A young European royal has died due to a rare genetic disorder at the age of 22.
Prince Frederik of Luxembourg passed away from a rare mitochondrial condition affecting two of his POLG genes that he had been diagnosed with at the age of 14.
His father, Prince Robert of Luxembourg, posted a moving message about his son to the POLG Foundation website, a foundation founded by Prince Frederik’s family to improve research into the disorder. Prince Frederik was the creative director of the foundation.
Why It Matters
Prince Frederik’s passing, one day after “rare disease day,” has brought more awareness to the mitochondrial disease, which per the POLG website, affects one in 5,000 people. After cerebral palsy, it is the second most commonly diagnosed serious genetic disease.
Research into POLG disease could also inform research into other degenerative conditions such as Alzheimer’s and Parkinson’s, as well as some cancers, depression and diabetes.
What to Know
Prince Frederik’s parents are Prince Robert of Luxembourg and Princess Julie of Nassau. The royal family of Luxembourg is currently led by His Royal Highness Grand Duke Henri, Duke of Nassau, Prince of Bourbon-Parma, who is Prince Robert’s cousin. Luxembourg is a constitutional monarchy, meaning the royal family has ceremonial powers, but the state is run by an elected parliament.
Grand Duke Henri is set to abdicate in October this year, to hand over the throne to his son, Hereditary Grand Duke Guillaume, after nearly 25 years as monarch.
Prince Frederik’s disorder prevented his mitochondria, known as the powerhouse of the cell, from being able to produce the energy needed to fuel the body. This led to organ dysfunction and failure. The POLG Foundation describes the disease as “having a faulty battery that never fully recharges and is in a constant state of depletion.”
He was diagnosed after his condition had already progressed, but even if he had been diagnosed earlier, there is still no cure or effective treatment for the disease.
In his tribute to his son, Prince Robert praised him as his “superhero,” saying ” Frederik was born with a special capacity for positivity, joy, and determination. When he was little, I would always say that if there is one child of ours that I would never need to worry about, it was him. He has social skills like no other, an amazing sense of humour, an emotional intelligence and compassion that were off the charts, a sense of justice, fairness and decency that knows no bounds.”
What People Are Saying
Prince Robert of Luxembourg: “After gifting each of us with our farewells – some kind, some wise, some instructive – in true Frederik fashion, he left us collectively with a final long-standing family joke. Even in his last moments, his humor, and his boundless compassion, compelled him to leave us with one last laugh….to cheer us all up.
“Frederik’s last question to me, prior to his other remarks was: ‘Papa, are you proud of me?’ He had barely been able to speak for several days, so the clarity of these words was as surprising as the weight of the moment was profound. The answer was very easy, and he had heard it oh so many times…. but at this time, he needed reassurance that he had contributed all that he possibly could in his short and beautiful existence and that he could now finally move on.
“Frederik knows that he is my superhero, as he is to all of our family, and to so very many good friends and now in great part thanks to his POLG Foundation, to so very many people the world over.”
What Happens Next
The POLG Foundation is committed to finding treatments for mitochondrial diseases, in the hope that its research will help not only PolG sufferers, but also people with other disorders that affect their mitochondria.
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