Adalia Rose Williams, a teenager followed by more than two million people on Youtube, died at the age of 15. The girl suffered from Hutchinson-Gilford syndrome, a rare genetic disease.
- In France, 3 cases of progeria have been reported. In Europe, this disease affects 25 people and worldwide, 100 patients suffer from it.
- Much research is currently being conducted on Hutchinson-Gilford syndrome to find effective treatments.
“On January 12, 2022, Adalia Rose Williams was released from this world. She was born into it and left it quietly, but her life was far from it.” These are the words used by the family of the famous youtubeuse, Adalia Rose Williams, in a publication on Instagram, to announce her death. The teenager, who shared her life on social networks, succumbed to Hutchinson-Gilford syndrome, also called “progeria”. It is a genetic disease that is characterized by premature aging, from an early age.
This pathology was detected in the American, originally from Texas, at the age of three months. Died at the age of 15, the young girl had just exceeded the average life expectancy of people suffering from this condition, set at 13 years. On the various platforms, Adalia Rose Williams was compared to Benjamin Button. A protagonist of The Curious Case of Benjamin Button who was born at age 80 and gets younger as the years go by.
“She touched millions of people and had a huge impact on everyone who knew her. She is no longer in pain and is now dancing to all the music she loves. We would have liked so much that it was not the reality but , unfortunately, it is”wrote her family, before thanking the Internet users who supported her and the health professionals who took care of the youtubeuse.
One in 4 to 8 million births
According to Inserm, one in 4 to 8 million newborns has Hutchinson-Gilford syndrome. This pathology is caused by the de novo mutation (not present in the parents) of a gene called “LMNA”. The latter was identified in 2003 by French researchers. “When the mutation occurs, this gene produces a truncated protein, called progerin, which remains anchored in the membrane of the cell nucleus, accumulates there, and ultimately leads to its deformation and dysfunctions”, can we read on the institute’s website.
Premature and accelerated aging
Children with progeria suffer from symptoms suggestive of premature aging. Manifestations include alopecia (loss of body hair), thin, hairless skin, cardiovascular disorders, joint pain, and slow growth. “However, their intellectual abilities are not impaired”, noted the Foundation for Medical Research. Atherosclerosis or stroke can occur and cause the death of the patient.
What are the treatments ?
For now, there is no treatment that can cure Hutchinson-Gilford syndrome. Medications prescribed to patients can only relieve the symptoms of progeria. “In order to limit cardiovascular complications, affected children are made to follow a low-fat diet and they are administered statins (substance indicated in the prevention of cardiovascular risks)”, develops the Foundation for Medical Research.
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