Pakistan’s Pervez Musharraf dies of amyloidosis: What is the rare protein build-up disease?

Pervez Musharraf, Pakistan’s last military ruler, survived many an assassination attempts. But on Sunday, the 79-year-old former four-star general passed away at the American Hospital in Dubai after a prolonged illness.The family of Musharraf — who had seized power in Pakistan in 1999 in a bloodless coup — known to be the architect of the Kargil War of 1999, has now filed an application in the Pakistani consulate in Dubai to shift the former leader’s body to Pakistan where he will be buried in a Karachi graveyard.Politicians in Pakistan expressed their condolences over Musharraf’s demise with former prime Minister Nawaz Sharif saying, “To Allah we belong and to Him is our return.”The former general’s demise came after he struggled with a rare disease called amyloidosis. His illness came to light in 2018 when his party — the All Pakistan Muslim League (APML) — had announced the same. And in June last year, he had been hospitalised in Dubai — where he lived in self-exile — for three weeks with his family stating that he was “going through a difficult stage where recovery is not possible and organs are malfunctioning”.Here’s a better understanding of the disease that claimed Musharraf’s life — from the causes to symptoms to treatment.What is amyloidosis?Amyloidosis is a rare disease that occurs when a protein called amyloid builds up in organs. This amyloid build-up can make the organs not work properly. Organs that may be affected include the heart, kidneys, liver, spleen, nervous system and digestive tract.As per the Cleveland Clinic, Amyloidosis is like debris floating down a river that snags on a rock or a tree limb. Over time, more and more debris catches in the snag, forcing the river to flow around the large snag. Just like debris caught on a snag, amyloid deposits accumulate within organs, eventually affecting organ structure and tissue function.The disease is very uncommon with healthcare providers estimating that about 4,000 people in the United States suffer from it each year.

As per Johns Hopkins, almost 70 per cent of people with amyloidosis are men and are usually between the ages of 50 to 65.

What causes amyloidosis?There are different types of amyloidosis and the causes of them vary. Amyloidosis may be secondary to a different health condition or can develop as a primary condition. It can exist on its own, or can be related to another medical issue. Johns Hopkins has reported that people receiving long-term dialysis are at some risk of developing a certain type of amyloidosis.According to doctors, Light-chain (AL) amyloidosis can be caused owing to an abnormality in the bone marrow cells. The abnormal plasma cells produce abnormal forms of light chain proteins, which enter the bloodstream and can form amyloid deposits.A person can develop Amyloid serum A protein if they have an inflammatory illness such as rheumatoid arthritis (RA) or inflammatory bowel disease (IBS). The third form of amyloidosis is Amyloid transthyretin protein or TRR protein and can develop if you inherit mutated DNA that makes TTR proteins more unstable. You may also develop ATTR as you get older.What are the symptoms of amyloidosis?It is very hard to diagnose amyloidosis and symptoms vary, depending on which organs are affected. Some general signs of amyloidosis are: feeling very weak, losing weight without trying, swelling in the belly, legs, ankles or feet, numbness or a tingling feeling in hands or feet, skin that bruises easily, purple spots (purpura) or bruised-looking areas of skin around the eyes, increased tongue size and shortness of breath.Most people who develop AL amyloidosis have a build-up of amyloid proteins in their kidneys, and are at risk of kidney failure.[caption id="attachment_12107732" align="alignnone" width="640"]

People watch a news report about the death of former Pakistani president General Pervez Musharraf at a TV shop in Karachi. AP[/caption]Is amyloidosis preventable?Unlike lifestyle diseases, one can’t prevent amyloidosis. People often develop some form of amyloidosis because they have an underlying condition.However, medical experts state that one can control how much amyloidosis affects your quality of life by understanding the risk for developing amyloidosis and treating the disease is in its early stages.How to cure amyloidosis?Unfortunately, there’s no cure to this disease. But there are treatments to stop more proteins being produced and give your body time to clear the deposits on the organs. This can help prevent organ damage.One of the treatments for amyloidosis is chemotherapy. Chemotherapy damages abnormal bone marrow cells and stops them from producing the abnormal proteins that form amyloid deposits. Doctors may also suggest a stem cell transplant.In the United States, the Food and Drug Administration has recently approved multiple medications for transthyretin amyloidosis. These medicines work by either “silencing” the TTR gene or by stabilising the TTR protein. As a result, further amyloid plaque should not deposit in the organs.With inputs from agenciesRead all the Latest News, Trending News, Cricket News, Bollywood News,India News and Entertainment News here. Follow us on Facebook, Twitter and Instagram.