Assessment of Cognitive Function and Exercise Capacity in Cystic Fibrosis

Exercise capacity is assessed using the 6-minute walk test (6MWT). The 6MWT is performed according to the criteria of the American Thoracic Society.

Arterial stiffness is determined using a oscillometric cuff device (Tel-O-Graph BT, I.E.M., Stolberg, Germany). Pulse wave velocity (PWV) and augmentation index (AIx) are measured by brachial pulse waves.

Cognitive function is measured using the Montreal Cognitive Assessment (MoCA) test. The MoCA test is a questionnaire developed to evaluate different cognitive functions and mild cognitive impairment. The maximum score that an individual can obtain from the test is 30, and a score of 26 above is considered as mild cognitive impairment.

Cognitive function is measured using the Mini Mental Test. A higher score indicates better cognitive function. The maximum score that an individual can obtain from the test is 30, and a score of 23 and above is considered as cognitive impairment.

Muscle oxygenation (SmO2) is assessed using a measuring and monitoring device (Moxy Fortiori Desing LLC, Minnesota, USA).

Body fat is measured using skinfold thickness assessment.

Testing procedures is in accordance with the guidelines published by ATS/ERS. A portable spirometer is used for assessment. Pulmonary function testing assesses forced vital capacity (FVC).

Testing procedures is in accordance with the guidelines published by ATS/ERS. A portable spirometer is used for assessment. Pulmonary function testing assesses forced expiratory volume in the first second (FEV1).

Testing procedures is in accordance with the guidelines published by ATS/ERS. A portable spirometer is used for assessment. Pulmonary function testing assesses FEV1/FVC.

Testing procedures is in accordance with the guidelines published by ATS/ERS. A portable spirometer is used for assessment. Pulmonary function testing assesses peak flow rate (PEF).

Testing procedures is in accordance with the guidelines published by ATS/ERS. A portable spirometer is used for assessment. Pulmonary function testing assesses forced expiratory flow from 25-75% (FEF25-75%).

Maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) are measured using a mouth pressure measuring device with personal antibacterial and antiviral disposable filters.

Knee extensor muscle strength is measured utilizing a portable device.

The Hospital Anxiety and Depression scale (HADS) is used to evaluate anxiety and depression. Higher scores correspond to more anxiety and depression symptoms.

Physical activity levels is assessed using the International Physical Activity Questionnaire-Short Form (IPAQ-SF). The IPAQ requires respondents to estimate time spent in various levels of physical activity during the previous week. Scores for walking and moderate and vigorous activities are calculated as durations and frequencies multiplied by known metabolic equivalents per activity. The results for all activity-based items are summed for the total physical activity score. The participants were then categorized as inactive, minimally active and sufficiently active according to the IPAQ categorical classification.

Sleep quality is evaluated using the Pittsburgh Sleep Quality Index (PSQI). The scale total score is between 0 and 21. A total score of 5 and above indicates poor sleep quality.

Disease-specific quality of life is evaluated using the Cystic Fibrosis Questionnaire-Revised (CFQ-R). The CFQ-R includes 50 items across 12 domains. Each domain is standardized on a 0-100 scale; higher scores indicate better quality of life.

Health-related quality of life is assessed using the Nottingham Health Profile (NHP). It is a general QOL questionnaire designed to measure perceived health problems and the extent to which these problems affect normal daily activities. It consists of 38 items in six dimensions (energy level, pain, physical mobility, emotional reactions, social isolation, and sleep). The total score for each section is 0-100. High scores indicate worse quality of life.