Rare disease bonds families
COTTAGE GROVE, Minn. -- Jennifer Fair said she tells her 10-year-old son that she loves him and that he is "my perfect boy."
She sees Alex, not the disease that will eventually take his life.
Most nights, Alex has trouble staying asleep. When he's having a bad night, Erik, does what most fathers will never have to do. He sleeps next to son's bed and when Alex tries to get up, Eric puts Alex's leg back into the bed and goes back to sleep.
Alex was born without an enzyme essential to maintain life. Every day, human bodies make new cells and break down old ones, but Alex's body can't do that.
There is a chain of enzymes that helps sustain life, but Alex is missing one of the crucial links and that affects all the enzymes downstream, Erik said in an interview last week with the Fair family of Cottage Grove and the Kell family of St. Paul Park. Like Alex Fair, the Kells' son Jack, age 3, has Sanfilippo syndrome.
The families are organizing a fundraising one-mile fun run, "Rays of Hope," for Sunday, Sept. 15, at Hamlet Park in Cottage Grove.
"We want to raise money to find a cure for children in future generations," Jennifer Fair said. "It's about hope."
The condition, rare and hard to diagnose, occurs in one child for every 100,000 births. It's genetic and referred to as an "orphan disease," in that it is too rare to get major funding to find a cure until recently when it was officially acknowledged by the federal Food and Drug Administration.
Bone marrow transplants and enzyme replacement therapies have not worked and life expectancy, as cells build up, is between 10 and 20 years.
So far, Alex isn't as far into the progression as many children are with the same disease. Erik and Jennifer believe Genistein, a soy protein, is slowing down the disease. The Kells are also giving it to Jack.
Hope takes many forms and Alex's family takes heart that, while non-verbal, he is staving off the need for oxygen or to use a wheelchair.
The eventual cause of death, if it's not from complications, is that a child forgets to breathe, Erik said.
Stephanie and Jeff Kell support the Fairs by commenting on how well Alex is doing compared to other children his age with the syndrome.
Their son, Jack, is at the active stage of the disease where he loves to jump and move around. He loves the mini-trampoline in the Fairs' family room.
One minute, a book will interest Jack and then he races from room to room.
Used to the sudden movements caused by the disease, Jennifer didn't blink when Jack bumped into a side table.
"Walmart, $15," she reassured Stephanie.
The Fairs live a life based on religious faith but it would be hard to take Alex to church. Five Oaks Community Church in Woodbury makes it easy, however, by providing a class for special needs children. "It makes you ask yourself why everyone doesn't do that," Erik said.
Both families said they are very grateful for the help of teachers in Early Childhood and Family Education, and special education teachers at Cottage Grove Elementary School.
The two families met nearly a year ago and immediately became friends. In addition to their sons, they have much in common. Both families have two daughters older in addition to their sons and dogs.
"We have the same wedding rings," said Jennifer and Stephanie in unison.
Finding solace in mutual acceptance of their sons' disease, they take time to laugh about behavior others might not approve.
Alex can be very quick, Erik said, demonstrating how he grabbed a cookie from an adjoining table at McDonalds. The Kells smiled and responded with a story about Jack.
People who have not experienced what it's like to live with a special needs child don't understand, according to Jennifer.
"It's not all about sadness," she said.
The Fair and Kell families are hosting "Rays of Hope," a one-mile family walk from 11:30 a.m. to 2:30 p.m. on Sunday, Sept. 15, at Hamlet Park in Cottage Grove.
There will be kids activities, live music, raffle and a silent auction. The event will raise money for research into a cure for Sanfilippo syndrome.
The cost is $15 per person with a maximum of $50 for a family of four or more. T-shirts are available for the first 200 people who register at http//www.mpsociety.org/?ee=111.
For more information, e-mail firstname.lastname@example.org.
If you can't attend but want to donate, please visit http://www.mpssociety.org/?ee=111.