Hayley O’Keefe doesn’t remember much about Thanksgiving Day in 2004. But her mother, Colleen, can recall every detail.

Soon after the family woke up that holiday morning, Colleen watched in terror as her 13-year-old daughter lost feeling in her left side, lost her speech, lost consciousness and lapsed into a coma.

All her life, Hayley had suffered from an autoimmune disease that brought on regular, high fevers and skin inflammation that led to welts and rashes. But neither her doctors nor her parents had ever witnessed the kind of crisis that Hayley was experiencing that morning.

“It was so fast,” her mother says. “Every organ in her body was involved. Her brain, liver, pancreas,” Colleen remembers. “We did think she was going to die.”

Local physicians airlifted Hayley, who is from New Jersey, to Children's Hospital of Philadelphia where she was treated by Edward Behrens, MD, a pediatric rheumatologist who has received research funding from the Arthritis Foundation since 2007, first as a post-doctoral fellow and in 2010 as the recipient of an Innovative Research Grant.

“He’s amazing. First of all he saved her life. But on top of that, he’s just really compassionate and really cares,” Colleen says about Dr. Behrens.

A Challenging Diagnosis, Too Often Missed

The O’Keefes consider themselves lucky that they found a doctor who recognized what was happening, which turned out to be a mysterious and rare condition called macrophage activation syndrome, or MAS.

Little is known about MAS except that it can kill within hours and that it tends to strike about 10 percent of children who have systemic juvenile idiopathic arthritis, or JIA. (Though it is most commonly seen in children with JIA, cases have also been described in adults and children with other kinds of rheumatic and autoimmune diseases.)

Dr. Behrens believes MAS was first recognized in the medical literature as culture-negative sepsis because it shared many of the same characteristics as the life-threatening, body-wide infection, in which bacteria overwhelm the bloodstream.