3 1/2-year-old dies after fight with rare fatal bone disease

TOPSHAM — A 3 1/2-year-old Topsham boy whose plight touched people throughout Maine has lost his long battle against a rare bone disease.

Derek Steeves died last week of Hurler’s Syndrome, a fatal disease marked by the body’s inability to regenerate certain enzymes and rid itself of others.

The effects include deformed bones, stunted growth and mental retardation.

Derek’s parents, Kim and Fred Steeves, and his sister, Ashleigh, were at his bedside at the University of Minnesota Hospital in Minneapolis when he died.

“It’s been a long two and a half years,” his mother said Wednesday. “We’re going to miss him very much. I miss him already.”

Mainers had rallied behind Derek, raising more than $36,000 to help pay for his treatment. Service clubs in the Brunswick and Lewiston-Auburn area held bake sales and car washes and placed donation jars at stores and restaurants.

Derek underwent two bone marrow transplants, but his own poisoned marrow regenerated itself after the first operation and the second also proved unsuccessful.

From the start, doctors said the chances that a transplant would succeed were 50-50.

Family and friends attended a private burial and memorial service Monday in Topsham.

Mrs. Steeves said Derek was, for the most part, “healthy and happy” and appeared unaware that anything was amiss.

“He handled it very well. You wouldn’t know there was anything wrong. He was like a normal 3-year-old kid, a good little kid,” she said.