“I was scared because first I couldn’t talk (because of the blisters in her mouth) and now I was in the hospital not even knowing what was going on,” she said. Among the physicians who examined her was an eye doctor, who knew immediately what she had. He said one of his employees had had the same thing a month earlier. He said, “She has Stevens Johnson Syndrome (SJS),” he said. Hannah quickly wrote down, “What the heck is that?”

“No one could really explain it to me,” she said. “They were taking blood and giving me morphine and drugging me up and they didn’t even know what had made me so sick.”

By the time Hannah was able to go home three weeks later, she learned what caused her SJS – it was a severe adverse reaction to her bipolar medication, trileptal.

Trileptal, also approved for the treatment of epilepsy, recently amended its safety labeling to include a warning of the risk of SJS and its more severe form, toxic epidermal necrolysis (TEN). SJS and TEN are hypersensitivity disorders that begin as a rash that blisters over and causes the skin to peel away. Mucus membranes also can be affected with blisters causing dehydration, infection, blindness and even death.

It has been almost a year since Hannah became ill. She has recovered but the memory of SJS still lingers in her mind. Her skin is almost completely healed, but now she must avoid the many medications that have been associated with SJS and TEN for fear of a relapse. “I can’t take Midol or Advil anymore,” she said. “Now, my mom tells me what to take.”

SOURCE: Stevens Johnson Syndrome › Teenager shares story of serious reaction to medication

SJS and TEN are hypersensitivity disorders that affect the skin and mucus membranes. It begins as a rash that blisters over, causing the skin to peel off in sheets. The mouth, eyes and other orifices can be affected with blisters as well. The condition can be life-threatening.

Oxcarbazepine is relatively new anti-seizure treatment for epilepsy. It works by blocking the channels that electrical signals use to get into brain cells. According to the update, individuals treated with oxcarbazepine who experienced the reactions had a fever and a rash. Some patients had enlarged lymph nodes and some experienced symptoms such as itchiness and joint pain. The rash usually appeared within three weeks of starting treatment with oxcarbazepine.

More than 200 medications have been linked to SJS and TEN. Many anti-seizure medications have been associated with the severe reactions. Those on oxcarbazepine or any other treatment for epilepsy should contact their doctors immediately if they experience any of the warning signs of SJS or TEN, including raised lumps, flaky skin, swollen face, painful skin, purple blotches, sores on the lips or inside the mouth, or any asthma symptoms such as wheezing and difficulty breathing.

SOURCE: Stevens Johnson Syndrome › Oxcarbazepine/Trileptal epilepsy meds linked to SJS/TEN

Researchers like Wen-Hung are asking why.

SJS is a life-threatening hypersensitivity condition that causes rashes to form over the body. The rashes blister over, causing the skin to peel off in sheets. SJS also can affect the mucus membranes, causing blisters to form in the mouth and eyes. Death can result in about 10 percent of patients with SJS. If it progresses to its most severe form, toxic epidermal necrolysis, (TEN) the death rate soars to 40 to 50 percent. SJS/TEN is linked to more than 200 medications, most commonly ibuprofen, antibiotics and anti-seizure medication.

A group of Chang Gung researchers were aware that the anticonvulsant carbamazepine was linked to many cases of SJS, but through its own study using Taiwanese patients, researchers discovered a possible connection – “a high correlation between hypersensitivity to carbamazepine and a single allele, human leukocyte antigen B1502,” according to the report. As many as 80 percent of Taiwanese carry the allele. The discovery lead to an FDA warning in the U.S. of the increased risk of SJS/TEN for patients of Asian ancestry with the B1502 allele who take carbamazepine.

Since the discovery, researchers began focusing on developing a test to determine whether a patient has the B1502 allele, and lead researcher Wen-Hung turned his attention on a treatment to halt the progression of SJS/TEN.

SOURCE: Stevens Johnson Syndrome › Taiwanese researchers pinpoint genetic marker for SJS

Beatham’s girlfriend of two years, Louise Armstrong, came down with flu-like symptoms in late December. The morning of New Year’s Eve, Beatham woke in their Penrith, Australia, home to find his partner gravely ill with blisters covering her body. He called the ambulance, but it was too late. Armstrong had died.

According to the News & Star, Beatham was made to wear a paper body suit while detectives waited to hear word from the pathologist on the cause of death. The pathologist determined the cause was natural, and that she had died from a rare but severe adverse reaction to medication called Stevens Johnson Syndrome, or SJS.

SJS, and its most severe form, toxic epidermal necrolysis, or TEN, has a reported incidence of about one case per million people each year. It affects people of all ages, including young children, and is most often caused by common medication such as ibuprofen, antibiotics and anti-seizure medication, to name a few. Defined as a hypersensitivity disorder, SJS/TEN affects the skin and mucous membranes, causing the skin to blister and peel away in sheets. Blisters also can form in the mouth or eyes, and can lead to severe eye problems or blindness.

Armstrong’s parents, Ted and Angie, were distraught about their daughter’s death and puzzled why police would think their daughter’s boyfriend was to blame. At the time of their daughter’s death, they were dealing with the sudden serious illness of their son, Chris, who as hospitalized at the time with epilepsy.

“It hasn’t yet hit us fully,” Mrs. Armstrong said of her daughter’s death.

SOURCE: Stevens Johnson Syndrome › Australian woman dies from adverse reaction to medication

But the blisters got worse. They spread down her throat and little Julie was unable to drink her bottle because of the pain. Jean rushed her daughter to the hospital, where doctors continued to administered the phenobarbatol for her seizures. But Julie’s health continued to decline. Four days later the little girl’s lungs collapsed and her skin began coming off in sheets. That’s when a nurse realized what was going on. “This is Stevens Johnson Syndrome (SJS),” she said. Jean was relieved to have a diagnosis, but the doctor said, “This is extremely serious. She could die.”

Jean shared her story earlier this year to Voice America’s Disability Matters radio show with host Joyce A. Bender. Jean appeared on the show with SJS survivor Elizabeth Boxer and her father Mark.

When Jean’s daughter was diagnosed with SJS, she was told the adverse reaction to medication such as anti-seizure medication, ibuprofens and antibiotics was so rare that she would probably never hear of it again. But Jean refused to believe that the illness that threatened her daughter’s life and left her blind in one eye and visually impaired in the other – a condition caused by common medications – was as rare as doctors described. She made it her mission to found a community where others touched by SJS could share their stories. Thus, the Stevens Johnson Syndrome Foundation was established.

Shortly after founding the organization, Jean discovered that several people were suffering from the disease. Many, like Jean, were looking for answers as to how medicine designed to help can actually cause more harm.

“This is wrong,” announced Jean on the radio program, where she took questions from listeners. “(SJS) is not rare. What’s rare is having it reported to the FDA.”

SOURCE: Stevens Johnson Syndrome › SJS Foundation established to give answers to those with SJS

Kim experienced a rare reaction to the antibiotic that started with a headache and progressed quickly to toxic epidermal necrolysis (TEN), a severe form of Stevens Johnson Syndrome (SJS), a reaction to medication where the skin blisters and peels off. Ninety percent of the time the reaction is caused by drugs such as ibuprofen, antibiotics and anti-seizure medications.

Kim lost 70 percent of her skin and was put into a drug-induced coma. She was given a 30 percent chance of survival by one doctor. She managed to beat the odds, and was released from the hospital this week.

“Why aren’t people told that they could die if you have a reaction to this drug?” Kim’s father, Art Oake, posed to CBC News.

Nearly one in a million Canadians are treated for SJS/TEN each year. One in six die, according to the story. Toronto dermatologist Dr. Neil Shear urges patients who take antibiotics or drugs used to treat epilepsy to look for signs of adverse reactions, such as fever, tender skin and blistering or sores in the mouth. Patients who experience any reaction should stop taking the drug immediately and call their doctors, he said.

SOURCE: Stevens Johnson Syndrome › Father asks why he wasn’t warned of possible reaction to drug

Lamotrigine is an anticonvulsant drug to treat partial seizures, primary and secondary tonic-clonic seizures in epilepsy and seizures associated with Lennox-Gastaut syndrome. It also is FDA-approved to treat bipolar disorder.

Peer View cited a session presented at the American Epilepsy Society’s annual meeting, which detailed a study, conducted between January 2000 and December 2007, involving 403 children treated with lamotrigine in addition to existing therapy. Twenty-one of the children in the study experienced adverse skin reactions including moderate diffuse mascular-papular or erythematous rash, severe urticarial skin eruption and mild Stevens Johnson Syndrome (SJS).

There were no cases of toxic epidermal necrolysis (TEN), a severe form of SJS, however the medication has a black box warning about life threatening skin reactions, including SJS and TEN. The manufacturer says the reactions usually occur in the first 2 to 8 weeks of therapy and if medication is suddenly stopped and then resumed at its regular dosage.

According to Rx List, the incidence of these serious skin rashes in response to therapy for epilepsy is about 8 in 1,000 in pediatric patients younger than 16 years of age and about 3 in 1,000 in adults. For treatment of bipolar disorder and other mood disorders, the rate is about 1.3 to 8 in 1,000 for adults.

SOURCE: Stevens Johnson Syndrome › Risk factors may indicate serious skin reaction to lamotrigine

In a recent Information for Healthcare Professionals sheet, the FDA described the increased risk of SJS/TEN with another antiepileptic drug, carbamazepine, in patients of Asian ancestry with the HLA-B*1502 allele. HLA-B is a human gene that provides instruction for making a protein that plays a critical role in the immune system. It is part of a family of genes called the human leukocyte antigen (HLA) complex. This complex helps the immune system distinguish the body’s own proteins from proteins made by viruses or bacteria. Several variations of the HLA-B gene are associated with adverse reactions to certain drugs.

SJS is a disorder of the skin and mucous membranes and often begins with flu-like symptoms followed by inflammation of mucous membranes and a painful red or purplish rash that spreads and blisters. It eventually causes the top layer of skin to die and shed.

Until the FDA completes its review, the agency warns healthcare providers who are considering the use of phenytoin or fosphenytoin for their patients to be aware of the risks and benefits described in the current prescribing information for the drug. Healthcare providers should consider avoiding phenytoin and fosphenytoin as alternatives for carbamazepine in patients who test positive for HLA-B*1502.

SOURCE: Stevens Johnson Syndrome › FDA warns of link between anti-epilepsy drugs and SJS