I am known as 'Warrior Princess' because I have chosen to fight like a seasoned warrior the whole journey to the very end. I was officially named Payel Bhattacharya but Warrior Princess stuck with many.
Today you are you,
That is truer than true,
There's no one alive who is you-er than you.
After several misdiagnoses and diagnostic dilemmas in 2008, I was diagnosed with a very rare disease hardly known in India, called VHL or von Hippel Lindau during my landmark liver transplant. I can still recall the faces of the radiologists checking my tumor-studded liver with their probes and looking at my file with a weird name “VHL” with vacant faces and regarding me quizzically. Yes, the liver transplant was a life and death affair and we had no money to get the liver transplant done which was a whopping> 30 lakhs!
Many doubted why I needed a liver transplant? In the beginning, it was a clinical diagnosis from the brain tumor then Pancreatic cysts, and multiple hemangioblastomas in the liver. Liver transplantation is an important treatment option for selected patients with non-resectable multiple tumors.
I had a liver transplant owing to several tumors in the liver which could not be taken out individually causing excruciating pain due to frequent hemorrhages in 2008. The largest lesion caused splaying of the portal vein around the lesion. Hepatic veins were compressed and displaced by the segment 4&8 mass lesion. I had two episodes of bleeding in the hemangioblastomas and required hospitalization. There wasn't any awareness of rare disease back then, therefore I had to suffer from the pain of hemorrhages and fentanyl patches were prescribed for pain. The second time I had a hemorrhage I asked the attending doctor to either do the transplant or kill me by euthanasia. An X-ray of the intestines was done but thankfully blood had not spread to the intestines. The transplant which was delayed due to lack of money was preponed.
The transplanted liver needs the immune system to be suppressed so that it isn’t rejected like any pathogen. Immunosuppressive treatment begins during the surgery and continues throughout the patient's life. Regular blood tests and other maintenance strategies by which medicines at specific doses are adjusted periodically by constant monitoring to prolong the transplant recipient's life and prevent acute or chronic rejections of the graft.
All immunosuppressants leave the patient more susceptible to infections and less able to fight them off.
Soon after discharge I got viral infection varicella and was treated with Zovirax.
I am an MDR- TB survivor with pulmonary, lymph, and bone involvement.
We are poor and abandoned and always have to think about the next day's food and medicines. But I was approached by AIIMS New Delhi to take part in a research project primarily for pheo and para. I readily accepted because I don't have money for any genetic testing.
The first email came
"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 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"
I was advised by the doctor to get a cyberknife done before they grow and put pressure on the brain. I got it done accordingly with the help of a well-wisher but the MRI after 6 months showed leptomeningeal hemangioblastomas.
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.
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 brain are hemangioblastomas.
I get stereotactic radio-surgery/radiation therapy before the growing tumors 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 of the optico-hypothalamic tumor because of financial reasons and also because I was diagnosed with an RCC at the same time.
I have Supraventricular tachycardia since time immemorial. Hypertension was regulated with few common brand medicines. But during a surgery, I had terribly high blood pressure. Regular medicines didn't work. The cardiologist prescribed another unusual medicine.
In 2019 I developed bilateral trigeminal neuralgia and got a cyberknife for it on 17/3/2020. In between, I got radiation therapy for two more growing tumors and after MRI it showed 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.
We returned after Trigeminal Neuralgia cyberknife to our city on 18/3/2020 and from the 24th lockdown commenced. But the left side remains untreated. Yet due to the pandemic, I and Mum went into voluntary isolation and haven't been out since nor do we let anyone enter our rented flat.
Since then several problems started,
- Difficulty speaking or loss of voice
- Pain in the tongue
- Voice becomes hoarse
- Trouble swallowing food and medicines, Here I'd like to state The cranial nerves associated with the swallowing process are the trigeminal (V), facial (VII), glossopharyngeal (IX), vagus (X), accessory (XI) - usually not considered - and hypoglossal (XII).
It should be emphasized that the structures involved in the swallowing process are pairs, both anatomically and/or functionally, due to the dual-side innervation.
Anatomically unique, the tongue, palate, pharynx, and larynx are functional pairs, each side having independent innervation.
- I have to crush every medicine.
- For about the past month after almost every meal I run to the loo. I am confused why I have fatty stool or watery stool. The cysts in the pancreas were noted down in the liver transplant Summary but the recent PET CT done without intravenous contrast showed nothing save mild FDG avidity in the lungs.
- Trouble drinking liquids
- Pain in the ear and behind it
- Hear odd wheezing sound sometimes which feels like listening to my own breathing. There are three types of important respiratory nerves:
The phrenic nerves: The nerves that stimulate the activity of the diaphragm. They are composed of two nerves, the right and left phrenic nerve, which passes through the right and left side of the heart respectively. They are autonomic nerves.
The vagus nerve: Innervates the diaphragm as well as movements in the larynx and pharynx. It also provides parasympathetic stimulation for the heart and the digestive system. It is a major autonomic nerve.
The posterior thoracic nerves: These nerves stimulate the intercostal muscles located around the pleura. They are considered to be part of a larger group of intercostal nerves that stimulate regions across the thorax and abdomen. They are somatic nerves.
These three types of nerves continue the signal of the ascending respiratory pathway from the spinal cord to stimulate the muscles that perform the movements needed for respiration.
Damage to any of these three respiratory nerves can cause severe problems, such as diaphragm paralysis if the phrenic nerves are damaged. Less severe damage can irritate the phrenic or vagus nerves, which can result in hiccups (which I do have in excess)
- Unusual heart rate (I already take Embetta XR 50 a beta-blocker crushed).
- Abnormal blood pressure and pulse.
- Problem with gait
- Touching my legs makes me fall, can't stand for a long fall anyway.
- Nausea or vomiting
- Abdominal bloating
- A metallic sound in my ear.
- Problem with gait, walking in a zigzag way I banged my forehead on the bookshelf.
They are increasing every day and more symptoms are getting added like the back of the neck and head pain with burning in the right temple.
Recently a virtual, video consultation with the doctor resulted in a prescription of Creon( enzymes) and probiotics.
Now my tummy feels better and I don't need to run to the loo after every meal and no watery stool.
There are myriad challenges of having a malady. If you have the most unusual diseases known to humankind it becomes a fateful condition where you can't afford to be ignorant and continue being tired if you desire quality and extension of your life.
Few diseases of the present have little in common with the diseases of the past because of their rarity but like an owl, you have to pore over abstruse medical documents and make it your pastime with a great penchant for general science with an interest in medicine because in our country most doctors aren't familiar with the mode of treatment and government policy is to support only children with lysosomal storage disorder. There's an argument going on in the Parliament about treating children with skin disease. They understand only problems with intellectual and physical development or deformity.
My father left us homeless and penniless. I don't get treatment or monthly blood chemistry/ radiological tests done in time because I can't get money.
I beg from Facebook acquaintances and some say " will send you in a few days" but that never happens.
National policy for rare diseases 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.
Government don't understand when a medicine is socialized, then you have true health care. When everyone's treatment is the same regardless of 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.
This newly minted policy is an irony that the government is taking care of a handful of citizens.
This is not helping, but 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. Tragically, the government thinks the "life and health of a citizen an unfair burden".
The tumors scattered inside my brain 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(Prednisolone) 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…
I consulted with Dr. Russell Lonser emailing my details with the last MRI of the brain for my leptomeningeal hemangioblastomas and the symptoms.
His reply
"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 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 have appealed to the government to grant money for my treatment.
I was once a child because I am not Benjamin Button. I grew up suffering all the time from a variety of symptoms and fatal diseases. Yet I have uniquely become a baby, not crying or a whimpering one but a gaily smiling one sometimes speaking in a mignon voice, sometimes a hoarse voice, and sometimes voice doesn't come out. Eating crushed and smashed food, sometimes baby food because I can't swallow and spew out food with a soft gurgling sound. Also, burp as I can't digest food.
My life depends on if they stick to their decision about only children with lysosomal storage disorder or someone who fought cancer and several other problems but never gave up.
I have a right to live, this is my fundamental right and provide "EQUALITY of status and of opportunity;" from the preamble of the constitution which has vanished into thin air.
Here healthcare is a profession and treatment is merchandise and life is commodity. They are quantifying life that can be saved.
I am a writer in Times of India digital but I have been stopped writing against the government.
No Justice only Tyranny prevails. My question to the government is those rare disease patient who you think are burden to the society, a bad tooth to be pulled out "Why not make a gas chamber for the citizens to whom you deny healthcare and right to live?"
Original Hippocratic oath as taken by hypocrites has mutated over the time to:
satyameva jayate nānṛtaṃ satyena panthā vitato devayānaḥ yenākramantyṛṣayo hyāptakāmā yatra tat satyasya paramaṃ nidhānam
In English Translation,
TRUTH alone triumphs; not falsehood. Through truth the divine path is spread out by which the sages whose desires have been completely fulfilled, reach to where is that supreme treasure of Truth.
The Truth referred to is the Paramatma. For sages who speak the truth they proceed closer to attaining ‘Moksha’ or oneness with that supreme Brahman is its real meaning.
The oath of the ministry happens to be:
Well, then Coca-Cola, IAM, Being Human, Tata trusts, Hans Foundation etc should support life without delay. This would never happen because Director and ambassador of Coca-Cola was in school with me, Tata trusts vaguely denied in 2020, IAM helped me once but now according to them they only help children although government is helping pediatric diseases, Being Human is unreachable, Hans Foundation approved my cyber knife but then denied because cost was very high 5 lakhs in 2020.
Voluntary crowd-funding for treatment
Human beings are no longer humane and they don't work hand in hand now and if you ask twice get irritated. They love a lavish life and collecting money. Therefore crowdfunding is an outrageous thought and every platform, Milap, Ketto has their own charges unless you collect a large amount it's useless. The decayed society with affluence. Crowdfunding for 5 lakhs becomes difficult for cyber knife of Trigeminal Neuralgia and when someone donates from abroad gateway fee etc by RBI deducts most of the sum.
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