Prince Frederick of Luxembourg died at the age of 22 of porgumitochondrial disease, a rare genetic disorder in which he was born, his family announced.
Frederick de Nassau passed away in Paris on March 1, the day after the internationally recognized day of Rare disease that occurred on February 1, Luxembourg’s Prince Robert said in a statement posted on the Polg Foundation’s website. Prince Frederick established an organization in 2021 to raise awareness about the situation.
Courtesy: polgfoundation.org
Prince Robert. His wife, Princess Julie of Nassau. And Prince Frederick’s brothers, Alexander and Charlotte, were able to say goodbye to him on his last day, the patriarch said. One of the last things Frederick asked him was, “Daddy, are you proud of me?” According to Prince Robert.
“He barely could speak for a few days, so the clarity of these words was astounding as the weight of the moment was profound,” writes Prince Robert. “The answer was very simple, and he had heard it many times… But at this time he needed the peace of mind that he would contribute everything he could with his short, beautiful presence and that he could ultimately move on.”
The young prince was remembered as a pleasant person with “undoubted desires for life.” He is a huge fan of the American “office” adaptation, Prince Robert said he has seen it over a dozen times in its entirety.
What is Polg Mitochondrial Disease?
Polg is the name of a gene that provides instructions for creating an active portion called the alpha subunit of a protein called polymerase gamma, according to the National Library of Medicine.
There are multiple POLG-related diseases and conditions, including Alpers-Huttenlocher syndrome, ataxia neuropathy spectrum, and pediatric myoma liver damage spectrum.
Frederick was born with Porg’s disease, but he was not diagnosed until he was 14, his father said.
According to the Polg Foundation, mitochondrial disorder Frederick “deprives the somatic cells of energy” and can cause a variety of symptoms, including progressive dysfunction and multiple organ failures.
“We might compare this to breaking down a battery that is not fully charged, being in a constant exhaustion and ultimately losing its strength,” the Polg Foundation said on its website.
Experts estimate that around 2% of the population carries the POLG mutation. However, the total number of people with Polg-related diseases is unknown. One study of Australian adults with Polg mutations found that 10% suffer from Polg-related disease.
