The Facts About
STEVENS-JOHNSON SYNDROME (SJS)
SJS: WHAT IS IT?
Stevens Johnson Syndrome (SJS), and TENS (Toxic Epidermal Necrolysis Syndrome)—another form of SJS—are severe adverse reactions to medication. Adverse drug reactions (ADR’s) account for approximately 150,000 deaths per year in the U.S. alone, making drug reactions the fourth leading cause of death in the United States. SJS is one of the most debilitating ADR’s recognized. It was first discovered in 1922 by pediatricians A.M. Stevens and F.C. Johnson after diagnosing a child with severe ocular and oral involvement to a drug reaction.
WHAT CAN CAUSE SJS?
Almost any medication including over-the-counter drugs, such as Ibuprofen, can cause SJS. Most commonly implicated drugs are anti-convulsant, antibiotics (such as sulfa, penicillin and cephalosporin) and anti-inflammatory medications.
WHO CAN GET SJS OR TENS?
Although SJS afflicts people of all ages, a large amount of its victims are children. More female cases have been reported ed than male, however it does not dis criminate against anyone. The SJS Foundation hears from people around the world who suffer from SJS and TENS.
SJS: KNOW THE SIGNS!
Recognition of the early symptoms of SJS and prompt medical attention are the most invaluable tools in minimizing the possible long-term effects SJS may have on its victims.
SYMPTOMS:
- Rash, blisters, or red splotches on skin
- Persistent fever
- Blisters in mouth, eyes, ears, nose, genital area • Swelling of eyelids, red eyes.
- Conjunctivitis
- Flu-like symptoms
- Recent history of having taken a prescription or over-the-counter medication Target lesions are not always seen in SJS!
IF YOU NOTICE TWO OR MORE OF THESE SYMPTOMS, CONTACT A PHYSICIAN IMMEDIATELY!
The Stevens Johnson Syndrome Foundation is a non-profit organization. Your donations are tax deductible and will provide invaluable aid to a worthwhile cause.
To contribute to the SJS Foundation, please contact us at:
Stevens Johnson Syndrome Foundation
P.O. Box 350333
Westminster, CO 80031
E-mail: sjsupport@aol.com / phone: 303-635-1241
www.sjsupport.org
RISKS
SJS and TENS are life-threatening reactions. If left untreated, they can result in death. Complications can include permanent blindness, dry-eye syndrome, photophobia, lung damage, chronic obstructive pulmonary disease (COPD), asthma, permanent loss of nail beds, scarring of the esophagus and other mucous membranes, arthritis, and chronic fatigue syndrome. Many patients’ pores scar shut, causing them to retain heat. These are just some of the side-effects that have been reported.
TREATMENT
First and foremost, affected persons must stop taking the offending drug immediately to prevent complications. Treatment for SJS is good supportive care. Because patients literally burn from the inside out, burn, infectious disease, ophthalmology and dermatology teams are recommended. IV fluids and high calorie formulas are given to promote healing. Antibiotics are given when necessary to prevent secondary infections such as sepsis. Pain medications such as morphine are administered to make the patient as comfortable as possible. Most SJS patients can be managed in medical ICU or pediatric ICU. Patients with TENS should be treated in a burn unit.
DRUG REACTIONS:
A SERIOUS ISSUE Drug reactions are one of the leading causes of death in the United States. Yet less than one percent are reported to the FDA, because there is no mandatory reporting system in effect for post marketing adverse drug reactions. Similarly, no one has an accurate count of the cases of SJS and TENS. Although SJS is listed as a rare disease, it may be more prevalent than previously thought.
WHO WE ARE
The SJS Foundation was founded to be a resource to SJS victims and their families. Our mission is to provide support services, and compile and distribute valuable information about SJS to the public and medical professionals regarding treatments and therapies that may prove beneficial to SJS sufferers. We work to promote awareness about the signs of SJS so that a quick diagnosis can be made, and the offending agent stopped as soon as possible.
©2006 SJS Foundation Duplication of the information and photographs contained herein is strictly prohibited. All rights reserved.
Click this link to learn more about SJS Foundation!
Last December 28th my 22-year-old daughter Angela died of a rare condition called Stevens Johnson Syndrome. I took her to ER on Christmas morning with an eye infection fever and rash. She was dead 3 days later. At her visitation and funeral, we accepted donations for research in lieu of flowers. We received nearly $10,000 at that time. My son had a benefit concert for her in February and we received another $5,000.
I then began my search for a place to send the money to. I went online and found the Stevens Johnson Foundation https://sjsupport.org/. I called them and talked to the mother of a child that had survived SJS twice. I asked her about research for SJS. She said that there was no research being done in the United States. She said that the Foundation’s purpose was to spread public awareness, and she suggested that we send our money to one of the burn centers that treats SJS patients such as the Shiners’ Hospital in Boston. She said that the Foundation didn’t need that much money, but we made a small donation to them anyway. I was disheartened that no research was being done on the disease that took my daughter.
I went back online and found that On March 3-4, 2015, the National Institutes of Health (NIH) and the Food and Drug Administration (FDA) had sponsored an international workshop - Research Directions in Genetically Mediated Stevens-Johnson Syndrome/Toxic Epidermal Necrolysist https://www.genome.gov/27560487/research-directions-in-geneticallymediated-stevensjohnson-syndrometoxic-epidermal-necrolysis/ At that site was a list of all the participants of that workshop. I copied the names of the 68 participants that were in attendance, Googled their names for their email addresses and phone numbers. I then began contacting as many of them as possible. I first heard from Dr. Teri Manolio from the NIH (National Institute of Health). She told me that she did not know of anyone doing research in the U.S. She suggested that I contact doctors in Southeast Asia where some research was being done. I did contact researchers from 3 countries but found that they either weren’t interested in the money or that the money would only be used to benefit research in their own country.
My first hint of research being done in the U.S. was in an email from Dr. Elizabeth Phillips of Vanderbilt University Medical Center: in an email to me, she said,
“On a positive note, we are actively gathering researchers within the United States and internationally to develop a multidisciplinary and active research network. This is happening currently as we have been surveying across the United States to get a coordinated effort over the last 2 weeks. There are potentially new drugs of interest that can be trialed in the treatment of SJS/TEN and this type of infrastructure will be absolutely necessary to study and effect change in a disease as devastating as SJS/TEN.”
In subsequent emails from Dr Phillips, I received a preliminary proposal for her intentions to start SJS research. She also invited us to visit her at VUMC. In May 2016 my wife, my son and I headed to Nashville to meet her. We were surprised when we got there that we were met with Dr Phillips and team of five doctors in a conference room. They proceeded to explain what kind of research they would be doing for the next hour and a half. They then gave us an extensive tour of their labs. At that time, we had accumulated $16K in donations. It was an amount we thought insignificant in the world of research where millions of dollars are spent. But they were impressed by our endeavors and said, “It’s a start!” After that, our son Tim opened up a Go Fund Me account on Facebook and I opened one also and donations continued to roll in.
On December 13, 2016, nearly one year since Angela’s tragic death we met with Dr Phillips again and presented her with a check for $22,000. This is an accumulation of all of the donations we have received from family, friends and concerned strangers. $22,000 is also a significant number because it represents $1,000 for each year of Angela’s shortened life.
More money is still needed to continue the fight against this dreadful disease. If you would like to add your donation to continue this research, you may donate by clicking here. Donate!
The Research Team
Left to Right-
- Simon Mallal, M.D. – Professor and Executive Director, Centre for Clinical Immunology and Biomedical Statistics at Royal Perth Hospital and Murdoch University; Professor and Director, Institute for Immunology and Infectious Diseases Murdoch University; Major E.B. Stahlman Chair in Infectious Diseases, Professor of Medicine and Pathology, Microbiology and Immunology, Director, Center for Translational Immunology and Infectious Diseases, Director, Center for AIDS Research, Vanderbilt University School of Medicine
-Elizabeth Phillips, M.D. - Professor of Medicine & Pharmacology, Director of Personalized Immunology, Oates Institute for Experimental Therapeutics, Vanderbilt University; Clinical Professor, School of Pathology & Laboratory Medicine, University of Western Australia
-Katie White, M.D. Ph.D. – Infectious Disease Fellow, Physician Scientist Program at Vanderbilt University
-Sarah Garon, M.D. - Clinic Fellow, Allergy/Pulmonary & Critical Medicine at Vanderbilt University
-Elizabeth Ergen, M.D. - Dermatology Resident at Vanderbilt University
-Kristina Newgarden, RN, BSN – Research Nurse Specialist at Vanderbilt University
