What Is the Life Expectancy for ATTR Amyloidosis

What Is the Life Expectancy for ATTR Amyloidosis?

Amyloidosis is a condition where abnormal deposits of proteins called amyloids build up in the body’s cells. These proteins are toxic to healthy tissue and cause various diseases such as Alzheimer’s disease (AD) or Parkinson’s disease (PD).

The average life expectancy of someone with amyloidosis is between 10 and 15 years, but it varies from person to person. People with the most common form of amyloidosis, known as familial amyloid polyneuropathy (FAP), have a life expectancy of around 20 years. However, some people live much longer than others.

People with other forms of amyloidosis may live anywhere from 2 to 30 years after diagnosis. There is no set rule for how long they will survive without treatment, though most patients die within 5 to 10 years after their initial diagnosis.

How Does Amyloidosis Affect the Heart?

Amyloidosis affects many organs including the heart. When amyloids accumulate in certain parts of the heart, they affect its function. For example, when amyloids build up in one part of the heart, it slows down blood flow to other areas of the body. This causes a drop in blood pressure and can lead to death if not treated quickly.

The rapid decrease in blood pressure sends fluid rushing into other parts of the heart. This fluid build up pushes against the walls of the heart and makes it bigger. Over time, this can cause the heart to fail completely, which can also lead to death if left untreated.

How Is Amyloidosis Treated?

Most treatment options are focused on the types of amyloidosis you have and how advanced it is. There are a number of organs that can fail if left untreated because of amyloid buildup. Each one will be treated differently to help slow disease progression and prevent organ failure.

Lungs and airways: If amyloids have built up in your lungs and airways, you may be given a medication to help thin the mucus in your airways so it can drain easier.

Heart: If amyloids have built up in the heart and blood vessels, you may undergo a procedure to open up the blood vessels that are blocked by amyloids. This procedure is called angioplasty. A catheter is guided through a blood vessel in your groin up to your heart. The catheter has an inflatable balloon at the tip. Once in position, the balloon is inflated and the doctor watches on a video screen for the proper location.

Once in place, the balloon is slowly inflated until it pushes against the amyloid build up. This clears the blockage and allows blood to flow through normally again.

Liver and bile ducts: If amyloids have built up in the liver and bile ducts, it can cause a liver failure. In these cases, a liver transplant may be needed.

Nervous system: If amyloids have built up in certain parts of the nervous system, such as the spinal cord or brain, symptoms can range from pain to difficulty walking.

Sources & references used in this article:

Expert consensus recommendations for the suspicion and diagnosis of transthyretin cardiac amyloidosis by MS Maurer, S Bokhari, T Damy, S Dorbala… – Circulation: Heart …, 2019 – Am Heart Assoc

Safety and efficacy of a TTR specific antisense oligonucleotide in patients with transthyretin amyloid cardiomyopathy by MD Benson, NR Dasgupta, SM Rissing, J Smith… – Amyloid, 2017 – Taylor & Francis

CNS involvement in V30M transthyretin amyloidosis: clinical, neuropathological and biochemical findings by LF Maia, R Magalhães, J Freitas, R Taipa… – Journal of Neurology …, 2015 – jnnp.bmj.com

Misfolded transthyretin causes behavioral changes in a Drosophila model for transthyretin‐associated amyloidosis by M Pokrzywa, I Dacklin, D Hultmark… – European Journal of …, 2007 – Wiley Online Library

Transthyretin cardiac amyloidoses in older North Americans by K Dharmarajan, MS Maurer – Journal of the American Geriatrics …, 2012 – Wiley Online Library

Nearly 200 X-ray crystal structures of transthyretin: what do they tell us about this protein and the design of drugs for TTR amyloidoses? by SK Palaninathan – Current medicinal chemistry, 2012 – ingentaconnect.com