The Double Danger

Dr Able Lawrence, professor and head of clinical immunology and rheumatology at Lucknow’s Sanjay Gandhi Post Graduate Institute of Medical Sciences, has heard at least a few young husbands say “marne do, main doosri shaadi kar loonga” (let her die, I will marry someone else) when their young wives were diagnosed with severe lupus, which typically requires expensive and continuous treatments to keep them alive.

Lupus, an autoimmune disease that mostly affects young women and, in severe cases, several organs, often puts the patient in “double danger”, Lawrence says. The first of these dangers is contracting a disease that is getting more frequently diagnosed now than before thanks to the progress medicine is making in rheumatological and autoimmune diseases. The second is the fact that the patients are women, who are still seen in some parts of Indian society as expendable. Lupus, of which the most common is systemic lupus erythematosus (SLE), according to American medical authorities, is more common in women than men by nearly a 10-to-1 ratio. In India, it is estimated to be 3.2 to 60 cases per 100,000. But the rheumatologists Open spoke to say the incidence is higher than the official estimates suggest, making it not as rare as it is made out to be. The disease predominantly affects women in their reproductive years.

For his part, Lawrence, who says he comes across lupus patients often, asserts that delayed diagnosis leads to deaths and disability. He says that a section of policymakers tends to underestimate the true burden of disease because of its so-called “low prevalence”—an unfortunate outcome of improper diagnosis —in India, despite its high incidence comparable to anywhere else in the world.

As a result, notwithstanding high mortality, the extent of prevalence appears low. “Also, we do not have many celebrities speaking up for this issue, and female celebrities with lupus keep silent about the disease,” says Lawrence, explaining why lupus isn’t getting as much attention as other autoimmune diseases and several cancers that are less prevalent. In fact, he refers to news reports that Congress leader Shashi Tharoor’s late wife Sunanda had lupus. “I know the rheumatologist who had treated her [Sunanda]. She probably died from NPSLE and it leaves nothing for the pathologist in autopsy,” Lawrence claims. NPSLE, or neuropsychiatric systemic lupus erythematosus, refers to neurological and psychiatric conditions caused by SLE. Reports from the time of her death in January 2014 had also suggested that Sunanda Tharoor had lupus.

Dr Padmanabha Shenoy, a renowned rheumatologist who is the founder of the CARE (Centre for Arthritis & Rheumatism Excellence) Hospital in Kochi and had earlier worked with the Amrita Institute of Medical Sciences in the city, says that several tests, including the antinuclear antibody (ANA) test, to confirm lupus were not common in India until 15 to 20 years ago. This meant that many of those who contracted the debilitating disease either died without getting diagnosed or were misdiagnosed. Shenoy, who has spent time as a student as well as a practitioner in several parts of India, notes that Andhra Pradesh is considered a hotbed of lupus cases where, by several official and unofficial estimates, many patients still go undiagnosed and untreated and die young. Dr Shenoy attributes delayed diagnosis of lupus cases to the slow development of rheumatology as a discipline worldwide compared with others, perhaps because of a lack of awareness and incentive for investing in this segment—the momentum only picked up over the past few decades.

But then things change only slowly in most parts of India, cautions Dr Chengappa KG, a senior rheumatologist and clinical immunologist based in Mysuru who had earlier studied and taught at Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) in Puducherry. Speaking about the disease which gets deadly in advanced cases, he notes, “In India, no disease is rare because the numbers are often high. It is true that lupus is not as common as diabetes or TB, but in my seven years in JIPMER we have diagnosed more than 3,000 cases, sometimes five to seven people in a week. That is not rare.”

Chengappa adds that the tendency to label it rare could perhaps also be because it affects women more, who in many parts of the country are not seen as equal to men. “The problem is also that families aren’t often ready for the long journey because this disease needs continuous treatment, often involving expensive medicines, and the burden is there for the next 10 to 20 years,” he points out, adding that the absence of rheumatology departments or rheumatologists in many government hospitals adds to the woes of those suffering from lupus, be it diagnosed or undiagnosed. “There are dermatologists who dismiss it as rash or an allergy due to detergents… in Karnataka, there are no government hospitals that have rheumatology practice. Authorities also shun our efforts to do a free OPD. Non-rheumatologists tell patients that it is a condition that has no treatment without referring them to specialists like us for specific treatment,” Chengappa states. He adds that although generic medicines for lupus treatment are made in India, many of them tend to be expensive, contributing to the perceived burden of managing the disease. Mycophenolate mofetil is one such medicine besides infusions, some of which are costly.

I met Vachasamrita S at her home in Kochi several months ago when she narrated the “long journey of her life” as a 26-year-old who was just 13 when she was diagnosed with lupus and her parents would do anything to see her get well. Her movements were slow, but she had a bright smile as she delved into her illness, along with what she does as cofounder of Lupus Trust India, which, launched in 2018, helps lupus patients across socio-economic backgrounds avail themselves of medical assistance and spread awareness about the disease. She also cofounded the Kochi Book Reading Club and helps her father at EduBrisk, a digital learning platform he launched.

Vachasamrita says she dug deeper and deeper into the disease and consulted doctors from various disciplines, including palliative care, because of her suffering at a time when other teenagers were busy partying, having sleepovers and making ambitious plans for life. “I have seen the monster side of lupus,” she tells me via a video call recently from her hospital bed after having contracted over the years chickenpox, shingles and meningitis. She says that the sense of helplessness transformed her into a bio-nerd, prompting her to learn more about lupus, only to discover the glaring lack of awareness about the disease in India. With the help of doctors, lupus victims, officials and others, she became a campaigner. Her family remained highly supportive and she took part in a documentary on what it is like to live with lupus made by a team of doctors on the YouTube channel Apothekaryam-Doctors Unplugged.

While the young lady talks about the “monster side”, there are others who have experienced mild versions. For instance, many years ago, CNN reported Lady Gaga as telling show host Larry King that she had tested “borderline positive” for lupus. She went on to add that she didn’t have lupus, however, due to the varying symptoms of the disease. The same report rightly said that no two cases are alike. Severity varies from mild discomfort to death and symptoms vary from swelling and rashes to joint pain and hair loss to inflammation of the kidneys, the heart, lungs, blood vessels and the brain.

In her case, Vachasamrita was referred to an Ayurvedic practitioner by none other than a gastroenterologist, a man of modern medicine, and the so-called alternative medicine did great harm to her body and health, she recalls, adding that the progress of the disease couldn’t be stopped early on. However, due to the persistence of her family, she says, she decided to turn to lupus advocacy. She wrote on social media that her next goal—after having collaborated with doctors and researchers in India—is to work with organisations pursuing genetic screening nationally and internationally. “As of now, early diagnosis in lupus is hampered by a shortage of testing facilities, lack of proper access to the specialist and financial problems,” she points out, reeling out details of freight charges for medicines earlier when her parents had to import them from places such as Switzerland and Turkey. She describes the disease as a “surreptitious, clandestine, shady mimicker”. SLE is often called the “great imitator” because it gets misdiagnosed thanks to its broad and variable symptoms.

Doctors and patients across the country whom Open spoke to, as well as research papers from around the world, dwell at length on how an illness turns into debt, as seen in lupus and other chronic conditions. Known generally as catastrophic health expenditure, the problem is growing in India as private hospitals and investors, including many new entrants, chase maximum profit while public health infrastructure is neglected by the authorities.

Meanwhile, Thiruvananthapuram-based Dr MR Rajagopal, a Padma Shri recipient and physician often referred to as the “father of Indian palliative care,” speaks animatedly about the life and work of Vachasamrita. “The girl is a phenomenon,” he says, stating that she has been through a sea of suffering. The veteran doctor and author, who has been the subject of a documentary, notes that healthcare needs to be integrated with pain and trauma care to ensure affordable and better treatment for health conditions such as lupus. Rajagopal, founder of Pallium India, who played a pivotal role in the creation of the National Program for Palliative Care (NPPC), says serious health-related suffering can be fought only through such a multi-disciplinary action, primarily because suffering is not just physical, but also social and mental. As he sees it, “It has to be a multi-stakeholder activity.”