The Prince of Luxembourg died of a genetic disease 22, which led to his short life and raising awareness.
Prince Frederick, the second cousin of the heir to the Luxembourg throne, discovered at the age of 14 that there was a mutation in the Polg gene, which is involved in DNA replication and repair. He passed away in Paris on March 1, his father, Prince Robert, said in a statement from the Polg Foundation.
Estimates vary, but it is believed that about one in 10,000 people have POLG, or polymerase gamma, the disease, according to the Mitochondrial Association Foundation. It is a mitochondrial disorder, a group of conditions that affect the way mitochondria in a cell produces energy. Polg disease can affect multiple organs and, among other symptoms, can lead to seizures and poor coordination.
“We might compare it to breaking down a battery that doesn’t fully charge, being in a constant exhaustion and eventually losing power,” Prince Robert said.
Porg deficiency is a particularly cruel type of mitochondrial disease, according to Sir Doug Turnbull, a professor emeritus of neurology at Newcastle University and a member of the Porg Foundation’s scientific advisory board. “It’s very merciless progressive,” he said, “sadly attacking so many different systems with the same conclusion.”
Prince Frederick was the founder and creative director of the Polg Foundation. The organization used his DNA in his research, began researching and made short films about the disease. Frederick also designed the clothing line to raise funds for research.
Frederick was born in Aix en Provens, France and grew up in Switzerland. He is a fan of the American television show The Office, his father said, and watched the full series 10 times.
On his last day, Frederick was in the hospital with pneumonia and another serious infection that he couldn’t move, but he still could look out the window, Prince Robert said.
“Through a small mesh-covered window, he saw the Eiffel Tower shine with a dance of lights per hour,” his father said. Frederick asked his younger brother Alexander to take a picture of the sunset.
Frederick was Prince Guillaume’s second cousin. He will take over the Luxembourg throne when his father, Grand Duke Henri, retired in October. Frederick and Guillaume share great grandparents: Charlotte, the Grand Duchess of Luxembourg, who reigned from 1919 to 1964, and her husband, Prince Felix.
“When you’re a child, you have all these dreams, all these aspirations,” Frederick said in a film produced by his foundation. “I don’t know if I understood all the outcomes of Polg when I was first diagnosed. It’s more subtle and slowly the world is getting smaller and smaller.”
