Prince Frederik’s Courageous Fight Against POLG Mitochondrial Disease. Over the weekend, Prince Robert of Luxembourg shared the heartbreaking news about his 22-year-old son, Prince Frederik, who lost his battle with POLG Mitochondrial disease, a condition he had been diagnosed with at the age of 14.
The Luxembourg royal released this statement on the official website of the POLG Foundation, a charity that Prince Frederik founded and where he served as creative director.
In discussing the foundation’s creation, Prince Robert mentioned that his son was passionate about contributing to the search for a cure and helping others facing similar struggles.
“Frederik expressed his gratitude for having this disease,” Prince Robert recalled. “In a conversation with a friend, Frederik said, ‘I am thankful that I am the one who was born with this disease. Even though I will ultimately lose my life to it, and regardless of whether my parents have the time to save me, I find comfort in knowing they will be able to help other children.'”
What is POLG Mitochondrial disease?
POLG mitochondrial disease is a genetic disorder caused by mutations in the POLG gene, which plays a critical role in maintaining mitochondrial DNA. Mitochondria are often described as the “power plants” of our cells because they generate the energy needed for essential bodily functions. When the POLG gene mutates, it disrupts this energy production process, leading to cellular dysfunction and damage across multiple organ systems.
This condition can impact the brain, muscles, liver, nerves, and even vision. While it’s one of the most common inherited mitochondrial disorders—affecting approximately 1 in 10,000 people—it remains largely underrecognized. Symptoms can emerge at any stage of life, from infancy to adulthood, and their severity varies widely depending on the mutation involved.
“One might compare it to having a faulty battery that never fully recharges and is in a constant state of depletion. The disease is uncommon, so no one knows how many patients there are.”
Symptoms of POLG Mitochondrial disease
The symptoms of POLG mitochondrial disease are diverse and depend heavily on when they first appear:
Early-Onset Symptoms (Before Age 12):
- Seizures
- Cognitive regression
- Motor impairments
- Vision loss
- Feeding difficulties
- Liver dysfunction
Adolescent/Young Adult Symptoms (Ages 12–40):
- Seizures
- Impaired coordination (ataxia)
- Peripheral neuropathy (nerve damage)
- Late-Onset Symptoms (After Age 40):
- Drooping eyelids (ptosis)
- Muscle weakness
- Parkinsonism-like symptoms
- Difficulty with eye movement
Other possible symptoms include developmental delays, hearing loss, depression, respiratory failure, and slurred speech. Over time, the disease often progresses to involve multiple organ systems, making it increasingly difficult for patients to manage daily life.
Prince Frederik’s Battle with POLG Disease
Diagnosed at age 14 after experiencing worsening symptoms, Prince Frederik faced his condition with remarkable courage. Despite the physical and emotional toll of POLG mitochondrial disease, he remained a symbol of hope for others. Frederik founded the POLG Foundation to raise awareness about the disorder and support research efforts aimed at finding better treatments.
Frederik’s family remembers him as someone who approached life—and his illness—with grace and humor. In his final days, he used Rare Disease Day on February 28 to share heartfelt goodbyes with loved ones while continuing to advocate for those affected by rare conditions. His passing is a stark reminder of both the devastating impact of POLG disease and the urgent need for medical breakthroughs.
Can POLG Mitochondrial Disease Be Cured?
Sadly, there is no cure for POLG mitochondrial disease at present. Treatment focuses on managing symptoms and improving quality of life rather than addressing the root cause. Common approaches include:
- Medication: Anticonvulsants are used to control seizures but must exclude valproate due to its risk of liver failure.
- Physical Therapy: Helps maintain muscle strength and coordination.
- Nutritional Support: Specialized diets or feeding tubes may be necessary for patients with severe swallowing difficulties.
- Speech Therapy: Addresses communication challenges caused by slurred speech.
- Respiratory Support: Breathing aids like CPAP or BiPAP may be required for apnea or respiratory failure.
While these treatments can help alleviate symptoms, they do not stop disease progression. However, recent research offers hope. A clinical trial led by McGill University has explored Deoxycytidine/Deoxythymidine Combination Therapy—a promising experimental treatment that has shown improvements in energy levels and motor function in early studies. Though still in development, this therapy could represent a significant step forward for patients living with POLG-related disorders.
Prince Frederik’s passing
Prince Robert shared his deep sorrow over the loss of his son, saying, “Frederik faced his illness with incredible bravery right up to the end. His relentless love for life helped him withstand the toughest physical and mental challenges.”
The prince also mentioned that in the months before his passing, Frederik had been hospitalized due to pneumonia and another serious infection.
While the diagnosis was made when Frederik was 14, his father explained that he was born with the condition, which became more apparent as his symptoms worsened and his illness intensified.
On the subject of the disease, Sir Douglas Turnbull, a respected professor and member of The POLG Foundation Scientific Advisory Board, stated, “I have treated many patients with mitochondrial disease, and it is clear that POLG deficiency is one of the most severe forms. It progresses relentlessly, impacting various systems and often leading to the same heartbreaking outcome.”
Amidst his profound grief, Prince Robert found a way to honor his son’s spirit, ending his message with, “Frederik passed away on March 1st, 2025, in Paris, France, the City of Lights. One light has gone out, but many others continue to shine.”
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