In Italy, the death of Sammy Basso, a man suffering from progeria, or Hutchinson-Gilford syndrome, generated a flood of tributes. Aged 28, he was the oldest patient suffering from this incurable and extremely rare disease. For those who have it, the average life expectancy is 13 years.
Progeria, derived from the Greek “geron” for “old man”, sometimes also called Hutchinson-Gilford syndrome, or “Benjamin Button syndrome” is an extremely rare disease. But unlike the film in which Brad Pitt plays the leading role, those affected do not get younger over time.
Risks of early cardiovascular disease
Resulting from a mutation in the LMNA gene, progeria causes accelerated aging from the first or second year of life. Hutchinson-Gilford syndrome affects one in 4 to 8 million births. In 2009, Inserm listed 3 cases in France, 25 in Europe and around a hundred worldwide. The Progeria Research Foundation estimates that 400 children suffer from this disease.
Symptoms include stunted growth, alopecia, and a characteristic facial morphology marked by a small head, small jaws and a pinched nose. Also, the teeth do not grow properly, the venous network appears little by little and the nails are atrophied. “Children reach on average a maximum height of 125 cm and 25 kg (…) and have a remarkably similar appearance despite different ethnic origins,” explains the Progeria Research Foundation.
As children age, they develop accelerated atherosclerosis (the formation of plaques in the inner lining of arteries) and cardiovascular disease. Mental development, however, is not affected. Progeria is currently incurable.
Even if this disease remains extremely rare, Sammy Basso participated in its media coverage. Thus, Giorgia Meloni, the head of the Italian government, paid tribute to him: “His commitment to research on progeria and his ability to inspire others will forever remain a model to follow,” she wrote in particular on The Italian singer Jovanotti praised him on Instagram “his intelligence, his passion, his culture and his ability to harmonize scientific knowledge with unshakeable faith, his formidable humor and his colorful spirit come to mind”.
The American YouTuber Adalia Rose Williams, who died in 2022 at the age of 15, also suffered from progeria and played a large part in giving more visibility to this rare disease. His YouTube channel still has more than 3 million subscribers.
