Voice of a rare disease patient about 'hopeless' and 'flawed' healthcare policy which does not treat patients as equals.

India is a secular federal republic governed in a democratic parliamentary system. The preamble of our Constitution states,

WE, THE PEOPLE OF INDIA, having solemnly resolved to constitute India into a SOVEREIGN SOCIALIST SECULAR DEMOCRATIC REPUBLIC and to secure to all its citizens

JUSTICE, social, economic and political;

LIBERTY of thought, expression, belief, faith and worship;

EQUALITY of status and of opportunity;

and to promote among them all FRATERNITY

assuring the dignity of the individual and the unity and integrity of the Nation.

India seeks social, economic and political justice to ensure equality to its citizens.

We often hear about activists or common people fighting for their human rights. But what are these rights? And who gave us these rights? Why do people have to fight for their rights? 

Fundamental Rights are considered as basic human rights of all citizens, irrespective of their gender, caste, religion or creed. etc. These sections are the vital elements of the constitution, which was developed between 1947 and 1949 by the Constitution of India.

India seeks social, economic and political justice to ensure equality to its citizens.

Right to Equality ensures equal rights for all the citizens.

Equality means the absence of privileges or discrimination against any section of society. 

The Preamble provides for equality of status and opportunity to all the people of the country.

Then why is the government discriminating on the basis of disease, even a rare disease?

Article 21 says that no one shall be deprived of life and personal liberty. “Right to life” included the right to lead a healthy life so as to enjoy all faculties of the human body in their prime conditions.

Right to Medical Care.

In Pravat Kumar Mukherjee v. Ruby General Hospital & Others[li], it was held that a hospital is duty-bound to accept accident victims and patients who are in critical condition and that it cannot refuse treatment on the ground that the victim is not in a position to pay the fee or meet the expenses or on the ground that there is no close relation of the victim available who can give consent for medical treatment.

The court has laid stress on a very crucial point, viz., the state cannot plead lack of financial resources to carry out these directions meant to provide adequate medical services to the people. The state cannot avoid its constitutional obligation to provide adequate medical services to people on account of financial constraints.

We know that

The most common rare diseases identified in India are 

• Haemophilia --A disorder in which blood doesn't clot normally.

When blood can't clot properly, excessive bleeding (external and internal) occurs after any injury or damage ...treatment - clot promoter medications.

• Thalassemia-- blood disorder that causes your body to have less haemoglobin than normal. Haemoglobin enables red blood cells to carry oxygen. Thalassemia can cause anaemia, leaving you fatigued. Treatment -Mild forms may not need treatment. Severe forms may require blood transfusions or a donor stem-cell transplant, 

• Sickle-cell Anaemia-- A group of disorders that cause red blood cells to become misshapen and break down.

• With sickle cell disease, an inherited group of disorders, red blood cells contort into a sickle shape. The cells die early, leaving a shortage of healthy red blood cells (sickle cell anaemia) and can block blood flow causing pain (sickle cell crisis).

•  Primary Immuno Deficiency -- Primary immunodeficiency disorders — also called primary immune disorders or primary immunodeficiency — weaken the immune system, allowing infections and other health problems to occur more easily. Treatment -- Treatment for immunodeficiency disorders commonly includes antibiotics and immunoglobulin therapy. Other antiviral drugs, amantadine and acyclovir, or a drug called interferon are used for the treatment of the viral infections caused by immunodeficiency disorders.

If your bone marrow isn’t producing enough lymphocytes, your doctor might order a bone marrow (stem cell) transplant.

• Lysosomal Storage Disorders such as Gaucher Disease, Fabry Disease, Hunter Syndrome and Pompe Disease --Lysosomal storage diseases (LSDs; /ˌlaɪsəˈsoʊməl/) are a group of about 50 rare inherited metabolic disorders that result from defects in lysosomal function. Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other parts of the cell for recycling. This process requires several critical enzymes. If one of these enzymes is defective, because of a mutation, the large molecules accumulate within the cell, eventually killing it. Treatment --No cures for lysosomal storage diseases are known, and treatment is mostly symptomatic, although bone marrow transplantation and enzyme replacement therapy (ERT) has been tried with some success.

The company is committed to supporting faster diagnosis and better access to treatments for patients with rare diseases.

"As a company, we are committed to serve patients suffering from rare diseases like Hunter's syndrome, Gaucher and Fabry and support them in their disease journey to ensure improved quality of life," said Vineet Singhal, General Manager and Country Head, Takeda India.

India Charitable access program is our free drug program through which we provide free Enzyme Replacement Therapy to patients with any of the four Lysosomal Storage Disorders (LSDs) - Gaucher disease, Pompe disease, Fabry disease and MPS I disease. Since 1999, INCAP has provided support to more than 100 patients in the Country. The program is guided by an expert medical committee that remains closely involved in participating patients' care and progress.

So we can see that only a handful of rare diseases get support and those which can be treated with medications or bone marrow transplant, if severe which is available in government hospitals...the cost of medicines may well be crores of rupees per annum." In India bone marrow transplant costs Rs.10-20 lakh in private hospitals, while in government hospitals it is much cheaper -  Rs.3-6 lakh - depending on the type of procedure, he said."

Read more at

I happen to have a rare disease 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 tumour and in certain cases, it turns cancerous. It is a genetic form of cancer VHL patients battle a series of tumours throughout their life.

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.

VHL, or von Hippel-Lindau disease, is a genetic disorder characterized by tumours in up to ten areas of the body. Under normal conditions, the VHL gene controls the cell's oxygen-sensing mechanism, keeping cancerous tumours at bay. When the gene is missing or mutated, the body's tumour suppressor signalling does not function correctly. The tumours can be benign or cancerous and appear and grow unpredictably throughout a patient's life. 

Most of these VHL tumours are benign, but that does not mean they are problem-free! In fact, benign VHL tumours can still be very serious. As they grow, these tumours and the associated cysts can cause increased pressure on the structure around them. This pressure can create symptoms including severe pain.

I had strange symptoms within four month after brain surgery and vascular tumours were detected in my liver but due to lack of money and the rapid growth of the tumours, they studded the liver in a way that transplant was the only way out.

The transplanted liver needs the immune system to be suppressed so that it isn’t rejected like bacteria and viruses. Immunosuppressants are expensive life-saving medicines. I am on immunosuppressive medicines for life. 

Right after I was discharged from my successful liver transplant, I got herpes zoster (shingles) with a high fever. I survived that because I had a long way to go. 

Here I might clarify the difference between immunosuppressants and immunotherapy. People are asking me why? Immunotherapy is available in government hospitals why not go for it...well, immunotherapy is a type of cancer treatment that helps your immune system fight cancer whereas immunosuppressants are drugs or medicines that lower the body's ability to reject a transplanted organ. Another term for these drugs is anti-rejection drugs.

I  take up to 20 pills throughout the day for my liver. HypoPara, brain, endocrine problems and associated problems like hypertension and tachycardia. 

If I don't take them I end up in the emergency room like getting a tetany attack or getting some grim infection with 105 F fever or diarrhoea. 

I battled MDR TB after my father's death because of prolonged usage of immunosuppressants and living in unhygienic houses with dripping roofs with moss and fungi all over the walls. We changed 7-8 houses in our eleven years of stay in Delhi.

When I was diagnosed with a couple of brain tumours right after a tetany emergency and infection which required daily injections of expensive antibiotics. I found myself floating in a leaky boat in the roaring sea without an anchor. I had no notion which way my life was taking me but I had no choice. I used my own hands as oars and my willpower to push the boat in a steady direction and stop it from sinking. 

Blimey! I thought this is the most horrendous thing that could ever happen. My father could never have imagined I would face the same situation of a brain tumour in my life. I felt like I was born with a noose around my neck. 

I'm a goose,

With an irksome noose,

Round my neck,

Zinging the tatty track

With an oooooh,

Volubly yet I say,

Wondrous abbreviated time,

Acrid stress,

The drag race,

‘live forever or die in the attempt’... says my selfish gene.”

This is what I wrote before my first cyberknife after my father's death. The worry about my brain tumour for which he eventually got a heart attack killed him after my liver transplant because he could not afford the medicines, leave alone the bypass surgery he required. Could he have imagined I will face the same situation over and over again? 

I was diagnosed with leptomeningeal hemangioblastomas since, 2013 Dec after the cyberknife.

Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Few studies have reported leptomeningeal involvement in sporadic HB or in HB associated with von Hippel ­Lindau syndrome.

Between 1902 and 2013, approximately 132 cases were reported. 

Loss of visual acuity or field and sudden visual loss or changes led to the diagnosis of an optic nerve tumour. Brain and spine MRI showed a mass in the kidney which was eventually diagnosed as kidney cancer in PET SCAN. I was diagnosed with both of these things at the same time in 2015 and I chose to get partial nephrectomy for my kidney cancer first.  I ran from hospital to hospital but couldn't convince the doctors that I can't give consent for a fine needle biopsy before the surgery as it may spread cancer to different parts of the body. Ultimately I found a doctor willing to do a laparoscopic partial nephrectomy without a biopsy prior to the surgery. During which a battle of the house was going on with our landlord who had asked to vacate without considering that I needed kidney cancer surgery and optic nerve radiation therapy.

But due to the immunosuppressants I take, wound healing is slowed down and in 2016 it led to incisional hernia repair and a debridement surgery. Anyway, after the partial nephrectomy was successfully completed, I had to wait for optic nerve tumour radiation therapy. By the time I got money for the treatment I was completely blind with my right eye. The treatment stopped the growth of the tumour but didn't shrink it. Hence, I can see with my left eye only. Now I also see black floaters and blue flashes in my left eye, and I haven't got my retina tested since 2917 because of financial constraints.

 In 2016 after the incisional hernia repair because of less blood flow to the skin tissue, I had a huge necrosed area in the abdomen. When, after a lot of effort, the attention of the doctors was drawn to the area and they identified it. The necrosed tissue was removed by a debridement surgery in a matter of fifteen days. I survived the back-to-back surgeries, but I required injections worth Rs 10000 daily. 

Following this, I had radiation therapy for two growing brain tumours putting pressure on the surrounding area and causing headaches. I could not afford a cyberknife and chose to go for radiation therapy. After six weeks of radiation and clumps of hair falling off which never grew back after the treatment was completed. Even now I have a bald spot on my scalp. Then in the following routine MRI, it showed white matter damage and a chronic ischemic brain. Meanwhile, electricity started zapping through my right eye then upper jaw then lower jaw and pressure built up in the forehead. 

I was diagnosed with bilateral trigeminal neuralgia.

 

Battle with Trigeminal Neuralgia

A bolt of pain shot up the face,

Reached the farthest limit,

With disdain without grace,

Sneaking into the throat,

As quiet as a mouse

Huddling behind the ear,

Pulsating in the mouth,

Strange tic

Distorting face so quick, 

Drilling teeth, eye swelling,

Making it tormented house,

 A distressful dwelling--

Jumping off, crossing sides

Jumps and attacks and hides

It's a race against time,

A gloomy struggle all-day

As the predicament shook me to the core

It's a dreadful dream, a nightmare

That I battle every day and more

I got a cyberknife in order to avoid surgery and a biopsy which can cause a threat to my life by causing meningitis, bleeding and spreading the cells. 

I found a doctor in Mumbai who said he would do my cyberknife in two parts. So, the right side is done now and pain is almost gone except a swallowing problem and numbness of the neck muscles. The left side remains to be done.

This is my medical odyssey where surgery, advanced technology and expertise of the doctors have saved me thus far. 

I have further untreated symptoms like tinnitus, vertigo, ear fullness, dental issues, facial pariesis and the vascular tumour detected in my neck.

Life for me is a challenge to be met with courage. Life is a game of skill with an element of chance. Life comes to a standstill if you give up hope. The power of volition originating from the energy of creation residing inside us made us move onward in small steps and little by little without thinking about the future or what may come tomorrow we advanced without stopping, without crying, thus like every life from desiring to live making tough decisions I fought on. 

I chose to trudge the hills less trodden by;

With head held high;

Being cognizant;

The turf is uncertain;

So is the terrain;

Still hanging on

When I want to sigh;

I get up with head held high;

Choosing a path never trodden by,

“To leave a trail and make a difference…

I came across a news article on the internet.

Which speaks should the government pay for treatment of rare diseases? Well, I spoke to the advocate mentioned in the news article. She is appointed by the court to help the court decide this matter. 

According to her in absence of government policy, the court has stepped in to provide treatment to lysosomal storage disorder patients. How the law has developed, and the rare diseases are handled in court so far are only lysosomal storage disorders.

The government can procure medicines but not where there is a need for surgery or advanced technology to deal with a disease. Who is to pay for it?

  Did I muse, why so? Is that because someone related to the government has those conditions? The government should pay private hospitals for specialist treatments. Why not? 

"I am not saying it is a sort of open and shut legal battle for you but it's not like the government will be able to fund every cancer patient." So now to get the specialised treatment, required for my disease I need to approach the court. I do not have my rights to proper treatment. Just an appeal will not do, I understand as it was said subtly.

According to my experience government hospitals are breeding-ground for germs, bacteria and viruses and with my low immunity, seeking treatment in government hospitals is welcoming a death sentence. Moreover, the treatment of most cases of VHL usually involves surgery to remove the tumours before they become harmful. Certain tumours can be treated with focused high-dose irradiation. Individuals with VHL and organ transplant need careful monitoring by a physician familiar with the disorder to control the plethora of illnesses that organ transplants and VHL affliction brings. I can't leave the doctors familiar with my case.

All which is needed to lengthen the period and quality of life is a good doctor who is thinking about your health and happiness and not treating you like a guinea pig and not working for his own good and fame. I got the opportunity to choose between good and better doctors four times in my life, during my liver transplant- who didn't say transplanting the liver will cause the tumours to grow once again and it will be a futile effort;

my MDR TB treatment-cured me of MDR TB which other doctors couldn't and because those doctors failed because of whom I can't walk properly as I had bone TB and I still need the help of a stick to walk;

my kidney cancer- who understood before taking out the cancerous tumour in kidney doing fine needle biopsy could spread cancer to other parts of the body

and trigeminal neuralgia-understood risks of MVD surgery on a leptomeningeal hemangioblastoma patient.

Perhaps the medical community needs to hear your thoughts, experiences and suggestions order to change for the better. Instead of accepting whatever the doctor says, as Gospel keeping oneself well-informed and getting treatment of one's choices will improve the quality of life and lengthen it. Your voice needs to be heard.

Also, I am not every second cancer patient. LSD patients have a delay in intellectual development and learning problems which I never had. I have written in nature Asia

and Sahitya Akademi  

for the latter one, I received two emails of appreciation.

And authored three books

This doesn't mean I am disrespecting or I don't want to demean any disease and their struggle but I am stating facts that despite my struggle with poverty and a unique disease I never keep ranting about it all the time.

I did not want to spend time living like a disabled person spending time commuting between the house and hospitals and shifting houses because landlords don't care or understand. I could have chosen to sit at home by myself. Every day, day after day, isolated from the world but I took life as a challenge and strove to write detective fiction, a thriller novel, and my survival story.

 

As the letter states, "I appreciate your efforts put in to give to the society an interesting and captivating book 'The adventures of mum and princess' that people of all ages enjoy reading. I would highly recommend this book to the students of classes 8,9,10 in my institute 'Monika's Institute of English Language "... 

If I am not denied proper treatment, I would give more such books to the society. My brain is riddled with tumours and I have written these books with my one-eyed vision.

.

As for who will pay for my treatment? Well, why does the government levy taxes? Even on crowdfunding platforms? They charge GST or Goods Services Tax.

Depending on which platform and plan you use you have to pay them from what you gather for your own treatment. 

Ketto, Milaap are alike, it differs in what percentage they charge. They take a huge sum in the name of gateway charges. 

Gateway fee: This is usually a monthly fee for using the payment gateway, the service that passes credit card data around the internet to either authorize or deny a credit card charge.

RBI could help with the gateway charges and the government could have spared GST so that whatever we can raise through crowdfunding would go entirely to our treatment. Not only this every medicine we buy, and for every test, we have to pay the cost plus tax. Then why not, government pay for my treatment from all the taxes I have been paying and my brother has been paying to be a freelancer? When I travel to the hospital, we pay toll tax on the Delhi Gurgaon border each time. My treatment doesn't amount to crores of rupees so it can well be paid from taxpayers’ money.

The Central Government spent close to Rs 10000 crore in 16 years from 2002-03 to 2017-18. 

The government spent over Rs 5,700 crore on total advertising between May 2014 and March 2019. So why can't a tiny amount go for treatment and saving one's life?

Read more at

https://www.indiatoday.in/india/story/government-spending-more-ads-hindi-newspapers-rti-1596821-2019-09-08

https://factly.in/the-central-government-spent-10000-crore-on-publicity-in-the-last-16-years/

Court happens to pronounce my death sentence for not having resources for my treatment! Well, do you think Justice is being served? Disparity judged by statistics of a malady and modes of treatment; their availability isn't fair play. How can I avail my monthly follow-ups and treatment with mother's pension which is just Rs 7000?

There are many NGOs in our country who care about orphans, child education, farmers but no one cares for a rare disease patient. Most of them prefer to help on a mass scale to get their names printed on the front page of the leading dailies.
Healthcare is very expensive for those who don't have the wherewithal to pay but does that mean they are destined to die. Those with the rare chronic disease can't be like a bad tooth waiting to be pulled out of the society, they can't be treated like unwanted. They are in need of better treatment for a better life.

You never realize how terrible it is unless you are in the middle of it. Life is hard and I have learnt to accept and adjust. 

My mum taught me to hold on like she does gripping whatever she can to keep it for another day. She does not think about the next week or the next month, just today. That's what she tells me that's how we have pulled so long by just keeping it for today. But now she is 67 yrs old but she's still fighting with the sword called courage. She's my God constantly saving me from my ailments and fighting the pathetic situation of our medical procedures which have become slaves of currency notes in most of the hospitals. She's my caregiver, my friend, my life. She inspired me to be a warrior princess. She helps me to gain sustenance for my mind, body and spirit so that I can pursue my dreams and travel to the very end of the road. 

But no, I feel everybody is sleeping their own sleep. We need to wake up, our tired souls and see the truth. We don't need more sleep; we need to wake up and live then only magic will happen. 

Hope springs eternal to a defiantly optimistic person. It's the indomitable life force that keeps me moving. Different people, different lives, different personalities all can be full of sunshine with equitable decisions. 

I have had a melancholy start
 But I fought through 
Give me a pleasant middle 
So that the end becomes joyful 
Not the saddest part.

Can any person understand the reality of my strife? Feel the unsaid words trapped between the lines? I am going on even when I lack the strength to…the pain inflicted by the endless thought of how to keep going with my treatment...health doesn't always come from medicine...mostly it's the peace of mind, peace in the heart and peace of soul which is not my case. The shocking cliché has always been money and I constantly have to think about it. Will anybody speak up for me? 

A Warrior fights the battle of life ---all its dreadful possibilities without a whit of fear without thinking that it’s unfair. Before we are inevitably visited by death and thrust to total chaos a warrior’s mind gracefully dances to the rhythm of the music of creation and life. Thus, A Warrior Dies Dancing, That’s Who I Am… The upshot is life belongs to us but we belong to death meanwhile dance away the time you get.

I never knew what life had encrypted in my genome. My mind whispers you have come so far " Go confidently in the direction of your dreams. Live the life you have imagined…. 

Myriad ways to learn,

Turn mirrors into a window,

We are hypnotized by our vociferous wretched selves,

Reside in a sea of blindness,

The semblance of truth,

We are at a standstill, you know.

Restful in ignorance,

The bold step to welcome change,

Choreographs a dance of awareness,

Adhere to orthodoxy,

Makes you an architect of self-decay and you rot,

I choose to gallop not trot,

We seek equity,

Elevate others from drudgery,

Kindle that inner voice,

Many reside in partiality in malady.

Ref

https://www.sanofi.in/en/about-us/empowering-life/fighting-rare-diseases

https://www.outlookindia.com/newsscroll/takeda-launches-enzyme-replacement-therapy-in-india/1575399

https://m.timesofindia.com/city/bengaluru/govts-reluctance-to-bear-treatment-cost-rattles-rare-disease-patients-kin/amp_articleshow/73257600.cms