
Nick Jonas' childhood pal and neighbor Maya Kibbel passed away at 30 years of age. She was suffering from a rare genetic condition called Wilson's disease.
Kibbel's mother wrote a note for her daughter's friends and Nick being one of them commented, "My sister forever. Here for you always @coach_kibbel."
Reportedly, Kibbel shared a good bond with Nick and the Jonas family. In fact, the Kibbel family used to post throwback images of Nick and Maya from their childhood days. Sharing a snap of two of them, Kibbel once wrote, "Throughout all these years and living separate lives across the world, they have kept in touch and kept their friendship. That's how it is with friends and family! No matter what happens in the world, we care and love each other. What comfort to know someone cares and loves you as we experienced something we have never had this year!"
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Moneycontrol team spoke to Dr Sunil Rana, Associate Director and Head of Internal Medicine (Unit III), Asian Hospital, to understand more about the rare genetic disorder. He shared that in this condition, the body is not able to remove excess copper via bile, resulting in its deposition in various organs of the body.
"What one must know is that accumulation of copper in the body can damage liver, brain, and other tissues within the body. This is why timely diagnosis and treatment assume a lot of significance," he said.
Dr Rana shared that the onset of symptoms typically occur in one's teen years or early 20s. Symptoms can vary considerably among patients. Common symptoms include fatigue, jaundice, abdominal distention, tremors, difficulties with speech, motor coordination problems, mood changes, and changes in behavior.
Patients may also develop a golden-brown ring around the periphery of the cornea, referred to as the Kayser-Fleischer ring, which is observable during routine eye exams, Dr Rana added.
"When copper accumulates excessively in the body, it becomes toxic to the tissues in which it resides. The liver may develop an inflammatory process, form scar tissue, or develop cirrhosis due to the accumulation of copper. The accumulation of copper in the brain may damage the areas of the brain responsible for motor and behavioral control which may lead to tremors, dystonias, and other neurologic deficits," Dr Rana told Moneycontrol. He further added that if the disease is left untreated, kidneys, eyes, and joints can also get damaged.
"Commonly, doctors advise medications, such as Penicillamine or Trientine, to promote the elimination of copper from the body. In some cases, Zinc acetate, a medicine, is also given to decrease the amount of copper absorbed from food. Other elements of an effective treatment plan include a low-copper diet and regular monitoring of the patient," he concluded.
1. What is Wilson’s disease?
Wilson’s disease is a rare inherited condition in which the body cannot properly remove excess copper. As a result, copper builds up in organs such as the liver, brain, and eyes, potentially causing serious health problems.
2. What causes Wilson’s disease?
The condition occurs due to mutations in the ATP7B gene, which normally helps the liver remove extra copper through bile.
3. Which organs are most affected by Wilson’s disease?
The disease mainly affects the Liver, the Brain, and sometimes the eyes and kidneys.
4. What are the early symptoms of Wilson’s disease?
Symptoms may include fatigue, jaundice, abdominal swelling, tremors, difficulty speaking, poor coordination, or mood and behavioural changes.
5. What are Kayser–Fleischer rings?
Kayser–Fleischer Rings are copper deposits that appear as dark rings around the eye’s cornea and are a common sign of Wilson’s disease.
Disclaimer: This article, including health and fitness advice, only provides generic information. Don’t treat it as a substitute for qualified medical opinion. Always consult a specialist for specific health diagnosis.
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