Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Individuals

Physiological Responses to Exercise Tests in Primary Ciliary Dyskinesia Compared With Healthy Children and Investigation of the Relationship Between Exercise Capacity and Quality of Life in Primary Ciliary Dyskinesia

The individuals with primary ciliary dyskinesia (PCD) have lower aerobic fitness and anaerobic performance than healthy individuals. Cardiopulmonary exercise test (CPET) provides an integrated assessment of integrative exercise responses involving the pulmonary, cardiovascular, hematopoietic, neuropsychological, and skeletal muscle systems that are not adequately reflected by measurement of the function of organ systems. Maximum oxygen consumption is measured by performing gas exchange analysis with CPET, offering an objective measure of cardiorespiratory fitness. The six-minute walk test (6MWT) and the shuttle walk test (SWT) are field tests that produce oxygen consumption results similar to those during CPET. There is a need to determine the physiological responses to CPET, SWT and 6MWT in individuals with PCD. Additionally, the physiological responses of individuals with PCD to different exercise tests will be compared with the responses of healthy individuals. Low aerobic capacity in individuals with PCD may affect the physical, emotional, and social lives of individuals. This influence may cause a decrease in the quality of life of individuals with PCD. The relationship between exercise capacity and quality of life in individuals with PSD will be investigated with this study.

Inclusion Criteria:

• Having been diagnosed with PCD in the Pediatrics Department, Pediatric Chest Diseases Unit of Hacettepe University Faculty of Medicine and being routinely referred to Hacettepe University, Faculty of Physical Therapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit for physiotherapy applications and pulmonary rehabilitation program,
• Being clinically stable,
• Being between 8-18 years old,
• Having a forced expiratory volume in the first second (FEV1) ≥ 40%,

The healthy group will be composed of individuals who do not have any known disease and volunteer to participate in the study. For healthy individuals, volunteers from acquaintances and/or relatives of the researchers will be included in the study.

Exclusion Criteria:

• Having unstable clinical condition
• Having severe neuromuscular and musculoskeletal problems,
• Having any congenital heart diseases other than situs inversus,
• Unable to cooperate