Keto Diet is the best treatment for GT1 Deficiency [2020]

The ketogenic diet is currently the only known treatment for children with Glucose transporter 1 deficiency syndrome. It is also used on a short-term basis to treat other types of seizure disorders. The ketogenic diet is a special high-fat diet that relies on oils, heavy cream, butter, and mayonnaise to provide the necessary fat to produce ketone bodies. These ketone bodies help to control seizures. The ketogenic diet is also being used as a treatment for cancer, ALS, Alzheimer’s and dementia.

keto diet for GT1 deficiency

Why does the keto diet for GT1 deficiency

Why does this diet work for kids with Glut 1-DS? The human body normally burns glucose (sugar) for most of its energy. In children with Glut 1-DS, glucose does not make its way to the brain to provide fuel for it to function properly because of the defective transporter named Glut 1. Ketones can reach the brain, however, providing the energy the brain needs to function and to control seizures. When fat breaks down in the body, it forms ketones; hence the effectiveness of this extremely high-fat way of eating.

In its most restrictive form – typically required for people with Glut 1-DS – the ketogenic diet totally eliminates sweets, such as candy, cookies, cakes and other desserts. In addition, carbohydrate-rich foods such as bread, potatoes, rice, cereal and pasta are not allowed. All other foods such as proteins (meat/bean/nuts), fruits and vegetables are limited. All the foods eaten must be consumed with 4-5 times the amount of fat as there are protein and carbohydrates combined. The fats need to be taken simultaneously with any other foods. They cannot be taken at another time.

Think that sounds like a diet you would want to be on? Consider that to reach that ratio, kids often have to swallow spoonfuls of mayonnaise or vegetable oils – plain. All meals need to be weighed on a scale and each food item is measured in grams. Even medications such as pills and lotions have to be monitored for their carbohydrate counts. When kids with Glut 1-DS are sick they must rely on IV fluids and IV medications that do not affect blood glucose levels.

How long can the Keto diet help the patients?

It is unknown how long the ketogenic diet will continue to control seizures and remain active as it has rarely been used long-term (most children are on the menu for 5-10 years at the longest). What is known is that the diet is certainly not without risk and harm. Because of the nutritional deficits children on the ketogenic diet experience, they often require supplements such as calcium, alpha-lipoic acid, and Carnitine and a sugar-free multivitamin to name just a few. Given the high-fat nature of the diet, blood lipids need to be measured regularly, and high cholesterol is a concern.

Children on a diet can experience kidney problems, including the development of painful kidney stones. Maintaining the food can be psychologically gruelling and physically agonizing as well. The strict nature of the diet makes eating out in public – at restaurants, birthday parties and family gatherings – a challenge both logistically and psychologically for kids who just want to fit in with their peers. When weighed against the alternative, however – near-constant seizures and severe developmental regression – the ketogenic diet currently offers the best course of treatment for children with Glut 1-DS.

 
Next Post

About The RSG1 Foundation

Leave a Reply

Your email address will not be published. Required fields are marked *