October 6, 2005

Couple creates a support group after their child dies
SUMMARY: Disease
West Linn parents' efforts help and inform others about a rare genetic disorder

After their youngest daughter, Darcee, died of complications following a bone marrow transplant intended to slow the disease, they started a foundation to provide information and support for families affected by metachromatic leukodystrophy, or MLD.

The condition affects the development of the myelin sheath, the fatty covering that acts as an insulator around nerve fibers. If the condition begins early, toddlers gradually lose the ability to walk and talk, and their cognitive development is stunted.

Children, such as the Suhrs' oldest daughter, Lindy, may be misdiagnosed with attention deficit disorder. Others may begin to stumble and lose muscle control. If the disease begins in adulthood, it can cause a range of muscular and cognitive problems.

"When we went through this, we were pre-Internet, and we felt truly alone," said Dean Suhr, a 46-year-old electrical engineer who has become an entrepreneur.

"We were pretty private people up to that point," focusing on family and friends at their church, he said. "After we lost Darcee, we decided it was our mission to try to reach out to others and provide information."

The West Linn couple started the nonprofit MLD Foundation in 2001, and Dean Suhr began an online discussion list that now connects more than 150 families from more than 14 countries. They call the foundation "a listening ear and a caring heart for families affected by metachromatic leukodystrophy."

The foundation is one of several organizations participating in Meier & Frank's 25th Charity Shopping Day on Oct. 25 in the remodeled Washington Square store. Participating organizations will share proceeds from the event, Teryn Suhr said.

Suhr knows of fewer than 10 MLD cases in Oregon and southern Washington, but 10 years ago she knew of none.

The Suhrs had struggled for seven years with Lindy's baffling behavior, beginning when she was a second-grader having difficulty following all the steps in assignments. By the fifth grade, she was diagnosed with attention deficit disorder, but medication failed to improve her symptoms.

As she entered her teens, doctors were trying to determine what psychosis she might have. Suhr feared that her daughter had a brain tumor because she complained that her head was spinning, as if she were on a merry-go-round. The mother urged the doctors to do a CAT scan, which revealed unusual white patterns in her brain that might be myelin sheath problems.

A Portland physician who had worked in Minnesota with one of the country's experts in MLD ordered blood and urine tests that confirmed Lindy's condition in July 1995, Teryn Suhr said.

Within two months, the Suhrs learned that 10-year-old Darcee also had inherited the two recessive genes that bring on the disease, although she hadn't yet shown obvious symptoms. Their middle daughter, Jclynn (Jay-cee-lin), didn't have the disease and didn't carry its recessive gene.

Jclynn, then 13, provided the bone marrow for Darcee's transplant with the Minnesota specialist. But two weeks after the surgery, Darcee reacted adversely to an anti-rejection drug. She died in 1995, shortly before Christmas.

To the amazement of her neurologist, Lindy recently celebrated her 25th birthday, still talking and walking fairly well, Teryn Suhr said. But she has begun using a feeding tube because she has trouble swallowing.

Jclynn, now 23, lives with her husband and three children in Florida, Suhr said.

The Suhrs speak of their life with MLD as a journey. MLD Foundation offers support and information for other families, they said, "on their MLD journey."

ILLUSTRATION: Color Photo by BRENT WOJAHN / The Oregonian