Rare POLG mitochondrial disease explained after Prince Frederik of Luxembourg dies aged 22

Earlier this month Prince Frederik of Luxembourg died aged 22 after being diagnosed with POLG mitochondrial disease aged 14.

The late prince, who is the son of Prince Robert of Luxembourg and Princess Julie of Nassau, passed away at the start of this month (1 March) but his death was announced last Friday (7 March) after his father took to the official POLG Foundation’s website to share the sad news with a touching statement.

In the statement, Robert explained a little more about the rare POLG Mitochondrial disease and its symptoms and made sure to praise Frederik, who was the POLG Foundation Founder and Creative Director, for his positivity in the face of adversity.

Prince Frederik of Luxembourg died aged 22 after being diagnosed with POLG mitochondrial disease (POLG Foundation)

"This is the battle that Frederik fought, and this is the burden that he had to carry throughout his life," the father-of-three wrote. "He always did so with grace and with humour. When we asked him if he wanted to create a foundation to find a cure and help others like him, he jumped at the opportunity.

"Though he always made it very clear that he did not want this dreadful disease to define him, he nonetheless immediately identified with and helped define the mission of The POLG Foundation."

What is POLG mitochondrial disease?

Robert explained that POLG disease is 'a genetic mitochondrial disorder that robs the body’s cells of energy', which, in turn, causes 'progressive multiple organ (brain, nerves, liver, intestines, muscles, swallowing and ocular function, etc.) dysfunction and failure.'

"One might compare it to having a faulty battery that never fully recharges, is in a constant state of depletion and eventually loses power," he added.

His father explained more about the illness (The POLG Foundation)

In the statement, Robert explained that Frederik was born with the rare disease, noting: "As is the case for 300 million people like Frederik worldwide, these diseases are usually hard to recognise even by physicians, and patients’ families may never know what they are suffering from as they may only be identified very late in their progression."

What are the symptoms of POLG mitochondrial disease?

In the statement, Robert shared that Frederik was only diagnosed with POLG at the age of 14, when his symptoms were 'showing more clearly' and 'when the progression of his disease had become more acute'.

Frederik received the diagnosis age 14 after his symptoms were 'showing more clearly' (POLG Foundation)

The symptoms can be mild to severe and most often include ophthalmoplegia (a condition that causes weakness or paralysis of the muscles that move the eye), muscle weakness, epilepsy and liver failure.

Is there a cure for POLG mitochondrial disease?

Robert explained in the statement: "Because POLG disease causes such a wide range of symptoms and affects so many different organ systems, it is very difficult to diagnose and has no treatments much less a cure."

The official POLG Foundation website adds: "For all patients with PolG disorders, the future is deeply uncertain. There are no effective treatments, let alone a cure that might save them.

"Every day they witness their own degeneration and progressive loss of function, a harrowing life experience."