What Is the Life Expectancy With ATTR Amyloidosis?

Getting diagnosed and treated for transthyretin amyloidosis as early as possible is key to adding years to your life.

Transthyretin amyloidosis (ATTR) is not one disease, but a cluster of related conditions. At their core, they stem from a disorder that develops when normal proteins produced by the liver suddenly begin to clump together. Eventually, these abnormal protein clusters build up in or around your organs or tissues.

In some cases, the buildup can happen in the heart, causing the walls to stiffen and weaken, so it’s harder for the heart to pump blood throughout the body. In other cases, the buildup occurs in the nerves or the lining of the brain and spine. ATTR can also affect your kidneys.

To make things even more complicated, ATTR can be inherited: The genetic mutation that causes this buildup of proteins can be passed down in families. In the United States, this mostly affects Black Americans. There’s also another kind, known as wild-type ATTR, that develops for no known reason.

Because ATTR comes in a lot of different forms, gauging life expectancy following a diagnosis is tricky. “Life expectancy varies widely,” says Amgad Makaryus, MD, a professor of cardiology at the Zucker School of Medicine at Hofstra/Northwell in Hempstead, New York. It also depends on a number of factors.

For example, wild-type ATTR usually shows up much later in life, and progresses more slowly, than hereditary ATTR, Dr. Makaryus explains. So life expectancy tends to vary by type, he notes.

“Other factors influencing life expectancy include how early the disease is diagnosed and, therefore, how early treatments may be employed,” Makaryus adds.

While predicting the course of ATTR is difficult, research has revealed some rough estimates for how long someone can live following a diagnosis.

What the Science Says About Life Expectancy

While ATTR can affect any part of the body, it most commonly shows up in either the heart or the peripheral nerves, which branch out from the brain and spinal cord. When the heart is affected, people have ATTR cardiomyopathy (ATTR-CM), which often leads to heart failure.

When ATTR develops in the peripheral nerves, it’s called ATTR polyneuropathy (ATTR-PN). People with ATTR-PN have a range of symptoms, including sensitivity to cold, numbness, and digestive issues.

The type you have, among other factors, can affect how long you’ll live with the disease.

Life Expectancy With ATTR-CM

For people with wild-type ATTR-CM, diagnosis usually occurs very late in life — normally after age 70. Without treatment, researchers have estimated that people with ATTR-CM live, on average, 5 to 7 years following diagnosis.

“Life expectancy with the hereditary form can vary widely, depending on the specific mutation, the age of onset, and other health factors and comorbidities,” Makaryus says. If the condition is left untreated, the average life expectancy for people with hereditary ATTR-CM is 7 to 10 years following diagnosis, the same research shows.

Of course, treatment can make a difference for people with ATTR-CM. The earlier treatment begins, the better the prognosis, Makaryus says. But again, that also depends on other factors, including:

The stage of the disease: People diagnosed with ATTR at stage 1 lived longer than those diagnosed at later stages in one study. Meanwhile, body mass index and measures of heart function were also related to changes in life expectancy, according to the study.
Your health: “Overall health of the patients matters,” Makaryus says. So does age and the type of mutation driving the ATTR, he adds.

Life Expectancy With ATTR-PN

Studies have shown that people with untreated ATTR-PN live for roughly 10 years after the start of the disease. The genetic mutation you have can also affect how long you’ll live.

But treatment does make a difference. A study published in 2020 found that people who were treated long term lived about 8 to 9 years longer. The researchers estimated that was 11 to 14 years after onset of the disease.

A liver transplant can also boost survival and sometimes add decades to someone’s life, versus not being treated at all.

Living Longer With ATTR

Staying on top of the condition with a doctor’s guidance is one of the best ways to add years to your life.

“It is essential for patients diagnosed with ATTR to work closely with their medical provider to manage symptoms, receive appropriate treatment, and monitor the progression of the disease,” Makaryus says.

Treatments can include stabilizer medications that try to stop the abnormal buildup of proteins, as well as drugs that lower the production of those proteins in the liver, says Makaryus. An organ transplant, such as a new heart or liver, is another form of life-extending medical treatment, he adds.

Lifestyle changes can also help boost survival. For people with ATTR-CM, Makaryus says that maintaining a healthy diet, exercising regularly, and not smoking can all help improve life expectancy and quality of life.

Promising new research on ATTR is also on the horizon, Makaryus adds. “Most importantly, people need regular follow-ups with their physician,” he says. That way, you’ll be able to know about new treatments and ways to live longer and better with ATTR.

The Takeaway

Life expectancy with ATTR varies widely from person to person. It’s based on the type you have, its severity, how quickly it progresses, and how early it’s diagnosed and treated. Staying proactive with treatment and adopting a healthy lifestyle can have a positive impact on your longevity with ATTR.

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