
Frederik was diagnosed with POLG Mitochondrial disease, which has no cure, at 14 (Pic: Instagram/iStock)
Prince of Luxembourg Frederic died at the young age of 22 due to severe health complications from a rare genetic condition in which the body’s cells become devoid of any energy. Prince Frederick, who lived in Switzerland, passed away earlier this month following a long battle with POLG mitochondrial disease, his father Prince Robert announced in a statement shared on the POLG Foundation website.
“It is with a very heavy heart that my wife and I would like to inform you of the passing of our son,” the statement began. In the statement, Robert recalled Frederik had "called us in his room to speak to him for one last time" on Feb. 28. He said the 22-year-old "found the strength and the courage to say goodbye" to his family, including brother Alexander and sister Charlotte, among others.
Frederik was diagnosed with POLG mitochondrial disease at 14, Robert explained in the statement, "when his symptoms were showing more clearly and when the progression of his disease had become more acute."
"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," Robert wrote. The condition affects multiple organs, including the brain, muscles, and liver, and is usually caused by mutations in the genes.
What causes POLG mitochondrial disease?
According to experts, POLG disease, which robs the body’s cells of energy, happens due to the effect on mitochondria working in your body. Mitochondria make energy in your cells, and when they are not able to produce enough energy that your body needs, it affects how your organs function. It then leads to progressive multiple-organ dysfunction and failure.
Statistics say an estimated 1 in 5,000 people have POLG mitochondrial disease. It’s common for mitochondrial diseases to receive a misdiagnosis due to the number and type of symptoms and organ systems involved, so this number may be underestimated.
Signs and symptoms of POLG mitochondrial diseases
According to experts, the symptoms of mitochondrial diseases vary based on the type and location of the affected cells. They can range from mild to severe and could include:
- Poor body growth
- Muscle weakness, muscle pain, or low muscle tone
- Vision and hearing loss
- Developmental delays or issues with cognitive development
- Diarrhoea or constipation
- Unexplained vomiting
- Persistent acid reflux
- Swallowing difficulties
- Seizures
- Migraines
- Breathing issues
- Fainting
Symptoms of mitochondrial diseases can be present at birth, but they can also arise at any age. A healthcare provider usually detects symptoms affecting more than one organ or organ system at the same time. Symptoms of the same disease can vary from person to person, even among family members.
Is there a cure for POLG diseases?
Doctors say there is no cure for POLG disease at this time. However, treatments focus on managing symptoms and improving quality of life. A few common treatments include:
- Anticonvulsant medications for seizures (although valproate must be avoided)
- Pain medication and muscle relaxants for comfort
- Small, frequent meals or a feeding tube for nutritional support may be helpful, and the ketogenic diet is sometimes used to help control seizures.
- Physical therapy for declining motor skills and muscle strength
- Surgery or special glasses to correct drooping eyelids
- Speech therapy for slurred speech
- Breathing tube and/or artificial ventilation for respiratory failure
- CPAP or BiPAP for apnea
- Physical and occupational therapy for motor involvement
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