What is POLG Mitochondrial Disease? Luxembourg’s Prince Frederik Dies at 22 From Rare Genetic Condition- wna24

Luxembourg City: Prince Frederik, the youngest son of Prince Robert of Luxembourg and Princess Julie of Nassau, died in Paris at age 22 on March 1, the royal family announced. 

Prince Frederik was suffering from a rare genetic condition known as POLG Mitochondrial Disease. 

In a heartfelt tribute, the royal couple said, “It is with a very heavy heart that my wife and I would like to inform you of the passing of our son, The POLG Foundation Founder and Creative Director, Frederik. “Last Friday, February 28th, on “Rare Disease Day”, our beloved son called us in to his room to speak to him for one last time. Frederik found the strength and the courage to say goodbye to each of us in turn—his brother, Alexander; his sister, Charlotte; me; his three cousins, Charly, Louis, and Donall; his brother-in-law, Mansour; and finally, his aunt Charlotte and uncle Mark.”

Prince Robert recalled the last conversation he had with his son. 

“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,” Prince Robert stated. 

POLG disease is a mitochondrial disorder caused by mutations in the POLG gene. It typically affects multiple organs, primarily the brain, nerves, muscles, and liver, and can affect vision due to involvement of brain structures. It is also one of the most common inherited mitochondrial diseases. Up to 2% of those of Northern European descent may carry disease-causing POLG mutations, and the frequency of POLG disease is estimated to be 1 in 10,000.