By Reeti Mahobe,
What’s common between the Oscar winning movie, Life of Pi, and her? That continuously ongoing struggle not just of life but for life. But her indomitable spirit is unmatched for. She is rare yet very special. This is about Payel Bhattacharya who has seen 33 years of life with each day being a mystery to unravel bringing in either new hopes or more often the new hurdles to conquer through. On this occasion of Rare Disease Day, she expresses her intent to spread awareness on VHL or Von Hippel Lindau Disease, the condition with which she has lived for all these years.
When she was just seven years old, while dancing at home, she suddenly felt excruciating pain on one of her feet, post which she couldn’t remember. Her doctors initially couldn’t diagnose the real cause behind the pain and tagged it to be sprain. Later she was diagnosed with an extremely rare disease called as Von Hippel Lindau Disease with a frequency rate of 0.001. This disease leads to formation of blood-filled benign tumors in vital organs of the body. While the disease is congenital in nature, in some cases, it might occur due to gene mutation during fetal development which seems to be the case in Payel’s. That pain on her foot was also result of formation of tumor. More than 16 organs of the body have had got affected because of this disease. There also remains fear of these tumors turning malignant at some point. She had also undergone her liver transplant four years back at Sir Gangaram Hospital with the financial help from PM’s fund. Her support system through all these odds has been her family. But this did not end here. Being shocked at take-over of his house by his brother, Payel’s father suffered heart attack and passed away just after her transplant. In order to avoid rejection of new organ in body she was administered with immunosuppressant that brought down her immunity immensely so much that now she has developed MDR TB totally unrelated with VHL.
Payel Bhattacharya
Payel’s living condition hasn’t been fair either. She stays in rented house at Delhi with hardly any ventilation with her mother. She also faced dejection from her earlier landlord who got her out of the house as she had contracted with MDR TB. She originally hails from Kolkata, but shifted to Delhi for her treatment. She has to get her treatment done from three different hospitals viz. Medanta Hospital, Gurgaon, AIIMS and Sir Gangaram Hospital. She is grateful to her doctors who have been life-savers. Even after so much of pain every now and then she managed to finish her school but still laments over not being able to go to college. She has excellent English. She has written over few poems published on her facebook profile which paint up a picture of her struggle. She currently has lot of brain tumors because of which doctors fear if she anytime gets excited, she might go into Comatose state. She has to regularly get her cyber knife surgery (radio-surgery) done. She has depended on some kind souls for covering her huge medical expenses but hasn’t been fortunate enough to get enough bucks everytime she needed. Only support currently has been from her brother who is a freelancer, and sometimes he himself doesn’t have a job.
She feels agitated over why government shouldn’t be covering her treatment expenses when she doesn’t have any financial backing! Why is there so much apathy against those suffering from such rare disorders? There’s not even any provision for it under much hyped about government sponsored health insurance schemes; neither for diagnosis nor for treatment expenses, forget about research purposes. It’s simply not understandable on how we could envision an inclusive society when no heed is being paid to all such persons who have struggled since forever for every minute of life.
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Payel Bhattacharya writes her experiences:
What is a rare disease?
A rare disease is a life-threatening or chronically debilitating disease. The common denominator of rare diseases is the infrequency of their occurrence in the human population. The likelihood where in a physician has encountered perplexing medical conditions and tackled it with expertise is rare.
My experience with a rare disease:- My encounter with a multi-organ rare disease and with physicians is that they themselves admit of getting 2 or 3 cases at the most hence even after diagnosing they don’t keep themselves updated with diagnostic assays and treatment modalities. I’m a rare disease patient causing tumorigenesis and have undergone 9 surgeries including a brain hemangioblastoma , liver transplantation due to vascular malformation( identified clinically as VHL or Von Hippel–Lindau syndrome which is treatable but has no cure), Cyber knife Radiosurgery of two more brain hemangioblastomas on 14th July 2013 and a and a communicable disease unrelated to VHL, disseminated latent MDR TB under immunocompromised state to avoid liver graft rejection.
About Von Hippel-Lindau disease:- Von Hippel-Lindau disease, or von Hippel-Lindau syndrome, is a rare genetic disorder characterized by visceral cysts, benign masses, and the potential for malignant transformation in multiple organ systems. Imaging plays a key role in identification of abnormalities and subsequent follow-up of lesions. The spectrum of clinical manifestations of the disease is broad; About 40 different lesions in 14 different organs have been described. Imaging plays a key role in the identification of abnormalities and their subsequent follow-up, in the screening of asymptomatic gene carriers, and in their long-term surveillance. The importance of screening is emphasized because the lesions in VHL disease are treatable; thus, early detection enables more conservative therapy to be performed and may enhance the patient’s length and quality of life. Because VHL varies so widely, there is not a consistent set of symptoms in each person. Every incidence of the disease has its own diagnostic evaluation. Being multi -ordinal disorder getting help for treatment of a VHL person is pie in the sky within the abbreviated time, so that growth of lesions doesn’t cause undue complexities especially neurologic ones. It can be emphatically said speed in detection and follow up through imaging and tests is absolute imperative case of VHL to better the quality of life in a debilitating condition. Whereas all my disease/ disorders are treatable but not curable provided no lag period happens in diagnosis thereby not deferring the proper treatment, at which point it might incur infirmity making the quality of life poorer with every passing day. I’ve been able to avail the authority in each manifestation of different organs presently dealing with my condition. My walking infirmity incurred due to TB has amped the expenses.
Current Status of tumors:- 1. Recent evaluation after cyber knife radiation is, my brain is riddled with tumors (hemangioblastomas) consequence – balancing and co ordination problems. 2. There is infiltration/fibrosis in left upper and mid zones in lungs – no evidence of TB as per radiological conference 3. Right lobe of thyroid : Remnant of the right lobe shows atleast 4 hypoechoic nodules measuring ~ 10mm in size with vascular flow seen within as well as at the periphery on CDF images. 4. Vision problems exist but not proper detection yet, save Posterior subcapsular cateract 5. Tetany emergencies : Rocaltrol and tayo (TDS current mode of control) I moved forth without regression with cadge from friends well-wishers and not willing to give up.
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. My long strife has stolen my life, hence I approach to revamp my life,long stolen due to my strife. I’m defiantly optimistic and reach out for a helping hand as I’m certain a way can be improvise to help a rare disease patient who is 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.
More information on her could be sought here https://plus.google.com/114374871539962440874
She could be reached at [email protected]
Facebook Profile: www.facebook.com/payel.bh