The "glass bone disease", Guillaume Bats had made it his strength, his asset, his trademark. If we do not yet know the causes of his death at 36 years, the comedian suffered from osteogenesis imperfecta, in one of its severe forms. According to the High Authority for Health, this disease affects one in 1,000 births in France.
Also called "Lobstein's disease", "glass bone disease" is a rare condition characterized by bone fragility and osteopenia (loss of bone capital). More concretely, the patient with osteogenesis imperfecta has very fragile bones and joints and therefore luxuriate quickly, but also suffers from skeletal deformities. Symptoms appear mainly in early childhood, but can also be discovered in adulthood in more attenuated forms.
⚫ Comedian Guillaume Bats died at the age of
➡️ 36 https://t.co/TPQHIlJLTn pic.twitter.com/iLS8P48bDG
— Le Parisien (@le_Parisien) June 2, 2023
These symptoms can also be "extra-skeletal" and affect teeth, hearing, blood circulation. In total, nearly 8 forms of osteogenesis imperfecta are listed in the scientific literature. In its most severe forms, the disease awakens in the first months of pregnancy and is characterized by prenatal fractures, until perinatal death.
A lifelong treatment?
In 90% of cases, "glass bone disease" is due to a mutation in one of the two genes that encode type I collagen, the protein in the fibers that help bone structure. This pathology is considered hereditary by the High Authority of Health. It is therefore recommended to consult early enough if cases of illness are known in the family, even in the absence of symptoms.
On the side of medical treatments, none exists to cure completely, but some can concretely improve the comfort of life, with a reduction in pain and better bone growth. Bisphosphonate infusions can be performed during 3 days of hospitalization every 4 months, for a maximum of 4 years.
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As a sign of the complexity of the treatment of the disease, the HAS has set up a national protocol for diagnosis and care (PNDS) to "better explain to the professionals concerned the current optimal diagnostic and therapeutic management and the care pathway of a patient with osteogenesis imperfecta".
