Prince Frederik of Luxembourg, the youngest son of Prince Robert of Luxembourg and Princess Julie of Nassau, passed away in Paris at the age of 22 on March 1st, just one day after Rare Disease Day, due to a rare genetic disease known as POLG.
His father announced his passing on March 7th in a statement posted on the website of the POLG Foundation, which Frederik co-founded in 2022 and served as the creative director of.
“Frederik valiantly fought his disease until the very end,” Robert wrote. “His indomitable spirit propelled him through the most challenging physical and mental obstacles.”
Frederik, who is survived by his family and pet dog Mushu, was remembered as a resilient fighter and a passionate advocate. “Frederik knows that he is my Superhero,” Robert wrote. “Part of his superpower was his ability to inspire and lead by example.”
Robert expressed gratitude for having his disease, stating that Frederik felt there were many amazing people he would have never met if it wasn’t for his condition. He once said, “Even though I’ll die from it … and even if my parents do not have the time to save me, I know that they will be able to save other children.”
What is POLG?
POLG disease is a mitochondrial disorder caused by inherited mutations in the POLG gene, which plays a critical role in cell replication and DNA repair. The disease, which has no cure, affects multiple organs and can have wide-ranging and debilitating symptoms. Robert described it as having a faulty battery that eventually loses power.
Diagnosing POLG is challenging, as it is one of the most common inherited mitochondrial diseases, affecting around 1 in 10,000 people. Symptoms can vary widely, making it difficult to identify, even for physicians. Frederik was diagnosed at age 14.
Doug Turnbull, a professor of neurology at Newcastle University and a member of the POLG Foundation’s scientific advisory board, described POLG deficiency as the most aggressive form of mitochondrial disease. It can impact vision, mobility, and speech, and is often fatal.
Life expectancy ranges from three months to 12 years from disease onset.
What does the POLG Foundation do?
Over three years, the foundation has provided $3.6 million in funding for four major research projects focused on understanding the disease mechanisms and potential treatments. They have also partnered with Columbia University and developed tools to assess disease progression in mitochondrial disorders.
Frederik played an instrumental role in advancing POLG research, not only as a founder of the foundation, but also by contributing his own DNA for further investigation.
Mitochondrial research has implications for other diseases such as cancer, neurodegenerative and immunologic diseases, and even aging.
Frederik and his mother, Julie, who raised awareness about the disease for 15 years, made a documentary about POLG from the perspective of Frederik and other patients.
The day before his death, Robert wrote, Frederik asked his father, “‘Papa, are you proud of me?’”
“We are all so very proud of you, Frederik,” Robert replied, providing reassurance that he had made the most of his beautiful but short life.
