A disorder in disguise

In SS,the bodys immune system white blood cells attacks its own secretory glands and tissues,causing the classical symptoms of dryness of eyes and mouth. Severe fatigue is another hallmark symptom of SS.

DIFFICULT DIAGNOSIS

While nine out of 10 SS patients are women in the age group of 20-40,the delay in its diagnosis averages six-eight years.

The symptoms of SS are subtle and non-specific in the beginning and a patient,therefore,visits a number of specialists eye doctors,dentists and physicians, says Dr Rohini Handa,president,Indian Rheumatology Association.

The problem is not the prevalence of SS but its underdiagnosis underlines Dr Sapan C Pandya,a well-known rheumatologist based in Ahmedabad.

Ahmedabad-based Kirtida Oza,49,says: I struggled through myriad physical distresses like debilitating fatigue,respiratory problems,joint pains,skin rashes,dental problems,dryness of mouth and eyes,for about 7-8 years before getting diagnosed.

What was more frustrating was to look well yet feel so terrible,and to be told by doctors that I was imagining my illness.

Dr Pandya admits there is an underlying lack of awareness amongst the medical fraternity about the syndrome. Our undergraduate medical training in this respect is not up to the mark.

Rheumatology,the specialty which deals with SS,is not very well taught in the curriculum, agrees Dr Handa,who is also a Senior Consultant at Indraprastha Apollo Hospital,New Delhi.

Oza began looking for a name for her imaginary illness herself. After reading an article on Sjogrens Syndrome sourced from a medical library,I was convinced I had SS and sought to confirm it from a rheumatologist in Mumbai.

She says she was finally diagnosed with SS in 2002 when she was headed for an assignment to teach at a college in USA. There,she learnt about the Sjögrens Syndrome Foundation SSF that is working for empowering SS patients for over 25 years. She returned to India in 2003,with the desire to learn to manage her own illness as well as to extend her learning to other patients in India. In July 2006,she co-founded a support group for SS patients in Ahmedabad one of its kind in India with another patient friend,Purvi Doshi.

Today,Sjögrens India SI works for empowering patients in India and abroad,by providing education and counseling support,public awareness and networking with healthcare professionals.

A SPECTRUM OF SYMPTOMS

Smita Sharma name changed,60,of South Delhi,was told she had Rheumatoid Arthritis RA and Systemic Lupus Erythematosus,another auto-immune disorder,for 13 years. It started with joint pain in 1993; then there was dryness of eyes and vagina; mouth ulcers and dry skin followed.

Since the symptoms were throughout the body,I could never really explain them properly to the doctors. My eyes were itchy. I would often feel that something was stuck in my throat. I was visiting an ophthalmologist,dentist and a rheumatologist.

In 2006,it was her dentist Sharma had decaying teeth due to insufficient secretion of saliva who suspected Sjogrens Syndrome. A few tests later,Sharma says,it turned out that she had had primary Sjogrens all these years,

Doshi,39,of Ahmedabad,says she would often get rashes on her legs since she was 15. She also suffered from oral ulcers and recurrent mumps.

In 2004 when I was in the US for my brothers wedding I developed high fever and swelling on my right knee. My sister-in-law,a rheumatologist,suspected SS and prescribed some tests which I got done once I was back in India.

WIDESPREAD EFFECTS

Patients affected by SS are more prone to pneumonia,liver failure,kidney failure and other internal organ damage. Dr Handa says: Women may suffer from dyspareunia painful intercourse because of dry vagina. A small percentage of patients can even develop cancer of the lymph glands.

MANAGING THE ILLNESS

Sharma says: I hadnt ever heard of Sjögrens Syndrome but having a name for my illness was indeed a relief. When my mouth goes dry,I know the secretory glands are acting up,and I reach out for my medicines. The dosage prescribed is in proportion to the dryness.

Her mouth ulcers have gone and she avoids foods like almonds and cashew.

Doshi says: Now nobody tells me that its all in my head. Of course,I have my bad days and good days,but I am at peace. I am also suffering from bipolar depression,but I have been trained to manage my dosage according to my mood swings.

Oza,too,always carries a water bottle and eye drops whereever she goes.

Getting Better

Oza says while statistical data on patients in India is not available,the number of people attending the Sjogren Indias programme has risen from 20 in 2006 to over a 100 from all over Gujarat and other states in 2010.

Dr Pandya says there is an increased recognition of this chronic disease now. Earlier this week,I saw my 149th SS patient since 2004.

There is a need to make people and the medical fraternity aware about SS.

Dr Handa adds: At present,I have around a dozen patients of SS. There are effective treatments but the need is for working towards better patient-doctor awareness.

WORLDWIDE

Originally listed in the National Organization for Rare Disorders registry,recent population studies in the US and in Britain have documented Sjögrens Syndrome as the second-most common autoimmune rheumatic disease,behind rheumatoid arthritis.

The disorder was first described in 1892 in a 42-year-old man and called Mikuliczs syndrome. But the diagnosis fell into disuse because it seemed to encompass so many conditions. The syndrome was resurrected in 1933 by Henrik Sjögren,a Swedish ophthalmologist,who described 19 women suffering from dry mouth and dry eyes.