Does Friedreich Ataxia Affect Life Span? Can It Be Fatal?

A diagnosis of any condition can leave you wondering how it may affect life span, and Friedreich ataxia (sometimes abbreviated FA or FRDA) is no exception. Friedreich ataxia is a progressive degenerative condition, meaning it gets worse over time.

Fortunately, new and improving treatment options are helping people with Friedreich ataxia live longer. However, the average life span is still shorter than that of the general population.

Keep reading to learn how Friedreich ataxia affects life expectancy and what steps may help lower the risk of early death.

How Long Does Someone With Friedreich Ataxia Live?

The average life expectancy for someone with Friedreich ataxia is 40 to 50 years, although some people live into their 60s or longer. Life expectancy may be shorter than in the general population because of complications that arise as the condition progresses.

As treatments for Friedreich ataxia and its complications continue to improve, average life expectancy is increasing. Studies from the 1980s estimated the average life expectancy for people with Friedreich ataxia to be in the mid-30s. Today, the average has increased by about 10 years.

What Factors Affect Life Span With Friedreich Ataxia?

Not everyone with Friedreich ataxia has the same life expectancy or risk of premature death. Several factors can affect life expectancy with Friedreich ataxia.

Condition Severity

The severity of Friedreich ataxia and its complications can affect how long someone is expected to live. More severe symptoms and a wide range of complications, including diabetes and heart disease, may reduce life expectancy.

Age at Onset

About 85 percent of people with Friedreich ataxia are diagnosed between ages 5 and 25. In rare cases, the condition is diagnosed in infancy or later in adulthood.

People diagnosed at younger ages typically have a shorter life expectancy than those diagnosed in late adolescence or in adulthood. Early-onset Friedreich ataxia (when symptoms begin before age 7) is linked to faster disease progression and more severe ataxia symptoms.

Heart Involvement

Heart complications are the leading cause of early death in people with Friedreich ataxia. More than half of people with the condition develop heart complications, and those who do tend to have a shorter life expectancy.

What Causes Premature Death in Friedreich Ataxia?

Friedreich ataxia can be fatal because of life-threatening complications. Because Friedreich ataxia is progressive, many people develop complications over time.

Cardiac Dysfunction

Congestive heart failure and arrhythmias (abnormal heart rhythms) that lead to heart failure account for over 50 percent of deaths in people with Friedreich ataxia. Many people also develop other heart conditions that can affect life expectancy, including ischemic heart disease (narrowing of the heart’s arteries) and hypertrophic cardiomyopathy (thickening of the heart muscle).

Aspiration Pneumonia

As Friedreich ataxia progresses, increasing nervous system dysfunction can make it harder to chew and swallow. This can lead to aspiration pneumonia, a serious lung infection that happens when food, liquid, or saliva enters the lungs instead of the stomach.

Diabetes Complications

Many people with Friedreich ataxia eventually develop diabetes, which can cause life-threatening complications if it isn’t well managed. One possible complication is a diabetic coma, which can result from dangerously high blood sugar or dangerously low blood sugar.

How Can You Reduce the Risk of Premature Death in Friedreich Ataxia?

If you or a loved one has Friedreich ataxia, you can take steps to help lower the risk of early death while improving quality of life. Managing Friedreich ataxia and any complications can improve your prognosis (outlook) and may help you live longer.

Routine Cardiac Monitoring

Routine cardiac monitoring involves regular tests to check how well your heart is working. These tests can help detect heart problems as quickly as possible so your care team can treat them. Your Friedreich ataxia specialist may recommend the following tests:

• Electrocardiogram (ECG) — An ECG measures the heart’s electrical activity and can detect arrhythmias. Many doctors and researchers recommend having an ECG at least once a year.
• Echocardiogram — An echocardiogram uses sound waves to create moving images of the heart. Getting an echocardiogram at least once a year is generally recommended.
• Cardiac MRI scan — This imaging test provides detailed pictures of the heart muscle. It can show changes such as thickening and scarring.

Treatment of Heart Complications

Your Friedreich ataxia specialist may prescribe medications such as ACE inhibitors or angiotensin 2 receptor blockers to help manage heart complications.

If you develop heart failure, your doctor may recommend an implanted device, such as a pacemaker, to help your heart work properly. In severe cases, a heart transplant may be an option.

Diabetes Screening and Management

Because around 30 percent of people with Friedreich ataxia develop diabetes, many health experts recommend annual diabetes screening. This usually includes blood tests that measure blood sugar levels, such as a fasting glucose test.

If you or your child develops diabetes, treatment should become part of your overall Friedreich ataxia care plan. Diabetes management focuses on keeping blood sugar in a healthy range and may include insulin or other medications.

Mobility and Swallowing Support

As Friedreich ataxia progresses, you or your child may need extra support for mobility. On average, people need a wheelchair about 10 years after symptoms begin. Mobility aids, occupational therapy, and physical therapy can help lower the risk of falls and serious injuries that can happen when Friedreich ataxia affects walking.

Speech therapy can help with swallowing problems as Friedreich ataxia progresses. This may reduce the risk of choking and aspiration pneumonia.

Support From a Neuromuscular Specialty Center

Getting care at a neuromuscular or ataxia specialty center can help you or your child receive comprehensive care. Specialists have experience in treating Friedreich ataxia and can offer personalized treatment recommendations.

Specialists can also connect you with a multidisciplinary Friedreich ataxia care team that may include neurologists, cardiologists, and other specialists, since the condition affects multiple body systems.

Genetic Counseling for Families

If your child was recently diagnosed with Friedreich ataxia, genetic counseling can help you better understand the diagnosis and what to expect. Genetic testing can confirm the diagnosis and may help guide treatment.

If you’re planning to have children, genetic testing can show whether you’re a carrier of Friedreich ataxia. A genetic counselor can explain the results and discuss your family planning options.

Talk With Your Doctor

Talk to your doctor or healthcare team about your or your child’s life expectancy with Friedreich ataxia. Because Friedreich ataxia affects everyone differently, treatment should be individualized. Your care team can recommend screenings and treatments that may help you live longer and maintain a better quality of life.

Join the Conversation

On MyAtaxiaTeam, people share their experiences with ataxia, get advice, and find support from others who understand.

How do you manage Friedreich ataxia and its complications? What has helped improve your quality of life? Let others know in the comments below.