Lacy Neff, a radio broadcaster from West Virginia, recently underwent a heart transplant as part of his treatment plan for amyloidosis, a rare disease that occurs when amyloid proteins accumulate in the organs. The MGH is one of only seven hospitals in the United States studying cardiac amyloid transplants – and the only one in the Northeast.
The journey home
NEFF, RIGHT, AND HIS WIFE DANA
Lacy Neff smiles every time he sees a ladybug. He adopted an affinity after the recent death of his mother – an avid admirer of all things resembling the brightly colored beetle.
“Every time I got good news – like my amyloid count went down – I would see a ladybug,” he says.
Neff, 48, has amyloidosis, a disease few have heard of and often can’t pronounce, so sometimes it is simply called “amyloid.” But there is nothing simple about this disease.
“Light chain amyloidosis occurs when components of antibodies called light chains form amyloid deposits,” says Marc Semigran, MD, director of the MGH Heart Failure/Cardiac Transplant Program in the Institute for Heart, Vascular and Stroke Care. “These abnormal proteins build up in one or more of the organs in the body, including the kidneys, liver, spleen, nervous system – and in Lacy’s case the heart.”
A fairly rare disease, Semigran and his team see roughly 30 amyloidosis patients a year, often sent by specialists from around the globe. “Some of these patients are referred from the Boston University Amyloid Center, with which we jointly work to treat patients with advanced cardiac amyloid,” says Semigran.
GARCIA, LEFT, AND SEMIGRAN
The MGH is one of only seven hospitals in the United States studying cardiac amyloid heart transplants – and the only one in the Northeast.
“We have become a referral center,” Semigran says. “Amyloid is a serious disease that moves at a rapid pace and often is not caught soon enough. In my opinion, anyone with heart failure without a clear underlying cause should be assessed for amyloid.”
So without hesitation, the day before Thanksgiving, Neff and his wife of two years, Dana Neff, left their jobs, packed their things, and traveled nearly 600 miles to live a stone’s throw away from the MGH.
That was eight months ago.
Neff’s amyloidosis journey, however, didn’t begin in Boston. It began in Morgantown, West Virginia, where pretty much anyone who listens to the radio knows Lacy Neff. For more than 25 years, he has been an on-air personality at WVAQ. On Oct. 2, 2013, his public life turned into a private battle.
“I couldn’t walk without taking a deep breath or getting dizzy, and if I stood up quickly I would pass out,” he says.
Neff was in and out of his hometown hospital before he was sent to another hospital in Pennsylvania for more tests.
A heart biopsy confirmed amyloidosis.
“I had less than a year to live,” Neff says. “My heart looked like shattered glass. The amyloid was like little swords going into my heart and sticking it. That’s when we began ‘the tour’ to try to find a cure.”
At a hospital in Ohio, Neff learned his heart was functioning at 12 percent and also learned he was not only battling amyloidosis but also multiple myeloma, a cancer of the plasma cells. Neff needed more than just chemotherapy; he needed a heart transplant and a stem cell transplant to treat both. He was referred to the MGH.
“We committed to stay in Boston for a year. It was one of the conditions,” says Neff. “Dr. Semigran asked if we were willing to do what it takes to care of my heart – I said, ‘Absolutely.’”
There was some debate as to whether Neff would qualify for the transplant list because it was the first time the MGH had an amyloid transplant candidate who also had multiple myeloma.
“The MGH has achieved the reputation as a center of excellence where a good number of patients received a heart transplant followed by bone marrow stem cell transplantation, and a great majority of them are doing very well years after these combined strategies,” says Neff’s hematologist and bone marrow transplant physician, Bimalangshu R. Dey, MD, PhD, of the Division of Hematology/Oncology. “For the MGH to continue to lead the way in the treatment of cardiac amyloidosis we have to learn to deal with unusual cases such as Lacy’s – they are becoming more and more common.”
As he waited in the hospital, Neff was visited by another member of his care team, Jose Garcia, MD, surgical director of the Cardiothoracic Transplantation and Artificial Heart and Lung Program.
“When I met Dr. Garcia, I asked him the stupid question of how many transplants he has done – he’s done hundreds, so I said ‘that’s the guy I want.’”
On Dec. 29, Neff was placed on the transplant list. Almost four months later he received a new heart.
“If there’s a better hospital, you’ve got to show me,” he says. “Dr. Semigran is my Superman.”
Neff’s journey is not over. Now he must wait another four months so he can gain strength and weight for a stem cell transplant, which Semigran hopes will wipe out the amyloid for good. “There is a 30 percent possibility of recurrence, but I think Lacy’s prognosis is excellent,” he says. “We assess all of our patients regularly for recurrence of amyloid protein production. The development of new drug therapies now allows us to suppress abnormal light-chain production before it can impair a patient.”
As Neff waits, he and his wife – for the first time in a long time – think about the future. He often reflects on a special moment while recovering from his heart transplant.
“A nurse brought her little girl for a visit,” he recalls. “She came into my room and told me, ‘I want to draw you a picture.’ I said, ‘I would love that.’ She looked at me, seriously, and asked ‘Do you like ladybugs?’ I looked at Dana who began to cry, and I said, ‘You know what? I love ladybugs.’”
Read more articles from the 08/01/14 Hotline issue.