Prince Frederik of Luxembourg has died on the age of twenty-two from POLG mitochondrial illness, a uncommon genetic dysfunction that he was born with, his household introduced.
Frederik de Nassau died in Paris on March 1, a day after internationally acknowledged Uncommon Illness Day, which takes place on the final day of February, Prince Robert of Luxembourg stated in an announcement posted to the POLG Foundation web site. Prince Frederik based the group in 2021 to assist increase consciousness concerning the situation.
Courtesy: polgfoundation.org
Prince Robert; his spouse, Princess Julie of Nassau; and Prince Frederik’s siblings, Alexander and Charlotte, have been all in a position to say goodbye to him on his final day, the patriarch stated. One of many final issues Frederik requested him was, “Papa, are you happy with me?” in response to Prince Robert.
“He had barely been in a position to communicate for a number of days, so the readability of those phrases was as shocking as the burden of the second was profound,” Prince Robert wrote. “The reply was very simple, and he had heard it oh so many instances…. however right now, he wanted reassurance that he had contributed all that he probably may in his brief and delightful existence and that he may now lastly transfer on.”
The younger prince was remembered as a joyful individual with “indomitable lust for all times.” He was studying Italian and was a giant fan of the U.S. adaptation of “The Workplace,” which he has seen over 10 instances in its entirety, Prince Robert stated.
What’s POLG mitochondrial illness?
POLG is the identify for a gene that gives directions for making the energetic piece, referred to as the alpha subunit, of a protein referred to as polymerase gamma, in response to the National Library of Medicine.
There are a number of POLG-related ailments and circumstances, together with Alpers-Huttenlocher syndrome, Ataxia neuropathy spectrum and Childhood myocerebrohepatopathy spectrum.
Frederik was born with POLG illness, however he wasn’t identified till he was 14 years previous, his father stated.
In response to the POLG Basis, the mitochondrial dysfunction Frederik had “robs the physique’s cells of power,” and may trigger quite a lot of signs, together with progressive dysfunction and failure of a number of organs.
“One would possibly examine it to having a defective battery that by no means absolutely recharges, is in a relentless state of depletion and ultimately loses energy,” the PoLG Basis stated on its web site.
Specialists estimate that about 2% of the inhabitants carry POLG mutations. Nonetheless, the whole variety of individuals with POLG-related ailments is unclear. One study of adults in Australia with POLG mutations discovered that 10% of them had POLG-related ailments.
