Stevens-Johnson Syndrome & The Eye: A Journey of Resilience and Progress

SJS Awareness Day: Shining a Light on a Rare and Devastating Disease

Today is SJS Awareness Day! – the 18th of August, and this entire month serves to create awareness regarding a disease that is relatively rare but causes devastating damage in the affected individual.

The Acute Stage: A Battle for Survival

Stevens-Johnson Syndrome (SJS) is a life-threatening condition that most commonly occurs as a reaction to medication, and sometimes to infections. The first few weeks are an ordeal, with survival as the primary focus. The reaction causes the shedding of skin both externally and internally, including the delicate conjunctiva that covers the eyeball. Under such circumstances, in most instances in the ICU of the hospital, accessibility to and evaluation by an ophthalmologist or ocular surface specialist is highly challenging, though changing.

The Ocular Aftermath: An Ongoing Struggle

Only after the vitals stabilize does the focus shift to the eyes. Once life is saved and the patient walks out of the hospital, triumphing over the disease by surviving, the ocular sequelae and its resultant morbidity raise their ugly head. The effects range from mild dryness to corneal blindness, and the ocular manifestations are myriad. Patients often experience an inability to tolerate light, a constant foreign body sensation akin to having a fistful of sand in the eye, and the need to instill lubricating drops as frequently as every 10 minutes. These symptoms can be crippling. Despite having good vision, these symptoms prevent the affected from opening their eyes to see. Losing vision is heart-wrenchingly painful—beyond words—but to have vision and not be able to use it is beyond comprehension.

The Unseen Burden: The Impact on Patients and Families

With the physical troubles come frustration, loss of morale, and interruption in education and career, as most affected individuals are young and in the prime of their lives. The psycho-socio-somatic-economic impact of the disease is indescribable. Life turns upside down for the affected and their family. Initially, there is a lot of sympathy, which slowly fades into the background given the chronicity of the disease. It then becomes a battle fought primarily by the affected individual and their closest kin. One parent or one sibling often takes on the responsibility of providing never-ending support—whether it be multiple clinic visits, aiding in the instillation of medications, or helping the affected ambulate—sometimes inside the confines of home if the resultant blindness is significant.

A Turning Point: The Evolution of SJS Management

Unfortunately, the medical field did not have too many options to improve comfort or vision until the late 90s and the turn of the millennium. This is when my journey with SJS began 21 years ago- with Dr Bhaskar Srinivasan. With constant guidance from mentors and encouragement from colleagues, we knew we had to make a change and address the unmet needs. And thus began the journey...

This with contributions from several EXPERTS across the country and globe who attempted and succeeded in addressing several factors that would improve not just vision but also comfort.

My Perspective: Two Decades of Fighting for Better Outcomes

Today, we can capitalize on the windows of opportunity the course of the disease provides us with to enhance the quality of life and prevent a further downhill course. Today, we are able to provide options for corneal blindness through artificial corneal transplants. We are also a step ahead in understanding the possibility of preventing the disease someday in the future. We still have a lot of ground to cover—but we shall. SJS is one of the most challenging conditions that defies what we refer to as the natural course of a disease.

Honoring the Heroes

Having been associated with the management of the ocular sequelae of SJS for more than two decades now, I could not resist penning this down on this day. This is an ode to all the heroes in the medical fraternity who have contributed in a big or small way to the betterment of treatment options—the scientists, researchers, geneticists, clinicians, optometrists, paramedical staff, and specialists from several allied fields who work as a team.

Of course, the true heroes are our patients—who endure the wrath of this disease. They suffer silently, but rather than resigning to fate, they become resilient and strong, and with the help of now available management options, several of them have soared in their respective fields.

A Call for Awareness

To me, it is a huge sense of pride to see the tiny tots now grown up into young confident adults pursuing their careers of choice, the teens in well-placed jobs, and the middle-aged satisfactorily retired after availing of the various medical measures that have been available. They still face hurdles, have their moments of despair, but have risen to the challenges posed by the disease.

More than a scientific piece, this was intended to be an empathetic emote—to create public awareness about the disease, its effects, and the treatment, so that every individual affected by the condition avails of the best possible management tailored to his or her needs.

Dr Geetha Iyer, Medical Director CODE Eye Care in T Nagar, Chennai - alongside Dr Bhaskar Srinivasan - is a world renowned cornea and ocular surface disease expert.