took his concerns to the transplant team at UMass Memorial Medical Center - University Campus. Dr. Giacomo P. Basadonna, director of transplantation, and transplant surgeon Dr. Marc E. Uknis remove kidneys and transplant donated replacements all the time, but Nate's case presented an interesting challenge.

The easiest way to cure his high blood pressure would have been to simply remove the problem kidney. People can live a normal life with just one kidney, but what if something went wrong with his other one? No one knows why he developed renal artery stenosis in his right kidney, so his doctors couldn't rule out the same thing cropping up in his left kidney, even though examinations found no evidence of a congenital defect.

"We know from living-organ donation that people give up one kidney all the time and live a pretty normal life, but we have only about 20 years of follow-up,'' said Dr. Uknis. "Nate's an 11-year-old boy and he has a life expectancy of 60 or 70 years. We don't know what that would be like having only one kidney.''

The transplant surgeons began to craft a plan to preserve Nate's kidney by correcting the problems in the blood vessels leading into it. They called in Dr. Bruce S. Cutler, chief of the division of vascular surgery, to do it .

"It was clear what needed to be done,'' Dr. Cutler said. "But I wasn't sure that we were able to do it at that point.''

Nate's aneurysm was located in a difficult place to expose for the delicate surgery required. And his kidney is only about a third to a half the size of an adult kidney.

"We thought repairing the artery while the kidney was removed from the body and then restoring it would be the best plan,'' he said.

The surgery, called "ex vivo,'' or out of the living body, is not unusual in adult transplant surgery, Dr. Basadonna said. Surgeons sometimes need to correct donor organs that have damage or malformations before they implant them in the recipients. They do that "on the bench,'' away from the patient in the operating room.

Dr. Hans W. Sollinger, chairman of the division of transplantation at University of Wisconsin Hospital in Madison, endorsed repairing the kidney outside the body and retransplanting it, a technique pioneered 30 years ago by Dr. Folker O. Belzer, with whom he trained, at the University of California, San Francisco.

"I think it's a great decision to take the kidney out, cool it down and really take your time and do a very, very careful repair,'' he said. "The precision of reconstruction outside the body is much higher.''

Doing ex vivo surgery in a child is rare, but the UMass Memorial surgeons adapted techniques used in adults. Preserving kidney function for the time the organ was outside his body was crucial. A chilled solution was perfused through the renal arteries into the kidney in the same way that donated organs are protected from deteriorating. Then Dr. Cutler and Dr. Basadonna could proceed with repairs.

"In bench surgery, I can sit down with good light and magnification and with everything still and be able to do the best job,'' Dr. Cutler said.

So on Feb. 5, Nate was slated to become both donor and recipient of his kidney, whose blood vessels would be repaired in between. There was an outside chance only the kidney removal would be done, but his surgical team was intent on preserving both kidneys and correcting the cause of his high blood pressure.

Dr. Cutler's task was to remove the aneurysm and replace the affected artery with a vein taken from Nate's leg. That meant sewing four small branches of the artery to this substitute artery. The branches ranged from 1 millimeter to 1.8 millimeters in diameter, about the width of a pencil lead.

Dr. Cutler knew what to expect from three-dimensional arteriograms performed before surgery. Computer-generated images allowed him to rotate the kidney and the aneurysm in space to see and measure what he had to deal with.

"It's as if you're standing inside the aneurysm and can see the opening of each of the small branch arteries that come off the aneurysm,'' he said. "You can know precisely what you are going to see when the kidney and artery are removed.''

When the three-hour bench surgery was complete, Dr. Uknis returned the kidney to Nate's body. Dr. Cutler was watching to see if the four branch arteries were doing their job. The kidney, pale white when chilled, immediately became pink all over from new blood flow. He knew then that the repair was working. Another good sign came when the kidney started to make urine.

Nate's mother was waiting in agony for word. When she heard things were going well, she got into her running gear and logged five or six miles around the hospital.

"It was definitely the worst day of my life,'' she said. "Nate told me, `It's only a minute for me. I go to sleep and then wake up. But it's worse for you.' "

She asked Nate to show off the long scar curving from his lower belly, rising up under his rib cage and disappearing beneath his red T shirt. Dr. Uknis said he should tell all the girls it's a shark bite, his mother said.

Otherwise, he seems no worse for the wear, now that fatigue after such major surgery has faded. He's back at school after missing a month. He will need to have regular follow-up ultrasound exams with Dr. Cutler to check on his kidney and blood flow as he grows. His blood pressure returned to normal right away.

Dr. Primack said the biggest issue was telling Nate not to snowboard this season.

Ms. Mahitka looked ruefully at Nate showing off the Christmas snowboard. But she's thrilled to have her healthy, active son back.

"I feel like singing from the rooftops, I'm so happy,'' she said.

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