I, miss Payel Bhattacharya was an unusual child. I was never concerned with petty things, the trivia, the dregs in life. My tangential connections with friends and relatives left me isolated but I was blessed with a wonderful mother. I grew up in the shadows of my Wonder-mum, my extraordinary mother who helped me wipe out the bad memories and realize the beautiful, prettier side of life.

I was born into a privileged family but those unfeeling relatives...when I needed them most…the foxy ones conspired against me so that I don’t get my rightful inheritance in a time of great need and that existence eat me up like a cat eats a canary and I meet my death. Other relatives… the rabbits turned tail and fled fearing I might ask them to help me or stand by me. But I didn’t despair and kept looking for hope and I found hope amongst mankind not tied to me by any blood bond.

The tumours in the liver known as hemangiobkastomas were compressing and distracting the vital vessels.

It is their combined effort that pulled me out of death’s grip when I needed a landmark liver transplant to save my life. It was then that I was diagnosed with a disease known as VHL or von Hippel-Lindau.

Von Hippel-Lindau disease (VHL) is a rare, genetic multi-system disorder in which non-cancerous tumors grow in certain parts of the body. Slow-growing hemgioblastomas -- benign tumors with many blood vessels -- may develop in the brain, spinal cord, the retinas of the eyes, and near the inner ear. Cysts (fluid-filled sacs) may develop around the hemangioblastomas. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas. Symptoms of VHL vary among individuals and depend on the size and location of the tumors. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, deafness in one ear, and high blood pressure. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer.

There was a regrettable incident though because it killed my father...not my transplant but the cunning uncle who took our house from us. In order to save my life, my father couldn’t afford his bypass surgery and expired leaving us penniless and homeless for my sly uncle took our house by crafty means.

Surprisingly after his death my cogs and circuits malfunctioned, and time cheated on me. I needed those life-saving anti-rejection medicines and acquired the infectious consumption disease(MDR TB) under suppressed immunity

The fun ingredient of life is not only you get bombs in your lungs wherein you gotta lie low hoping they won't go off; they can be diffused by a squad with proper knowledge. Looking forth to sunshine so that the landmines (leptomeningeal hemangioblastomas) don't blow me off with an utter BOOM!

within a month after his death. Strange feelings came over me and it seemed strange and lonely sitting in those unhygienic shack-like houses. Now, after a few years sitting in my chair it seems strange that I survived 15 surgeries and radio-surgeries under such bizarre conditions.

My mother’s belief and boundless energy sustained us and not only we faced shocking poverty where we didn’t have money for food the next day, but I also overcame MDR- TB and its expensive treatment along with two sessions of radiation therapies (in brain for leptomeningeal hemangioblastomas-Between 1902 and 2013, approximately 132 cases ) which went on for six weeks, one cyber-knife and not forgetting the kidney cancer. Resection for these numerous tumours can cause excessive bleeding and tumour cell spillage thus non-invasive Procedures with little blood loss may bring rapid prognosis and till now it has been proven to improve the quality of my life thus far in my case. Whenever any of these multiple tumours become symptomatic or starts growing their growth is contained by radiation. Sometimes they start shrinking little by little after some time.

All my life-saving surgeries and treatments and diagnosis happened at the nick of time. I’ve had numerous surgeries, not dismissing from mind subtotal thyroidectomy which led to HypoPara (hypocalcemia due to low parathyroid hormone level). We get by with arranging all those expensive immunosuppressant (anti-rejection) medicines for the liver every month and laboratory and radiological tests with the divine power helping us because we don’t despair. The divine power is in every human.

Strangely enough, I became something of a connoisseur of the bizarre and the grotesque which led me to write the novel "Midnight-a thrilling novel". I haven’t forgotten everything of my past because my struggle hasn’t ended, every day of suffering, part of which remained in my circuits and when those fateful memories turn up, the electrons in the brain gets charged and misfires and I start remembering extra my mother channelizes that bottomless energy and I start writing and spinning out tales, thus I became an author. I can’t get back to my childhood days where I never enjoyed the hop-skip-jump of those days. Realizing such a strange tour is out of the question, I decided to wage a war against the rogue protein molecules( VHL or Von Hipple Lindau) that brought me into a condition that a faulty part had to be changed and the system rewired. Thenceforth everything went wrong from the sub-atomic level and other parts started sprouting unwanted malicious codes (tumors). They disrupted or inflicted some other harmful action on the network.

I survived kidney cancer but currently my pancreas is causing problems and the medicine Creon SD micro granules are very expensive and doesn't last more than a few days. One of those malicious tumors sitting atop my optic nerve in the brain makes me partially blind. Not even those killer sessions of radiation therapy could shrink it. Those tumors,especially the nasty ones popping up in my brain makes living every day a constant fight.

A new chapter started in my life story.It was a warm October morning and I was in a merry mood having a good time because of the approaching Durga Pujas. I always try to make most of the time with my mother. The season is very inviting and makes us happy and less anxious. I sat down with a book determined to enjoy it thoroughly but suddenly out of the blue something zapped across my right eye. The jolt of lightening appeared repeatedly. It lasted for a few seconds to a few minutes but it was difficult to keep my right eye open. This continued for the next few days but the pain was gone as abruptly as it appeared. I underwent an MRI scan. Leptomeningeal hamangioblastomas started compressing the nerves then I started fighting bilateral trigeminal neuralgia which is characterized by a sudden, recurring stabbing, electric current-like pain usually unilateral in nature. Bilateral presentation is rare. It is also known that it has something to do with an artery being near a nerve.

It made living every day a constant fight.

It was confirmed by the doctor that the left-hand side pain which is milder than the right-hand side is due to an artery being very near the nerve. Thus I have bilateral trigeminal neuralgia. The trigeminal nerve has three branches: ophthalmic, maxillary and mandibular. According to the doctor it is rarely seen that bilateral trigeminal neuralgia with pain in all three branches on both sides.

A new medicine was added to my list of medicines which made my blood pressure drop instantly with the intake of the first pill.

I was told that I should come perhaps at the last moment… I had a feeling of history being repeated. When my father was alive he went to most hospitals across India for my growing brain tumor causing partial seizures but I was told these very words “Come at the last moment when the tumor grows to the size of a Deus ball” it was also added that there was a chance of getting paralyzed for life. I didn't want to be a guinea pig. But the major thing which happened is after the medicine was added my BP plunged down. I have tachycardia and high BP most of the time but never low BP. I take medicine for that. I stopped the medicine and drank lots of fluids but to no avail. Doctors persisted in their decision of choice of medicine... They keep commanding in a military-like way that I need to take those medicines and my only respite from the overwhelming pain is the MVD surgery. I wanted to avoid the surgery because of my rare condition of multiple or numerous brain tumors, why don’t they understand that? I presume this is all due to a lack of awareness. How many of us know 29th February is celebrated as a rare disease day all over the world? Hardly a handful of Indians do.

All my life I have kept looking for hope. I have undergone 14 surgeries including cancer and a few life-threatening ones but I have never stopped hoping. Hope sustains us. I got this hope from Dr. Shankar Vangipram of HCG Khubchandani Cancer Center Mumbai who said he could definitely treat me by cyberknife radiosurgery. In a Cyberknife instead of Cobalt-60, a 6 MeV linear accelerator is mounted on a robotic arm that moves around the patient and fires radiation from different angles. The patient is immobilized but does not require a frame to be screwed to the head.

The soul of the warrior honors the cosmic spirit. A Warrior fights the battle of life ---all its dreadful possibilities without a whit of fear without thinking that it’s unfair. My trust in the Cosmic spirit who created me and my will to enjoy this beautiful world with Mum where little birds still sing on the trees and there is still warm sunshine waiting for me made me bold enough to fight the cruelty of the pain gnawing into me and destroying my life.

I got support and kindness and the cyber knife of the right side-- the most painful side happened about a month ago and we returned in time. Within four days of the nerve being shot with high dosage of radiation to destroy it, I wrote the poem--

Fall of the contagion

Mind is a powerful weapon they say,

Where knowledge is the mainstay.

As the catastrophe happened,

Prepare, be aware,

Avoid public places,

Refrain touching faces,

Life is not unfair,

Soon days will be brightened.

In jeopardy may humans stand,

But by maintaining distance,

Not traveling to any distant land;

Ensuring safety of the vulnerable,

Being responsible, together we stand.

Isolation is not perdition.

Not being utterly hopeless,

Being cognizant;

The turf is uncertain;

So is the terrain;

Can bring nemesis of pathogenesis.

The sinister brute would cast off its crown,

As time frown,

Monsters, cruel, fierce and base;

Causing deaths of life-forms

Would decay and fall,

Die a death approved by all.

To make people come to terms with the reality of Covid19.

I can't say about my entire pain while typing the lines of my books with my one-eyed vision. There with swelling in my eyes and eyes which felt like on fire with tears streaming down. There was pain at the back of my head and the ear and even the pinna. Pain hasn't entirely gone but in a month, I can say I am 80% better. Pain comes back little with weather changes or emotional strain or remembrance of life spent in great duress or thinking about life's wrongs, mistakes, miscues, misunderstandings and muddles . But when thoughts try to destroy the peace of my mind or cause pain, I think of something else--- the times I enjoyed with my mum.

I can't let it happen like before when I could just remember taking painkillers apart from the medicines I was on, but still, the pain remained. The agony of the soul as it shrieks in pain was indescribable at night. I know everything will eventually fall in place. Albeit there's a problem with stiffness in the neck muscles and trouble swallowing but that can be solved by practicing a few neck exercises and swallowing food with a sip of water. But still thoughts about getting cyber knife of the left side remains. After all nothing can be completely accurate. All which is needed is a good doctor who is thinking about your health and happiness and not treating you like guniea pig and not working for his own good and fame. I got the opportunity to choose between good and better doctors four times in my life, during my liver transplant, my MDR tb treatment, kidney cancer and trigeminal neuralgia.

I hope for aid from the government to cover this.and the high cost of subsequent medicines including anti-rejection medicines, radiological and lab tests required for brain radio-surgery, liver transplant and kidney cancer. Since it is impossible to predict how the disease will present or progress, active surveillance is exceptionally important for people living with VHL.

Treatment for VHL varies according to the location and size of the tumor. In general, the objective of treatment is to treat the tumors before they grow to a size large enough to cause permanent problems by putting pressure on the brain or spinal cord. This pressure can block the flow of cerebrospinal fluid in the nervous system, impair vision, or create deafness. Treatment of most cases of VHL usually involves surgery to remove the tumors before they become harmful. Certain tumors can be treated with focused high-dose irradiation. Individuals with VHL need careful monitoring by a physician familiar with the disorder.

I think one shouldn't believe in a doctor with eyes closed, rather be aware of what one is facing and the right doctor can definitely treat the disease. Dr. Arvinder Singh Soin didn't say transplanting the liver will cause the tumours to grow once again and it will be a futile effort. Dr. Randeep Guleria cured me of MDR tb which other doctors couldn't and because those doctors failed because of whom I can't walk properly as I had bone tb and I still need the help of a stick to walk. Dr. Sanjay Gogoi understood before taking out the cancerous tumour in kidney doing fine needle biopsy could spread the cancer to other parts of the body and similarly Dr. Sankar Vangipuram understood risks of MVD surgery on a leptomeningeal hemangioblastoma patient. I would like to get treatment for such an understanding doctor not only for my left side treatment of the trigeminal neuralgia but if any of my countless tumours because symptomatic.But doing a treatment by radio surgery will take time to let the pain go away. It can't vamoose like a magician's trick.

Our destiny is not always determined by what happens to us, rather how we react to what happens and surely not by what life brings to us, but our attitude towards it. The outcome of positive attitude is always positive thoughts and eventually leading to positive events. Life for me is a challenge to be met with courage. Life is a game of skill with an element of chance. Life comes to a standstill if you give up hope thus like every life from desiring to live she fought on. Like Asterix and Obelix traveling the untrodden track, I jest with Mum about the crazy Romans we met in our lives.

Journey through the gravelly road of life

I make my own roads,

Every stone on the way has a significance,

If I trip I gain more experience,

My remembrance

Helps me to use my diligence,

To make my road better.

To tackle my sorrow,

So that my eyes don't get wetter,

Thus building a brighter tomorrow.

Without hurt, without tear,

Without pain , without fear,

Without despair,

Where everyone will care for each other,

Angst and pain will disappear.

Everyone will love one another.

My missing liver which has been stitched back with a replacement; my left kidney, part of which is gone forever; my brain which has been burnt by radiation beams. resulting in an ischemic brain and the tumours scattered inside sparkling like a star punched sky yet the "little grey cells" had worked out an exceptional detective book during lancinating pain with one-eyed vision and typing with the index finger of the right hand because after the had got palsy and was treated with high-dose steroids( omnacortil/Prednisolone ) by Dr Shamsher Dwivedee but the hand doesn't work like before and the left had after recovering from paralysis during the craniotomy didn't get back the sense of touch and both halves of brain lost coordination and refuse to work together.

Also writing for Times of India digital spreading awareness, positivity and hope without honorarium...

Once upon a time the unusual birth of an eye-catching child of fond parents after a miscarriage and the following embryo getting problems while in the womb, were lost in the thought that behind the outer shell never thinking 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.

I was a precocious little girl. I started walking and speaking fluently at the age of nine months. The grim humour was a disease that was lurking inside with considerable firmness to follow me through each doom and see my reaction, that if I turn a victim or a warrior.

After my father passed away Poonam Gupta, student of Dr AC Ammini got in touch with me for research purposes.

I got an email from AIIMS.

"Hi, Payel. I have found a mutation in VHL gene from the DNA extracted from your blood. We have to further confirm the mutation by a different process & it will take a month because the enzyme used for that analysis is not available in India & we have to order that from Sigma, USA. It will be great if you can send me the sample of your brother & mother then it will be very easy to confirm the mutation."

Next email

Next time

"You are suffering from VHL Syndrome that has been confirmed from your genetic testing of VHL gene.

You may need the following test to be done each year.

1. MRI Whole body

2.PET-Scan

3. Plasma/Urinary Metanephrines

4. routine analysis of blood chemistry

5. Regular chart of B.P"

Von Hippel-Lindau disease is a neurocutaneous syndrome. A neurocutaneous syndrome causes problems that affect the brain, spine, and nerves (neuro) and the skin (cutaneous).

In Von Hippel-Lindau disease, tumors most commonly develop in the brain and retina of the eyes. These tumors, called angiomas, consist of blood vessels. Other types of tumors develop in other organs and include tumors in the adrenal glands (pheochromocytomas) and cysts in the kidneys, liver, or pancreas. As people with the disorder age, the risk of developing kidney cancer increases. By age 60, the risk may be as high as 70%.

Hemangioblastomas of the CNS are solid or cystic vascular-rich tumours, most common in the cerebellum, less frequent in the brainstem or spinal cord and rare in supratentorial locations with meningeal involvement.

Bakshi et al5 suggested that this phenomenon occurs as a result of release by tumor cell factors that promote aggressive angiogenesis or increase vascular permeability.

Hemangioblastomas of the central nervous system are the most common tumours seen in patients with von Hippel-Lindau (VHL) disease.

Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Between 1902 and 2013, approximately 132 cases were reported.

Few studies have reported leptomeningeal involvement in sporadic HB or HB associated with von Hippel Lindau syndrome.

Detecting and treating the condition of leptomeningeal hemangioblastoma without delay seems to help survival, though the number of patients analyzed is small. The most common cause of death was respiratory failure due to pontomedullary or cervical cord compression.

Because no case of de novo development of disseminated HB without previous surgery has been reported, it is strongly suggested that the spillage and spread of tumour cells through the CSF space may be an origin of hemangioblastomatosis in patients with a genetic predisposition to the condition, Care should be taken to avoid tumour cell spillage during surgery.

A 68Ga-DOTANOC PET-CT based SSTR imaging because VHL syndrome associated hemangioblastomas frequently express SSTR

(Somatostatin Receptor)confirmed that the floating lights in the patient's brain are hemangioblastomas.

Early detection and aggressive management can enhance the prognosis of patients with CNS leptomeningeal dissemination of HBs. Imaging surveillance, by contrast-enhanced spinal or brain MRI, should be considered in patients with a large amount of tumor bleeding during initial surgery.

After 2017 I also have a chronic ischemic brain. 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.

"I have encountered the problem that you describe below. We have used craniospinal radiation in some cases and/or adjuvant chemotherapy for leptomeningeal hemangioblatomatosis. Unfortunately, I can not open your film files. Russ"

The opinion of Dr Russell Lonser is very important.

.https://wexnermedical.osu.edu/find-a-doctor/russell-lonser-md-47618

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 tumour but also the healthy tissue that the beam encounters on its way towards the tumour and beyond the tumour. 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 tumour 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 tumour.

Not to worsen the condition further, I sought the help of Dr Jalali

The hypoxia- inducible transcription factors have also been shown to play a crucial role in tumor promotion in other cancers including breast, brain, colon, gastric, lung, skin, ovarian, prostrate, renal, pancreatic. Increased levels of growth factors result in angiogenesis.

VHL is the key to know how tumours grow and how potential therapies can inhibit angiogenesis.

It may sound complicated, but having a VHL diagnosis shouldn’t consume a patient’s life. In fact, monitoring through routine screenings is one of the most important components of care for patients with the inherited condition, which puts them at an increased risk for developing tumors.

While prognosis for children with VHL-associated disease is excellent, the challenge and essential component of care for these patients is surveillance, as the tumors can slowly grow and impinge on organs.

We are poor and abandoned with only Rs 7000 mother's pension which is spent mostly on food and my mother's health needs as she is almost a septuagenarian and the nagging worries about rent, electricity and water bills, food and then medicines with surveillance and doctor's fees and conveyance charges. My personal income is almost nil after my rare thoughts and manuscript of a good book " Adventures of Mum and Princess" - an anthology detective story was stolen by the owner of 3 English speaking schools for the purpose of syllabus of classes 8, 9,10. I wrote this book at the peak of pain of Trigeminal Neuralgia.

After this incident my personal income is nil.

I beg from Facebook acquaintances and some say " will send you in a few days" but that never happens.

NPRD gently and politely smashed hopes for continuing my treatment by choosing a handful of diseases by foretelling and quantifying the quality of human life. Not considering what I have done despite several symptoms of vHL. I am a warrior not a victim.

Our government doesn't understand when medicine is socialized, then you have true health care. When everyone's treatment is the same regardless of the socioeconomic position or age there will be justice. Otherwise, there will always be a tormenting distress about someone somewhere else getting what you are not.

Swamping those who are rare and need to fight daily for life, not just recuperate but also keep thinking about how to live, how to arrange food, medicines and a roof above the head and how to be relatively healthy. It's tragic that the government thinks "life and health of a citizen is an unfair burden".

My mental states and processes are supposedly made up of a different sort of stuff and take place in a different sort of realm. I disregard the view of this prevalent tendency to equate mind with brain, and the accompanying assumptions about the spatial boundaries of the human cognitive realm. My brain is naturally adaptive and the natural plasticity and unique brain's circuitry, partially busted, makes it plastic par excellence and makes me endure anything and not be a victim but a Warrior Princess.

I am very stimulated. My mind never stops. I write because reading and writing has been my passion. The beautiful simplicity of the ideas attract me , astounds me and penning them down gives a blaze of joy and exultation. My ideas have novelty and an unusual polish like locked room detective stories or investigation of reincarnation , time traveling and doing investigation of a murder in the past or spooky novels with a dash of spy thriller.

Before I became partially blind I could draw several pictures.

All which is needed to lengthen the period and quality of life is a good doctor who is thinking about your health and happiness and not treating you like a guinea pig and not working for his own good and fame. I got the opportunity to choose between good and better doctors four times in my life

I wrote my entire autobiography which is about my struggle in crossing a thunderstorm amid thunderclaps and heavy rainfall with the help of those who felt an immediate and deep connection with my struggle. I named it ' A Warrior dies dancing...That's who I am. ". Some people found it funny but my explanation is ---Life is a joust where the opponent you are riding against is death. When a seasoned warrior steps into the battlefield fighting valiantly, she delights in the spirit of it and it's glory. The soul of the warrior honors the cosmic spirit. A Warrior fights the battle of life ---all its dreadful possibilities without a whit of fear without thinking that it’s unfair. Before we are inevitably visited by the death and thrust to total chaos a warrior’s mind gracefully dances to the rhythm of the music of creation and life. Thus, A Warrior Dies Dancing, That’s Who I Am… The upshot is life belongs to us but we belong to death meanwhile dance away the time you get.

I also wrote ‘The Adventures of Mum and Princess” which is a really thrilling book, unlike the deadly one (Midnight)you just read about. My war is still going on.