The purpose of the study is to determine if an oral ketone beverage is safe and well-tolerated during moderate intensity exercise in participants with long-chain fatty acid oxidation disorders and if it will raise blood ketones to levels similar to that reported among normal healthy subjects.

Purpose: Subjects with long-chain fatty acid oxidation disorders (LC-FAOD) do not make ketones during fasting or with exercise. Ketones are an important alternative energy substrate during moderate exercise, sparing the oxidation of glucose and providing a source of ATP to the central nervous system and exercising muscle. Fatty acid oxidation in the liver is required to make ketones. Subjects with a LC-FAOD cannot generate ketones because of their block in fatty acid oxidation during exercise. Providing ketones in an oral ketone beverage may increase blood ketones with exercise to levels normally observed in humans.

Aim: To determine the safety and tolerability of an oral ketone beverage during moderate intensity exercise among subjects with a LC-FAOD compared to an isocaloric maltodextrin beverage, and to determine blood ketone concentrations.

Hypothesis: Oral consumption of a ketone beverage before moderate intensity exercise will be safe and well-tolerated, and will raise blood ketones among subjects with a LC-FAOD to concentrations similar to that reported in the literature among normal healthy subjects.

Inclusion Criteria:

• confirmed diagnosis of VLCAD, LCHAD/TFP or CPT2 deficiency
• speak English
• willing to complete 2 moderate intensity exercise treadmills

Exclusion Criteria:

• subjects actively participating in another research study that prohibits their participation
• pregnant females
• subjects with diabetes or taking medications to treat diabetes

Participants will be provided with creatine kinase lab test and body composition measurement. The results of their creatine kinase lab test will be placed in their medical record. Results of the study will be written in a published manuscript and summarized in a cover letter to be sent via mail or email to the participant after publication of the data.

The published manuscript will be available approximately 6-9 months after the conclusion of the study.

not applicable. A reprint will be sent to participants.

Sachets of the ketone or maltodextrin powder are coded and packaged identically. The bionutrition unit will prepare the beverage and maintain the randomization code and the code for the ketone and maltodextrin products.