Payel Bhattacharya (Hippel-Lindau Syndrome): Fighting multiple battles together

Payel Bhattacharya (Hippel-Lindau Syndrome Warrior)

A test of inner strength awaits me as the pressure builds upon the nerves along with various other problems, which may exhaust my energy. With the winter months’ approach, a certain bored feeling sets in, let alone the loneliness.

I had a liver transplant due to several liver tumors that could not be taken out individually, causing excruciating Pain due to frequent hemorrhages in 2008. The largest lesion caused splaying of the portal vein around it. Hepatic veins were compressed and displaced by the segment 4&8 mass lesion. I had two episodes of bleeding and in the last one, asked the doctor to get euthanasia done. Bleeding in the hemangioblastomas required hospitalization and was excruciatingly painful.

Certain medications are nothing short of miracles for the patients who need them. Medications such as anti-rejection drugs, chemotherapy, and corticosteroids are life-saving for many patients, but like many treatments, these pharmaceutical marvels come with side effects. And at least one of these side effects requires some potentially significant lifestyle adjustments.

The side effect in question? These medications may increase your risk of infection.

In other words, drugs like sirolimus, prednisolone, Cyclosporine, Mycophenolic, or tacrolimus jeopardize your immune system. They are immunosuppressant drugs.

It sounds alarming, but what does it even mean?

Conversation with VHL winner Payel Bhattacharya
Conversation with VHL winner Payel Bhattacharya
/

I have come across people who have confused it with Immunotherapy. Immunotherapy is a type of Cancer Treatment that boosts the body’s natural defenses to fight cancer. It uses substances made by the body or created in a laboratory that makes our immune system to find and destroy cancer cells.

It means the medication can interfere with the body’s processes that prevent infection, and this interference is what allows the medication to work. How and why this happens depends on the particular drug. In general, however, the medication causes all or part of your immune system to “turn off so that your body doesn’t go into attack mode, waging war against whatever it sees as a foreign invader.

This almost implies that if you are taking some of these medications, you will fall ill every time you pass by someone with the sniffles along with the big stuff, like flu or tuberculosis. Are you going to need to live in a bubble from here on out?

What are the side effects of immunosuppressants?

Fortunately, in most cases, an actual bubble won’t be necessary. Unless there’s a pandemic going on and you are ill-fated that the army to protect you is disarmed. However, it is essential to understand the outcomes of living with a compromised immune system so you can protect yourself.

Side effects of immunosuppressant medications may include gastrointestinal problems like diarrhea, nausea, and Vomiting. However, the most severe side effect of taking an immunosuppressant is the risk of infection.

That could mean catching every single bug your family member brings home from work or the possibility that a flu diagnosis will land you in the hospital. You are also more likely to experience complications from food-borne illnesses, bug bites, and environmental hazards (like mold). Oh, and you know all of those recent H1N1 outbreaks? You could always be at risk. Immunosuppressants also leave you at risk for very rare and difficult-to-treat infections, molds, fungal pneumonia, and certain types of Lymphoma.

Being diligent about basic hygiene procedures such as hand-washing is the only way out.

Everyone knows that hand-washing is the best thing you can do to avoid getting sick, but it is even more important for those who are immune-compromised and anyone who comes into contact with them.

Make sure to wash fruits and vegetables.

Avoid people who have active infections (don’t be shy about telling people to keep their distance, either).

You may need to wear a mask at times (if you are on an airplane and people are coughing, for example), and it is also wise to avoid large crowds.

Staying up-to-date on all of your vaccinations is extremely important.

Adopt healthy lifestyle practices (get plenty of sleep, exercise, and eat a healthy diet).

If you think you might be sick or show any signs of infection, call your doctor immediately. The wait-and-see plan that applies to so many other people does not apply to the immunocompromised population. This is especially true with fevers.

If there is a high fever, they need to run to the emergency room to see a healthcare provider. Still, I got a mysterious fever when I got to the emergency room at 2 am because I was getting tetany and needed a Calcium drip.

Soon after discharge from the hospital after liver transplant, I got viral infection varicella and was treated with Zovirax, which was my first experience of viral infection.

To my plight, I was diagnosed with RCC H1N1 that was raging in Delhi, and I had to commute to different hospitals because the doctors wanted to do a needle Biopsy before taking the tumor out. I traveled to different hospitals and got a cuckoo fever, which wouldn’t go. I couldn’t get my blood tested for H1N1, and the clock was ticking because my RCC was 2.8 cms, just a little below the threshold. The Surgery was confirmed with a doctor at FMRI, but he still said he couldn’t perform Surgery with an infection in the body.

By God’s grace, I thought of calling another experienced doctor then, who gave me some medicine over the phone and green cough syrup. I believe it’s thanks to his experiences and expertise that I recovered and became ready for the Surgery.

The ultimate example of the risk of infection is getting MDR-Tb. I was losing more weight than the exercises I was doing and the food I was taking. I never had outside food, but still, the doctors didn’t think of that. Two years after the liver transplant, I had a bewildering high fever, which puzzled the doctors. After three months of continuous fever with a swollen lymph node made the physicians think. The lymph node Biopsy showed TB infection(AFB+). More than four months of being on anti-Tb-treatment resulted in my lung condition worsening. HAIN test confirmed that the bacteria is resistant to Rifampicin, Isoniazid and ethambutol; hence a change was implemented, but it didn’t work, and I gradually stopped walking due to immense Pain from the pelvis onwards. The doctors removed the TB bacteria-infected lymph node through the Surgery of lymphadenopathy.

Doctors changed my medicines to the highest degree antibiotics, and the expensive drugs cured MDR-Tb, but I need a walking stick to walk and can’t do most of the daily jobs, for which I have to depend on others.

I always wore a mask when I went out and carried a hand sanitizer in my handbag. You can never take enough precautions. I always use tissues to wipe my face because I was advised not to use a handkerchief as if you fold it in and use it, the infection on the other side would be enough to make me sick. My faithful troops have been disarmed or perhaps just turned off. I always wash my hands properly but in the freezing winters or when outside, I use hand sanitizer to control my infections.

Hippel-Lindau Syndrome

I have the rarest brain tumors – Hippel-Lindau syndrome. The Hippel-Lindau syndrome is so rare that between 1902 and 2013, approximately only 132 cases were reported. Few studies have reported leptomeningeal involvement in sporadic HB or HB associated with von Hippel­Lindau syndrome.

Because no case of de novo development of disseminated HB without previous Surgery has been reported, it is strongly suggested that the spillage and spread of tumor cells through the CSF space may be an origin of hemangioblastomatosis in patients with a genetic predisposition to the condition. Special care should be taken to avoid tumor cell spillage during Surgery.

A Ga-DOTANOC PET-CT based SSTR imaging confirmed that the floating lights in the patient’s brain are hemangioblastomas. With this, the true nature could be seen, and the diagnosis was confirmed. A Biopsy isn’t required for diagnosis as it may cause meningitis and blood loss, thereby cell spillage.

A fellow remarked after he saw the picture of the scan of my brain tumors, “You have more tumors in the brain than people have lice in hair.

There is no data on the surgeons’ competence and equipment used in the initial CNS surgery, so trying to determine if any incompetence caused the cell spread during my brain Surgery (craniotomy) in 2006 would be extremely difficult, if not impossible, to prove beyond a reasonable doubt. The fact that each tumor’s physiology is different and the number of cases is so small means that an accurate comparison of surgeons is impossible.

I have lost vision of my right eye for not being able to avail Radiation therapy at the right time because of financial reasons and also because I was diagnosed with an RCC ( kidney cancer) at the same time.

Trigeminal Neuralgia (TN)

Trigeminal Neuralgia (TN), also known as tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve). It is characterized by attacks of intense, stabbing Pain affecting the mouth, cheek, nose, and other areas on one side of the face. Sometimes there’s a constant dull aching or burning Pain. Both types of Pain can occur in the same individual, even at the same time. In some cases, the Pain can be excruciating and disabling. If untreated, Trigeminal Neuralgia can have a profound effect on a person’s quality of life.

In most cases, Trigeminal Neuralgia develops due to a blood vessel pressing against the trigeminal nerve, but sometimes no underlying cause can be identified (idiopathic). It can also be idiopathic due to the trigeminal nerve’s compression or can occur due to a known underlying cause such as a tumor or multiple sclerosis. Trigeminal Neuralgia can usually be managed through medications, Surgery or injections, or Stereotactic Radiosurgery.

Despite the striking strength of this pain, Trigeminal Neuralgia isn’t particularly well known. Most people never hear of it until they or a relative develop it.

Sometimes the Pain comes out of nowhere with no trigger at all. While a classic attack is sudden and sharp and then gone altogether, sometimes a low-grade ache or burning Pain will persist in its wake for an hour or more. In some patients, the constant aching, burning Pain is their initial complaint.

It was a warm October morning, and I was in a merry mood because of the approaching Durga Pujas. I always try to make the most of the time with my mother. The season is very inviting and makes us happy and less anxious. I sat down with a book determined to enjoy it thoroughly, but suddenly out of the blue, something zapped across my right eye. The jolt of lightning appeared repeatedly. It lasted for a few seconds, but it wasn’t easy to keep my right eye open. This continued for the next few days, but the Pain used to go as abruptly as it appeared. I went to the neuro-ophthalmologist thinking about my optic nerve tumor, but he said that the optic nerve doesn’t cause Pain and told me that it looks like trigeminal neuralgia and asked me to visit a neurologist immediately. The neurologist did his examination and confirmed Trigeminal Neuralgia and asked for an MRI. I underwent an MRI scan the next day, and it confirmed the diagnosis of Trigeminal Neuralgia.

Trigeminal Neuralgia is also known as “suicide disease since many patients with the condition sometimes choose to kill themselves to escape the Pain.

Hypoparathyroidism

I had a subtotal thyroidectomy from which emerged Hypoparathyroidism, a rare endocrine condition in which insufficient or inactive levels of parathyroid hormone (PTH) are produced by the four tiny parathyroid glands in your neck.

It may be due to a congenital, genetic, or autoimmune disorder that affects the function of the parathyroid glands. More commonly, it may occur as the temporary or permanent result of Surgery to the neck where there is removal or damage to the glands.

Insufficient parathyroid hormone leads to low Calcium levels in the blood, or hypocalcemia. It causes electrolyte imbalance and can be a life-threatening condition if untreated.

Why is Calcium so important?

Calcium is vital to life and affects every cell in the body. Most people know about teeth and nails connected with calcium, but its effects are on the whole body – nerves, muscles, and organs. It helps blood to clot and is important in energy production. Calcium is crucial to us, which is why the body has special mechanisms like the parathyroid glands to keep Calcium levels constant.

Treatment with Vitamin D analogs and Calcium supplements is not ideal and can lead to long term renal problems. Calcium levels fluctuate, but home Calcium testers are not available, so monitoring this condition can be challenging.

My recent problems are with swallowing and tackling. I have lost weight up to 10 kgs, and if I am forced to talk, I get Pain in my larynx area and my voice changes.

Parting Message:

Everywhere there is a glimmer of hope. Hand-holding fantasies are not there in my life; instead, everyone would like to swat me out of the way. Sometimes I feel the need for someone, not to take away or bear my Pain but just to support and care about me. From my childhood journey to middle-age, I became a warrior, but nobody stood by me. When I needed support from everyone, I rarely got it. Thanks to the internet and social media, few people don’t turn their faces when I need support. The rest peacefully ignore the agony of others.

There’s always a glimmer of hope. There is cancer, depression, but everyone can get out of it. The glory and brightness of life! When you arise in the morning, think of what a precious privilege it is to be alive to breathe, think, and enjoy being alive, so please don’t keep wringing your hands.

LEAVE A REPLY

Please enter your comment!
Please enter your name here