There are countless challenges to having a disease. I have the most unusual diseases known 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.
The name of my diseases sounds thrilling but when you start explaining, they resemble deadly babble where the prognosis is like a psychic scream.
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 tumors throughout their life. VHL may occur in up to 10 organs of the body also there is a possibility of neuroendocrine tumors.
Most Indians pooh-pooh vHL but when cells perceive a lack of oxygen (hypoxia), such as through a defect in the tumor suppressor gene (VHL), the transcription factor, HIF (Hypoxia-Inducible Factor), is not allowed to bind to the VHL protein. HIF is thus protected from degradation.
The research professionals and members of the medical community around the world to better understand the VHL gene and its impact on cancer development. The outcome is Belzutifan (MK-6482; PT-2977)
As stated by Dr. Kaelin, “When you are studying about von Hippel-Lindau disease, you are not just studying about [the] …. disease … you are also now touching other diseases as well, where we can use the VHL gene to understand what is happening.” As such, the understanding of HIF involvement in tumorigenesis provides hope for the 40% of the world’s population who will be diagnosed with cancer at some point in their lives.
Every part of our body – skin,
bones, hair, blood, muscles, organs – they’re all made out of cells, and
inside each of the tens of trillions of cells that make up your body is an
identical copy of your DNA, a molecule that basically are the blueprint
for building us.
When we get injured, the way your body repairs that injury is that the healthy cells around it divide, making copies of themselves to replace the damaged cells. This is also how we grow and make things like hair and blood.
Every cell in our body has this capacity to copy itself. But every time a cell divides, there’s a small chance that it won’t go exactly as planned. What can result, then, is an unplanned cluster of cells somewhere in your body. We call this cluster a tumour.
So, “tumour” can be a scary word, even benign – meaning non-cancerous – tumours can sometimes be uncomfortable, can sometimes even make us sick and need to be dealt with.
Sometimes a tumour has a glitch in its DNA that inspires it to not just copy itself uncontrollably, but to cause other cells it comes in contact with to do the same, causing chain reactions of uncontrolled cell growth to spread wider and wider around your body. This is called cancer, and as we all know it is a big deal because it can often mean that these out-of-control clusters of dividing cells can grow in such a way that they prevent the organs from
functioning properly and they become cancerous when they develop the potential to spread to other places.
Neuroendocrine cells are present all over their body. They’re mainly in places like
These cells make up a system that control a bunch of important bodily functions – things like heart rate, blood pressure, air
flow, digestion, and lots of other things. They do this by being the meeting
points between two different kinds of signalling systems that keep the body
running.Some of the cells in the body communicate with each other by sending and receiving chemical signals, such as hormones, which are produced
by the endocrine system.
Other cells though, such as the cells in your brain, they run on an entirely different system of electrical signals – sending
sparks of electricity between the different neurons.
So, there are cells in the body that are neurons, they’re nerves, and other cells in the body that are endocrine cells that form
parts of glands, and these are all essential for normal function.
And in many of these places, like in the glands that make those hormones,
there’s a need for a group of cells that can bridge the gap between those two
kinds of signalling systems – receiving an electrical message from the nerves
and turning it into a chemical message. These intermediaries are our
neuroendocrine cells. It’s a translator, so it translates a neurologic – which is
really an electrical communication to a hormonal – which is a molecular – communication, where a molecule is released by a cell and goes and attaches to a different cell and in so doing sends a message to that cell to do something.”
Just like any other kind of cell in the body, these neuroendocrine cells need to divide and replace themselves periodically, and when they do there is a chance that a mistake might be made that will cause them to form a malignant tumour.
The first place that most NETs spread into the lymph nodes that are nearest to the primary site. Lymph nodes are a kind of gland that work as filters for harmful substances in the body. They carry oxygen and other nutrients to cells and carry away waste products that flow out of the cells.
Primary growing into other areas, cancer can form metastases through the bloodstream and the lymphatic system. This is a system of thin tubes (vessels) and lymph nodes that run throughout the body in the same way blood vessels do. The lymph system is an important part of our immune system as it plays a role in fighting bacteria and other infections; and destroying old or abnormal cells, such as cancer cells. The lymphatic system also contains organs, some of which feature regularly in NETs. If cancer cells go into the small lymph vessels close to the primary tumour they can be carried into nearby lymph glands where they stick around. In the lymph glands they may be destroyed (that is actually one of the jobs of the lymph glands) but some may survive and grow to form tumours in one or more lymph nodes.
Neuroendocrine tumours are sometimes called cancers in slow motion, because even neuroendocrine tumours that have spread to other parts of the body, can be indolent and not grow for months or even years.
Because of the gradual growth physicians until very recently tended to misunderstand just how dangerous NETs actually are.
Neuroendocrine tumours were actually all classified as being benign.
Thus, so did doctors taking care of these patients, and so then patients had this belief that these cancers would never come back. And we now know that these in fact are cancers, and they can grow and spread, and therefore they need to be treated as such. Neuroendocrine tumors, in general, are all malignant. They all have malignant potential. Most lethal malignancies in the world, with mortality rates being close to the incidence rates.
Hence early detection and treatment could be advisable.
Not only Irrfan Khan and Steve Jobs fought neuroendocrine cancer, I am fighting it too. Life is not a party after several misdiagnoses since childhood.
Brain metastases (BM) are rarely reported in patients with neuroendocrine carcinoma (NEC) of neuroendocrine tumors (NET) of the gastroenteropancreatic (GEP) or bronchopulmonary system. However, symptomatic brain metastases are associated with dismal prognosis, so early detection and treatment could be advisable.Somatostatin (SST) and somatostatin receptors (SSTRs) finding them in my brain play an important role in diagnosis in the brain.
Dr. Hukku diagnosed my NET and sent me to a great oncologist Dr. Sajjan Rajpurohit.
Symptoms
- without any reason for my choking and hoarse voice.
I can't stand fall
I vomit suddenly
I have neck pain and headaches
Swallowing problem.
I got choked on solid food and coughed my lungs out.
I bit my tongue while eating or speaking and the dentist said to use anaesthetic mouth wash
My gait and I bumped into stuff and hit my skull. Last hit was my loving bookshelf and got a bump.
My right legs have been hurt so many times and if you touch I fall
Dizziness
Blurry vision.
Stinging of the calf muscles and toes, recently fingers as well.
I am confused because of symptoms of the diarrhea and brain. I am going to the toilet after every meal.
One red eye in the right side, right cheeks rosy, lips bent twitching.
difficulty speaking or loss of voice
a voice that is hoarse or wheezy
trouble drinking liquids keep coughing
pain in the ear, liquid coming out and getting deaf with every passing day.
Unusual jaw pain
unusual pain in the middle of the chest
decreased/increases appetite
Fear of food that again I would run to the loo
nausea or vomiting
abdominal bloating /pain
Also I get diarrhea.
cough
tiredness
headache especially I started falling on my Birthday last December
aches and pains
a rash on skin,
red or irritated eyes are very common for me
difficulty breathing or shortness of breath
loss of speech or mobility, or confusion
During my craniotomy my parents noticed loss of consciousness and jerking of the left leg.
Difficulty speaking or loss of voice
voice that is hoarse or breathy
trouble drinking liquid
unusual heart rate
abnormal blood pressure
decreased production of stomach acid/ exocrine function
nausea or vomiting
abdominal bloating and cramps
Also I get acute diarrhea and lost 20 kilos
My wheezyness feels like I am hearing my own breathing
After a meal, I have eaten
I leap to the bathroom
The poop smells like perfume. As I get weak.Feel dizzy and sick.
After 6 chemotherapy treatments I came back from the jaws of death and I believe because I got back to Mum.
I had a massive stroke/ seizure which caused a hemorrhage in my brain. I was admitted to the emergency at BL K by an ambulance called by the kind inmates of Golf Links Residency, Dwarka.
I went to the Neuroendocrine cancer department because the whole thing started after three sleepless nights after chemotherapy. Although the advice from eminent doctors is to get a PRRT. I was getting problems to avail the treatment because I am the only patient with several brain metastases. My genetic mutation was established under Dr. AC Ammini in a pheochromocytoma and paraganglioma research at AIIMS.
Oncologist sent me to neurologist Dr Atul Prasad and I was advised to take IV levipil( anticonvulsant ) at a higher dose than I was taking. He sensed a seizure was about to occur. I felt a premonition and told Mum in a low voice that I was feeling unwell. There was alternate light and darkness. Mum went out with me in a wheelchair and I told her to get admitted for the IV after a while. As Mum went to talk with the secretary I was pulled by someone and my right hand went rigid clutching the left and trying to pull it out of the socket. I saw volcanoes erupting in darkness meanwhile for 45 mins I was out of sight of Mum and nobody could say where I was. She searched for me like a madwoman and found me in front of the medicine shop. What I experienced was unimaginable. In the darkness sparks rose and as if someone twisted my neck almost breaking it. I gave up fighting but thoughts appeared about going back to Mum. It appeared to be out of the body experience. Which some would find ludicrous and pestering worsen my health but the darkness, my inability to talk, droning buzz, explosions, intense light is reality.
I got conscious for a while and told Mum to take a room when she found me. I started speaking fluent English after another spell of darkness. I was telling someone to kill me because of the neck pain. Confusion and. weakness ruled with intermittent seizures for days.
A 12-year retrospective clinical study of patients who received peptide receptor radionuclide therapy (PRRT) for malignant neuroendocrine tumors demonstrated the long-term effectiveness of this treatment, which also allows patients to maintain a high quality of life. The study was published by Gabriel et al in (The Journal of Nuclear Medicine.)
It is most consistent neurobiological abnormality implicated in the genesis of suicidal behaviour is that of serotonin. Abnormalities in number of serotonergic neurons, serotonin transportation, receptor binding and serotonin levels in key brain areas have all been linked with suicide.
I am a Warrior Princess…a zebra…a unicorn.
Being a sparse-haired, mutant wide-eyed kid with a weird disease early in life a tumour was sawed off from the brain.
Now I run to the loo
Holding my belly crouching like a kangaroo
At times I drool
Bumpety bump
I feel a lump
I don't jump
But call Mum
When I need to pee
I wonder how deftly she handles me
Bedwetting Bedshitting
Head and body rocking
My eternal protector
My Mum toiling for my better
Doesn't it matter? A life?
The crazy rhythm in a winding walk
People who enjoy adventures and like to laugh.
And now is the time to laugh.
For a day and a half
I am a zebra, not a giraffe.
The mysteries of the human soul can josh, annoy but I learn to start living instead of merely existing. With a cruel effort of will heave myself up to the "don't be chicken-hearted", "don't scare easily" situation. Life is what you make of it. It's always when you think you have enough determination victory is yours.
Thinking " Alone, alone, alone terribly alone" is not fruitful because God has made me and repeatedly saved me from the clutches of death presumably with some good end in mind and is watching over you. There's no turning back. "Selume Proferre"- towards the light, as you live you learn… under the whims of fortune, the wreckage of life will be refurbished in time through messengers of God.
Justice, Purity, Equity
Where are people of integrity?
Uncertainty in my everyday life feels like life has thrown a punch at me but willpower has the strength to make my life turn around because there’s still so much more to see beyond my wildest dreams. Finding the love of God, understanding the power of God delighted me; although I need a stick to walk, sometimes in a wheelchair, I could see in my mind’s eye I am on top of the world.
I was given a one-way ticket to this world but we could have got the life of a vermin- a mere bug being crushed under the feet or not having enough intellect to enjoy the sun poking its adorable head from behind a cloud…the star-studded clear blue night sky… or the grand sound of the thunder and with it the soul waking up or watch the iridescent play of colours of the rainbow.
Maryfilomena E Rita Viñcenza of Ex NYPD's ' comment honoured me
"Payel Bhattacharya you are what I call a beautiful soul . You see, I knew !!!! I see such light coming from you . There is a special reason why God saved you and uses you for his glory . I am so honored to have Met you . May God bless you . You have lightened up my mind and spirit ; you see , it is 20 years from 9/11, my love of my life Til death did we part , Mark , died from being a first responder a NYPD Sgt , who got kidney cancer from working months there . This puts my gift of life into perspective once again . I like you always chose to find the flowers the birds the breeze the music in life in the lil moments of the day . I have lost so much of my light since Mark died nearly 8 years ago . This is how I have written poems in the past and more importantly how I have spoken to encourage and enlighten others in their dark moments . Thank you . I’ll pray blessings over you . 💝"
I haven't been given a choice in a life with mountains of books and chocolate bearing trees so why not face this life head on?
Rare disease patients and caregivers often shoulder a considerable burden for their disease and find it necessary to educate the society when we hear people asking "is Metastatic Neuroendocrine Cancer a contagious disease?" Itching and scratching diseases get full attention of our great countrymen whereas my cancer is beyond comprehension.
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 mother who has cared for me as nobody would. She is not just my caregiver but my only companion.
The universe is not outside me
To protect you an' defend you
From cruelty, apathy, and wrong,
Someone's there to open each and every door,
I knocked loudly at each door
When it's time someone will open the door
Hard times, hard times, come again no more.
I have been through nightmares before
The universe always responded when I needed help,
Leading up to the right door
