"Someday Somebody Would Really Understand My Rare Condition"

Payel Bhattacharya, 38 suffers from a rare condition called VHL and has undergone countless surgeries and a liver transplant to overcome her various challenges. She is a warrior and here she recounts as to how tough her going has been

Please tell us a bit about yourself, your condition, as in what you are suffering from and when you were diagnosed.

I am Payel Bhattacharya. I was born and raised in Kolkata, but I am currently residing in New Delhi with my mother for the past decade. Ten years ago I underwent a landmark liver transplant, which is still the only VHL liver transplant in the world at Sir Gangaram Hospital New Delhi under Dr.A.S.Soin and his team. Shortly after that my father expired at Sir Gangaram Hospital due to cardiac arrest and since then we are residing in Delhi.

I was born with 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 tumor and in certain cases it turns into cancer. VHL can affect up to ten parts (blood-rich organs) of the body, including the brain, spine, ears, eyes, lungs, liver, pancreas and kidneys. After a lot of misdiagnosis and diagnostic dilemma I was diagnosed with the disease during my liver transplant. My liver was studded with hemorrhaging tumors which individually couldn’t be resected. Thus, I survived a very expensive (30 lakh) liver transplant which went on for about eighteen hours with a team of thirty doctors.

VHL disease is a unique disease. It is different for every patient. Since it is impossible to predict exactly how and when the disease will present for each person, it is very important to check regularly for possible VHL manifestations throughout a person’s lifetime.

Most of these VHL tumours are benign. Some VHL tumours, such as those in the kidney and pancreas, can grow to a stage where they become cancerous. Benign VHL tumours can still be very serious. As they grow, these tumours and the associated cysts can cause an increased pressure on the structure around them. This pressure can create symptoms including severe pain. The goal is to identify these lesions when they are small before they spread to other parts of the body.

What is your present condition?

I am a kidney cancer survivor which is a manifestation of VHL. I have very rare form multiple brain tumours called leptomeningeal hemangioblastomas which is a manifestation of VHL and till now I haven’t encountered anybody who has similar tumours in brain. I have had brain surgery, radiosurgery and several radiation therapies but still my brain is riddled with tumours. I can’t see with my right eye because a brain tumour on the optical nerve has claimed my vision. I have short term memory loss. I had an incisional hernia repair surgery which went wrong. Due to lack of blood flow to the skin it had necrosis which required a debridement. After the debridement I need to wear an abdominal binder for support and it acts as a buffer against any sudden jerks. My bones have weakened after my MDR-TB (under immunocompromised condition) necessitating me to walk with the help of a stick. I have undergone countless surgeries.

I have a tumour on the trigeminal nerve in my brain causing trigeminal neuralgia, which is also known as suicide disease because of the intense pain it causes. VHL and an organ transplant bring along a plethora of illnesses. I have had several complications from anaemia to hormonal imbalance.

What medications are you on? Were there any side-effects of the medicines?

VHL has no cure. The medicines I am on are to treat the symptoms of VHL. I am a liver transplant patient on immunosuppressant (anti-rejection) medicines which are lifesaving medicines used for a transplant patient so that the new transplanted liver is not rejected. Since my immune system is suppressed I cannot go to any place or eat anything because I am susceptible to several diseases. I contracted MDR-TB under my immunocompromised condition. I had to take ATT for four years. MDR-TB attacked my lymph nodes and via lymph nodes it attacked my bones. I had pulmonary, lymph and bone TB. I require medicines for my trigeminal neuralgia to control the pain.

You have written in your blog that because there is usually no physical deformity involved in this, people find it hard to be convinced of your state. What would you like to say about it?

We are judged mostly by how we look. I never look afflicted. Therefore, when I approach people for help, they think I am faking the disease! They also don’t understand the recurring nature of the disease. Perhaps if I had a missing limb they would have readily helped or perhaps if they saw me in bed writhing in frustration and pain. But they hardly realize what I lack inside. My missing liver which has been stitched back with a replacement; my left kidney, part of which is gone forever; brain which has been burnt by radiation beams and will never grow back, and the part which is vacant inside the roomy cranium. When I ask some people for help, they remain mute, as if I don’t exist and sometimes there are a few kind souls who help without asking. But I should add that I don't despair. Maybe that's why somebody sometimes helps, and I survive. I can't even say how many people came in my life when I desperately needed help and I got kind help from them. Then poof! They vanished! This process continues in my life, but I always hope people would someday really understand my condition.

Have you learnt anything in managing this condition that you wish you knew before? What kind of specialists do you consult and how often? What resources are available to you in your city to help you in managing the condition?

Since the disease is a multi-organ syndrome, I need to visit various doctors in different fields at regular intervals. Hospitals in Delhi-Gurgaon are well-quipped with good doctors and techniques like cyberknife to deal with my disease. I underwent cyberknife radiosurgery in 2013.

Have you had to make some changes to your lifestyle because of your condition?

I had to restrict myself in many ways after my liver transplant because I am susceptible to infections with my suppressed immunity. I can’t go near people who has viral or any other infections for fear that I may get it and no roadside food or eating in the open.

How have you managed to channelize your energies into writing?

I took it as a challenge to write the crime fiction stories of Mum and Princess (Mum and Princess go spying, Sweeter than Revenge, Mum and Princess in Mystic Land) which are available on Amazon in kindle format. I wrote my first long poem, “The Warrior Princess” which sowed seeds for a later fictional account of my life story “A Warrior Dies Dancing, That’s who I Am” published by Sahitya Akedemi in the September-October 2018 issue of their Indian Literature Journal. I wanted to let people know of my struggles and the unpleasant experience of having a rare disease along with the injustice meted out to me. Thus, I wrote my life story, of the same name which is also available on Amazon in kindle format.

What was the hardest part of suffering from VHL?

You get to live your life once. You can’t go into your past and relive any moment. So why waste it? I always live every moment brim-full with hope. I am afflicted with a rare disease, but I don’t look like I am “afflicted” because I don’t writhe in frustration. My father didn’t leave us any money or house and I need help to carry on my treatment. I have no relatives. Sometimes when I ask people for help during fundraising, people stay mute, as if I don’t exist. The ignorance of people, and the society. Landlords presenting us with court notices when I was facing my cancer in kidney and a tumour in brain. The injustice being meted out to me. My relatives depriving us of our right to our own house has been the hardest part of coping with a bizarre disease.

Have you tried complementary medicine or therapies?

My father tried everything with the fondest hope of curing me. From taking me to several allopathic and homeopathic doctors to ayurvedic ones, he did everything. He even took me to religious places and followed their advice praying that I would be cured yet nothing happened, and things got worse and beyond control.

Has it been difficult emotionally to cope with your condition? How has your family supported you in this? Did you see a counselor for support? 

After knowing about my condition, I doused my fear and marshaled my thoughts to a successful battle of survival with my parents beside me. After my father has gone my mother means the world to me. She assists me in everything from inside the bathroom to even discussing plots of my crime stories. She is my inspiration. She is the person based on whom I created my problem solver lady Mum and I am her Princess. With my Mum beside me I wouldn’t ever need a counselor and counselors for VHL don’t exist in this county I suppose.

What kept you going? How has this changed your perspectives in life?

My mother keeps me going. We need to enjoy the small joys of life. My parents performed such superhuman feats when I needed a 30-lakh liver transplant for the faintest hope that I will stay alive, that it has made me realize the importance of the one life granted to us. After I survived countless life-threatening surgeries, I realized we can’t go back in time to see if any bitter moment can be expunged completely or relive any event of our life.

Please reach out directly to Payel Bhattacharya