A prince of Luxembourg has died at the age of 22 due to a genetic disease for which he dedicated his life to raising awareness.
Prince Frederik, a second cousin of the heir to the Luxembourg throne, was diagnosed at 14 with a mutation in his POLG gene, responsible for DNA replication and repair. He passed away on March 1 in Paris, as confirmed by his father, Prince Robert, through the POLG Foundation.
POLG, or polymerase gamma, disease is estimated to affect approximately one in 10,000 individuals, according to the United Mitochondrial Disease Foundation. This mitochondrial disorder impacts how cells produce energy and can affect multiple organs, leading to symptoms such as seizures and poor coordination.
Prince Robert described the condition as akin to "having a faulty battery that never fully recharges, is in a constant state of depletion and eventually loses power."
Sir Doug Turnbull, an emeritus professor of neurology at Newcastle University and a member of the POLG Foundation’s scientific advisory board, characterized POLG deficiency as a particularly severe form of mitochondrial disease, stating, “It is so relentlessly progressive, attacking so many different systems with sadly the same conclusion.”
Prince Frederik was the founder and creative director of the POLG Foundation, which utilized his DNA for research, initiated studies, and produced a short film about the disease. He also designed a clothing line to generate funds for research.
Born in Aix-en-Provence, France, and raised in Switzerland, Frederik was known for his fondness for the American television show “The Office,” having watched the entire series ten times, according to his father.
In his final days, Frederik was hospitalized due to pneumonia and a serious infection that left him unable to move. However, he was able to gaze out the window, where he observed the Eiffel Tower's illuminated display. He requested his brother, Alexander, to take a photograph of the sunset.
Frederik was a second cousin to Prince Guillaume, who is set to ascend to the Luxembourg throne upon his father, Grand Duke Henri's, retirement in October. Both share great-grandparents, Charlotte, Grand Duchess of Luxembourg, who reigned from 1919 to 1964, and her husband, Prince Félix.
In a film produced by his foundation, Frederik reflected on his childhood aspirations, stating, “When you’re a kid, you have all these dreams, all these aspirations. I’m not sure I understood all the consequences of POLG when I first got diagnosed. It’s more subtle, where slowly the world is getting smaller and smaller.”