Complication of Still's

[Guest guest]

I want to share with everyone something that happened to us recently.

Our daughter, age 17, has had systemic JRA(Still's disease) for

almost seven years. She was on enbrel, mtx, folic acid and

naprosyn. There is a rare complication that can occur with systemic

JRA, and I believe adult onset Still's as well. I think everyone

should be made aware of it. It is called Macrophage Activation

Syndrome. Its symptoms are similar to a flare, and that is the

danger. You have to move very quickly with Macrophage Activation

Syndrome(MAS) or the patient is lost. There is little known about

it. Many times an infection will throw the person into MAS. Mono,

chicken pox, and hepatis A can trigger it. The physical symptoms are

fever, sometimes vomiting, not feeling well, all kind of the same

symtoms as a flare. The lab symptoms are extremely high ferritin,

low platelets, high triglycerides and LDH.

n caught mono. The day after diagnosis we brought her to the ER

and alerted her pediatric rheumy that we were coming. Thankfully, he

was on the lookout for MAS and ordered the correct blood tests. She

was diagnosed in two hours. She still ended up in ICU for seven days

on a ventilator fighting for her life. Her lungs filled up with

fluid, around her lungs, around her heart, and her kidneys starting

going bad. She was is the hospital a total of two weeks. The

treatment for MAS is high dose steroids (she got 1000mg/day for five

days) and Cyclosporine(an organ transplant rejection drug) . If any

other doctor treated n, they would have no idea what to do.

I hope this never happens to anyone again. But I would suggest to

everyone to make sure your rheumy knows what MAS is and knows how to

quickly treat it. Adult rheumies may never have heard of this, and

it is a danger for any Still's patient. n can get it again, and

when she moves to an adult rheumy-you bet we will be careful who we

choose for her.

Take care, (mom to n, 17, still's)