Things I've learned since Austin's SJS experience

1) Doctors all say "it's rare" - yet we have heard so many other stories on the SJS support website.

2) Many doctors have no clue how to recognize SJS or what it truly is.

3) I need to be more "in your face" when it comes to my family's health care. Doctors and PA's really don't know everything and I shouldn't be so trusting.

4) Rainbow Babies and Children's Hospital is an excellent facility with excellent people working there.

5) I have a new perspective on many things...

6) We were very lucky Austin's rounds with SJS weren't more severe than what they were. Many people are not as fortunate.

7) Although many people get SJS from ibuprofen or any other anti-inflammatory drug, sulfa based drug or Tamiflu, Austin's seems to be brought on by a virus.

What is SJS? Who does it affect?

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.

Almost any medication including over-the-counter drugs, such as Ibuprofen, can cause SJS. Most commonly implicated drugs are anti-convulsants, antibiotics (such as sulfa, penicillin and cephalosporin) and anti-inflammatory medications.

Although SJS afflicts people of all ages, a large amount of its victims are children. More female cases have been reported than male, however it does not discriminate against anyone. The SJS Foundation hears from people around the world who suffer from SJS and TENS.

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 ascomfortable as possible. Most SJS patients can be managed in medical ICU or pediatric ICU. Patients with TENS should be treated in a burn unit

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 mucousmembranes, arthritis, and chronic fatigue syndrome. Many patient’s pores scar shut, causing them to retain heat.Th ese are just some of the side-effects that have been reported.

SJS Symptoms

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!

Austin's Timeline

February 4, 2009 – Took Austin to see Dr. for swollen knee - either a snowboarding or basketball injury – was told to take ibuprofen for a week - every 6 hours for a week and not to play basketball or snowboard.

February 9, 2009 - fever and sore throat – went to Pediatrician who swabbed him for strep and mono. – both negative - stayed home from school

February 10-11 – still has fever (still taking Ibuprofen) - didn't go to school

February 12 – feeling a little better –but really tired (no school because of wind storm)

February 13 – went to school – at 2:30 p.m. after school was out, Austin came to my office to get a ride home. I noticed his lips had swollen to about double - I had a flashback to 2 years ago when he looked similar. Knowing now what I did not know then - 2 years ago he also had SJS - but we had never heard of it - doctors never diagnosed him with SJS or ever mentioned it. That night – the roof of his mouth “felt funny” – had a white coating on it - looked like thrush.

February 14 – woke up with one lesion on his lip – took him to the doctor, as at that moment we knew it was a repeat of what happened 2 years ago – she diagnosed stomatitis and possible yeast infection – put him on Diflucan and an anti-viral - by that night lesions got much much worse. Did not want to eat or drink anything. Started with ice packs to his lips.

February 16 – called doctor b/c Austin not able to eat or drink b/c of the swollen lips and lesions covering his lips, inside of his mouth, and tongue – she said take him to ER for fluids – saw a Physician's Assistant – we asked what the lesions were that we had just noticed in the ER on his arms and hands– P.A. said “part of the virus.” He was given a bag of IV fluids, pain meds (which were not a good idea with him not eating for 3 days) and sent home. Very weak. Started squirting water down his throat with a syringe.

February 18– evening – started coughing blood - thought it was from the lesions - still don't know for sure - that day we had him start squirting Ensure down his throat since he still was unable to eat or drink anything. Still has not complained. Washer going 24/7 with cleaning the bloody towels. Ran to Convenient on runs for bags of ice.

February 19 – that evening started coughing blood even more - lesions progressed to his legs, feet, on palms of hands

February 20 – took him to doctor first thing in the morning - he can barely walk – she said SJS as soon as she saw him (had not seen her 2 years ago) – she x-rayed him for pneumonia (negative) – admitted him to Rainbow's Babies and Children's Hospital. When they weighed him upon admit, was the first time I really lost it - he had lost 18# - and anyone who knows Austin, knows he is very tall and very thin to begin with. Not knowing for sure it was SJS, he got a private room. Infectious diseases, dermatology, opthamology, rheumatology, all in to see him, lots of interns.

February 20-March 6 - They finally rule everything out except SJS. It is a dermatology disease, so that was the lead department taking care of him, but ophthalmology was in daily for first week to watch his eyes, because many many people come out of SJS with blindness or some other eye complications. He was lucky. Austin was on the highest level of pain killers (can't remember the name - 2 levels above Morphine) - thru a personal pain management pump (he was able to press for meds as needed), was on a PICC line to receive his fluids with fats, proteins, sugar). Thought he'd have to go home with it - but he was able to be weaned down from the pain meds and able to drink the required 8 Ensures (yuck) a day to get his nutrition. Still did not complain, but the middle of one night he finally had enough after waking yet again to coughing up more blood. He had to wean off the ice packs too... he had them on almost 24/7 for about 2 weeks before weaning down from them (dermatology said it was fine as long as it wasn't directly on his skin - for fear of frostbite). He used a suction machine like the dentist uses to get all the saliva and blood out of his mouth since he couldn't swallow. The doctors cannot do a whole lot to treat SJS - they can only make the patient as comfortable as possible with pain meds and make sure the nutritional needs are being met. Went home March 6th - had follow up visit in the next week and month.

March 6- April 3, 2009 - went to Physical Therapy to start regaining his strength

April 3, 2009 – finally able to not use suction machine and started eating and drinking out of a cup -up until this time he had still been using syringes we got from the hospital. We made our own contraption - got a large syringe, cut down a catheter - put it on the end of they syringe and it was easier to squirt down the thick Ensure and V8 Fusion. V8 was his "treat" for the day when he finished all of his Ensures. He loved the strawberry-banana flavor.

April 14 – Finally went back to school after Spring Break. He had an at home tutor come about 3 times a week - but caught up on his own.

April 20 – started noticing his fingernails peeling - as predicted would happen.

April 22– finally gained all lost weight back (18#)!

August 1, 2009 - Austin is doing great - you'd never know 6 months ago what he went through. But if you look, you can still see the scars on his hands, arms and legs, his mouth is still sensitive to spicy foods, and his gums have still not receded completely back yet.

He did, however, pass 8th grade with a 3.8 GPA taking all honors classes and playing "catch up" the whole last grading period, he was able to participate in about 2 track meets, golfed this summer in a junior golf league, he's playing percussion in marching band and taking bass guitar lessons. I'm happy to report at 5'11", this summer he has finally surpassed me in weight (yea)! And he's still one of the sweetest boys you will ever meet!

Our whole family is grateful for the support of family and friends through that very rough period. Please pass this information about SJS on to your family and friends to educate everyone about this type of reaction to some medication that causes all types of health complications and could lead to death. The earlier it's caught when symptoms start appearing, the earlier medication can be stopped and hopefully the progression is stopped or not as severe. Thank you.

SJS Support

Go to www.sjssupport.org for more information and to see other SJS survivors and their stories - Austin and other kids' stories are at
http://www.freewebs.com/sjskidssupport/sjskidspics.htm

To read one brave little boy's struggle or to donate to his care go to http://www.milnesjs.com/