Sarah Goss can't eat meat, cheese or even ice cream.
The 17-year-old Jasper, Ga., resident isn't on a restrictive diet to lose weight. She isn't a vegetarian or vegan. And she doesn't have an eating disorder. Sarah can't eat these foods and others high in protein because she was born with a metabolic nutritional disorder called phenylketonuria or PKU.
PKU, Maple Syrup Urine Disease (MSUD) and Isovaleric Acidemia (IVA) are metabolic disorders that affect how the body breaks down protein. For people with PKU, MSUD or IVA, eating too much protein found in meat, poultry, fish or dairy products, could have devastating consequences including irreversible brain damage or death. They must adhere to a low-protein diet for the rest of their lives consisting mainly of a medical formula, fruits and vegetables.
"A typical day in my life is not much different from anyone else's," Sarah says. "I just can't eat meat and some other foods high in protein."
One of the biggest challenges though for Sarah and her family has been figuring out what she can add to her diet besides the special formula, which she drinks twice a day. They've received quite a bit of help over the years from a team of nutritionists and geneticists at Emory University, who staff an annual summer camp for young women living with metabolic disorders.
Sarah has attended the Metabolic Camp for the last six years. This summer she'll join 31 campers, ranging in age from 12 to 51, for the camp scheduled June 18-23 on the Emory campus. The campers will participate in sports activities and local field trips. They'll also receive intensive education from a team of Emory nutritionists and genetic counselors, and have the chance to try out healthy recipes that fit into their restricted diets. The recipes have all been carefully tested.
"The Metabolic Camp has introduced me to foods that I can eat and taught me ways to stick with my diet," she says. "The most important lesson I've learned at camp would be to never go off my diet!"
"We've noticed a tremendous effect on patients, and a decrease in isolation just during the one week of camp," says Rani H. Singh, PhD, RD, camp director and assistant professor of genetics in the Emory School of Medicine. "We're working to develop a national system of networking and resources to continue beyond camp."
As a biochemical nutritionist, Dr. Singh and her staff provide nutrition management to every PKU and MSUD patient in Georgia from birth to childhood. After years of addressing the special needs of afflicted children and their parents, Dr. Singh created the model, research-based metabolic camp in 1995 for young female patients.
Most PKU, MSUD and IVA children can't attend conventional camps because they're not set up to meet their special dietary needs, Dr. Singh says. And although many boys in Georgia live with these disorders, the Emory camp targets teenage girls since they are at high risk for delivering severely compromised babies should they become pregnant, she adds.
For Sarah, the annual camp has been life changing.
"My parents won't always have to see that I'm taken care of or worry that I'm eating right because I'm learning to be independent and responsible for myself," she says.
But other than some dietary restrictions, Dr. Singh says teens like Sarah with metabolic disorders can lead normal, healthy lives if they have the information and resources to manage their disorder.
"Ultimately, we want to impact the quality of life for the campers," says Dr. Singh. "As they enter adulthood, they need to develop diet self-management skills and become more independent."
For more information about the Metabolic Camp and other innovative programs available to individuals living with metabolic disorders, contact the Emory Genetics Metabolic Nutrition Program at 404-778-8521.