FATAL REACTION:
THE HORROR OF STEVENS JOHNSON SYNDROME

BY STEPHEN BYRNES, PhD

Jean Farrell knew something was wrong. The Denver travel agent had left for work in the morning and noticed that her 11 month-old daughter, Julie, had a swollen eye. Julie, an epileptic, had been placed on phenobarbitol, a widely used anti-convulsant drug, about two weeks prior. Farrell asked her mother to watch Julie during the day. But when Farrell returned from work later that day, however, both of Julie's eyes had swollen completely shut. The concerned mother quickly called Julie's pediatrician who asked Jean to bring her into his office. As Jean and Julie were leaving, Farrell noticed small blisters on Julie's lips, as well as red spots forming at various places on Julie's skin. Julie was also running a high fever.

"The pediatrician looked at Julie and told me she had a viral exanthem," Farrell said. "The doctor checked Julie's ears and noticed that pus-filled blisters had formed on Julie's ear drum. He thought it was unusual. He then changed the diagnosis to chicken pox with an ear infection. He told me to keep giving her the phenobarbitol and also prescribed an antibiotic for the ear infection."

But this course of action did little Julie no good. "The next day," Farrell continued," Julie had developed large blisters on her lips, as well as inside her mouth and ears. Her eyes were still swollen shut, the red spots on her body had grown to the size of quarters and were ulcerating. She had so many blisters in her mouth that she couldn't swallow." Farrell, knowing that Julie had not had anything to drink for several hours, thought she might be dehydrated and took her to the emergency room. "The hospital staff told me that Julie had 'a REALLY bad case of the chicken pox.' They placed her on an IV for her fluids and, when they learned Julie was epileptic and on phenobarbitol, added that into her IV treatment."

Julie got steadily worse. "My baby's face was unrecognizable. She looked like she had been deep-fried," said an agitated Farrell, obviously upset at having to relive the horrific events. "Finally, an older, very experienced nurse boldly said to the doctor and me that Julie did not have the chicken pox, but Stevens Johnson Syndrome. She said she knew because she had seen other cases before. The doctor did not believe her and left the room. I was bewildered: "What is wrong with my little girl?"

A short while later, another doctor came into Julie's room to find Jean Farrell sobbing hysterically. "He asked me what was wrong with my daughter and I told him that nobody seemed to know. Then, I mentioned that the nurse thought Julie had Johnson's disease. The doctor looked at me and said, 'Stevens Johnson Syndrome?' I blurted out 'That's it.' He took one look at Julie and immediately removed the IV with the phenobarbitol. He then called the Burn Unit, an opthamologist, and an infectious disease specialist. Finally, somebody knew what was happening."

While Jean was at last relieved to discover what her daughter's problem was, the doctors involved were not. They told her, "Jean, this is not a good thing." And Farrell would find out why in the next three weeks that Julie was in the hospital. "Julie's lungs had to be constantly suctioned to remove fluid and mucous that kept accumulating there. Her skin and mouth continued to blister and ulcerate for weeks and she was completely wrapped in bandages. She was on morphine for the incredible pain. In order to work on her eyes, I had to hold my baby down on the bed while the doctors pried her blistered eyes open with glass rods. When they did this, the skin under her eyelids literally sloughed off. There was blood everywhere and Julie was in agony; this happened on her first birthday. A one year old child not knowing why this was happening to her."

Despite several close calls, Julie survived her ordeal, but not without permanent damage. She is completely blind in one eye and has limited vision in the other. She must constantly wear sunglasses as her eyes are very light-sensitive. While her skin has mostly healed, she still has noticeable scars on her legs and back. She may have sustained kidney damage as well. Jean Farrell started the Stevens Johnson Syndrome Foundation to educate the public about the dangers of allergic drug reactions: "I don't want anyone to go through what me and Julie did."

WHAT IS STEVENS JOHNSON SYNDROME?

Stevens Johnson Syndrome, or SJS, is an extreme allergic reaction, usually to a drug, but also to certain bacterial and viral infections. "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."

While there are a number of infectious causes that can initiate an SJS reaction, the most common culprits are drugs. The drugs most implicated are anti-convulsants (like phenobarbitol), sulfonamides, or sulfa drugs, non-steroidal anti-inflammatory's (NSAIDS), including ibuprofen, barbiturates, and antibiotics such as amoxicillin and tetracycline. "We just lost a 17 year old boy in Kansas City to SJS apparently caused by tetracycline, " Farrell said ruefully. "He was taking it for his acne. We also have a woman whose daughter went blind after taking ibuprofen. People need to realize that over the counter drugs can cause SJS too."

DIFFICULT TO DIAGNOSE

Dr. Cohen, a pediatric dermatologist and medical advisor to the SJS Foundation, explained, "Typically, the reaction begins within the first two weeks of taking the drug (if that is the true cause). The eyes will get puffy, swollen, and gritty. There will usually also be cracking or small blisters on the lips and mouth. Usually the person has a fever and red patches on the skin. Most people think they have the flu." Or the chicken pox as Jean and her daughter found out. "Chicken pox is the most common misdiagnosis, at least for children. Its amazing how many doctors and nurses don't know what SJS is," she pointed out.

For Sherry Callejo, a secretary at the University of Hawaii, her doctor's ignorance about SJS almost cost her her life. Callejo took a fourteen day course of a sulfa drug, for a sinus infection. "About two days after I finished the treatment, my face and lips got tingly and splotchy. The next day my lips were darker, and two days later they were completely black and my mouth was full of sores. My face was bright red. My doctor, an internal specialist, did not know what was happening and he sent me to an infectious disease specialist who correctly diagnosed me with SJS. By then, all my mucous membranes were inflamed and blistering, but especially my mouth and genitalia. My torso and neck were covered in splotchy bruises." Although Callejo sustained some corneal scarring, her vision was not affected. She continued, "I've seen pictures of people with much worse reactions than me. I consider myself very lucky."

STANDARD TREATMENTS

If and when SJS is diagnosed, the offending drug must be removed at once. It is common to prescribe steroids like Prednisone to quell the overactive immune system, but both Dr. Cohen and Ms. Farrell stated that there is no conclusive evidence that steroid treatment halts an SJS reaction though Callejo definitely felt it helped her: "I felt much better after three days on the steroids."

Since the skin is literally burning from the inside out, with extensive blistering and ulceration, standard burn treatments are common. Many SJS patients are referred to Burn Treatment Centers. "Care must be taken to prevent staph infections of the skin from taking hold," Dr. Cohen said. "Intravenous fluids to prevent dehydration are a must," he continued, "As the person is usually unable to eat or drink due to the mouth blisters."

Both Farrell and Cohen admitted sadly that there really is nothing to halt an SJS reaction once it has begun. All that can be done for the unfortunate person affected by it is to deal with the secondary manifestations of the disease.

LONG TERM DAMAGE

While the acute SJS reaction may be finished in less than a month, its effects can last a lifetime. For the 15-20% of SJS victims whose eyes have sustained damage, the Scleral Lens, developed by Dr. Perry Rosenthal of the Boston Foundation for Better Sight, offers considerable relief. "We have many clients of the Foundation using the scleral lens. Its enabling Julie to see right now, " Farrell elated.

"Usually we tell SJS survivors to use extra sunscreen and to stay out of the sun for awhile as they are more susceptible to skin cancer," Farrell went on. "Patients' immune systems are in overdrive and shock after an SJS episode so they usually end up with several allergies. We've had a few clients use alternative therapies to good effect. We have a girl whose nails fell off now using acupuncture and Chinese medicine; the nails are growing back."

Dr. Cohen added that sometimes SJS victims sustain permanent lung and esophegal damage. Callejo, for example, has esophegal scarring and frequent coughing that she controls with a steroid inhaler.

More severe damage occurs sometimes in people who survive TENS. Often, they become heat sensitive and lose the ability to sweat--the skin damage can be that extensive.

Less severe after effects from SJS can linger for months. "Its been 14 months since my episode and I still don't have all of my energy back," said a frustrated Callejo. "I feel like something in me is missing, almost spacey."

Betty Ellen Ford, an 84 year-old Honolulu resident, concurred: "Its been 18 months and my legs are still scarred. I still have flare-ups in my mouth making it difficult for me to eat sometimes." Ford had an SJS reaction after being given a common drug for a respiratory infection.

A RARE DISORDER?

Medical textbooks commonly state that SJS and TENS are extremely rare disorders. But Farrell strongly disagrees: "SJS is NOT as rare as we are led to believe. Since setting up the SJS Foundation and launching our website, we've been contacted by thousands of individuals. During the winter months, we were learning of 15 new cases a week, and that's only through the Internet. How many affected people were out there without access to the Internet? After Julie's episode, I was told by almost every doctor that SJS was so rare that I would never meet another person who had had it. Well, after the local news in Denver ran a story on Julie, I was contacted by several people whose children had been through it. That was the start of our support group. Just last week I was contacted by a man with HIV who had contracted SJS by taking the currently recommended "drug cocktail" for AIDS. We've been contacted by people as far away as China and South Africa. This disease is not rare--its on the rise."

Many people thank God for Farrell's organization. "When I got some strength back, I did a search on the net for SJS and found Jean's foundation," Callejo said fondly, "It and Jean were heaven sent. I felt so good to find others who could relate to what I was going through and I've benefited from the support group and on-line chat room the website supports."

How does Farrell keep the Foundation and website going? Ironically, through a generous grant from United Airlines. Farrell did approach several drug companies about funding, but was curtly turned down. "I first approached the companies who manufactured the drugs that were the most implicated in SJS. I made it clear that we were not looking to blame anyone; we just wanted more knowledge about SJS and more research done so that it could be stopped." Despite this cooperative approach, the response she got was, nevertheless, defensive: "This one company told me something like, 'There are so many environmental issues surrounding SJS. How can you be sure it's coming from our drug?' I couldn't believe this answer! I mean, let's blame Mt. St. Helen's for SJS! It is true that the package inserts for several drugs do mention SJS as a remote risk factor, but how many people know what SJS is? How would they know they were having a reaction that could possibly kill them?"

NATURAL THERAPIES FOR SJS

If there is a desert of information in allopathic medicine on SJS, there is an even greater lack in natural therapies. Its important to realize that SJS is a modern disease and that it is not associated with herbs, supplements, or homeopathics. It is also imperative to point out here that someone with SJS needs to be in a hospital under acute care. Nevertheless, natural therapies can be employed to help with the rebuilding process.

Joan Scott-Lowe, a registered nurse and practicing homeopath in Alabama, feels that homeopathy could be a useful adjunct to standard treatment: "Allergies are dealt with in homeopathy by giving a homeopathic dose of the substance to build up resistance to it. In the case of SJS, since the person is hypersensitive to a drug, the drug would have to be prepared homeopathically at a dose no less than 15C or 24X to be sure that no gross substance remained. Theoretically, this could help." "Theoretically" is the operative word here: no one has ever tried this approach. Lowe also added that standard homeopathic remedies for more serious burns such as Cantharis and Urtica urens could be administered under the direction of a trained practitioner.

Supplements of vitamins A, E, C, B complex, zinc, and essential fatty acids could be employed to rebuild the immune system and skin damage. Vitamin A is particularly crucial for the eyes and mucous membranes, and vitamin E is known for its anti-scarring properties. Farrell spoke of a few mothers of SJS-affected children who had placed a drop of vitamin E oil directly on their child's scarred cornea, often to good effect. Farrell also reported that Julie's frequent infections after her SJS episode disappeared once she began taking daily multivitamin/mineral and vitamin C supplements. Julie's epilepsy was brought under control, without the use of anti-convulsants, through a ketogenic diet that restricted carbohydrates of all types.

Mouth lesions could be soothed by holding liquid aloe vera in the mouth, and by gently washing the mouth out with 1 oz of water with a few drops of tea tree oil added. Aloe could also be applied to the skin. Despite positive anecdotal stories, more research is needed here, however.

A NIGHTMARE THAT CAN END

SJS is definitely a nightmare, but there is hope. With adverse drug reactions being the 4th leading cause of death in North America, it behooves each person to think carefully before taking something. If a blood relative has had an allergic reaction to a drug in the past, even a mild one, consider yourself at risk and avoid the drug. "I was shocked to find out after my reaction that my father and brother both had developed skin rashes when they took sulfa drugs for short periods in their lives," Callejo said. "If only I'd known." It makes more sense, however, to seek drug-free alternatives for one's ailments if and when they arise.

Nevertheless, knowledge is power and all those interviewed for this article felt that more education about drug reactions and SJS were needed. Farrell said forcefully, "It is so tragic. You're warned not to give aspirin to children. How can you not tell people about a life-threatening reaction to a drug . . . any drug?"

For more information, contact the Stevens Johnson Syndrome Foundation at PO Box 350333, Westminster, CO 80035, (303) 635-1241; (303) 487-9359 (Fax). Info packs are available, please send your name and address, along with a $5 donation to cover copying and mailing expenses.

The SJS Foundation website is: http://www.sjsupport.org
Email: sjsupport@aol.com

Originally published in WellBeing Magazine (Australia), September 1999