Thursday January 24, 2019

This woman has survived major tumours and is still fighting the rare Von Hippel-Lindau disease

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payel

By Shreya Upadhyaya

Have you ever heard of Von Hippel-Lindau? At 35, Payel Bhattacharya has already endured eight major surgeries including a liver transplant and brain tumour operation due to it.

Payel suffers from a rare genetic disorder called Von Hippel-Lindau (VHL), in which blood tumours constantly form in blood vessels. The disease is so rare that out of over a billion Indian population, only 70 have been detected with the syndrome so far.

VHL syndrome is caused by a gene mutation and leads to an abnormal capillary growth in internal organs like the lungs, heart, pancreas, liver and intestines. Normally, our capillaries branch out like trees. In VHL patients, little knots of extra capillaries form tumors that have to be removed.

Unaware of the genetic disorder at first, Payel was detected of it at the age of three. She underwent her first surgery at the age of 12 to remove a tumour in the left foot. 23 years later, she underwent a brain tumour surgery and then doctors confirmed VHL.

The biggest shock came in four months later, when she was diagnosed with tumours in her liver. Since then Payel has suffered from a minor stroke and paralysis of the left side after her brain surgery. Till now she has had tumors removed from her foot, knee and brain which was followed by a liver transplant surgery.

After this, she had to take regular doses of immunosuppressant drugs which greatly weakened her immunity. She was diagnosed with tuberculosis of the bones, which is resistant to any possible treatment. She had to undergo a surgery on her neck for the removal of an enlargement. She has now turned partially blind as the tumours have spread over her eyes and is on a life-long medication of three anti-convulsants.

Biggest Loss

Her father’s death in 2009 made her life even more difficult as she lost a strong pillar of support. And the reason of his death was that he could not undergo a heart surgery due to lack of funds because all his retirement benefits were exhausted on Payel’s previous surgeries.

Over the years Payel and her mother have moved several houses. From their 2,200 square yard ancestral home in Kolkata to a windowless one bedroom home in a crowded and suffocating unauthorized colony in South Delhi, hers has been a tough journey. Her previous landlord asked her to leave as he thought hers is a contagious disease.

However, Payel has been and continues to be a fighter and her condition has not weakened her passion for life.

True Grit

She has been optimistic. She says that she “loves life and wants to live it to the fullest.” She adds that she is aware that her genetic disorder is incurable, not terminal.

But financial worries constantly loom large over Payel as given her genetic disorder, emergency can crop up any time.

“Many donors pay for one or two months. But I need lifelong medical support. I can lead a normal life with medicines, but my resources are limited and fast exhausting,” says Payel.

Next Story

Life-Threatening Liver Disease May be Caused Due To Fatty Diet: Study

Not only does this study define how fat and cholesterol shape the progression of liver inflammation and scarring, but it also identifies potential pathways

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Cholesterol -- a molecule normally linked with cardiovascular diseases -- may also play an important role in the onset and progression of Alzheimer's disease, researchers have found.
Junk Food is highly rich in Cholesterol, pixabay

High fat and high cholesterol diet may trigger changes in the immune system that can lead to a serious form of non-alcoholic fatty liver disease (NAFLD), a study warns.

An estimated 20 per cent of people with NAFLD have non-alcoholic steatohepatitis (NASH), according to the study published in the journal Hepatology.

NASH can eventually progress to cirrhosis or liver cancer, especially in those with obesity or Type 2 diabetes. Patients with NAFLD are also at an increased risk of cardiovascular disease, they said.

“Despite its increasing prevalence and burden to the health care system, there are currently no US Food and Drug Administration-approved therapies for non-alcoholic fatty liver disease,” said Hugo Rosen, a professor at University of Southern California in the US.

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Skip Oily Food in The Morning. Pixabay

“There’s an urgent need to better understand the causes of non-alcoholic fatty liver disease progression so that successful therapeutics can be designed and brought into clinical practice,” Rosen said.

The study illuminates how a toxic combination of dietary fat and cholesterol impacts the behaviour of macrophages, a type of white blood cell, in the liver.

Using a mouse model, the study details the cascade of events in the immune system that eventually leads to the type of liver inflammation and scarring that is commonly seen in patients with NASH.

After feeding mice diets with varying levels of fat and cholesterol, the team found that the combination of both had a synergistically detrimental action on the genes regulating liver inflammation and scarring.

Liver
The study illuminates how a toxic combination of dietary fat and cholesterol impacts the behaviour of macrophages, a type of white blood cell, in the liver.

Oxidised low-density lipoprotein cholesterol, in particular, directly altered gene expression in both human and mouse macrophages associated with inflammation and scar formation.

Also Read: Omega-3 Fatty Acids in Diet can Prevent Cancer From Spreading

The group also identified a novel type of reparative macrophage that counteracts the inflammation.

“Not only does this study define how fat and cholesterol shape the progression of liver inflammation and scarring, but it also identifies potential pathways that can be targeted for future therapies. That could bring us closer to finding a treatment for a disease that impacts millions of lives around the world,” Rosen said. (IANS)