The disease caused Prince Frederik to die causing symptoms such as epilepsy, dysfunction, multi -organ failure, and there is no cure.
Luxembourg’s Prince Frederik died in his 20s after the battle with a rare genetic disease called Polg. “With a heavy heart, my wife and I would like to notify our son’s departure,” Luxembourg’s Prince Robert, Frederik’s father, wrote.
Robert’s notice was posted on the Polg Fund website, an organization established by Frederik to seek treatment and cure this disease. In the notice, Prince Robert said Frederik called his family into the room to say goodbye to the last “rare day” on February 28. He died on March 1.
Because of a rare disease, experts have not determined the total number of people in the world with Polg.
Polg is a genetic disorder that removes cell energy, causes dysfunction and multi -organ failure. The patient’s body “is like an error that is never fully charged and always in a state of exhaustion”.
The cause of polg is derived from mitochondrial failures. Mitochondria is a cell energy factory, converting food into energy for the body to function. They contain their own DNA, to multiply the polymerase γ (Polg) enzyme that is encrypted in the gene.
The mutation in these genes weakens the multiplication of the mitochondrial DNA, causing symptoms that may start from childhood to adulthood.
Prince Frederik had to sit in a wheelchair due to illness before his death. Image:polgfoundation
The manifestations of Polg disease are very diverse and appear in many different organs. In the nervous system, patients may have epilepsy, loss of movement, muscle weakness and intellectual impairment. The digestive system is also affected, with symptoms such as liver dysfunction, pancreatitis. In addition, the muscle system is also affected by muscle weakness, fatigue and reducing muscle tone. These symptoms often appear in the period of young children, but may also start in adulthood, depending on the degree and position of mutations in the polg gene.
Because polg leads to many problems and affecting different organs, it is difficult to diagnose and treat. The diagnosis of Polg is usually based on combining clinical manifestations, mitochondrial tests and genetic analysis to identify mutations in polg genes.
There is currently no specific treatment for polg. Disease management focuses on symptomatic treatment and function support for patients. For example, anti -epileptic drugs can be used to control seizures, physiotherapy therapies that help improve motor function.
Mitochondria diseases in general affect one over 5,000 people worldwide, a serious genetic disease that is the second most diagnosed after cystic fibrosis. By supporting the study of mitochondrial dysfunction found in Polg mutations, experts are creating a knowledge store that can provide information for the development of treatments and the ability to cure many other diseases, including Alzheimer, Parkinson, diabetes, depression, some cancers and aging in general.
For all patients with Polg, the future is very uncertain. There is no effective treatment or treatment that can save them. Every day, they witnessed their own degeneration and losing their own functions, rated as a heartbreaking life experience.
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