What is the life expectancy of someone with HGPS?

Exploring Life Expectancy in HGPS: Key Insights

HGPS (Hutchinson-Gilford Progeria Syndrome) is a genetic disorder that accelerates the ageing process in a child. It is an extremely rare condition. Children with progeria appear healthy at birth and start to show symptoms within the first two years.

HGPS does not come from parents but occurs due to a mutation in the LMNA gene. The mutated gene creates an abnormal protein called progerin. This process causes cells to become unstable and age prematurely.

This article answers all common questions about the life expectancy of people with HGPS and the promising treatments on the horizon.

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What Is The Average Lifespan Of Someone With HGPS?

The harsh reality of progeria is its fatality. The average lifespan of someone with HGPS is 14.5 years. Some children have died at 6 years, while some have lived till their mid-twenties.

HGPS does not affect brain development but compromises physical health so severely that fatal complications become inevitable. The disease attacks the body's bones, organs, and blood vessels. Death occurs as a result of heart attack or stroke.

What Is The Reason For Their Short Life Expectancy?

Hutchinson-Gilford Progeria Syndrome (HGPS) creates internal complications. The children, at an early age, develop conditions that affect a normally ageing human in their mid-40s.

• Atherosclerosis: Plaque buildup in the inner lining of the arteries narrows the path of blood flow, disrupting the flow of blood and causing heart attacks.
• Spontaneous bone fractures: People with HGPS have rapidly declining bone density, leading to osteoarthritis and broken bones. Hip dislocation is another common complication of this disease.
• Insulin resistance issue: In progeria, the body stops responding to the insulin produced in the pancreas, leading to diabetes and heart issues.

Are There Any Treatments to Increase Life Expectancy?

There is no cure for Hutchinson-Gilford Progeria Syndrome (HGPS). However, recent research has shown promising results that can increase the average lifespan by at least 2.5 years.

Lonafarnib (Zokinvy), developed for cancer, is seen as having a positive result on people with progeria. The drug prevents the abnormal buildup of progerin in cells, thus helping to improve the symptoms of the disease. A patient, 11 years on Zokinvy, has lived 4.3 years longer.

Besides medications, regular cardiovascular monitoring, physical therapy, and a healthy diet are crucial.

Has Anyone With HGPS Lived Beyond Their Average Age Expectancy?

Although the life expectancy is low, some people with progeria have lived till their late 20s. Sammy Basso was diagnosed with HGPS when he was 2. He lived till the age of 28 and died in 2024. He was the longest-known survivor of progeria, living beyond the average life expectancy for individuals with the condition.

Progeria doesn't come from family, which makes it difficult to predict. The diagnosis of a child with HGPS is devastating news for the family. But there is hope.

Researchers all over the world are working on gene modification and modern drugs. With early diagnosis, regular monitoring, recent medications, and support from family, an improved and healthier life is possible for someone with HGPS.

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