Burn Sunnybrook Magazine - Spring 2018

Network of experts gives patients with rare skin disorder a fighting chance

Sonia Whyte-Croasdaile, former TENS patient

Sonia Whyte-Croasdaile, pictured above, suffered from a rare toxic epidermal disease, which affects every surface of the body and requires extensive treatment from a multi-disciplinary team of specialists.

On a wall of the intensive-care burn unit where Sonia Whyte-Croasdaile was a patient for six weeks – her skin sloughing off her face and body – was a full-size photo of Sonia taken when she was healthy.

“I had to bring in a picture, so they could see who she was,” says her husband, Joseph Croasdaile.

Sonia’s illness began with flu-like symptoms. Less than a week later, her body was fully covered with burn blisters.

The results of a skin biopsy confirmed the suspected diagnosis. Sonia was one of the two in a million people struck by the little-known but life-threatening Stevens-Johnson Syndrome (SJS), a rare condition, often triggered by an adverse drug reaction.

“The term SJS is used for several similar diseases,” explains Dr. Neil Shear, head of Dermatology at Sunnybrook. “Some are viral-induced and mostly involve the eyes, mouth and genitals. Some are drug-induced and can involve the body skin as well as the mouth, eyes and genitals. SJS in any of its manifestations is a horrific disease.”

When the disease becomes even more severe, as it did in Sonia’s case, with a mortality rate as high as 40 per cent, SJS is referred to as toxic epidermal necrolysis spectrum (TENS).

According to Dr. Shear, early diagnosis and treatment by an interdisciplinary medical team, “people with experience who understand the nuances of the disease,” is key for a positive outcome.

The challenge in treating TENS is that every surface of the body is affected, including all the mucous membranes – mouth, throat, airways, eyes, nose, genitals. It can also affect the kidneys and cardiovascular system.

At Sunnybrook, patients with TENS are treated in the Ross Tilley Burn Centre, where the multidisciplinary medical team is led by Dr. Shear and the centre’s medical director Dr. Marc Jeschke.

Dr. Neil Shear

Dr. Neil Shear, head of Dermatology at Sunnybrook, developed the first Canadian protocol for dealing with the often fatal disease SJS/TENS.

The Sunnybrook team includes not only dermatologists and burn specialists, but also ophthalmologists, respirologists, gynaecologists, urologists, ear, nose and throat specialists and plastic surgeons.

Dr. Shear and his team of specialists have developed the Sunnybrook Protocol for SJS/TENS. The first Canadian protocol for dealing with the disease, it’s been published in the Journal of Cutaneous Medicine and Surgery.

“We would like to share our management, experience, and present our treatment protocol to help guide treatment of these complex patients, not only in Canada but also worldwide,” Dr. Shear and his colleagues explained in the protocol document.

The protocol is designed to prevent further decline, heal debilitating symptoms and provide supportive care.

Recommendations for supportive care include increasing the temperature in the patient’s room to 30-32°C, applying white soft paraffin to the lips every two hours and a topical steroid four times daily, along with oral rinses, including lidocaine for pain.

Fluids are replaced intravenously, along with pain medication, while appropriate nutrition may be provided through a feeding tube through the nose.

Involving an ophthalmologist and a gynaecologist or urologist as soon as the diagnosis is made is also important.

According to the protocol, daily follow-up by an ophthalmologist is essential. Eyes are treated with artificial tears, lubrication, topical antibiotics and steroids and, when necessary, daily sweeping of the inside of the eyelid with glass rods to prevent it from sticking to the eye itself.

“It is a miserable thing to see. I can’t overstate how horrible this disease is,” says Dr. Shear. “It’s a burn from the inside out. It looks like a burn, but it’s the immune system causing it, as opposed to high temperature.

“What happens is the immune system gets fired up and starts attacking the outer layers of skin, the eyes, genitals, throat, lips. It’s like a flesh-eating disease with cell digestive enzymes eating the skin.”

Of the 300 patients treated every year at the Ross Tilley Burn Centre – the largest burn centre in Canada – up to 10 are admitted with SJS/TENS. These patients spend about four to six weeks at the centre.

“It’s a very challenging patient population,” says Dr. Jeschke. “But for the patients who do survive, healing is quite good. The organs recover, and [there is no] scarring of the skin.”

Unlike temperature burns, SJS/TENS affects only the epidermis, the upper layer of the skin. The dermis, or structure of the skin, which is damaged with third-degree temperature burns, remains intact with SJS/TENS.

Sonia’s skin is now smooth and luminous. “The blisters dried up miraculously and disappeared,” says Sonia, now 50 years old. But seven years after her diagnosis, she is still bothered by bright lights and wears dark glasses day and night. “The normal feeling in my eyes is like when wind blows grit in your eyes on a hot summer day,” she explains.

She also lacks energy and can no longer eat the spicy food she used to enjoy because it burns her mouth and throat. “Before, she used to eat the hottest peppers,” recalls Joseph. “Now we give away most of the ones we grow.”

The scars of TENS, according to Dr. Shear, are actually on the inside. “Patients rebound but become very anxious. Almost always, survivors suffer from post-traumatic stress disorder, and it’s also traumatic for the family.”

He adds, “There is a tremendous sense of fear of future illnesses and the potentially frightening prospect of the unknown risks from any drug. This affects the survivor and their loved ones.”

For Sonia, rebounding from the disease has taken a positive direction. With encouragement from Dr. Shear, she has become a crusader for SJS/TENS awareness and support for patients.

“I want to mobilize the survivors and bring hope to SJS/TENS patients,” says Sonia. “They can heal. They can be beautiful again.”

Are you at risk?

Stevens-Johnson Syndrome (SJS) is a serious disease that causes blistering and sloughing of the skin and mucous membranes, affecting all body surfaces. It can also quickly develop into toxic epidermal necrolysis spectrum, or TENS, which can be fatal if not treated promptly in a burn unit by an interdisciplinary medical team experienced in treating the disease.

The mortality rate is 30 to 40 per cent.

The disease is an immune reaction that, in some cases, is triggered by certain drugs interacting with specific genetic markers. People can be screened for these genetic markers before they’re prescribed the drugs known to trigger the disease. One of these drugs is allopurinol, often prescribed for gout.

Certain populations are particularly prone to SJS/TENS, according to Dr. Neil Shear, head of Dermatology at Sunnybrook. Those who are most at risk are people from South Asia, southern Pakistan, southern China and the Philippines.

Even having one parent from those areas increases risk. People from Japan, Korea and northern China are not in the high-risk group.

Being in the higher-risk group doesn’t mean you have the known genes that can interact with drugs that may trigger SJS/TENS, says
Dr. Shear, nor does it mean that if you have the gene, you would develop the disease if you take the drugs.

However, his recommendation is that everyone in those populations at risk should be screened.

“There are genetic aspects to this disease that we don’t know about yet,” he points out. “It’s very tough to predict.” He recommends using alternative drugs, even if the patient is not in the high-risk group. But in some cases, the trigger is never determined.

Patients should also be aware of the early symptoms of the disease, including rash, eye irritation and flu-like symptoms, especially if they have started a new drug.

All photography by Kevin Van Paassen

About the author

Judith Gerstel