Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance in Patients With Cystic Fibrosis

Study Overview
=================
Brief Summary
-----------------
The primary aim of the study is to evaluate upper extremity exercise capacity, muscle oxygenation, balance and physical activity level in patients with cystic fibrosis.. The secondary aim of the study is assessment of functional exercise capacity, respiratory function, peripheral and respiratory muscle strength, respiratory muscle endurance, quality of life in patients with cystic fibrosis and compare them with healthy controls.

Detailed Description
-----------------
Cystic fibrosis (CF) is an autosomal recessive genetic disease. Although it is a monogenetic type of disease, its phenotype varies widely. CF is characterized by progressive lung disease, malabsorption of fat and protein, fatty stools, pancreatic insufficiency resulting in gastrointestinal malabsorption, intestinal abnormalities resulting in malnutrition, growth retardation, sinusitis, and diabetes. The most important problems seen in patients are excessive secretion, decreased exercise capacity, dyspnea and muscle strength loss. Exercise capacity, respiratory functions, respiratory and peripheral muscle strength, respiratory muscle endurance, balance, physical activity, quality of life are poor in cystic fibrosis patients.There are insufficient studies on this subject in the literature. The number of studies using objective method is few. Cystic fibrosis patients have upper extremity muscle weakness. There is no study on the evaluation of upper extremity exercise capacity in these patients in the literature. Even if patients with CF have normal pulmonary function test results, there are studies showing that exercise intolerance has developed. These reasons have revealed the need to investigate oxygen metabolism at the cellular level.There are only two studies in the literature evaluating muscle oxygenation in patients with cystic fibrosis. There is no study evaluating with a Moxy monitor.The primary aim of the study is to evaluate upper extremity exercise capacity, muscle oxygenation, balance and physical activity level in patients with cystic fibrosis.. The secondary aim of the study is assessment of functional exercise capacity, respiratory function, peripheral and respiratory muscle strength, respiratory muscle endurance, quality of life in patients with cystic fibrosis and compare them with healthy controls. The study was planned cross-sectional. At least 30 cystic fibrosis patients and 30 age- and sex-matched healthy controls will be included in the study. Individuals' exercise capacity, respiratory functions, physical activity levels, balance assessments, peripheral and respiratory muscle strength, respiratory muscle endurance, muscle oxygenation and quality of life will be evaluated. Upper extremity exercise capacity will be assessed using six minute pegboard ring test, functional exercise capacity using six minute walk test, muscle oxygenation using Moxy monitor, balance using Biodex Balance System® and Y balance test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using The Revised Cystic Fibrosis Questionnaire (CFQ-R) (Turkish version). The assessments will be completed in two days.

Official Title
-----------------
Investigation of Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance and Physical Activity Level in Patients With Cystic Fibrosis

Conditions
-----------------
Cystic Fibrosis

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Patients; 6-18 years old Diagnosed with cystic fibrosis according to the American Cystic Fibrosis Association consensus report criteria Those who have not participated in the exercise training program planned in the last 3 months Healthy controls; 6-18 years old Exclusion Criteria: Patients; A history of lung or liver transplant Diagnosed vision, hearing, vestibular, or neurological problems that can affect balance History of hospitalization previously (1 month) History of coronavirus disease (COVID-19) History of smoking Diagnosed orthopedic problems affecting mobility or a history of musculoskeletal surgery Acute pulmonary exacerbation Diagnosed with allergic Bronchopulmonary aspergillosis Systemic corticosteroids use Pulmonary hypertension and cardiovascular instability Healthy controls; Trouble understanding and following the exercise test instruction A history of smoking

Ages Eligible for Study
-----------------
Minimum Age: 6 Years
Maximum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients<br>Upper extremity exercise capacity will be assessed using six minute pegboard ring test, functional exercise capacity using six minute walk test, muscle oxygenation using Moxy monitor, balance using Biodex Balance System® and Y balance test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using The Revised Cystic Fibrosis Questionnaire (CFQ-R) (Turkish version). | |
| Healthy controls<br>Upper extremity exercise capacity will be assessed using six minute pegboard ring test, functional exercise capacity using six minute walk test, muscle oxygenation using Moxy monitor, balance using Biodex Balance System® and Y balance test, physical activity using multi-sensor activity monitor, pulmonary function using spirometry, respiratory muscle strength using mouth pressure device, peripheral muscle strength using hand held dynamometer, respiratory muscle endurance using incremental threshold loading test, life quality using The Revised Cystic Fibrosis Questionnaire (CFQ-R) (Turkish version). | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Upper extremity exercise capacity | Upper extremity exercise capacity will be evaluated with the 6-Minute Pegboard and Ring Test (Total number of rings). There are a total of 20 rings on the 4 iron bars on the board to be used. Patients will be asked to wear the rings with both hands first from top to bottom and then from bottom to top. At the end of 6 minutes, the total attached ring will be recorded in terms of pieces. | First Day | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Functional exercise capacity | Functional exercise capacity will be evaluated with the 6- Minute Walk Test. 6- Minute Walk Test will be applied according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria. | First Day | 
| Muscle Oxygenation | Muscle oxygenation will be evaluated with the Moxy monitor (Moxy, Fortiori Design LLC, Minnesota, ABD). For measurement, Moxy monitor will be placed bilaterally at the 1/3 lower motor point of the quadriceps muscle group and on the deltoid muscle. A minimum of 3 minutes will be waited until the resting measurements and the skeletal muscle oxygenation (StO2) signal stabilize. The values (StO2 and total hemoglobin) will be recorded after the 6-minute walking test and the 6-Minute Pegboard and Ring Test. Data will be analyzed appropriately. | First Day | 
| Balance | Static balance will be evaluated with the Biodex Balance System®. Dynamic balance will be evaluated with the Y balance test. The data obtained from the system as a result of the test will be the general stability index, anterior / posterior (AP) stability index, medial / lateral (ML) stability index and their standard deviations. The tests will first be carried out on the hard ground with open eyes and then repeated on the soft ground. The measurements will then be repeated with eyes closed on hard and soft ground. | Second Day | 
| Physical activity (Total energy expenditure) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Total energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Active energy expenditure (joule / day)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Active energy expenditure (joule / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Physical activity time (min / day)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Physical activity time (min / day)will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Average metabolic equivalent (MET / day)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Average metabolic equivalent (MET / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Number of steps (steps / day)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Number of steps (steps / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Time spent lying down (min / day) days)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Time spent lying down (min / day) days) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Physical activity (Sleep time (min / day)) | Physical activity will be evaluated with the Multi sensor activity monitor (SenseWear®, Inc Pittsburgh, ABD). The patient will wear the multisensor physical activity monitor over the triceps muscle of the non-dominant arm for 3 continuous days. The patient will be informed about removing the device while taking a bath. Sleep time (min / day) will be measured with the multi-sensor physical activity monitor. The parameters measured over two days will be averaged and analyzed with the SenseWear® 7.0 Software program. | Second Day | 
| Pulmonary function (Forced vital capacity (FVC)) | Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to ATS and ERS criteria. With the device, forced vital capacity (FVC) will be evaluated. | First Day | 
| Pulmonary function (Forced expiratory volume in the first second (FEV1)) | Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to ATS and ERS criteria. With the device, forced expiratory volume in the first second (FEV1) will be evaluated. | First Day | 
| Pulmonary function (FEV1 / FVC) | Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to ATS and ERS criteria. With the device, FEV1 / FVC will be evaluated. | First Day | 
| Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%)) | Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to ATS and ERS criteria. With the device, flow rate 25-75% of forced expiratory volume (FEF 25-75%) will be evaluated. | First Day | 
| Pulmonary function (Peak flow rate (PEF)) | Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to ATS and ERS criteria. With the device, peak flow rate (PEF) will be evaluated. | First Day | 
| Respiratory muscle strength | Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength will be measured with a portable mouth pressure measuring device according to ATS and ERS criteria. | First Day | 
| Peripheral muscle strength | Isometric peripheral muscle strength will be measured with a portable hand dynamometer (JTECH Commander, USA). Measurements will be repeated on the shoulder abductors and knee extensors three times on the right and left. | Second Day | 
| Respiratory muscle endurance | Respiratory muscle endurance will be assessed by the POWERbreathe Wellness (POWERbreathe, Inspiratory Muscle Training (IMT) Technologies Ltd., Birmingham, UK) device and the respiratory muscle endurance test at increased threshold load. The test will be started with 20% of the maximal inspiratory pressure and the pressure will be increased to 40%, 60%, 80% and 100% every two minutes. Patients will be asked to continue breathing through the device during the test. During the test, the number of breaths delivered and the maximal time reached during each 2-minute period will be recorded. If the individual cannot breathe 3 consecutive times, the test will be terminated by the physiotherapist. The total duration of the test and the maximum pressure value at which it continues to breathe for at least 1 minute will be multiplied. The value found will be recorded as the respiratory muscle endurance value. | Second Day | 
| Life quality | Quality of life will be evaluated with the Turkish adaptation of the Revised Cystic Fibrosis Questionnaire (CFQ-R). The scale have well-established reliability and validity. There are four CFQ-R questionnaires for patients of three different age groups (6-10, 12-13, and 14 years and older). The CFQ-R children's version consists of 35 questions. Scale subtitles; It consists of physical functioning, emotional functioning, social functioning, body ımage, eating disorders, treatment burden, and respiratory and digestive symptoms. Items for child CFQ-R are rated according to a 4-point scale. Higher scores indicate better quality of life. | Second Day | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
cystic fibrosis, upper extremity exercise capacity, muscle oxygenation, balance, physical activity