In case you haven’t picked up your copy of SURGEON’S STORY yet, here’s a little excerpt to give you just a taste of what you’re in for when you step into the OR with Dr. Kristine Guleserian… not your average cases at all…
PATIENT: JENNIFER N.
Jennifer had her first heart attack at the age of two. It’s understandable. The upper limit for “normal” blood cholesterol levels is around 200 mg/dl (milligrams per deciliter). Jennifer’s cholesterol level was 1,020. She had an inherited disorder called homozygous familial hypercholesterolemia. Her liver couldn’t metabolize cholesterol. It is, literally, a one-in-a-million diagnosis. Both her parents carry the gene for the condition, and her maternal grandmother died of a heart attack at age 31. Her older brother Frankie was being treated for a milder form of the disorder.
At first her parents thought the scabs on her tiny ankles were just a skin condition. But her Miami pediatrician recognized it as a telltale sign of the disease she carried. The family moved to Texas to take advantage of new treatments being offered, including advancements in plasmapheresis, a filtering of blood plasma to remove cholesterol. Jennifer had her first three treatments at Cook Children’s Hospital in Fort Worth, and everything looked good. She had her heart attack during the fourth procedure. She was sent the 35 miles to Children’s Medical Center where, after they finished a lengthy workup, doctors told her family that Jennifer needed a rarely performed double transplant. She’d get a new heart and a new liver in the same surgery.
Drs. Michael Brown and Joseph Goldstein did groundbreaking research in cholesterol disorders at UT Southwestern Medical Center in the 1970’s. They broke down the genetics of hypercholesterolemia and found the mutation that created it. Their work won the Nobel Prize for Medicine in 1985, and Jennifer was a beneficiary of their efforts. She was admitted to Children’s in July, 2006 and listed for transplant August 11th. And that’s when the fates began to intervene.
Another name is added to national organ transplant waiting lists every 21 minutes. Waiting for one organ is agonizing enough. Waiting for two is exponentially worse. Especially since the organs have to come from the same donor, at the same time, so both transplants can be accomplished in one surgery. Organs might not become available for weeks, months, or years, if ever. Jennifer’s parents were given a pager and told that when it went off, that would be the signal that suitable donor organs had been found. In storytelling that’s called the “ticking clock,” the added twist of an element of running time. When will the bomb explode? When will the cavalry arrive? How long until the questions are answered? Two days of waiting was more than enough for Jennifer’s father Rich. He stood in her hospital room staring at the pager. He squeezed it and angrily said, “Why won’t you go off?”
And the pager beeped.
That would be enough for most screenwriters. But now the drama grows. She needed a heart-liver transplant. When you list a patient for a double organ transplant like that, the recipient waiting for two organs has priority over somebody waiting for only one. “Two days after listing, I got the page from the OPO (organ procurement organization). I was on the phone with them and Susan Daneman,” said Dr. G, “our nurse/transplant coordinator, was with me. I told them the heart was perfect and we’d take it. Susan left to inform the family it was a go. As I said, the dual transplant patient takes priority. Just as an afterthought I asked, ‘We’re getting the liver, too, right?’ They told me they’d already placed the liver in Houston. I said, ‘I don’t think so. Get me your medical director.’ And I told him, ‘You’re going to have make a very difficult phone call to the Houston team and tell them that we get the heart and the liver.’ And that’s what happened.”
The timing was more than good. Jennifer’s left anterior descending coronary artery was completely blocked. In the adult world, that would result in the “Widow Maker” heart attack. But tough little Jennifer hung on. The transplant took place August 14th. As the donor organs made their way to Children’s, Dr. G opened Jennifer’s chest and saw how things looked. Everybody knew Jennifer was sick, but even Dr. G was surprised at how sick she turned out to be. In her post-op report, Dr. G wrote, “… the patient’s ascending aorta was extensively diseased with fatty plaque deposits … the myocardium was pale.” In other words, just in time.
When her new heart was in place, and the clamp was removed from her aorta, the heart began beating in perfect rhythm. Her sternum was closed and wired shut. But she wasn’t taken to the CVICU. Instead, another surgical team came in and gave Jennifer her new liver. Dr. G stayed
in the OR through the second surgery.
Jennifer made a little bit of history as the youngest child in the U.S. to receive a combined heart-liver transplant. Her brother Frankie, who Dr. G remembers as always being
around, and always asking questions about everything, was so impressed by the events in his sister’s life that he went to medical school.