My determined looks don't betray the nature of my disease and I knew somewhere down the line it would bring tough luck and I would have to face up to it. Unless people look at you and cry out,
“Oh! You look so afflicted!”
They think you are faking the disease. I do my best to wave away the remarks,
“Oh, you don't look sick!” with a horrified revulsion. Life, even a life with illness, presents itself in stages but I believe as a citizen and a member of the community of my own disease I have a positive contribution to make. The first thing that I can think of while dealing with my disease is building awareness.
The preconceived beliefs of what being chronically ill looks like are what prevents people from thinking that the medical attention that patients need, receiving the benefits that they deserve, and being treated with dignity. There are people who cannot even get much-needed support during their illnesses because their people, government or society do not understand that being sick can present in many different ways. Sick looks different for different people on different days.
Let's start at the very beginning when I used to get headaches. Nobody cared in school but my father took me to plenty of doctors who put me on medications that made me drowsy and I put on weight. I was never thought to have a chronic illness. Assistive devices, such as walkers, wheelchairs, tubes, monitors, oxygen cylinders, and other medical devices can be an obvious signal that the person has a disease. Perhaps if I lacked a limb, the society would have treated me with more charity. However, there are also many chronic illnesses that are “invisible.” An invisible illness means that a person can be continuously sick, but may look just fine on the outside.
When I started getting partial seizures doctors thought they'd do an MRI and the scan showed a tumour has grown more than five times from a tiny dot which showed up in a routine MRI conducted to know the cause of my headaches. Shouldn't they have watched the tiny spot? Why should it be there in my brain in the first place?
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. Later, in life, I am now facing these ultra-rare brain tumours.
My father went to most hospitals across India for my growing brain tumour causing partial seizures. 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) 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.
Then within a month my father had a massive heart attack but survived after being on a ventilator for nine days and loosing twenty kilos.
Within four months I had strange symptoms and vascular tumours were detected in my liver but due to lack of money and the rapid growth of the tumours, they 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, a powerful regenerative ability known as a healing factor, and three retractable claws in each hand. With childish awe, I used to gaze at him for his abilities but I never knew what my future held for me.
I had endured two episodes of intense pain and in the last one asked the doctor to get euthanasia done. It was so painful.
I had faith in my Maker and he sent help at the nick of time when the tumours were haemorrhaging. I survived the transplant and the subsequent infection of herpes zoster after being discharged for being immunocompromised.
The transplanted liver needs the immune system to be suppressed so that it isn’t rejected like bacteria and viruses. Immunosuppressants are expensive life-saving medicines. I am on immunosuppressive medicines for life.
I was diagnosed with a 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, neuroendocrine tumours can also happen.
Currently, there are no approved drugs for treating VHL. Treatment for VHL varies according to the location and size of the tumour. In general, the objective of treatment is to treat the tumours before they grow to a size large enough to cause permanent problems by putting pressure on the brain or spinal cord. Treatment of most cases of VHL usually involves surgery to remove the tumours before they become harmful. Certain tumours can be treated with focused high-dose irradiation. Individuals with VHL need careful monitoring by a physician familiar with the disorder.
If I am a failed experiment of nature is for you to decide. I don't have the regenerative ability or the healing factor beyond a common man yet a flawed gene makes menacing tumours pop up in different parts of my body.
I have faced countless surgeries and there will be more on the way.
I had tetany emergency twice, brought on by dramatically low calcium levels, also known as hypocalcemia. Tetany is a condition marked by intermittent muscular spasms, caused by malfunction of the parathyroid glands and a consequent deficiency of calcium.
People think Tb has some noticeable symptoms and you always cough up blood but that was not in my case. I had high fever which baffled the doctors and ultimately a fine needle biopsy of the swollen lymph node diagnosed me with Tb but even then I was getting worse and not responding to medications. It was then that I was diagnosed with MDR Tb.
Due to the life-saving immunosuppressants or anti-rejection medicines I was prescribed after liver transplant, I acquired the infectious MDR TB, caused by bacteria that are resistant to treatment with at least two of the most powerful first-line anti-TB medications - isoniazid and rifampin - due to my suppressed immune system and living on rent in shabby houses with dripping roofs after father passed away leaving nothing, no money, no roof above our head. I got disseminated MDR TB with pulmonary ( lung), bone, and lymph involvement and I can't walk or climb stairs necessitating a walking stick.
At this point in life, I started needing an assistive device but still, people weren't convinced about my illness. Whenever people visit with help in mind they expect to see an ailing woman lying on the bed, breathing cold sighs but when they see that is not my case they forsake their cause and turn their back on me.
.When your struggling mentally singing
"IF THEY ANSWER not to thy call walk alone,
If they are afraid and cower mutely facing the wall,
O thou of evil luck,
Open thy mind and speak out alone.
If they turn away and desert you when crossing the wilderness,
O thou of evil luck,
Trample the thorns under thy tread,
And along with the blood-lined track travel alone.
If they do not hold up the light
When the night is troubled with storm,
O thou of evil luck,
With the thunder flame of pain ignite thine own heart
And let it burn alone." With more passion than Rabindranath Tagore became my mantra.
Accept yourself for who you are. Stand up for what you believe in. There are times when life seems unfair, but even on dreary days, it's possible to create your own sunshine with your willpower.
I have undergone two sessions of radiation therapies (in the brain for leptomeningeal hemangioblastomas which is very very rare and only 132 cases have been found between 1902-2013) which went on for six weeks, one cyberknife and not forgetting the kidney cancer. All my life-saving surgeries and treatments and diagnosis happened at the nick of time. I’ve had numerous surgeries, not dismissing from mind thyroidectomy which led to HypoPara (hypocalcemia due to low parathyroid hormone level).
I think I need to make it clear about my leptomeningeal hemangioblastomas. First, you got to know what a hemangioblastoma really is. Well, let's just know about it first.
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 Hippel Lindau (VHL) disease.
Now let's come to 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.
Now we're in a position to understand what are leptomeningeal hemangioblastomas and how can they be treated?
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 Hippel-Lindau syndrome. Diffuse infiltration of the leptomeninges is known as hemangioblastomatosis and has been observed both in VHL disease and in patients with spontaneous mutations.
Detecting and treating the condition of leptomeningeal hemangioblastoma without delay seems to help survival, though the number of patients analyzed is small. Patients may have other underlying health issues that may affect the data.
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 CSF under high-resolution MRI scans are all that is necessary for an accurate diagnosis. A biopsy isn't required for diagnosis as it may cause meningitis and blood loss thereby cell spillage.
I was diagnosed with supranational leptomeningeal hemangioblastomas in 2013.
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 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.
I get stereotactic radio-surgery before the growing tumours start putting pressure on the brain and become symptomatic. I have lost the vision of my right eye for not being able to avail of radiation therapy at the right time because of financial reasons and also 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.
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.''
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.
Unfortunately, my optic nerve tumour didn't shrink and I remain blind with my right eye.
Let's continue with the story now that you have understood the science behind it. If you are smart enough you can yourself deduce by reading the following articles…
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
So doesn't that mean I am a super rare case? I am a minority? I have tried to track anyone with my kind of brain tumours but till now I have been unsuccessful.
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 and it said: “ Thin vascular loop of SCA abutting cranial aspect of the right trigeminal nerve at the root entry zone is noted.”
In trigeminal neuralgia (TN), the trigeminal nerve's function is disrupted. It is the most excruciating pain known to man-- sometimes called the suicide disease. The compression of the nerve by vascular loops may lead to TN. TN 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.
Pharmaceutical science has medications to control the pain.
I was initially put on medicine but it mostly didn’t contain the pain. I had fleeting jabbing pain which would fade away in at the most few hours. Pulses of electricity travelled through my cheeks. Doctors upped and upped my dosage but I was not satisfied.
It is because of VHL that I underwent a liver transplant 12 years ago, I have side-effects like diarrhoea. I went somewhere else for consultation on less invasive procedures like damaging the nerve fibres with a heated electrode known as Radiofrequency Lesioning where an electrode is inserted through the foramen ( hollow or opening inside the skull) or Gamma- knife where beams of cobalt 60 radiation are used to damage the nerve. There it was confirmed by the doctor that the left-hand side pain which is milder than the right-hand side is also due to trigeminal neuralgia. Thus I have a bilateral manifestation of 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.
I revisited the neurologist who previously diagnosed it. A new medicine was added to my list of medicines. I asked him that if it's true I had bilateral trigeminal neuralgia...he gave a knowing wink and said but the pain in the left side is milder...why should he hide it from me? Who is he to decide the intensity of my pain? Mistaking the pain in the jaw for a dental pain I sought the help of a dentist. Because of my low immunity, I acquired some infection and got 105F fever. I wouldn't have suffered if he hadn't hold back the truth from me.
But the major thing which happened is after the medicine was added is my BP plunged down. I have tachycardia and high BP most of the time. I take medicine for that. I stopped the medicine and drank lots of fluids but to no avail. Doctor persisted in their decision of choice of medicine... He keept 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. It was because of that stubbornness that I ended up vomiting the entire night losing three kilos in the process. Oh! I wanted to avoid the surgery because of my rare condition of multiple brain tumours, why don’t they understand that? Why ask for a biopsy? I would never have given my consent. 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 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.
I applied to many trusts one said it's done but rejected at the last moment with words that "it is too expensive", next one just denied that there are many genuine cases waiting and the other one said they had helped me earlier but they have now started helping children.
The trusts refused in the most awful way but wished that I, get well soon. Pray, tell me how? They rejected my application and wished me to get well. Helping hands are better than praying lips. Quite sarcastic! They forgot that. I can't avail my treatment due to my financial circumstances, how can I get better? Healthcare is so expensive, especially for rare diseases.
Another incident of a woman getting in touch with me reminds me of something… calling oneself humans don't mean you are showing respect and dignity to a rare disease. They wanted to know a fixed expenditure for a month. Since it is impossible to predict how the disease will present or progress, active surveillance is extremely important for people living with exceptionally under conditions of HypoPara, Trigeminal neuralgia, leptomeningeal hemangioblastomas and so on; also considering the emergencies like when I had tetany emergency twice brought on by dramatically low calcium levels, because of my malfunctioning of the parathyroid glands and a consequent deficiency of calcium, or like the infection, I got after visiting a dentist which brought on 105F fever or the night when I kept on vomiting whole night losing 3 kilos in one night. Providing absolute monthly expenditure is next to impossible. Then again no response..whenever I call I get no answer.
There are many NGOs in our country who care about orphans, child education, farmers but no one cares for a rare disease patient. Most of them prefer to help on a mass scale to get their names printed on the front page of the leading dailies. If you approach them with blood cancer, they come forth and readily help you, but no one is aware of various forms of cancer. This is the bitter truth of our country.
I got support and kindness and the cyberknife of the right side-- the most painful side happened and we returned in time just before the covid19 lockdown. The nerve was shot with a high dosage of radiation to destroy it. But the pain in the left side remains as the left side is still pending. Also stiffness in the neck muscles and trouble swallowing.
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 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.
One might expect someone with never-ending pain and suffering from such rare diseases to spiral into a sea of woe, depression, and inactivity but in my case, it makes me a warrior who is fighting and suffering not just suffering fueling positivity & hope.
I don't get to recover from any of my rare diseases like you do when you get cold or flu. It’s a daily fight for me where hope and the will to survive saves me always. I have accepted surgeries, radiotherapy and infectious diseases would happen anytime but I wouldn't be tormented because I would survive if only I fight. I have chosen not to sit at home by myself. Every day, day after day, isolated from the world but I took life as a challenge and strove to write detective fiction and spooky novel. I decided it’s time to live my dreams and aspirations and took the challenge to write. Yes, despite my brain tumours, headaches, and one-eyed vision I knitted complex plots with intrigues.
After reading about my disease if you look at me with a confused mind and tell me you are sorry, it means you have wasted your time reading about my invisible illness. I don’t want sympathy. Sympathy, for me, implies you think I’m weak. And I am not weak. I am strong. I know you can’t see it when you look at me, but I have a rare disease that is not apparent to the eye.
We must enjoy life, every moment of it. Death will put an end to everything but facing several life-threatening surgeries, especially the liver transplant made my spirit wake up and lead a life in defiance of death knowing each moment might be the last …..and never brooding about my plight. I am always eager to grasp whatever I can get from every moment before it is too late, thus honouring life....someday we will die but on all other days, we will not!
No matter how much we wish to go back and relive an event or a happy moment or eradicate a sad moment that is never going to happen. So, live at this moment don’t lose it.
Little birds sing on the leafy branches, warm bright sunshine light up the cloudless sky and I love my life with my wonderful mum.
I chose to trudge the hills less trodden by;
With head held high;
Being cognizant;
The turf is uncertain;
So is the terrain;
Still hanging on
When I want to sigh;
I get up with head held high;
Choosing a path never trodden by,
I have faced life boldly and I am strong enough to know that I am rare and that implies I am a minority and I too have the same right to live. Everyone doesn't have money and means and every rare disease treatment needs that. Shouldn't the government sponsor my treatment as I lack the wherewithal to pay?
A doctor from Chennai told me that I have survived beyond expectation but I have a feeling that I want to live more than anyone's expectations.
Government is up to the ears with weighty matters mostly concerning minorities but being a minority I don't get their attention! They remain unresponsive and the cold attitude is I think because they don't have enough awareness about my diseases. Lack of social support, empathy is an additional trial throughout my life. Now my mother is ageing but she doesn't look like a 69 years old lady but that doesn't take away the years she has gained and I have survived 15 surgeries and radio surgeries and radiation therapies but I bear the pain and get my treatments at the nick of time or sometimes don't get it. Its time government 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.
I felt the need to write so I thought I would share it with you but please don't compare your story with mine or say sorry.
Perhaps if I face life with spirit, courage and boldness my Creator, will grant me the much-coveted healing and other 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.
Hats of to you Mam.
ReplyDeleteThere are no words which can describe how brave and motivating person you're.
Rather than having a film star as a role model people should look up to you as a Role Model for having such a Never-Giving up Attitude.