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Payel Bhattacharya (Hippel-Lindau Syndrome): Fighting Multiple Battles Together

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, and various other problems, which may exhaust my energy. With the winter months approaching, a certain bored feeling sets in, let alone the loneliness.

I had a liver transplant due to several liver tumours that could not be taken out individually, causing excruciatingPaindue to frequent haemorrhages in 2008. The most extensive 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, I 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?

I have come across people who have confused it withImmunotherapy.Immunotherapyis a type ofCancer Treatmentthat boosts the body's natural defences to fight cancer. It uses substances the body makes or creates in a laboratory that makes our immune system find and destroy cancer cells.

It means the medication can interfere with the body's processes that prevent infection, which 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 take some of these medications, you will fall ill every time you pass by someone with the sniffles and the big stuff, like flu or tuberculosis. Will you 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 a pandemic is going on and you are ill-fated, the army to protect you is disarmed. However, it is essential to understand the outcomes of living with a compromised immune system to protect yourself.

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

That could mean catching every bug your family member brings home from work or possibly 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 mould). Oh, and you know all of those recent H1N1 outbreaks? You could always be at risk. Immunosuppressants also leave you at risk for sporadic and difficult-to-treat infections, moulds, fungal pneumonia, and certain types ofLymphoma.

Consistently 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 critical 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 aeroplane 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).

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

They must run to the emergency room to see a healthcare provider if there is a high fever. Still, I got a mysterious fever when I got to the emergency room at 2 am because I was getting tetany and needed aCalciumdrip.

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

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 needleBiopsybefore taking the tumour out. I travelled 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 cm, just a little below the threshold. TheSurgerywas confirmed with a doctor at FMRI, but he still said he couldn't performSurgerywith an infection in the body.

By God's grace, I thought of calling another experienced doctor 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 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 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 nodeBiopsyshowed TB infection(AFB+). After more than four months of being on anti-Tb-treatment, I worsened my lung condition. HAIN test confirmed that the bacteria is resistant to Rifampicin, Isoniazid and ethambutol; hence, a change was implemented, but it didn't work. I gradually stopped walking due to immensePainfrom the pelvis. The doctors removed the TB bacteria-infected lymph node through theSurgeryof lymphadenopathy.

Doctors changed my medicines to the highest degree of 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 sanitiser 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 correctly, but I use hand sanitiser to control my infections in the freezing winters or when outside.

Hippel-Lindau Syndrome

I have the rarest brain tumour - 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 de novo development of disseminated HB without previousSurgeryhas been reported, it is strongly suggested that the spillage and spread of tumour 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 tumour cell spillage duringSurgery.

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. ABiopsyisn't required for diagnosis as it may cause meningitis and blood loss, thereby cell spillage.

A fellow remarked after seeing the picture of my brain tumour scan, "You have more tumours 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 determining if any incompetence caused the cell spread during my brainSurgery(craniotomy) in 2006 would be extremely difficult, if not impossible, to prove beyond a reasonable doubt. The fact that each tumour'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 in my right eye for not being able to availof Radiation therapyat the right time because of financial reasons and because I was diagnosed with an RCC ( kidney cancer) at the same time.

Trigeminal Neuralgia (TN)

Trigeminal Neuralgia (TN), or tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve). It is characterized by attacks of intense stabbingPainaffecting the mouth, cheek, nose, and other areas on one side of the face. Sometimes there's a constant dull aching or burningPain. Both types ofPaincan occur in the same individual, even simultaneously. In some cases, thePaincan be excruciating and disabling. If untreated, Trigeminal Neuralgia can profoundly affect 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 compression or can occur due to a known underlying cause such as a tumour or multiple sclerosis. Trigeminal Neuralgia can usually be managed through medications,Surgeryor injections, orStereotactic 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 thePaincomes 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 burningPainwill persist in its wake for an hour or more. In some patients, the constant aching, burningPainis 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, something zapped across my right eye. The jolt of lightning appeared repeatedly. It lasted for a few seconds, but keeping my right eye open wasn't easy. This continued for the next few days, but thePainused to go as abruptly as it appeared. I went to the neuro-ophthalmologist, thinking about my optic nerve tumour. Still, he said that the optic nerve didn't causepainand told me that it looked like trigeminal neuralgia and asked me to visit a neurologist immediately. The neurologist did his examination and confirmed Trigeminal Neuralgia, and asked for anMRI. I underwent anMRIscan the next day, confirming the diagnosis of Trigeminal Neuralgia.

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

Hypoparathyroidism

I had a subtotal thyroidectomy from which Hypoparathyroidism emerged, a rare endocrine condition in which insufficient or inactive parathyroid hormone (PTH) levels 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 lowblood calciumlevels or hypocalcemia. It causes electrolyte imbalance and can be a life-threatening condition if untreated.

Why isCalciumso essential?

Calcium is vital to life and affects every cell in the body. Most people know about teeth and nails connected with calcium, butCalciumfects are on the whole body - nerves, muscles, and organs. It helps blood to clot and is essential in energy production.Calciumto us, so the body has unique mechanisms like the parathyroid glands to keepCalciumlevels constant.

Treatment withVitamin Danalogues andCalciumsupplements is not ideal and can lead to long-term renal problems.Calciumlevels fluctuate, but homeCalciumtesters are unavailable, 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 getPainin 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; everyone would like to swat me out of the way. Sometimes I need someone not to take away or bear myPainbut to support and care about me. I became a warrior from my childhood journey to middle age, 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, and 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 breathe, think, and enjoy being alive, so please don't keep wringing your hands.

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