January 1, 2016
Payel was at Medanta Hospital today and had a vision field test by Dr. Digvijay Singh. It has showed some deterioration since last October’s result (shown), but is still a lot better than what it was before the radiation treatment. I hope the deterioration is temporary.
She was also seen by Dr. K.K Handa who treated Payel’s ear fungal infection caused by the radiation treatment.
VHL was decisively clinically diagnosed in me during my liver transplant in 6/aug/2008 carried out by Dr.A.S.Soin and team for symptomatic relief of multiple hemangioblastomas. Prior to that, I had several surgeries including subtotal thyroidectomy and open brain surgery or craniotomy of brain but misdiagnosed.
My introduction and accrual to surgical world started at early childhood. In
2013 I underwent cyber knife radiosurgery for two brain hemangioblastomas in one sitting.
Currently there’s minimum 6 neck paragangliomas which upon doppler study reveals high vascularity. And the problem which has reared its head is brain being riddled with tumours. VEP was done at the end of 2014, when I couldn’t see with right eye and visual field report was abnormal. VEP (Visual Evoked Potential)abnormality corroborates abnormality in right visual pathway. Next was MRI Performed of brain and Spine which inferred the reason of my vision loss to be a tumor in the optic nerve in brain (enhancing mass lesion in the relation to prechiasmatic-intracanalicular right optic nerve). Mild increase in rest of the tumors compared to August 20014 and a note of the radiologist ‘ Note is made of smooth ovoid 2.8×2.2×2.2cm solid lesion in posterolateral relation to upper third left kidney, abutting it” led to me getting me getting nuclear scans Dr. Ishita Sen in Fortis healthcare 68 Gallium Dotanoc and F18 FDG on separate days outcome of which is the kidney mass being intensely fdg avid enhancing lesion. For this I underwent partial nephrectomy of renal cell carcinoma by Dr.Sanjay Gogoi in Fortis and biopsy confirmed the malignancy.
Von Hippel- Lindau (VHL) is a disorder without outward manifestation, only detected upon periodic screening wherein mushrooming tumour generally called hemangioblastomas, grow in
central nervous system, i.e. brain and spine and
visceral organs, i.e. kidney, pancreas, liver; being genetic, can’t be halted and the mainstay is diligent monitoring by adept doctors and surgical intervention or irradiation at right time.
VHL has no resort in India, save deft monitoring and aggressive intervention; spiralling expenses: there’s no NGO, government help. Only treatment is removal of tumours at right size before the spread to increase longevity, else vascular tumours located adjacent to a vital structure can pose threat, symptoms varying according to the area compromised. Although, a stuttering growth, they may rupture without any telltale sign.
This genetic flaw is an evolutionary misfire; the mischance on genetic disorder finds its explanation as a basic evolutionary compromise. Random misprints are made while handing down the vast genetic library which is essentially programs with molecules placed in specific order being encoded to carry on the life-process. Mutation is the engine of evolution, without that we never would have evolved. So if there were no mutation, we’d still have been apes. The trade-off being hyperactive cells, endowed with survival down to their innate molecular core. VHL being rare scientists are certain, small advances in less frequent tumours may translate into major advances in more common tumours.
What is VHL?
Willem Dafoe talks about VHL
Not only no NGO/Govt support why I need help-
I moved forth without regression with the cadge from friends well-wishers and not willing to give up.
Multiple operations have the serious problem of postoperative morbidity in the CNS and visceral organs. But I’ve the verve to fight life’s impediments despite my infirmity incurred due to the time lag between treatments; the most important one amongst them is my fiscal situation.
I’ve no paraphernalia; I’m not wistful prone to enervating rather mightily satisfied with my vitality. I possess a candid mind equable belligerent nature. After my Father left us on 6/ Apr/2010, I’ve no kith and kin, no accoutrements, vittles, not even a roof above my head.
With foreseeable vissicitudes and lifes mutability I’ve moved forth and I’m dead earnest.
I’m defiantly optimistic you can not willing to give up, no matter what. I have the right to live and life is granted once beyond my ken, but certainly has a purpose.