A prince of Luxembourg died at 22 of a genetic disease for which he spent his short life raising awareness.
Prince Frederik, the second cousin of the heir of the Luxembourg throne, discovered at the age of 14 who had a mutation in his Polg gene, which is involved in the replication and repair of the DNA. He died on March 1 in Paris, said his father, Prince Robert, in a statement through the Polg Foundation.
The estimates vary, but it is believed that about one in 10,000 has Polg or polymerase disease, according to the United MitoCondrial Disease Foundation. It is a mitochondrial disorder, a group of conditions that influence the way the mitochondria in cells produce energy. Polg’s disease affects multiple organs and can lead to convulsions and poor coordination, among other symptoms.
“You could compare it with a defective battery that never recharges completely, is in constant state of exhaustion and in the end it loses power,” said Prince Robert.
The deficiency of Polg is a particularly brutal type of mitochondrial disease, according to Sir Doug Turnbull, emeritus professor of neurology at the Newcastle University and a member of the scientific consultative committee of the Polg Foundation. “It is so incessantly progressive,” he said, “attack so many different systems with unfortunately the same conclusion.”
Prince Frederik was a founder and the creative director of the Polg Foundation. The organization used his DNA for research, started his studies and made a short film on the disease. Federico also designed a clothing line to raise research funds.
Frederik was born in Aix-en-Provence, France, and grew up in Switzerland. He was a fan of the American television show “The Office”, said his father, and had seen the whole series 10 times.
In his last few days, Frederik was in hospital with pneumonia and another serious infection that left him unable to move, but he could still look out the window, said Prince Robert.
“Through the small window covered with mesh, he saw the Eiffel tower glitter with his hourly dance of the lights,” said his father. Frederik asked his brother, Alexander, to take a photograph of the sun at sunset.
Frederik was a second cousin of Prince Guillaume, who will take over the throne of Luxembourg when his father, the Grand Duke Henri, retires in October. Frederik and Guillaume share the great -grandparents: Charlotte, Grand Duchess of Luxembourg, who reigned from 1919 to 1964, and her husband, Prince Félix.
“When you are a child, you have all these dreams, all these aspirations,” said Frederik in the film produced by his foundation. “I’m not sure I understand all the consequences of Polg when it was diagnosed for the first time. It is thinner, where the world slowly is becoming smaller. “