Dempsey’s Encouraging Words: “There’s Help Out There!”

Dempsey Worner’s atrial fibrillation (AFib) turned out to be caused by transthyretin amyloid cardiomyopathy (ATTR-CM). Grateful for his ATTR-CM diagnosis and treatment, he wants to educate others about the condition.

A former high school football player, Dempsey Worner stayed fit and active throughout his life, working out three times a week and swimming every morning while at his lake cabin in Minnesota, where he and his wife, Kathie, have spent summers since they retired.

So Dempsey (83), previously diagnosed with heart failure, was caught off guard when, during a visit to his cabin in 2015, he could only swim a few feet with his head in the water before he had to come up for air. Even walking up a small hill to the cabin left him short of breath.

At his next bi-annual checkup, Dempsey mentioned his symptoms to the nurse practitioner, who ran a series of tests before diagnosing him with atrial fibrillation or AFib, a heart rhythm disorder that increases the risk for stroke.

To bring his heartbeat back into the proper rhythm, doctors performed a cardioversion, using electrodes to shock his heart. At first, it seemed to work, but just a few days later, the irregular heartbeat returned.

Looking for answers, he made an appointment with a cardiologist at a leading clinical center, who confirmed his AFib diagnosis, adding, “But that ain’t your problem,” Dempsey recalled. The doctor then explained to Dempsey that he had an often-misdiagnosed condition called transthyretin amyloid cardiomyopathy (ATTR-CM), a serious and underdiagnosed type of amyloidosis in which a protein called transthyretin (pronounced trans-THY-re-tin) becomes unstable and misfolds and can build up in the heart, nerves, and other organs over time.

The amyloid protein deposits cause the heart walls to become stiff, resulting in the inability of the left ventricle to properly relax and fill with blood and adequately squeeze to pump blood out of the heart.

Adding to the shock, the cardiologist told Dempsey there was no pharmacological treatment at the time of his diagnosis.

“It was profound,” he said. “What do you mean it’s not treatable? Am I going to die? A whole lot of things went through my head.”

There are 2 sub-types of ATTR-CM, wild-type, which occurs as you age and the hereditary form of ATTR-CM (hATTR-CM), which is caused by a gene mutation. 

Some people with wild-type ATTR-CM have limited symptoms while others develop end-stage heart failure.

Affecting mostly men, wild-type ATTR-CM can also cause a host of seemingly unrelated conditions, such as bilateral carpal tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture and neuropathy, or tingling and numbness in the extremities. 

A heart biopsy confirmed the ATTR-CM diagnosis, and genetic testing revealed Dempsey had the wild type of ATTR-CM.

“They don’t know where it came from,” he said. “In other words, stuff happens.”

In Dempsey’s case, the condition caused his heart to work less efficiently, causing fluid to build up around it.

Unlike others with ATTR-CM, however, Dempsey never experienced carpal tunnel syndrome or lumbar spinal stenosis, and he only has minor, infrequent tingling and numbness in his hands.

“I have to make a conscious effort to hold items as they have a tendency to slip out of my hands,” he said.

A former schoolteacher and college professor, Dempsey made it his mission to educate others about amyloidosis, speaking at seminars and doing local radio and TV interviews for World Heart Day.

“If sharing my experiences can help even one person, I’m happy to do it,” he said. “It’s important to be diagnosed early and accurately.”

“The medical profession is increasingly learning more about the different kinds of heart disease, so stay positive and live your life,” he said. “After all, I’m living proof that early diagnosis and proper treatment makes a difference. There’s help out there.”