New Heart Saves Patient with Amyloidosis

groth-banner.jpg

Ray Groth’s first significant interaction with University of Utah was in the 1960s as a student and football star. He came to Salt Lake City from Idaho Falls as a starting quarterback, and in 1970 the St. Louis Cardinals drafted him. He didn’t make it in the NFL, but that didn’t end his passion for football. To stay around the game he loves, he began coaching, first at the high school level, then in college, while raising three children with his wife Deidra.

Ray always loved working out, and when the grueling football workouts ended, he started running marathons. His brother eventually got him into cross-country skiing, where he excelled. During a cross-country training session about four years ago, he first noticed a problem.

“[During one race,] I took off and saw a guy up ahead and thought, ‘I’m going to try and keep up with him’. I came to the first hill and bonked out. That was a struggle, and it shouldn’t be—races are hard, but not that hard.”

He wasn’t sure exactly what the problem was, but having spent much of his life pushing his body to its physical limits, he knew something was off. He went to a doctor for some tests, but they couldn’t find anything.

Things continued to deteriorate for Ray during the winter of 2017. “I took off one day skiing with my wife and I couldn’t keep up with her,” he said. “I couldn’t breathe. I had a race three days later and nearly passed out on the course.”

That was the catalyst for his second significant interaction with U of U, this time at U of U Hospital. He saw Dr. Roger Freedman, a cardiologist who put him through a series of tests and noticed on the MRI that Ray’s heart walls were thickening. Dr. Freedman called his colleague, Dr. Jose Nativi-Nicolau, a cardiologist and director of the Amyloidosis Program at University of Utah Health and Huntsman Cancer Institute. Dr. Nativit-Nicolau recommended a heart biopsy to test for a rare but very serious heart condition called amyloidosis

Ray’s diagnosis was wild-type ATTR amyloidosis. His body produces excess protein chains called amyloid fibrils that cannot be absorbed or eliminated naturally. These fibrils were accumulating in his heart, causing the heart walls to thicken. There is no cure for amyloidosis, and in 2017 there was no way to slow or stop the disease progression. Without a heart transplant, the condition would be life-threatening.

“This disease is rare and can affect how long you’re going to live if it’s not treated,” Dr. Nativi said. “At the time we diagnosed him, there were no medications approved to treat it, so it was a devastating situation for Mr. Groth and his family. He was physically active all his life, and now he was facing a deadly condition.” 

At age 70, Ray was older than a typical heart transplant patient. But Dr. Nativi started collaborating with Dr. Craig Selzman, chief of the Division of Cardiothoracic Surgery at U of U Hospital, to discuss the possibility. This treatment isn’t common for amyloidosis patients because most are very sick by the time they get diagnosed. And their prognosis, even with a new heart, is not good.

“Historically, someone over 65 wouldn’t get a transplant,” Dr. Selzman said. “With Ray, you got the sense that he was someone who has fought his entire life. He was one of those guys that seems younger than his actual age, his level of activity, and his general attitude toward life."

“When a physician asks to evaluate a patient for advanced therapy, they go through a series of interviews—financial, nutrition, social work, psychology, pharmacy, and others—then we do a lot of tests,” said Sharon Ugolini, a nurse and transplant coordinator. The information is presented to a multidisciplinary team that includes surgeons and cardiologists, who evaluate whether the patient meets the criteria for transplant. If so, they’re put in the United Network of Organ Sharing (UNOS) database, which lists every donor that comes through and every recipient waiting.

The team approved Ray’s transplant, but his physical health was deteriorating. So doctors checked him into the intensive care unit (ICU) while he waited for a donor heart. He was fast-tracked because his disease was serious and there were no therapies or procedures to mitigate it. 

Five weeks later, a young man in Ogden who was an organ donor—and a match for Ray—passed away. Waiting for a heart donor can feel excruciatingly slow, but once the heart becomes available, things speed up significantly. The new heart was on its way and, within a few hours, was in surgery. Dr. Selzman began the procedure around 1 a.m. and worked through the night to transplant Ray’s new heart. 

Doctors kept him in the hospital for eight days before sending him home—a total of about six weeks in the hospital since he first checked into ICU)—but planned to keep a close eye on him for signs of rejection.

“The risk of transplant for him was low overall, but there is always a risk of rejection,” Dr. Selzman said. “There’s a constant ying-yang of your body trying to fight off a foreign object versus trying to find some kind of symbiotic harmony to live with this thing that came from the outside.”

When Ray first heard he was getting a new heart in December, he thought he would be able to race in February. The reality, though, was much different. Within 10 minutes of arriving home after his first hospital discharge, he got a call to come back after a biopsy showed signs of rejection. A few days later, doctors found bleeding in his lungs. The medication prescribed to prevent rejection also suppressed his immune system; a month later, Ray came down with the flu and pneumonia. But his whole care team was there to help along the way.

“When you’re at [University of Utah Health], you’re in phenomenal hands,” he said. “I am so impressed with the coordination of care.”

Ray has regular follow-up appointments to monitor for rejection and check on his amyloidosis. Getting a heart transplant did not cure the disease—it only removed the damaged heart. Fortunately, new medications are available that can slow or stop amyloid fibril production. So far, there is no evidence of build-up in his new heart.

Ray also had a chance to connect with the parents of his heart donor, a decision both the donor family and the recipient get to make. They exchanged letters, spoke on the phone, and eventually met in person to visit the donor’s gravesite.

“It’s tough talking to a parent who just lost a son,” Ray said. “I’m so thrilled because I’m living—and they’re devastated.” He keeps in touch with them, sending pictures of the cross-country skiing races, bike rides, and other activities that he—and the donor—are accomplishing together.

Ray is focused on making the most of life with his new heart. “Amyloidosis is a bugger,” he said. “It’s an unbelievable disease—such a complicated thing. Every day, I just keep getting better and better, doing something that will make it stronger and healthier. Some days are diamonds, some are stones—but you keep working at it.”

Give a Gift Online

comments powered by Disqus