Understanding the science behind rare brain tumour – leptomeningeal hemangioblastomas

 Brain tumours grow within a rigid, firm bony skull. Benign, slowly growing or malignant brain tumours may produce serious neurological symptoms and signs prior to treatment or cure. Although brain tumours rarely metastasize outside the central nervous system (CNS), disability and death occur with brain tumours when the intracranial contents exceed the intracranial space, causing herniation and compression of respiratory centres.

Fewer than 5% of patients with brain tumours have a predisposing genetic syndrome. The most common of these are von Recklinghausen's types I and II neurofibromatosis, tuberous sclerosis, von Hippel-Lindau disease, and the epidermal nevus syndrome. These dominantly inherited neurocutaneous syndromes are associated with an increased incidence of specific tumours.

There are more than 120 types of tumours of the CNS.

Neuroepithelial tumours are the most common and feared tumours of adult life and occur most frequently as astrocytoma, oligodendroglioma, and ependymoma.

Leptomeningeal hemangioblastomas are perhaps the rarest sort.

What is a hemangioblastoma?

Hemangioblastomas (HBs) of the central nervous system (CNS) are histologically benign, slow-growing tumours, which may occur as sporadic lesions or in association with von Hippel­Lindau (VHL) disease.

What is von Hippel-Lindau or VHL?

von Hippel Lindau is a very rare disease. 

von Hippel-Lindau or VHL which is a genetic defect that causes capillary growth to go out of control. While the tiniest blood vessels or capillaries usually branch out gracefully like trees, in VHL patients a little knot of extra capillaries forms a growth or tumour and in certain cases, it turns cancerous. It is a genetic form of cancer VHL patients battle a series of tumours throughout their life. 

VHL may occur in up to 10 organs of the body like liver, kidney, brain, spinal cord or retina, inner ear, pancreas, pheochromocytoma, paraganglioma can also happen.

What is the leptomeninges?

Meninges are the three membranous envelopes—pia mater, arachnoid, and dura mater, that surround the brain and spinal cord. Cerebrospinal fluid fills the ventricles of the brain and the space between the pia mater and the arachnoid. The primary function of the meninges and of the cerebrospinal fluid is to protect the central nervous system.

The two innermost layers of tissue, arachnoid mater and pia mater that cover the brain and spinal cord are together called the leptomeninges.

Leptomeningeal dissemination of Hemangioblastomas

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 al3 described a 55-year-old patient with disseminated intradural masses involving almost the entire spinal cord on magnetic resonance imaging. They reported both extramedullary intradural tumour with numerous leptomeningeal nodules and microscopic infiltration of the spinal cord and coined the term leptomeningeal hemangioblastomatosis to define this condition.

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 in HB associated with von Hippel­Lindau syndrome.

Diffuse infiltration of the leptomeninges is known as hemangioblastomatosis and has been observed both in VHL disease and in patients with spontaneous mutations. 

Treatment

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

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.

Prior to surgery of the initial tumour, planned embolization should be undertaken if possible to reduce blood loss. However, depending on the actual tumour structure embolization may be found not to be possible. Reducing blood loss may also help in reducing tumour cell spillage and spread.

 Looking at tumour cells in the spinal fluid under high-resolution MRI scans are all that is necessary for an accurate diagnosis.

When another mass was illegally cultivated on top of the kidney, a Ga-DOTANOC PET-CT based SSTR imaging because VHL syndrome associated hemangioblastomas frequently express SSTR confirmed that the floating lights in the patient's brain are hemangioblastomas. With this, the true nature could be seen and the diagnosis was confirmed.

A biopsy isn't required for diagnosis as it may cause meningitis and blood loss thereby cell spillage. 

My experience with leptomeningeal hemangioblastomas for 7 years? 

I was diagnosed with supranational leptomeningeal hemangioblastomas in 2013. I have been seeking anyone experiencing the same condition but didn't find anyone till now.

A fellow remarked after he saw the picture of the scan of my brain tumours

" you have more tumours in the brain than people have lice in hair.''

There is no data on the competence of the surgeons and equipment used in the initial CNS surgery so trying to determine if cell spread was caused by any incompetence during my brain surgery (craniotomy) in 2006 would be extremely difficult if not impossible to prove beyond a reasonable doubt. The fact that the physiology of each tumour is different and the number of cases is so small means that an accurate comparison of surgeons is impossible. 

These tumours are extremely vascular. A biopsy would make them bleed resulting in a stroke. After my first brain tumour surgery, done in 2006 I woke up with paralysis on the left side. After the craniotomy ( open brain surgery) I experienced total numbness in my arms and hands and I couldn't close my fingers and make a fist. But this was sorted out by proper physiotherapy and exercise and I got back the control of my limbs but even now I don't have sensations in my fingertips.

I get stereotactic radio-surgery before the growing tumours start putting pressure on the brain and become symptomatic. I have lost vision of my right eye for not being able to avail radiation therapy at the right time because of financial reasons and also because  I was diagnosed with an RCC ( kidney cancer) at the same time. 

Advanced radiation techniques, such as radiosurgery, are more effective than conventionally fractionated radiotherapy, but it is difficult to perform high-dose radiation therapy or radiosurgery for numerous lesions scattered throughout the brain. 

Radiosurgery appears to be safe and has prevented local recurrences in my case, with fewer sessions than conventional radiation which caused white matter changes representing chronic ischemic changes. I couldn't afford a cyberknife and underwent conventional radiation therapy in 2017 after which this happened. 

Sometimes tumours treated with radiation, on follow-up MRI imaging scans, appear stable without evidence of growth even mildly shrinking every time. 

LMD

Unexplored safe technology.

It is a new exciting technology which may help people trying to minimise collateral damage which with radiation treatments has always been the challenge. 

The precision of proton therapy can be critical for protecting the brain’s delicate tissues.

Proton therapy can limit the amount of normal brain tissue receiving radiation. That reduces the effects on important functions, such as vision and hearing.

Proton radiation may be less likely to damage nearby organs, such as the heart and lungs while treating areas around the spinal cord. This technique is safe and potentially effective.

Often, people with these recurrent tumours have received significant radiation doses in the past to important parts of the brain. These may include optic nerves, which are critical to vision, and the brainstem, which regulates many essential body functions, such as breathing, heart rate, and swallowing. 

To treat more people with proton therapy is by making the technology even more accessible. Currently, this technology is available only in Apollo Chennai and expensive enough to cost an arm and leg.

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. 

References

• Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature in journal Acta Neurochirurgica.

• Supratentorial leptomeningeal hemangioblastoma resection after preoperative embolization Lee, Ching-Yi, Chen, Shiu-JauLanguage: English Journal: Formosan Journal of Surgery.

• Journal of Korean Medical Science, article Arch Pathol Lab Med--Vol 132, January 2008- Intradural Extramedullary Leptomeningeal Hemangioblastomatosis and Paraneoplastic Limbic Encephalitis Diagnosed at Autopsy

• Journal Neuropathology -A report of supratentorial leptomeningeal hemangioblastoma and a literature review 

• Power and Precision: Proton Therapy Can Target Brain and Spine Tumors with Fewer Side Effects

Book lovers day : How books influenced me in different stages of my life

 All I can say is that I was born with a book in my hand. Reading was not only my passtime, but it was also my passion, my obsession and fodder for my active mind. Books stimulated my brain switching on the thinking process and the reasoning and led me to the peak of happiness.

I started with Enid Blyton and enjoyed her Famous five and Secret Seven series, also the wonderful magical adventures of Wishing chair, Faraway tree, Mr Pink-whistle's party which enhanced my vivid imagination to a brilliant and powerful one. Albeit damn interesting, books helped me pick up some confidence and optimism and admire the forces of nature, rain or sunshine, wind or quietude.

As I grew up in my aloneness books became my only companion. I spent a good deal of time in bookshops turning over the pages of books I used to think if only my life were a thousand years long I could read all these books. Two bookshops became my regular haunting place to which I went for an evening walk and returned loaded with wrapped up books. 

By this time I had read all Agatha Christie. Michael Crichton and Arthur.C.Clarke books I was reading as many books as  I could lay my hands on. I fell in love with their novels so much so that an idea lurked in my mind that one day I will be an author. After reading "Wheelers" by mathematician Ian Stewart and biologist Jack Cohen, I told my father " I am going to be an author." He took the book I was holding and looked at me happily. "Okay finish your studies and keep reading books. I will be the happiest person to find a publisher for your books", he gave me a frank smile that strengthened my confidence. While reading "The Name of the Rose" by Umberto Eco I discovered Philip.K.Dick and Neil Gaiman. I'd like to see shelves with books rather than furniture in my room. When Philip K Dick entered my life I had odd sensations, fidgety and nervous but didn't understand my feelings. I had odd nightmares but told my mother that aliens are trying to get in touch with me so just keep everything shush! I could hear things which others couldn't and see stuff which nobody did! I asked my mother to be sure of that and was convinced either I could see ghosts and hear them or aliens are trying to get in touch with me. I was happy with the thought.

After some time elapsed my mother saw me getting violent partial seizures while in sleep, she called my father and both tried to hold down my body getting spasms but the force with which it was happening they couldn't. After I woke up I didn't remember what happened to me at night. For this brain tumour, father ran across India looking for a suitable doctor. After fifteen days of admission,  when the doctor saw I was reading "Minority Report " in the hospital bed he denied doing the surgery but I was told these very words “Come at the last moment when the tumour grows to the size of a Deus ball” it was also added that there was a chance of getting paralyzed for life.

My father sought out a tiny nursing home in Kolkata with minimum facilities where ultimately the surgery of the tumour was done in 2006. After the craniotomy ( open brain surgery) These tumours are extremely vascular. A biopsy would make them bleed resulting in a stroke. I woke up with paralysis on the left side. After the craniotomy ( open brain surgery) I experienced total numbness in my arms and hands and I couldn't close my fingers and make a fist. But this was sorted out by proper physiotherapy and exercise and I got back the control of my limbs but even now I don't have sensations in my fingertips. Currently, I am suffering the consequences as Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Between 1902 and 2013, approximately 132 cases were reported. Without previous surgery such cases haven't been reported, it is due to spillage and spread of tumour cells through the cerebrospinal fluid(CSF) space in patients with a genetic predisposition to the condition. I made up my mind to keep myself well-informed so that I don't get into such situations.

Well, certain things are just fated, and I had a liver transplant but I had the ability to overcome it and the subsequent viral infection which was supposed to be fatal for me. But life offered a few moments of bliss, and happiness then. I read up " One Flew over the cuckoo's nest" and finished the Ring trilogy by Koji Suzuki. 

When my tough times began after my father perished books kept me away from the feeling of irritation and provided courage and strength. I read, "The Shadow of the Wind" but "The Lovely Bones" put my spirits down. I thought I'd better not read Isaac Bashevis Singer, Albert Camus or Jorge Luis Borges. Times change and when things get better I shall read those. I started with " Wise Guy", Jack Reacher novels when I discovered a marvellous thriller "Killer inside me".

Then I had a cyberknife. It was a time which had a lasting effect on my life. I had sudden brain fog, short-term memory loss and I became cranky. I wrote my poems and read and primarily stuck with crime thrillers. I possess a lot of unobtainable books and I am glad my father and brother always gifted me books.

I discovered "A Kiss before dying" by Ira Levin. After I finished the book I was sorry I had finished it so soon. I developed abililiophobia, a critical condition. The book reminded me of the Bollywood movie "Bazigar" but as we know books are always better than films. I made up my mind if my brain permits me to be an author I will write an entirely different book like no one has ever read before or can correlate to.

But then again, I was in dire straits as the landlord presented me with a court notice on my birthday as I was diagnosed with kidney cancer and optic nerve tumour but by a twist of fate I got cancer surgery but optic nerve radiation therapy was late making me blind with the right eye and left with mum within the stipulated time given by the court. 

While reading my favourite Mark Twain short stories and" Letters from the Earth" re-reading "Three men in a boat", " Dracula" I read Shirley Jackson's books.

I made up my mind after completing Roald Dahl that I'd write a ghost story someday. 

One sunny morning, sitting with a cup of coffee after the 2017 radiation therapy I made up my mind to write unique detective stories. I created unique characters drawn from my life and wrote about Mum with an unexpectedly brilliant and problem-solving mind enjoying the guise of a harmless little lady with a remarkable facility for observation and notation of the trivia which often leads her to solve impossible cases. Her daughter Princess is very ill but doesn’t spend time brooding over it. With her anxious searching gaze and flashing her brilliant determined smile twiddling with various theories, she helps Mum solve mysteries introducing Verma Sharma detective duo as comic relief and Arin as an inspector in the homicide department who takes the help of Mum for puzzling cases. Even Dr Ishita liked the stories.

Next time I thought of writing a paranormal story of a gifted child. She has an unusual ability. The power to see those spirits wandering on the margins of the world. The fabled sprites come to meet her, to play with her but this amazing ability makes her different. It doesn’t let her blend in with normal people. One day a vengeful spirit visits her and tells his story. The ghost was on a desperate quest of a murderous assassin to stop a crime against humanity when he fell into their trap and got killed. He seeks help from her to avenge his killers. But how did Mina go missing? The story is in the diary. It is a medley of ghost story and spy-thriller like never been written before.

But my disease is an albatross around my neck and I developed severe lancinating pain of bilateral trigeminal neuralgia and it felt like holding a live cable against my eye, cheeks, teeth. I got a cyberknife of right side but the left side is pending. I have several problems after the cyberknife but no way to get an MRI or follow up with the doctor. Hence, I'm again with my beloved pals. Reading and writing help to improve memory and get a good night's sleep. The entertainment part is an important part along with boosting courage. I have taken names of just a few of the books I have read, I have missed a lot of gems like Kazuo Ishiguro, P.G.Wodehouse, Keigo Higashino, Kanae Minato, Stieg Larsson and the classics which are a vital part of our lives.

Floccinaucinihilipilification

 

The most striking disclosure among the world of people is that we are connected. According to neuroscience, our brain is designed to make us sociable. The neural bridge lets us affect the brain and body of everyone we interact with and vice versa. The more strongly connected we are with someone emotionally the greater is the mutual fondness. Our most vital conversations occur with those people with whom we spend the greatest amount of time, day in and day out and those who care about us the most.

The result of the neural linkups has profound outcomes that vibrate through the core regulating our immune system and triggering the flight or fight hormone. Thus my childhood relations with cousins, school friends and family members had both favourable and unfavourable impacts on my life.

When I grew up I learnt that people who "used to be close to you...who had meals in your house, stayed over with you, enjoyed every celebration with you, travelled to the mountains with you"… gladly walk away when they see you in danger, they don't care if you live or die.

When I make a trip down the memory lane I wonder if humans have a mind to act hand-in-hand, to help others even when it requires their efforts? Or are they, in their very hearts, selfish creatures?

 I think for them life and lust are synonymous. Everyone has a similar hobby, that of collecting money. Even kinship and friendship can be pitted against the desire for large sums of money.

Money under most circumstances can't buy peace and happiness for you. Your Creator will provide just as much as you need for your own personal sustenance because that is what you deserve, asking for more is being greedy and is a punishable offence. We find everyone running after fame, prosperity, wealth and power which results in daily indigestion and therefore they have built-in gyms in their premises to work out, not just to build eight-pack abs by shedding out the extra fat but to get rid of menacing indigestion and get a peaceful night's sleep which seems to elude them always. You become a runner with extra fizz or a singer with a melodious voice without realising the timeless melody of life. There are a range of emotional traffic from sadness, anxiety to extreme joy. Mother's smile is a contagion and acts as a way to uplift the sport.

Neuroplasticity reshapes the brain with repeated experience with various relationships with the effect of either being chronically hurt or chronically angered or emotionally nourished. 

From the time we are infants, we are equipped with the survival instincts necessary to respond with fear when we sense danger or feel unsafe. But sometimes people facing constant challenges develop boldness boosted by the nourishing relations which can also buffer us from diseases. The vital nourishing relation in my life has been my mother. Mum had been exploited and overburdened by her in-laws, yet she had taken care of me and suffered herself as her own kith and kin turned their backs on her.  She had never known soundness, only struggle. With age, she too is getting tired and irritable tackling all the work single-handedly. She needs to be taken care of. She is my caregiver and she taught me to walk boldly through the terra incognita.  As you learn to control life it becomes joyful.

A Warrior Princess I am, 

I wage wars with head held high, 

A warrior, never afraid of wounds, 

A Warrior dies dancing that’s who I am.

 

Trudging hills never trodden by, 

Propelled into uncertain territory, 

Soul deeply wounded by black melancholy, 

Never stooping to fear or sentimentality, 

Chasing desires, not waiting for fate to show mercy;

Flowing forth with random delight in stark reality,

Gloom of doom come what may;

Banished all worries, doubts, 

I moved onward in the track valiantly, 

Imploding yet ebullient in a spree, 

Compounded difficulties, hiemal cold manner can’t faze me,

Benign friends express fervency.

Content with mysterious, meaningless, contradictory, hostile, 

Unexplainably warm and giving,

Fearfully enclosed minds,

I hurtled the absolute randomness, 

On the verge of uncertainty. 

 Proffered relations, friends, unfailing support,

 Bravado, mercy, pity, arrogance,

A loner rushed forth in the path of life.

Halt! Patience!

I hear people cry,

But I'm at a point of no return. 

 

I’m brave, I rave,

Grave only when repressed,

Crammed with memory, hoarding much,

Endured much, weary of wounds,

Feelings which built up in the War,

Hardened and died inside,

Soul searcher I am,

Reality seems absurdity,

Kind, affectionate friends,

Elevate me, eke out positivity. 

 

I still dare to change the things I can,

I resuscitate ceaselessly,

I wage wars with verve

In a point of no return

Yes, a Warrior Princess I am.

Wild bouts upended direction, 

Infallible friends formed my legion, 

Marched with me without regression, 

Flew fearless without reason, 

Melancholy replaced by undying optimism,

Turned to memory,

Worries banished, never looked back in history. 

 

 I welcome change with open arms,

Grow and live vibrantly not in a trance,

Soul dance,

Immense will billow like a towering tsunami;

Summon up the courage to live life once,

Regression dishonor efforts and makes them undone,

Thus I move on with fluency.

 

I hurtle the chaotic reality in Mum's glorious presence,

I resuscitate ceaselessly,

Wage wars from a point of no return.

A Warrior Princess, I am.”

There are few people who are born good, instinctively concerned with the welfare of others and they have realised my sufferings and struggles. I am blessed with a few true friends in India and across the world who encourage and help me. 

A friend is sweet when it's new,

 Sweetest when it's true.

Many thought that by helping a destitute they will use me as their portable step ladder to fame.

Prettify, nor falsify no one,

They are but for a season,

Gathered to escort the lost one,

By way of cosmic expression,

Collective train of thought mitigates privation.

 

Here comes the season

Of faces blurring and fading away

Like a dream, in illusion.

There are also a few doctors who cheer me, while some say they are proud of me others help me out with paying hospital bills or others who are very close listen to me and attend my Whatsapp messages every day. These interactions help me get along better with life. 

These actions are like grasping hands when pain and distress are tearing my soul apart and assuring that the whole world is so beautiful and smiling with me beckoning me to jump up and go watch the sun-kissed world and a spontaneous force pulls strings from within and I find my wounds healed.

 My brother's concern too helps lessen my pain and the daily struggle, his sudden bursts of positivity.

Life is a game of chance;

Granted once;

Supercalifragilisticexpialidocious! 

Life is so beautiful and precious!

I was dumbfounded to find social chameleons in my life, it was pathetic and ruthless, meanest, greediest, cruellest, backstabbing act knowing in the heights of my lancinating pain and how with my brain riddled with tumours I produced the intellectual property and taking the opportunity of the deathly silence of the pandemic earning their livelihood. I expect everything, the unexpected never happens, printing and training have become a game. " Love thy neighbour as thyself, help them with thy soul and thy mind"  is the eternal saying but days have changed. People with shabby ethics want to swamp a struggling differently-abled patient suffering continuous surgeries from her ailments but they have chosen the wrong soul war which began surely by stepping on the edge, in ‘extremis’ still continues.

Her cogs and circuits malfunctioned

She fought the war unlamented

She faces life uncomplaining, contented

A very long time ago, a lifetime before the present

A dragon came to blaze up her life, push her to perdition

She traversed Via Dolorosa holding tight

Inspired by glorious Mum's might

She fought the demon with Mum’s inspiration

With congested brain but plenty of imagination

Faced the momentous occasion

Putting her faith on Divine Providence

God's intervention

Even in unthinkable pain

She does not complain

All is but floccinaucinihilipilification

She smiles in jubilation.

Sketch by me

Turn on the sun, light up a life by organ donation.

Kings held a unique position in ancient Egyptian society, they were somewhere between human and divine.

Thus they believed that when the king died, part of his spirit (known as “ka”) remained with his body. To properly care for his spirit, the corpse was mummified, and everything the king would need in the afterlife was buried with him, including gold vessels, food, furniture and other offerings. Their riches would provide not only for him but also for the relatives, officials and priests who were buried near him.

The pyramid's smooth, angled sides symbolized the rays of the sun and were designed to help the king's soul ascend to heaven and join the gods, particularly the sun god Ra.

Likewise, do you plan to bury or burn your favourite car, washing machine and all your possessions so that you can use it after your death? If you are planning to build a mausoleum for all your favourite things you are free to try it out and see if you can use it in your afterlife, including all the wealth you have acquired.

I want to remind you of what I mentioned in my last post

"Devi Parvati sculpted a son to guard the door of her palace. When Lord Shiva tried to enter he drove him back and all the Gods. While Ganesha was involved in a duel with Lord Vishnu, Lord Shiva moved slowly from behind and cut Ganesha's head with his trident. Lord Shiva agreed to bring back Ganesha to life and grant him divinity. Lord Shiva did the first mytho-historic transplant surgery and Ganesha was brought back to life.

After the transplant surgery, Lord Shiva is believed to have given Ganesha an elixir to drink. One cannot help speculating if this was some potion containing an anti-rejection medication."

Also, how Prometheus unflinchingly bore the cruelties and the tortures inflicted by a bird of prey eternally for gifting fire to better the lives of mankind.

In every field of life, you enjoy the results of your work or suffer the results and you cannot escape it. This is your karma. You suffer or enjoy the results of your activities from time immemorial, but you can change the results of your karma and this change depends on the way you react. For every action, there is an equal and opposite reaction.

 A hug is like a boomerang, it comes back to you because if you hug someone you get hugged too. So instead of burying or burning hearts, lungs, kidneys, livers and all other organs why not pledge to donate them. 

Donate your eyes and help a soul to sense the beauty of nature. How little birds twitter on green leafy branches, the warm rays of sunshine light up the cloudless sky.

Don't say tut!tut!tut! Because lots of people need a healthy organ, today is Friendship Day and tomorrow is Rakhi, an auspicious day so why not gift a brand-new life to some struggling spirit? Your payback will be refunded.

Remember the liver is the only organ which you can donate while you are alive and kicking and even if 70% of your liver is is removed and can grow back to normal size within a few weeks provided the remaining 30 %is healthy. Thus, a living person can donate a liver out of affection and go back to normal life in a matter of days. This transplant is safe and possible due to this uniqueness.

This is also why half livers transplanted into recipients are very successful since they soon grow to normal size.

Our scriptures say

• Do your duty, but do not concern yourself with the results.

• The fruits of your actions are not for your enjoyment.

• Even while working, give up the pride of doership.

• Do not be attached to inaction.

Gift of life is the most wonderful thing on Earth. Light up the candle, the dia and make someone's life joyful and livable. No deed is greater than this. 

"Turn on the sun, turn on the sun

Open the doors, come tell everyone

Bad times are out, good times are in

Turn on the sun, let's smile again."

For more guidance on living liver donation please contact

Dr Arvinder Singh Soin

Chairman

Institute of Liver Transplantation and Regenerative Medicine

He's a wonderful person who will guide you and explain how safe it is!

Miracles and madness of life

I had a strange childhood. In my early childhood, I masked my unique feelings and emotions and hid it from my parents.

When I started getting partial seizures doctors thought they'd do an MRI and the scan showed a tumour had grown more than five times from a tiny dot from a previous  MRI. Shouldn't they have watched the tiny spot? They neglected thinking it won't harm in any way. My father went to most hospitals across India for the symptomatic brain tumour. After fifteen days of admission,  when the doctor saw I was reading "Minority Report " in the hospital bed he denied doing the surgery and told me “Come at the last moment when the tumour grows to the size of a Deus ball” it was also added that there was a chance of getting paralyzed for life.

My father sought out a tiny nursing home in Kolkata with minimum facilities where ultimately the craniotomy was done in 2006. 

Within four months I had strange symptoms and vascular tumours were detected in my liver but due to lack of money, we didn't get treatment. Meantime, tumours grew and studded the liver in a way that transplant was the only way out. 

I always felt captivated when I heard the lines " Mutation is the key to our evolution It has enabled us to evolve from a single-celled organism into the dominant species of the planet. The process is slow, normally taking 1000 and 1000 of years. But every few hundred millennia evolution leaps forward"

I had never loved any character as much as I loved Wolverine with his enhanced physical capabilities and powerful regenerative ability -- the healing factor. 

I had endured two episodes of intense pain and in the last one asked the doctor to get euthanasia done. It was so painful.

But the magic of myth guided my doctors and my life turned around.

When I read the story of Prometheus creating men, I never knew it would change my life. Initially, it was a sunless world, with land, air and sea mixed together reigned by a deity of Chaos. Prometheus was entrusted with the task of sculpting Mankind from mere clay.

 He went to make them wiser and better but was filled with grief as he found them living in caves holes shivering with the cold because there was no fire, dying of starvation, hunted by wild beasts and by one another.

In order to better the lives of his creation, he went into direct conflict with Zeus and when Zeus withheld fire he disobeyed him and stole fire from heaven as a gift to Man. Zeus, the ruler of Mount Olympus had no compassion for the man and he meted out punishment for this daring crime to Prometheus.

For this rebellious act, in his rage, Zeus chained Prometheus to the Great Caucasus Mountains where an eagle was set to feed upon his ever-regenerating liver.

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

I found hope as I remembered my maternal grandmother's words, 

Devi  Parvati sculpted a son from some clay out of a pond to guard the door of her palace. Ganesha stood as a sentry as Devi went for a bath. When Lord Shiva tried to enter he drove him back and all other Gods. Lord Shiva cut Ganesha's head with his trident. As Devi Parvati learnt of the tragedy she was ready to destroy the universe. To appease her Lord Shiva agreed to bring back Ganesha to life and grant him divinity. As the head was lost in the battle his companions went in search for a first living being they saw, it happened to be an elephant. Lord Shiva did the first mytho-historic transplant surgery and Ganesha was brought back to life.

After the transplant surgery, Lord Shiva is believed to have given Ganesha an elixir to drink. One cannot help speculating if this was some potion containing an anti-rejection medication.

In a similar way, I got a new lease of life through a landmark liver transplant with the funds acquired by donations. But then my father perished and my foxy uncle took our house from us in a cunning way and we were left penniless and homeless.

These days we are aware that the liver is the only organ in the body endowed with the power to regenerate itself after large portions of it are removed. Liver functions normally even when as much as 70% of it is removed and can grow back to normal size within a few weeks provided the remaining 30 %is healthy. Thus, a living person can donate a liver out of affection and go back to normal life in a matter of days. This transplant is safe and possible due to this uniqueness.

This is also why half livers transplanted into recipients are very successful since they soon grow to normal size.

It was then that my real tough times began and we had two choices ...death or preservation of life. Not only we faced shocking poverty where we didn’t have money for food the next day, but I acquired MDR- TB living in lousy cramped houses and changed houses seven times hounded by landlords but ended up with 15 surgeries with the rarest brain tumours.

I understood the frightfulness and the oddity of my nasty lingering illness and its larger economic implications...the humiliation of asking for financial help, money concerns for food, medicines and rent as if not just physical suffering was enough.

 My brain tumours are the rarest ones. A fellow remarked after he saw the picture of the scan of numerous lesions scattered throughout my brain,

" You have more tumours in the brain than people have lice in hair.''

After I returned from a cyberknife of the right side of my bilateral Trigeminal Neuralgia I asked help from an elite lady who has already earned her name but she desired to bask in the glory of her deeds being choosy... which duty to do… which will fetch more attention of the whole country and told me she could arrange for my immunotherapy because she knows Dr Randeep Guleria, the director of AIIMS...I was speeches about how people can be narrow-minded not knowing the larger world around them.

Immunotherapy works by boosting the immune system so it works harder or smarter to find and attack cancer cells. whereas, immunosuppressants are drugs or medicines that lower the body's ability to reject a transplanted organ just like any bacteria or virus.

Also, I'd like to mention I know Dr Guleria pretty well because he diffused the bombs of MDR TB in my lungs.

These days people understand only their own troubles and see their own perspectives. Soft-brained, weak-minded, chicken-hearted people get the best attention whereas a warrior is like an elephant in the room like something everyone is aware of, but which is being ignored because everybody finds discussion about it uncomfortable. I am afraid the differentiation between diseases, comparison of lives will stop when pigs fly.

While the world wages a war against COVID 19 my war intensifies.

There is a concern that immunocompromised patients are at a greater risk of morbidity and mortality due to COVID-19 infection, although data on liver transplant patients is limited at present [6].

Immunosuppression strategy during the COVID19 pandemic 

Although there is a concern that organ transplant patients may be at a higher risk of COVID-19 infection, there is no evidence as of now to modify the immunosuppression protocol. Standard immunosuppression should be followed in the post-transplant period until further data is available.

Follow up post-transplant.

Patients should follow up with their respective centres as usual. It is strongly encouraged that the patient should avoid hospital visits for routine follow up and consult online via telemedicine.

However, those patients with post-transplant emergencies should attend hospital as usual and should be provided with necessary standard care by the transplant team.

Recipients with symptoms such as fever, cough and breathing difficulty should be evaluated for suspected COVID-19 infection as per the national guidelines.

- a message from my doctor Dr Sanjiv Saigal, Senior Director Transplant Hepatology at Medanta the Medicity.

Thus, I assume being a prisoner in your own home isn't a good idea but a sensible one until it's safe outside.

 Prometheus unflinchingly bore the cruelties and the tortures inflicted by a bird of prey eternally, but so have I. Perhaps in the face of life’s mutability, its unceasing and unforeseeable vicissitudes if I face life with spirit, courage and boldness my Creator will grant me the much-coveted healing powers of Wolverine and then I will be the saviour of mankind. If not in this life perhaps the next but I tell you I will be a superheroine doing good for the world.

Image credit: Akash Sharma. 

The picture was taken with Dr Guleria in 2013.

From:https://timesofindia.indiatimes.com/blogs/warrior-princess/miracles-and-madness-of-life/