To fight a Super Rare Chronic Disease you need a rare brand of courage and rare friends.

Miss Payel Bhattacharya, was diagnosed with a rare disease VHL or Von Hippel-Lindau. It is a genetic form of cancer.

She is a Blogger at TOI , and has shared details of her Rare Conditions  – Understanding the science behind rare brain tumour in Times of India.

Payel has shared the following Blog to highlight her journey with this Rare Condition , and hopes to raise awareness about the Rare Disease and also to raise funds for  the high cost of the treatment involved.

Please do read and share maximum – so that it reaches the right mediums from where she can get assistance.

Milaap is running a Fund Raiser – Please share ers/support-payel-bhattacharya-1

To pin down meaning to life is a piece of cake compared to actually being alive, not just breathing. I was born with an intense sense of being alive.

To begin with, I always felt fringed by my narcissist relatives who had less than average intelligence. I had a stifling awareness of the silly-willy fringe stuff.

I experienced horrors that would give most people nightmares for life but the thing is if you face serious issues from a younger age you don’t cringe inside or give up on life. The older you get without ever having a real problem you don’t know how to handle it. Starting early gives you a perspective if you don’t die first.

The name of my disease sounds euphoric and when you start explaining it resembles deadly babble where the prognosis is like a psychic scream.VHL or Von Hippel-Lindau, which turns you into a tumor-producing factory. The gene is present in every human being but in those with the disease, it is a case of mutation, hereditary, de Novo, or mosaicism.

It’s like Jerome. K Jerome said “I sat and pondered. I thought what an interesting case I must be from a medical point of view, what an acquisition I should be to a class! Students would have no need to “walk the hospitals, ” If they had me. I was a hospital in myself. All they need do would be to walk round me, and, after that, take their diploma. ” in his novel “Three men in a boat.”

Since birth, all I can see is a struggle. Even before I came to the planet. My mother is a different being altogether. As she got pregnant, She was subjected to cruelty and ruthlessness by aunt and granny-tyranny. They used to make my mother do heavy jobs while she was carrying her first child and she had a miscarriage.

When I was in her womb she was again subjected to heavy jobs in the kitchen. Mother had developed certain problems with her pregnancy. She worked too hard to bring me into the earth. She cares for me like no one. She used to play with me and was my only playmate.

Mum and I still love one another. She is my motivation to fighting, which doesn’t necessarily mean throwing punches; it might stir me up and inspire me to combat unusual diseases unknown to humankind and my strife is intense, grave, tireless where you don’t have to worry only about healing because recovery is a tough process that takes time and patience leaving scars behind but you have to bother about financial demands to get a proper diagnosis and the desired treatment and surveillance.

Initially, I underwent a few surgeries but ultimately was diagnosed with a disease that is not terminal but will only get worse. Strange oddments of fate changed it into inevitable doom.

The unusual birth of the eye-catching child of fond parents after a miscarriage and foetus getting problems while in the womb, were lost in the thought that behind the outer shell there might lurk some sickness never known to humanity and the ill-fated, ill-starred child would have to endure the worst in all walks of life and face countless challenges my parents never thought of that.

It was never fun for me growing up. It’s quite true people say “Prosperity gains friends, adversity tries them”. I never had a friend and because no one would like to socialize or go out with a limping girl.

After my brain tumor surgery, I was diagnosed with tumors compressing and displacing hepatic veins, hemorrhaging inside so that I needed a liver transplant and my relatives thought of not giving money from our due inheritance because they have sold the portion of the estate allotted to us to the South Indian Singer Didi, not even the money in the bank and the jewelry according in the testament because they wanted to swat me out of their way. I was the Achilles heel for my parents.

The friends and relatives who used to drop in at odd times often to enjoy, especially the one whom father took to Manali for some refreshment went their own way. Few became busy with finding a groom, others just avoided.

Fie, I say! They only knew money.

In an escalating crisis, divine intervention gave me a new lease of life through a liver transplant but turned off my reliable troops, my immune system so that the organ isn’t rejected.

VHL is a genetic form of cancer and VHL patients battle a series of tumors throughout their lives. Most people are not aware in our country that the VHL gene is involved in many forms of cancer. Curing VHL brings us closer to curing many forms of cancer.

The anti-rejection protocol almost implies that if you are taking some of these medications you will fall ill every time you pass by someone with the sniffles along with the big stuff, like the flu or tuberculosis.

Yes, I was diagnosed with tuberculosis which was later found to be Multi-drug-resistant Tuberculosis.

The only treatment is the removal of tumors, irradiation, and monitoring but unfortunately, multiple tumors formed in the liver which compressed and displaced vital vessels and I had a liver transplant leading to turning off of the immune system.

The pharmaceutical marvel made my already bankrupted father take loans as his family denied paying the assets he deserved. His heart was 80% blocked, and he skipped his medicines to provide mine.

No merriment, no jokes, no laughter, no playful ways, nor any happy silliness. I had no life-long friends and learned how to face death or bereavement. “You can choose your friends but not your family”…but this thought didn’t happen to be true for me. Let those walk away who don’t want to stay because your destiny is not tied to them. Family is not about blood bonds, it’s about willing to hold your hands when you need them most.

When the money-minded, relatives got rid of us after my father perished, Mum with her neat and orderly mind kept her struggle with survival moving forward.

The early years were toughest: we had no money and no idea what to eat the next day. The relations with a twisted mentality are still fighting in court and trying to swat me out of the way.

The challenge that was thrown down our way has been quite an adventure, setbacks with hard work and luck involved.

When life is sheer willpower hanging by two or three threads, she tells of the wondrous things in my rare and strange life and we together have journeys and magnificent adventures mostly making rounds of hospitals.

Going down memory lane, how I survived so many surgeries including my landmark liver transplant, kidney cancer, MDR TB, and obviously fighting brain tumors more than folksies have lice on their head, is indeed a marvel, a wonder indicating God’s intervention. As mountains rose on my way it couldn’t startle and I didn’t quiver with fear.

I live in a realm where with Mum’s support I can move the mountains with determination and resolve.

Once you go for surgery the blood-filled tumors would bleed spreading tumor cells throughout the brain and spine and they, in turn, would travel with the fluid inside the brain which acts as a cushion for the brain. There’s also a risk of bacterial meningitis due to the absence of an immune system. I have parathyroid insufficiency or HypoPara causing Hypocalcemia and attacks of tetany

Detecting and treating the condition of leptomeningeal hemangioblastoma without delay seems to help survival, though the number of patients analyzed is small. Patients may have other underlying health issues that may affect the data.

Rare disease patients and caregivers often shoulder a considerable burden for their disease and find it necessary to educate physicians about their condition and serve as becoming their own advocates.

The complexity of their existence, the trials of maladies forced me to struggle to live.

The will to live is an unstoppable thing. Most people live only when they are about to die.

I am not after pain medicines that will make me gain weight, damage my transplanted liver and whatever I have left of my left kidney but I am after a treatment that will make a big difference in my life. The doctor’s response is a shrug.

If you don’t have enough knowledge you can’t survive such nefarious tumors.

Once you go for surgery the blood-filled tumors would bleed spreading tumor cells throughout the brain and spine and they, in turn, would travel with the fluid inside the brain which acts as a cushion for the brain. There’s also a risk of bacterial meningitis due to the absence of an immune system.

I was told I have 3-6 months in 2013 after diagnosis… if I had gone for surgery but I was clever not to choose surgery.

I used radiation therapy or cyber knife for stopping the growth or mildly shrinking of the tumors.

But the 2017 radiation therapy went wrong, my ‘ little grey cells’ are functional and I became an author of books and columnist with Times of India( an unpaid job which I took to quench my passion) but my white matter was damaged.

As I was exposed to a lot of radiation in the brain, the consequences of which are white matter damage, I got chronic ischemic brain leading to Cerebral ischemia or brain ischemia, and when there isn’t enough blood flow to the brain leading to limited oxygen supply it may lead to the death of brain tissue, or ischemic stroke.

But another thing happened: the nerves started getting damaged. My 5th nerve has been destroyed by cyberknife for my bilateral trigeminal neuralgia.

Trigeminal neuralgia (TN), also known as tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve). It is characterized by attacks of intense, stabbing pain affecting the mouth, cheek, nose, and other areas on one side of the face. Sometimes there’s a constant dull aching or burning pain. Both types of pain can occur in the same individual, even at the same time. In some cases, the pain can be excruciating and disabling. If untreated, TN can have a profound effect on a person’s quality of life. In most cases, TN develops due to a blood vessel pressing against the trigeminal nerve, but sometimes no underlying cause can be identified (idiopathic). It can also be idiopathic, due to compression of the trigeminal nerve, or can occur due to a known underlying cause such as a tumor or multiple sclerosis. TN can usually be managed through medications, surgery or injections, or stereotactic radiosurgery.

Other nerves too look like they are on the path of destruction. We have 12 cranial nerves right inside the neck region. If the tumors in their journey travel to the nerve controlling the lungs or heart, I will take off from the world bidding adieu to all.

The electrons can be made to strike a tungsten target within the head of the accelerator to create a beam of photons (or “X-rays”). These X-ray beams are then directed at the site of cancer. Photons have no charge or mass and can be regarded as small packets of energy. Photons deposit their energy along the entire path that they travel through the body.

Therefore, a beam of X-rays irradiates not only the area of the tumor but also the healthy tissue that the beam encounters on its way towards the tumor and beyond the tumor. X-rays used for treating cancer usually do not stop within the body. X-rays travel right through you. On the other hand, proton beam therapy is delivered by larger, much more expensive accelerators called cyclotrons and synchrotrons.

A proton beam directed at a tumor travels in a straight trajectory towards its target, gives off most of its energy at a defined depth called the Bragg peak, and then stops. While X-rays often deposit more energy within the healthy tissues of the body than within the tumor.

I need 40 lakhs for the treatment. I assume you’d help me out. I live with Mum in a rented house. Already my medicines are very costly. I am not afraid of death. Life has to end once you are born but I think of my 68-year-old mother. After her first miscarriage during my birth, there were problems but she wanted me and for 41 years she has cared for me as nobody would. She is not just my caregiver but my only companion.

Animals care for their offspring for some time and teach them to eat, walk, how to get food or fly. But at one point in time, they leave them to make their way in life but that’s why my mother is unique.

“An’ why? Because…because I got you to look after me, and you got me to look after you, and that’s why.” -John Steinbeck

If only, governments or some trust or Institute sponsors the high cost of my treatment. I could have a better and longer life and my quality of life would be better. 

About Payel Bhattacharya –

Miss Payel Bhattacharya, was diagnosed with a rare disease von Hippel-Lindau or VHL. It is a genetic form of cancer. VHL patients battle a series of tumors throughout their life. VHL may occur in up to 10 organs of the body with a possibility of neuroendocrine tumors.

Her autobiographical fictional account has been published by Sahitya Akademi. She also wrote, “The Adventures of Mum and Princess is her attempt to write mystery stories. Even with the rarest form of brain tumors, she took the challenge to write a paranormal novel with a dash of spy-thriller known as Midnight. She also wrote her survival story “A Warrior Dies Dancing That’s Who I Am…” She finds positivity and warmth through her life writing and poetry also through creating much-needed awareness.

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