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

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.

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.

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