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Sunday, May 1, 2016

Pro athletes, residents join together to fight Krabbe Disease

Thursday, August 28, 2003

Football players and racecar drivers seem an unlikely group to champion the cause of infants. But Pro Football Hall of Famer and former Buffalo Bills quarterback, Jim Kelly, and National Hot Rod Association's Top Fuel Dragster, David Baca, will be in Indianapolis over Labor Day weekend to do just that.

They'll be available to sign autographs at 4:30 p.m. Saturday and 3:15 p.m. Sunday in the American Racing Wheels T-shirt Trailer at Raceway Park, which is one mile west of Clermont on U.S. 136 (Crawfordsville Road).

Kelly will be there to promote the Hunter's Hope Foundation. The organization was established in 1997 by Kelly and his wife, Jill, after their son, Hunter, was diagnosed with Krabbe Disease.

Baca will be driving the Top Fuel car this weekend. He had the entire car painted for Hunter's Hope with a picture of Hunter on the side.

Brazil residents Daniel and Stacy Frye, will be attending the festivities with their daughter, Leandra, to meet Kelly and Baca. Their little 2 1/2-year-old girl was born with Krabbe Disease.

Daniel and Stacy noticed breathing and feeding difficulties in their daughter when she was just six weeks of age. It took weeks of repeated trips to their doctor then to Riley Hospital for a large battery of tests before a diagnosis was made.

Unfortunately, those weeks of delay may have cost Leandra a chance at a normal life. Krabbe is a genetic disease transferred by both parents. Any children conceived by carrier parents have a 1 in 4 chance of being affected.

This disease is found in all ethnic groups. The carrier rate in the general population is estimated to be 1 in 150. One in 100,000 live births in the United States are afflicted with Krabbe Disease. It is frequently misdiagnosed, but diagnosis can easily be made by testing the white cells from a blood sample.

Krabbe is a rare, degenerative enzyme disorder of the central and peripheral nervous system. Children who inherit the disease lack an important enzyme (GALC) that is a component of the white matter of the brain (myelin). This enzyme deficiency produces toxins in the brain, causing myelin loss and neurological damage. Progression of the disorder is rapid and death usually occurs in early childhood. The average age of death is 13 months.

Signs and symptoms of Krabbe may include colic, extreme irritability, stiffness, fevers, reflux, feeding difficulty, arrest of motor and mental development and loss of milestones. Early detection is crucial to a child's survival.

Thanks to a new, revolutionary treatment called a cord blood transplant, the lives of some children with Krabbe may be saved. However, the treatment is only effective when coupled with early detection. Most cord blood transplants are done at Duke University Medical Center in Durham, North Carolina.

According to a brochure, "Krabbe Disease, New Treatment, New Hope", newborn screening for Krabbe Disease is necessary at birth for detection and for the opportunity for effective treatment.

Babies transplanted as early as two to four weeks of age have had a high success rate with cord blood or bone marrow transplant treatment.

Research studies indicate that the use of transplantation therapy early in life provides healthy donor cells which produce enzyme in the blood and brain enabling children to live normal to near normal, healthy lives. Hunter's Hope is fighting for the institution of mandatory Newborn Screening for Krabbe Disease. Kelly has helped raise more than $3.5 million for the organization.

The cord blood transplant, if successful and done early enough, may prevent the neurological destruction caused by Krabbe. Even when it's done after damage has started, it may stop further damage from occurring, but it has not yet been able to reverse damage already done.

Leandra was not expected to live when she was first diagnosed. She was seven months old when she received her treatment.

Unlike her sisters, Lindsay, 7, and Lauren, four months, Leandra may never run, ride a bike and play childish games. But she is making new strides every day. Her parents are thrilled and will be in Indianapolis this weekend, with Leandra, Jim Kelly and David Baca to promote Hunter's Hope and the hope of every parent of a child with Krabbe Disease.

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