Not Writing - Caring for a Patient with Steven Johnson Syndrome

Steven Johnson Syndrome or SJS is a very rare and life threatening health condition which needs to be taken very seriously by the patient and by the attending doctors. It can be caused by a long list of medications and some illnesses, such as hepatitis.

I am not a doctor. I am not even a medical student. I did, however, fall ill with SJS in late December of 2008. Information about SJS treatment and care was so scarce, we had to make most of it up and there was a lot of trial and error involved. This webpage is designed for anyone else in my position—or for doctors in the same position as mine were, who have no idea what to do next.

Symptoms and History.

For me, the first symptom was several extremely itch spots on my feet. They appeared to be very small, compact insect bites that were abnormally itchy. They were also notable in that regardless of how I scratched them, the skin didn’t break like with most insect bites. This lasted for three days.

These spots—which were red/pink—soon spread to my hands and my lips began to blister and itch. I went to my doctor and he diagnosed hand, foot and mouth. A week later I ended up in ER, as I began to get week and lethargic. I was temporarily quarantined, given a blood test and sent home.

The blood test found abnormal liver function and I returned to my GP who consulted with another doctor who diagnosed Erythema multiforme. I was told to ‘wait it out’ and sent home with panadine forte. I was also finally taken of the trigger medication—tegretol.

The rash was continuing to spread and I was—for lack of a better term—very sickly. Sleep deprivation was becoming an issue and nothing would stop the itching. I could not walk on my blistered feet or hold things in my blistered hands. My lips were so swollen and red they looked fake. The blisters, by this stage, had spread up my arms, across my chest and across my back. My mouth was going numb.

I presented at another ER and went under the care of a dermatologist and his team. By this stage it was about two weeks since the spots on my hands had appeared.

How SJS Kills

SJS is a little like an allergy. The body attacks itself in an extreme reaction to some stimulus, such as a drug. Only when this is removed can the patient begin to recover and it took at least four weeks for the blisters to fade, four months for my skin to grow back and two years later I am still having ongoing health problems from the condition.

SJS kills in three possible ways:

1. Firstly, it can attack the organs causing organ failure. My liver, lungs and digestive tract were all damaged. Thankfully, in my case, many of my mucus membranes—eyes, mouth, genitals—were spared.

2. Secondly, the lesions on the skin, mucus membranes and organs can let in secondary disease, viruses or bacteria which, given the weakened state of the patient, are quickly fatal.

3. Thirdly, the blisters in the mouth, lips and throat can become so painful the patient refuses food and water—ultimately succumbing to malnutrition, dehydration or a combination of factors.

When treating a patient with SJS, these three factors must be kept in mind.

TREATMENT

I am unfortunately sensitive to most medications. Contracting SJS is a case in point. However there is not many treatment options open to patients with SJS beyond fighting the contraction of a secondary disease and offering pain relief.

SJS skin lesions should be treated like burns. Mine were about the thickness and caused the damage of second degree burns to about 30% of my skin. That meant a lot of my treatment was focused on the skin.

Skin

Ice and white paraffin. You will need a lot of both. During the worst of it, I slept with my feet over the side of the bed—resting in a bucket of ice and water with ice packs held in my hands, layered up my arms and resting on my chest. On top of all that, was about eight blankets.

This sounds horrible, but it only took me a day to get used to the ice—after that I could sleep and work with it quite easily. When the ice packs started to heat up, I couldn’t have them replaced fast enough.

White paraffin should be layered on the skin thickly. It’s worse than the ice because it gets on everything. During the day our method was to apply a thick layer of paraffin wax to my arms, lips and hands—wrap the latter in tea towels and then stack the ice packs on top of that.

My feet rarely needed paraffin because during the day they were always in a bucket of ice water.

Scratching did me no harm or good. Don’t break the skin and keep it as clean as possible. Once the blisters fade expect to peel repeatedly. Scratching less and caring for the skin during the peel with baby oil may minimize the scarring—don’t expect your skin to ever go back to what it was.

Food and Diet

First and foremost, keep the patient hydrated. Two liters a day, minimum. Water is best—something with salt and sugar for hydration is second. Bubbles may not be tolerated due to blisters. Anything hot, too cold or too acidic will also likely be rejected. Mild juices and room temperature water is best.

If the patient is eating well, it is critical to give them a balanced diet of fresh fruit and vegetables, protein and carbohydrates. The depletion on the body’s resources is monumental during SJS. Just growing back the skin will require vast amounts of vitamins and protein. And with the extreme sleep deprivation I suffered, finding the energy to go to the bathroom or walk to the shower was almost beyond me.

If the patient is resistant to food, anything they will eat is good. Puddings, chocolate mousse, cake—as long as they’re eating and getting fluids. Everyone was so keen to get me eating I lived almost exclusively on pizza, chocolate mousse and cherry soda.

If the patient is refusing food and water—they need to be hospitalized and put on a drip as quickly as possible. Do not let them go more than 24 hours without food and more than 12 without water. This is the kind of thing that will escalate quickly.

Please note, despite being numb in the mouth instead of in horrible pain, I still didn’t want to eat while I was sick. I don’t remember feeling hungry at all and would often only have a mouthful before I didn’t really want more. Patients cannot be trusted to monitor their own food intake.

Medications

My medications consisted of: Panadol, Panadine forte, Zyrtec (an antihistamine) and not much else. Zyrtec was great for lessening the itching and the primary function of the panadine forte was to let me sleep in half hour snatches between ice pack changes.

Steroids have been used in the past to treat SJS with mixed results. They act to stop the body attacking itself, thus limiting the damage to organs and lessening the blisters and itching. However steroids function by stopping the immune system. If a patient already has open lesions on their skin or in their mouth, eyes or genitals, it will be much easier for another illness to attack and potentially kill the patient.

I did not attempt to use steroids.

Mental Health

SJS is depressing, scary, exhausting and frighteningly lonely. It’s also very, very painful. And itchy. So itchy you will scratch with steak knives (I did, many times—I kept one at my computer desk with me). If you have someone caring for you, they will be stressed and exhausted too.

I laughed and joked the entire time I was ill. I joked with the ER staff, I joked with the doctors, I joked with my friends on the phone—clutching a handset slick with white paraffin. You NEED a good sense of humor. You NEED to see the funny side and to be optimistic and you NEED about 100 hours of TV and movies on DVD. Do not underestimate the damaging effects of boredom.

Be asleep or be watching something or talking to someone. Do not sit alone, awake in the silence. Noise and laughter is probably the only thing between you and a mental breakdown.

Final Note

SJS is not a disease the patient can handle alone. If you have SJS and you are reading this, be aware you will need either a dedicated person (or two!) caring for you full time for at least five weeks, or you need to be admitted to hospital for the same.

I was very lucky to have my mother care for me. She is a level five registered nurse with well over 20 years experience and I think my SJS did more harm to her than it did to me. She kept me fed and changed my ice and forced me to take medication with tireless dedication. I think it almost killed her.

At the time, you won’t care about that though—you’ll just want to be wherever has the most ice packs and the best painkillers. Which is why I recommend a well staffed hospital.

COPYRIGHT. Talitha Kalago, 2010. Do not reproduce without permission.