Patients: Terence Boyle, 53, lawyer; Brendan Boyle, 52, reference librarian; Patricia Spencer, 52, nurse; Mary Jane DiPaolo, 48, division director at Catholic Charities
Doctor: Dr. Alan Benvenisty, director of renal-transplantation program, St. Luke’s–Roosevelt Hospital Center
Benvenisty: The family has polycystic kidney disease, a heritable illness that can lead to renal failure, which can affect your heart, mental status, electrolytes, and even result in sudden death—it’s one of the more deadly genetic diseases. Their father had the disease. He died in his late thirties and passed it on to his children. Usually, not all the children will get it, but in this case, they all got it. I’ve performed six transplants on them. I’ve known them for 22 years.
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Terence: I was first diagnosed in 1972, when I was 19 years old. I had a summer job at a General Motors assembly plant and they gave me the perfunctory physical and one of the things they did was take my blood pressure, which was very high. The doctor had me fill out a form, and I put down that my father had died from polycystic kidney disease. He suggested that I see another doctor, and eventually we were all tested—my brother, Brendan; his twin sister, Patricia; and my youngest sister, Mary Jane. There was no indication that Brendan and Patricia had the disease. Mary Jane had the disease but she showed no symptoms.
Benvenisty: What happens is, as the patient ages, cysts gradually appear on the kidney and enlarge over time. You can usually diagnose it by the time the patient is 25. In this particular family, the kidneys grew to an impressive size. Terence’s kidneys were massive—each one was more than 20 pounds. A normal kidney weighs about a quarter of a pound, half a pound at the most. Mary Jane’s kidney was 26 pounds. Brendan’s kidneys are about three or four times normal size. Pat’s were not impressively enlarged.
Terence: I’ve had four kidney transplants. I’m pretty sure I’m the patient Dr. Benvenisty has operated on the most—unless someone else has come along in the meantime.
Benvenisty: Terry’s first transplant was in 1985. Ultimately, the transplant failed. Then in August of 1987, he had a second transplant. This one was a perfect match. But many kidneys fail over time.
Terence: And this particular kidney—the second one—was never ideal. A kidney has two arteries and a week after the transplant, the bottom artery collapsed. So I was really operating for those eleven years on two-thirds of one kidney.
Benvenisty: In 1998, I transplanted him with a kidney from his former wife. At the same time, I removed the second transplant kidney to make room for the third one. Then that didn’t do well. He developed thrombotic microangiopathy, an inflammatory condition of the blood vessels in the kidney that can cause kidney damage. His last transplant was in 2002. Brendan and Patricia’s surgeries, in 1997 and 1998, were pretty routine. They had one kidney transplant each.
Mary Jane: I was diagnosed when I was 15 and had kidney failure at 46, in 2004. My brothers and sister were all shocked and disappointed. I guess everybody thought it wasn’t going to happen to me ’cause I’m the baby. It was a big disappointment for everyone because no one was spared.
Benvenisty: Mary Jane was very ill with uremia when she finally got her transplant. Uremia is when your kidneys are not filtering things properly and toxins build up. She was very weak, had shortness of breath, was anemic, and was losing weight. I had to remove the kidney at the same time I did the transplant. Most of the time, the native kidneys stay in their place in the flank and the new kidney is placed right above the groin. But in this particular case, the kidneys grew so large that there was no room.
Terence: When each of them got their transplants, I was in the hospital that day.
Benvenisty: The whole family is very sensitive to medication. Terry and Mary Jane developed thrombotic microangiopathy. It’s thought to be a reaction to one of the medications. I was on guard with Mary Jane because Terry had thrombotic microangiopathy, so when it happened to her, I knew how to deal with it.