It’s not Lupus: It’s Lupus in India

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Time: 11:30 pm
Place: Emergency Department (ED) of a public hospital in Mumbai (average attendance ~ 550 patients per day).

A weary and teary mother wheels in her daughter ‘Asha’* who is in her early twenties. They have traversed 600 km from their remote district to Mumbai. Asha is pale, edematous, short of breath and her chest is full of crackles. She has not been able to lie flat since past 1 week secondary to difficulty breathing. She had been symptomatic for over a year with multiple complaints ranging from fatigue, fever, rashes to joint pains. The family initially consulted a local ‘tantrik’ (a faith healer who liberates possessed souls, see here for a notorious example). After that didn’t help much, she visited a local doctor who treated with her IV fluids for ‘the weakness’ and prescribed what she remembers as colorful tablets which were likely multivitamins. To note, ‘doctors’ in smaller villages and towns (and even cities) in India may or may not be qualified doctors, and even if qualified, may sometimes be homeopathic or ayurvedic doctors. It is difficult to comment as there is no formal record of any actual treatment administered. For her worsening joint pains, an orthopedic surgeon was consulted, who started her on steroids and NSAIDs. Asha had no recollection of any blood pressure measurement or urine examination being. At presentation in the ED, she is oliguric and is in pulmonary edema with a blood pressure of 180/90 mm Hg with a serum creatinine of 6.8 mg/dl. There are various striking features of ‘this’ presentation of lupus nephritis (LN) which is quite common in Indian population. It is well documented that there are substantial ethnic and socio-economic differences in presentation and outcome in Hispanics and African-American populations. Indian patients also have been reported to have lower eGFR and a higher chronicity index at presentation. Retrospective data from our institutional records (presented at ISNCON 2017) of 191 patients revealed a mean serum creatinine of 2.85 mg/d at presentation and 23% presented with rapidly progressive kidney failure requiring dialysis. However, apart from this inherently severe disease they suffer from, what is more disturbing is the fragmented care which they receive at various levels ranging from primary physicians to the specialists (rheumatologists, hematologists, dermatologists, orthopedics, gynecologists). Most of them don’t envision the disease in its entirety and even may be blissfully unaware of treatment prescribed by others (big thanks to poor documentation). This therapeutic muddle is amplified by the faith healers, whose treatment is mostly concealed by the patient.

Another aspect of this presentation is the delay in seeking medical care, which is massively influenced by the fact that lupus predominantly affects women. The gender bias in seeking medical care is multifaceted and is irrespective of economic constraints. It is deeply rooted in social prejudices such as a lower willingness of the family to spend their time and resources for the health of women, disadvantages in matrimonial prospects for a woman even after therapy, abandonment by ‘in laws’ after knowing about the chronic illness with difficulties in conception, etc. An interesting aside is that LN is not a favorable illness even for males, as it is often the last differential considered and hence often missed or diagnosed late.

Asha remained dialysis dependent for a week, received pulse steroids while her kidney biopsy confirmed class IV LN with crescents. Now, the biggest dilemma was that the available evidence for therapeutic options in such severe patients is scarce and most of the studies have excluded patients like Asha. Given that there is some evidence supporting ethnic differences in responses to induction immunosuppression, it is imperative that we opt our choices based on evidence from Indian patients. Also, not to forget that Asha’s body surface area (like most Indians) was 1.1m2 and the safer “low dose cyclophosphamide regimen” is equivalent to “high dose” (0.5- 1g/m2) for her.

Asha’s father couldn’t accompany her as he was himself afflicted with pulmonary tuberculosis. This alerted me to the rampant risk of infections which will be compounded by my immunosuppression. In my effort to salvage the kidney, I may end up losing the patient to infection. This is another reason why we need to have indigenous treatment guidelines which can prevent over-immunosuppression in this infection prone population.

After induction with pulse cyclophosphamide and steroids, Asha’s eGFR had stabilized and she was dialysis independent at discharge. However, managing her immunosuppression is very difficult as long-term therapy with MMF is (economically) out of her reach. Drug non-adherence was an independent predictor of progression to ESKD in our data. Monetary constraints topped the causes, others being- stopping drugs after feeling better, being fed up with long years of therapy, and social taboo. One young girl was so embarrassed to take medications in college that she regularly skipped afternoon doses. Some of them get confused with the complicated prescription and take vitamins but miss immunosuppressives. One important factor was the lack of universal availability of drugs and physicians. Asha’s district has no access to immunosuppressives and specialists within 500 km, hence the travel expenses for every visit are added to the cost of treatment.

Our experience of setting up a Lupus Nephritis Clinic 

Our nephrology unit caters to patients from all spheres of nephrology (general, pediatric, critical care, and transplantation). Initially, these patients were equally divided into five clinics over the week, each with an attendance of over 100 patients. With a vision to serve patients like Asha better, we started a dedicated lupus clinic every month with about 50-60 patients per clinic. This has improved patient satisfaction as their waiting time is reduced. Committed time for counseling on specific issues like the importance of long-term adherence, pregnancy, and contraception has helped us move from disease-centric to patient-valued goal directed care. In addition, group counseling sessions have proved quite effective and time efficient. Apart from enabling us to provide better care in the resource-limited setting, the specific lupus clinic model has also helped in streamlining and analyzing data to generate much needed information on indigenous populations of india to help fine-tune our practice patterns. 

Asha is now regularly following up in the lupus clinic and is in partial remission. Her initial desolation has decreased after meeting other lupus survivors in the clinic. She now also encourages other patients for seeking timely and regular care.

Divya Bajpai
Nephrologist
KEM Hospital
Mumbai, India
NSMC Intern Class of 2018

*Note- This is a fictionalized story based upon a composite of many real patients; names and other information is altered. The name ‘Asha’ means ‘Hope’ in Hindi.

2 comments

  1. Dear Dr Bajpai,

    Thanks for a terrific overview of some of the few challenges that as providers we had/ and continue to face in India. In the US too, the underprivileged population is no different. The cost of cytoxan in close to 500 dollars a month out of pocket. If the pts are un-insured, there is simply no way to get treatment. Post transplant care too becomes adversely affected since medicare drops off after 3 yrs, and younger individuals have found it hard to find jobs. The recent health care change has helped some of these patients too.
    Kudos to you and your team to be able to have the vision and empathy to provide your excellent service to the patients that need the most. Good luck !!

    Regards

    Gautam Phadke

  2. Dear Dr Bajpai,

    Thanks for a terrific overview of some of the few challenges that as providers we had/ and continue to face in India. In the US too, the underprivileged population is no different. The cost of cytoxan in close to 500 dollars a month out of pocket. If the pts are un-insured, there is simply no way to get treatment. Post transplant care too becomes adversely affected since medicare drops off after 3 yrs, and younger individuals have found it hard to find jobs. The recent health care change has helped some of these patients too.
    Kudos to you and your team to be able to have the vision and empathy to provide your excellent service to the patients that need the most. Good luck !!

    Regards

    Gautam Phadke

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