Life is granted once: Coping with the VHL genetic defect

After several misdiagnosis and other dilemmas, 10 years ago 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 quizzical faces of the radiologists checking my tumour-studded liver with their probes and looking at my file with a weird name in it, "VHL". The liver transplant was a life and death situation but we had no money to get it done, with the cost being a whopping Rs 30 lakhs. We reached out to the world through the media and the transplant took place just automagically.

Even now, 10 years hence, VHL remains the least understood disease in India. It is a genetic condition that results in abnormal capillary growth in the visceral organs of the body. Normally, our capillaries branch out gracefully like trees. In VHL patients, little knots of extra capillaries form tumors and, in certain cases, cancer. VHL can affect up to 10 parts of the body, including the brain, spine, ears, eyes, lung, liver, pancreas and kidneys.

Even before the liver transplant, I had multiple surgeries but doctors were always in a dilemma about the diagnosis. It was during my brain tumor surgery that the neurosurgeon suggested I was suffering from VHL. But back then, there was hardly any chronicled cases of VHL in India and my parents apparently don't have VHL, thus he found it difficult to reach a conclusive diagnosis.

My father died within a few months of my liver transplant, leaving me penniless and without a roof above my head. It was then that my real tough times began.

The transplanted liver needs the immune system to be suppressed so that it isn't rejected like bacteria and viruses. Immuno-suppressants are expensive life-saving medicines. VHL disease and an organ transplant requires constant attention and a strict radiological and lab testing schedule to identify new tumors or those that have re-grown or check if the transplanted organ is working perfectly. VHL requires surgeries or treatment for symptomatic relief of tumors, benign or malignant.

To control the plethora of illness that organ transplants and VHL affliction brings, it requires doctor's visits and regular monitoring. We were at a loss: How would we get along? Each day brought with it hope that if we try and don't cry we will live. I, with my mother, began wondering how we could get my treatment done when it was uncertain whether we would get the next day's meal. We didn't cry and reached out to the world with full faith in ourselves that we would live, that we were meant to live. Help came from unexpected quarters and we became survivors. We still need help and funds are difficult to come by, but we never lose hope, it is hope that propels us.

I'm also a MDR-TB survivor which I got under immuno-compromised conditions. I am also a kidney cancer survivor, which is but another manifestation of VHL. I have had so many major surgeries just at the nick of time that I have stopped counting. I wrote poetry and made sketches to express the way we are combating the world and the dire situations.

I wrote my first long poem "Warrior Princess" which sowed the seeds of my later autobiography "The Warrior Princess". After several radiation therapies and treatments for a very rare kind of brain tumor, when my brain fog lifted -- despite the fact that a brain tumor claimed most of my vision in the right eye -- I penned down my story, my experiences with a bizarre disease; and how I fought on.

Ten years ago I never thought I'd live to see this day. Life is granted once and I have an intense will to survive; after the aforementioned war I waged through life, I want to celebrate this day as "Survival Day".