The 'Skinny' Over Ketek and Stevens Johnson Syndrome

Denver, COIt's a condition that would remind you of the worst horror movie. Bleeding from the eyes. Skin sloughing from flesh. Like a severe burn from a fire, but there is no burn, or fire. This is Stevens Johnson Syndrome (SJS) and its more severe cousin, Toxic Epidermal Necrolysis (TENS). And it has been linked to various drugs, including Ketek.

Although considered rare by some, SJS and TENS can be triggered by an infection. However, it is more likely to be triggered by a reaction to certain drugs, and Ketek is thought to be one of them after a Canadian woman developed TENS after taking Ketek in 2007. It should be noted that Ketek is only suspected in the case, given that the woman had a history of allergic reactions to penicillin, as well as the drug class to which Ketek belongs.

Nonetheless, the suspicion of a causal link is there, which only adds to the dark cloud that already hangs over Ketek due to concerns over liver damage, and questions which surround pre-market clinical trials.

There is no question, however that SJS and TENS are a nightmare.

Jean Farrell is a travel agent from Denver whose daughter's story will rip out your heart. Julie was only 11 months when she was treated with Phenobarbital, a widely used anti-convulsion drug. Wee Julie was prone to epileptic seizures, and had been on the drug about two weeks before her mother noticed Julie had a swollen eye.

Then both eyes swelled shut, followed by blisters forming on the child's lips. Pus-filled blisters were forming on the surface of her eardrums. Within a day, the baby had developed blisters inside her mouth, and red spots on her body were growing, and had started ulcerating. The sores inside little Julie's mouth were so bad, she couldn't swallow.

Her condition worsened further. The child's face took on a look that her mother could only describe as 'deep-fried.' Fluid was building up in her lungs, and the child required a body wrap in bandages, much like a burn patient would be treated.

On Julie's first birthday, doctors had to pry open her blistered eyes with glass rods. The skin immediately beneath her eyelids sloughed off, and there was blood everywhere. In spite of morphine administered carefully for pain, the child was suffering horribly.

Happy first birthday, little Julie.

The good news is that the child survived. The tragedy is that Julie sustained permanent damage. She remains blind in one eye, with limited vision in the other. Her one good eye is light sensitive, so she always has to wear sunglasses. The child may have sustained permanent liver damage as well.

Such is the horror of SJS, and TENS—conditions which basically turn the body against its own skin, effectively burning the skin from the inside out.

"We usually distinguish between three forms," said Dr. Bernard Cohen, MD, of Johns Hopkins Hospital in Baltimore, "A milder form called Erythema Multiforme Minor, or EM, Stevens Johnson Syndrome, SJS, and TENS, or Toxic Epidermal Necrolysis Syndrome, the most severe form. With EM, which can be recurrent, there will be lesions on the distal extremities (lower legs and arms) and in the mouth; there is little mucous membrane involvement, however. With SJS, which is usually not recurrent, there are usually blistering ulcerations of the cornea, mouth, rectum, genitalia, skin, and urethra, usually accompanied by a high fever and generalized weakness. TENS involves the entire skin and mucous membrane; the skin literally sloughs off of the person's body."

And while the acute term of an SJS reaction can last for a month, the effects can last a lifetime. Dr. Cohen notes that SJS victims will sometimes sustain permanent lung and esophageal damage.

For survivors of the more serious TENS, there is often newfound heat sensitivity, and survivors lose the ability to perspire.

And there are those who believe that SJS and TENS are not as rare as some would lead you to believe. The mother of little Julie Farrell founded the SJS Foundation, and notes that since setting up the Foundation's website they have been contacted by thousands. Farrell notes they were learning of 15 new cases a week, and that's just over the Internet. She wonders at the number of cases of which they are not aware, from people who do not have access online.

"We've been contacted by people from as far away as China and South Africa. This disease is not rare," Farrell suggests, "it's on the rise."

Those who suspect a link between Ketek and SJS/TENS will be keeping an eye on that potential causal relationship with regard to a possible rise in the incident rate, as well. While the mystery of SJS/TENS remains, there is no mystery about the hell on earth that the condition represents. That said if you, or are a loved one develops this horrid condition at some point after starting a regimen of the antibiotic Ketek for infection, talk to your doctor.

Then, talk to your lawyer.