Tuesday, July 6, 2021

Vascularised, hopeful, lifetime of learning and living with vHL where cure for cancer lurks in my genes.

 





I was diagnosed with a rare disease von Hippel-Lindau or VHL. 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 also there is a possibility of neuroendocrine tumours.




Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Between 1902 and 2013, approximately 132 cases were reported.





Detecting and treating the condition of leptomeningeal hemangioblastoma without delay seems to help survival, though the number of patients analyzed is small. The most common cause of death is respiratory failure due to pontomedullary or cervical cord compression.








Early detection and aggressive management can enhance the prognosis of patients with CNS leptomeningeal dissemination of HBs. Imaging surveillance, by contrast-enhanced spinal or brain MRI, should be considered in patients with a large amount of tumor bleeding during initial surgery.




After 2017 I also have a chronic ischemic brain. Cerebral ischemia or brain ischemia, and when there isn’t enough blood flow to the brain leading to limited oxygen supply it may lead to the death of brain tissue, or ischemic stroke.



A proton beam directed at a tumour travels in a straight trajectory towards its target, gives off most of its energy at a defined depth called the Bragg peak, and then stops. X-rays often deposit more energy within the healthy tissues of the body than within the tumour.


Dr Jalali gave me an estimate of 40lakhs for both brain and spine. But when the pancreas came up he said he won't charge extra







I have no income. I write for Times of India digital without honorarium and my mother who is a pensioner draws barely enough pension to feed and clothe us, leave alone pay for my medical expenses.




After the cyberknife on 17/3/2020I got a swallowing problem. The cranial nerves associated with the swallowing process are the trigeminal (V), facial (VII), glossopharyngeal (IX), vagus (X), accessory (XI) - usually not considered - and hypoglossal (XII). 





The mutation of the gene is confirmed in an AIIMS.





There were multiple cysts in the pancreas already. They blocked the exocrine functions ( enzymes) and for a couple of months, I had to run to the bathroom after every meal. After Creon SD microgranules were prescribed my diarrhea feels better but you can confirm it is around Rs 3000 and lasts for only 2 days.






Pancreatic cancer is one of the most lethal malignancies in the world, with mortality rates being close to the incidence rates. The incidence rates of pancreatic cancer is 3%. Most patients with pancreatic cancer are diagnosed at the advanced stage due to the deficiency of a standard program for screening patients at a high risk of pancreatic cancer,leading to a poor prognosis with a 5-year survival rate of <7%. Therefore, it is very important to clarify the mechanisms of pancreatic cancer progression and develop novel therapeutic strategies to improve the overall survival of affected patients.




The VHL Alliance (VHLA, vhl.org) has been working for decades with cadres of research professionals and members of the medical community around the world to better understand the VHL gene and its impact on cancer development. The VHL Alliance funds research in numerous areas including work related to that of the newest Nobel Laureates.




Thanks to this research, the FDA has approved eight drugs for the treatment of kidney and breast cancers. These agents target the regulation of cellular growth factors (the downstream consequence of elevated HIF levels).




A HIF inhibitor is currently in clinical trials for VHL and metastatic kidney cancer. Due to our current understanding of HIF, there is reason to believe that this treatment may be effective in other forms of cancer, as well.


The will to live is an unstoppable thing. Most people live only when they are about to die. 




Well, if you ask me, does Covid impact brain tumor treatment? I'd say yes because I got Covid myself.


 Mansi Aggarwal and hubby are crazy. I deciphered that although Mum  started with CT level 21.05  because at 68 she developed moderate Covid perhaps because of collecting the deliveries of medicines or food kept in the bag hanging on the door. Perhaps some delivery men might be Covid positive and she collected stuff after an hour but aSARS-CoV-2 remained viable in aerosols throughout the duration of 3 hours, with a reduction in infectious titer from 103.5 to 102.7 TCID50 per litre of air. This reduction was similar to that observed with SARS-CoV-1, from 104.3 to 103.5 TCID50 per millilitre.



 As from 18/3/2020, we are under voluntary isolation to avoid the pandemic and just Mum collects the deliveries hanging on the bag outside the door after the person has gone for over an hour.




Also since Mansi Aggarwal had Covid below 20 and tested negative once they thought Mum had a high viral load and because she came out of her room to give my medicines and breakfast which happens to be a mere gruel.


Mansi Aggarwal thought her hubby will contract Covid and if I die everyone will be saved because Mum will not have to care for anyone.


That's why she hit me twice and said I have to die right now without a mask and screaming ' bitch you have to die right now"


Good logic. But I got Covid from Mansi Aggarwal after 3 days. Mum had symptoms on the 28th April She might have contacted it before as the incubation period is 2-14 days but I didn't get it from her because she wears a double N95 mask while coming out of her room.


Mansi Aggarwal was a patient with few symptoms for 26 days under isolation with high viral RNA load in the nasopharynx early in the course of disease.



CT count or cyclic threshold is inversely proportional to the viral load..more than 30 considered very low infectivity..less than 20 high infectivity..20 - 30 moderate infection- hence Mum had moderate infection which rose to CT count 28 and now vamoosed, gone!


Experts have also strongly suggested that solely relying on testing as a safeguard can make people ‘lax’ and take other safety measures, such as mask-wearing and social distancing lightly, which could be the two biggest factors which can spike up infections. Remember, the tests are a preventive measure and only effective, as long as you put in place other security measures. But she put my life at a risk by subtly killing me. 



 Blood tests at times are a good marker of inflammation and they can also give you and your doctor an idea of what is going on in your body, in particular, the C-reactive protein and the D dimer. Both of them are said to be a good testing ground for understanding what is happening in your body.




The RT-PCR test was not done in my case because I have trouble swallowing food and medicines, Here I'd like to state The cranial nerves associated with the swallowing process are the trigeminal (V), facial (VII), glossopharyngeal (IX), vagus (X), accessory (XI) - usually not considered - and hypoglossal (XII).


Hence it might be extremely hurtful as I get pain in the pharynx and larynx and often my tongue gets paralyzed. Voice becomes hoarse after speaking a bit. 


Both Naso and Oropharyngeal samples are collected. If there's a problem in nasopharyngeal then it can be done in Oropharyngeal sample only hence I got my CRP, D-Dimer and antibody tested.


I had so many life threatening surgeries and my realisation of Memento Mori   started very  long ago.


We desperately need in our own lives—a thought or an idea that we’d rather do everything to avoid and pretend is not true. Most often, our ego runs away from anything that reminds us of reality or, we are simply petrified to look at life’s facts as they are. And there is one simple fact that most of us are utterly scared to meditate, reflect on and face head on: We are going to die. Everyone around us is going to die.Such reminders  take part of Memento Mori—the ancient practice of reflection on mortality that goes back to Socrates...Memento Mori translated in English, “Remember you must die.” The point of this reminder isn’t to be morbid or promote fear, but to inspire, motivate and clarify. The idea has been central to art, philosophy, literature, architecture, and more throughout history. 


Memento mori is an artistic or symbolic reminder of the inevitability of death by keeping an object such as a skull


  


 Only after Madam Mansi hit me and said without a mask I should die right now within 3 days I got high D dimer and CRP but no antibody. Antigen couldn't be tested as   both Naso and Oropharyngeal samples were collected. If I have a problem in my nasopharyngeal then it can be done in an Oropharyngeal sample and it might hurt my throat.  Bilateral trigeminal nerve, vagus, Hypoglossal, Glossopharyngeal, facial, auditory are all compressed by tumours.

But I formed antibodies after a month and Jeevan Stambh NGO loaned an oxygen concentrator.


After getting 15 surgeries I can't give up on living. With leptomeningeal hemangioblastomas and Covid with the immune system turned off due to liver transplant I am at a high risk but I love life and my mother. I don't want to leave so early.


 I don't worry. I am not just struggling, I am a warrior. Worrying is a full-time job, requiring 24 hour work a day. Every day, you worry when you wake up, while at work, when you eat, even while you dream. Instead of a worrier woe-man, I chose to be a Warrior Princess, yes that's who I am.


  




A Warrior princess I am, 


I wage wars with head held high, 


A warrior, never afraid of wounds, 


A Warrior dies dancing that’s who I am.













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