Organic acidemia is an inherited metabolic diseases due to the deficiency of an enzyme or a transport protein involved in one of the several cellular metabolic pathways devoted to the catabolism of amino acids, carbohydrates, or lipids. Collectively, their incidence approximates 1 out of 3000 live births. Moreover, the demanding medical management of organic acidemia patients often dominates the efforts of their metabolic teams, impacting on time dedicated toward addressing aspects of care that could enhance functional independence and improve quality of life. While most published data on long-term organic acidemia outcomes have focused on medical endpoints such as survival and neurocognition, there have been fewer efforts to document functionality, independence, and social integration or the need for rehabilitation interventions that could maintain and maximize function. It's understood that delivering one form of meta-cognitive strategy training, the CO-OP approach, using a telerehabilitation format is feasible and shows similar positive effects to face-to-face delivery.~It was planned to include children between the ages of 7-12 who were diagnosed with organic acidemia in this study. In consequence of the statistical power analysis, the number of individuals to participate in the study was determined as 34 children diagnosed with organic acidemia, including 17 in the intervention group and 17 in the control group. The study will be conducted in a randomized controlled method. Children will be divided into intervention and control groups by using a computer-assisted online randomization application that provides an equal number and probability. Participants will be randomly numbered and randomly assigned to the intervention or control group without repeating the same number with the online randomization application available at randomizer.org. Both groups will be given a home program. Home programs will be prepared and sent to the person via e-mail. The Cognitive Orientation for Daily Activity Performance (CO-OP) Approach to the intervention group will be applied by video conferencing method in a total of 10 sessions, 2 sessions per week, through 5 weeks, and a total of 12 sessions with initial and final evaluations.~The children to be included in our study will be determined among the children with organic acidemia who are directed from Hacettepe University Metabolism and Nutrition Department. The contact information of the volunteers who agree to participate in the study will be given to the occupational therapist. The occupational therapist will provide information on how to download and use the video interview program called Zoom on his/her phone or computer. The session schedule will be determined after the program is set up. The program named Zoom was preferred because it is free, easy to install, and accessible compared to the other video conference programs.~The informed consent form will be sent to the individuals online by using Google Forms. After the participants approve the consent form, they will reach the evaluations to be prepared on Google Forms. One of the evaluation scales, the Canadian Occupational Performance Measure (COPM) will be made over the video interview. If they do not want to approve the consent form, they will be exempted from the study by checking the I do not approve option.~In this study, Canadian Occupational Performance Measure (COPM), Pediatric Evaluation of Disability Inventory (PEDI), The Child and Adolescent Scale of Participation (CASP), Quality of Life Questionnaire for Children (Kid-KINDL), Assistance to Participate Scale, and Sociodemographic Questionnaire will be implemented.
The aim of this study is to research the effect of Cognitive Orientation to daily Occupational Performance (the CO-OP Approach) on daily life activities, quality of life and participation in children with organic acidemia.~The hypotheses of the study are as follows:~Cognitive Orientation to daily Occupational Performance (the CO-OP Approach) applied to children with organic acidemia has no effect on daily life activities.~Cognitive Orientation to daily Occupational Performance (the CO-OP Approach) applied to children with organic acidemia has no effect on quality of life.~Cognitive Orientation to daily Occupational Performance (the CO-OP Approach) applied to children with organic acidemia has no effect on participation.