Upon joining the study, initial assessments will be conducted to establish baseline measurements. These assessments serve as reference points for comparison throughout the trial.

Various evaluations will be performed, including physical measurements such as height, weight, and body mass index (a calculation based on height and weight). An echocardiogram (a heart ultrasound test) will be conducted to assess heart function.

Functional assessments will be completed, including the modified Friedreich Ataxia Rating Scale, which measures movement and coordination abilities. The Upright Stability Sub-score specifically evaluates balance and stability while standing.

Additional questionnaires will be administered to assess daily living activities, overall health impression, and developmental stage using the Tanner scale (a measure of physical development).

This part of the study lasts 52 weeks (approximately one year). During this period, either omaveloxolone or placebo (an inactive substance with no medication) will be administered. The assignment to either group is random, and neither the participant nor the study team will know which treatment is being received.

The medication is provided as hard capsules containing 50 mg of omaveloxolone (or matching placebo). The capsules are taken orally (by mouth).

Regular visits will occur throughout this 52-week period to monitor progress and safety. At these visits, the same assessments performed at baseline will be repeated to track any changes.

Blood samples will be collected at specific timepoints to measure the levels of omaveloxolone in the bloodstream.

Safety monitoring will include tracking any side effects or adverse events that occur during treatment. Heart function will be reassessed through echocardiograms, and physical measurements will be repeated.

Questionnaires about suicidal thoughts or behaviors (Columbia-Suicide Severity Rating Scale) will be administered as part of safety monitoring.

Participants who complete Part 1 and meet safety criteria may continue into Part 2. In this phase, all participants will receive omaveloxolone (no placebo), and both the participant and study team will know that active medication is being administered.

The medication will continue to be provided as 50 mg hard capsules taken orally. The duration of this extension phase is designed to evaluate long-term safety and effectiveness.

Regular monitoring visits will continue throughout Part 2. The same types of assessments performed in Part 1 will be repeated, including movement and coordination scales, daily living activities questionnaires, and overall health impression ratings.

Safety assessments will remain a primary focus, with ongoing tracking of any side effects or adverse events. Echocardiograms will be repeated to monitor heart function over the long term.

Physical growth will continue to be monitored through height, weight, and body mass index measurements. Developmental stage assessments using the Tanner scale will be repeated.

The Columbia-Suicide Severity Rating Scale will continue to be administered to monitor mental health and safety.