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2024-10-22 19:35:30 UTC
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Permalink"Sammy Basso, Advocate for Progeria Research, Is Dead at 28"
"One of about 150 people known to have a rare condition that causes
rapid aging, he sought to raise awareness and participated in the search
for a cure."
By Trip Gabriel
Published Oct. 19, 2024
Sammy Basso, an advocate for research into progeria, an ultrarare fatal
disease that causes rapid aging in children, who was known for living
with gusto and humor with the condition as he faced the certainty of
premature death, died on Oct. 5 near his home in Tezze sul Brenta, in
the Veneto region of northern Italy. He was 28.
Dr. Leslie B. Gordon, medical director of the Progeria Research
Foundation, for which Mr. Basso served as global ambassador, said the
cause was complications of the disease. Mr. Basso was the oldest known
person living with classic progeria.
Mr. Basso, who lived with his parents, was diagnosed with progeria at
age 2.
He was one of only about 150 people worldwide identified with the
condition. He traveled internationally, most recently to China, to raise
awareness; gave TED Talks; and participated with scientists from Harvard
and the National Institutes of Health in a research group that is
seeking a cure.
“You couldn’t watch a presentation by Sammy without being captivated by
his courage, his spunk, his smarts and his sense of humor,” Dr. Francis
S. Collins, a former N.I.H. director who has long researched progeria,
said in an email.
Progeria, also known as Hutchinson-Gilford progeria syndrome, causes
children to undergo rapidly accelerated aging. Its effects include
baldness, wrinkled skin, hardening of the arteries and a wizened
stature. Mr. Basso was about 4 feet 5 inches tall and weighed about 44
pounds.
Individuals with the condition, whose average life expectancy is 14.5
years, do not experience senility. Profiled in The New York Times in
2022, Mr. Basso joked that mental clarity in the face of certain
premature death was either a blessing “or a damnation.”
The article pointed out that Mr. Basso, who founded the Italian Progeria
Association Sammy Basso, had become “one of Italy’s most recognizable
advocates for science and human dignity, regardless of age.” Pope
Francis called him by phone while he was in high school, and President
Sergio Mattarella of Italy awarded him a knighthood in 2018.
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Mr. Basso inspired Sammy’s Runners, a group of friends who ran marathons
to raise awareness of progeria while pushing him in a wheeled chariot.
He was also a bit of a prankster. He once posed outside a U.F.O. museum
in Roswell, N.M., in green “alien” eyeglasses, which accentuated his
egg-shaped head, to make tourists think he was a real visitor from outer
space.
“He made everyone around him feel comfortable with him and with
progeria,” Dr. Gordon said. “All he had to do was say two words and
you’d be smiling and laughing.”
A group of people running on a track in matching tie-dye T-shirts and
pushing Mr. Basso in a wheeled chariot.
The club Sammy’s Runners ran marathons to raise awareness of progeria
while pushing Mr. Basso in a wheeled chariot.Credit...Nadia Shira Cohen
for The New York Times
Sammy Basso was born on Dec. 1, 1995, in Schio, Italy, to Amerigo Basso,
a factory worker, and Laura Lucchin, who helped run his progeria
association. His parents are his only immediate survivors.
At age 12, Sammy participated in the first clinical trial, at Boston
Children’s Hospital, of a drug to slow progeria’s progression.
Dr. Collins credited that medication, lonafarnib, which the Food and
Drug Administration subsequently approved, with Mr. Basso’s relative
longevity, along with the fact that he “took really good care of
himself.”
He graduated from the University of Padua with a degree in natural
sciences in 2018, and three years later he earned a master’s in
molecular biology from the same university.
Mr. Basso was a patient, a research subject and a researcher of his
condition.
“The fact that the science of progeria has advanced so much is in part
due to Basso himself,” the medical news site Stat wrote after his death.
The latest frontier of progeria research is a gene-editing technique
developed by Dr. Collins, Dr. David Liu of Harvard and others. It would
repair a mutated gene that causes progeria and reverse its premature
aging. Scientists have shown the technology to work in mice.
The U.S.-based Progeria Research Foundation is working toward F.D.A.
approval for a human trial and hopes to raise an estimated $30 million
to make a drug privately, since ultrarare diseases do not interest
commercial drug companies.
Progeria affects only about one in 20 million people. At the same time,
there are some 7,000 genetic diseases with known mutations, and 85
percent are also ultrarare, affecting fewer than one in a million
individuals. It is hoped that a progeria breakthrough could help point
the way to cures for other diseases.
The researchers, who include Dr. Collins and Dr. Gordon, hold a video
call every Monday. Mr. Basso would join them from Italy. He kept the
group’s minutes and participated in scientific deliberations.
He once told The Times that he hoped his visibility would encourage
others with progeria to seek treatment and avoid despair.
“The thing I would like them to learn by my experience is that their
life is important,” he said. “They can be helpful for the world.”