It is anticipated that Oklahoma would detect three affected infants through newborn screening annually. Due to an enzyme defect in fat metabolism, affected children must use glucose as an alternative energy source instead of stored fat. This results in a severe intolerance to fasting and possible hypoglycemic crisis that can lead to seizures, respiratory arrest, cardiac arrest, and death. This disorder often goes undetected until a hypoglycemic crisis occurs. The goal of newborn screening is to detect affected infants in the newborn period and provide education to parents on the risk of hypoglycemic crisis during fasting, stress, and illness.
Treatment includes frequent feedings, and a diet low in fat and high in carbohydrates.
Early detection with dietary treatment allows these children to lead normal lives. It is anticipated that Oklahoma would detect four affected infants through newborn screening annually. Such issues as cost and comprehensive follow-up services for affected infants will need to be delineated before an effective expanded screen in Oklahoma is achieved. The council has 26 members and 17 ex-officio members from Oklahoma State Department of Health and Department of Human Services. The OGAC has six standing committees, with 82 participating members.