Investigation of the Effects of Pulmonary Rehabilitation in Children With Primary Immunodeficiency

Investigation of the Effects of Pulmonary Rehabilitation on Exercise Capacity, Muscle Oxygenation and Physical Activity Level in Children With Primary Immunodeficiency

Primary immunodeficiencies (PID) are a heterogeneous group of diseases that occur as a result of disorders that affect the development, differentiation and/or function of various cells and building blocks in the immune system. Among the symptoms and complications of PID, pulmonary complications are very common and significantly increase the morbidity and mortality of the disease.

Study Overview

• Status: Recruiting
• Conditions: Primary Immunodeficiency
• Intervention / Treatment: Other: Inspiratory muscle training; Other: Upper extremity aerobic exercise training; Other: Lower extremity aerobic exercise training; Other: Resistant exercise training; Other: Thoracic expansion exercises

Detailed Description

Among the symptoms and complications of PID, pulmonary complications are very common and significantly increase the morbidity and mortality of the disease. Recurrent respiratory infections are often the first warning sign in some types of PID and is a cause of mortality in adults with PID. Presence of 2 or more pneumonias per year is one of the 10 warning signs of PID. Respiratory system diseases are mainly caused by acute and chronic infections. Non-infectious respiratory system diseases and complications are asthma, bronchiectasis, bronchiolitis obliterans, interstitial lung disease, granulomatous lung disease and malignancies. These diseases significantly affect the quality of life of PID patients, limiting their ability to work and their physical and social activities. Health-related quality of life in PID patients is also significantly affected by delays in the diagnosis and treatment of infections. As survival from infections increases, non-infectious pulmonary complications are more common in PID patients. Permanent lung damage is seen at a rate of 20-40%, especially in PID patients with antibody deficiency. The main causes of exercise intolerance in patients with lung disease include isolated or associated factors such as increased symptoms (fatigue and shortness of breath in the lower extremities), development of dynamic hyperinflation, peripheral muscle dysfunction, abnormalities in oxygen transport and progressive loss of physical condition, physical inactivity. There are no studies evaluating exercise capacity, respiratory and peripheral muscle strength, inspiratory muscle endurance, and muscle oxygenation in children with PID. In studies conducted with post-infectious bronchiolitis obliterans patients, it has been shown that exercise capacity is reduced in these patients. There are no studies in the literature on pulmonary rehabilitation practices and efficacy in PID patients.

The primary aim of this study: To investigate the effects of pulmonary rehabilitation on exercise capacity and muscle oxygenation in children with primary immunodeficiency.

The secondary aim of this study: To investigate the effects of pulmonary rehabilitation on physical activity level, respiratory functions, peripheral and respiratory muscle strength, inspiratory muscle endurance, shortness of breath, fatigue and quality of life in children with primary immunodeficiency.

Primary outcome measurement will be oxygen consumption (cardiopulmonary exercise test).

Secondary outcome will be muscle oxygenation (Moxy device), physical activity level (multi sensor activity device), pulmonary function (spirometer), functional exercise capacity (six-minute walk test), respiratory (mouth pressure device) and peripheral muscle (hand-held dynamometer) strength, inspiratory muscle endurance (incremental threshold loading test), dyspnea (Modified Borg Scale (MBS)), fatigue (Modified Borg Scale) and quality of life (The Pediatric Quality of Life Inventory (PedsQL)).

• Study Type: Interventional
• Enrollment (Estimated): 40
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Meral BOŞNAK GÜÇLÜ, Prof. Dr.; Phone Number: 03122162647; Email: meralbosnak@gazi.edu.tr
• Study Contact Backup: Name: Betül Yoleri, Pt. MsC; Email: betulyoleri@gazi.edu.tr

Study Locations

• Turkey
  • Çankaya
    • Ankara, Çankaya, Turkey, 06490
      • Recruiting
      • Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
      • Contact: Meral BOŞNAK GÜÇLÜ, Prof.Dr.; Phone Number: +903122162647; Email: meralbosnak@gazi.edu.tr
      • Contact: Betül Yoleri, Pt. MsC; Email: betulyoleri@gazi.edu.tr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

-Patients aged 6-18 years with primary immunodeficiency

Exclusion Criteria:

• Acute pulmonary exacerbation, acute upper or lower respiratory tract infection
• Serious neurological, neuromuscular, orthopedic and other systemic diseases or other diseases affecting physical functions
• Participating in a planned exercise program in the past three months
• Cognitive impairment, which may cause difficulty understanding and following exercise test instructions

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: Triple

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Pulmonary Rehabilitation Group; Pulmonary rehabilitation practices (inspiratory muscle training, aerobic exercise training, resistance exercise training) will be performed 3 sessions a week for 6 weeks under the supervision of a physiotherapist to the training group.	Other: Inspiratory muscle training; Inspiratory muscle training will be performed with Power Breathe®. Inspiratory muscle training will be given to the training group, starting from 50% of the MIP, and 2 sessions/day, 15 minutes/session. Patients will be asked to check breathing for 4-5 breaths after 8-10 consecutive breathing cycles. The patient will continue this cycle for 15 minutes.; Other: Upper extremity aerobic exercise training; Upper extremity aerobic exercise training will be performed 3 days/week, 1 session/day, 15 min/session using arm ergometer device accompanied by a physiotherapist. Aerobic exercise training workload will be 60-80% of maximal heart rate. In this study, the perception of dyspnea will be between 3-4, arm fatigue and general fatigue perception will be between 5-6, warm-up and cool-down periods will be 5 minutes, and pedaling speed will be 40-50 rev/min, according to MBS.; Other: Lower extremity aerobic exercise training; Lower extremity aerobic exercise training will be performed 3 days/week, 1 session/day, 15 minutes/session using the treadmill device, accompanied by a physiotherapist. Aerobic exercise training workload was set at 60-80% of maximal heart rate, dyspnea perception according to MBS was between 3-4, arm fatigue and general fatigue perception was between 5-6, and warm-up and cool-down periods were 5 minutes.; Other: Resistant exercise training; Upper and lower additional limb strengthening training will be performed 3 days/week, 1 session/day, 10 repetitions/sessions from the first day by using bullion weights in the presence of a physiotherapist. The upper extremity strengthening program will consist of a progressive exercise program to strengthen the shoulder flexors and abductors, and the lower extremity strengthening program to strengthen the knee extensors. The training workload will be increased progressively. For strength training, the workload will be adjusted so that the perception of fatigue is between 4 and 6 according to MBS.
Sham Comparator: Control Group; Control group will be given breathing exercises as a home program for 6 weeks.	Other: Thoracic expansion exercises; The control group will be asked to do thoracic expansion exercises seven days/week and 120 pieces/day for six weeks.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Oxygen Consumption	Cardiopulmonary Exercises Test	First Day

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Pulmonary function (Forced vital capacity (FVC))	Pulmonary function will be evaluated with the spirometry. Dynamic lung volume measurements will be made according to American Thoracic Society (ATS) and European Respiratory Society (ERS) criteria. With the device, forced vital capacity (FVC) will be evaluated.	Second 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.	Second 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.	Second 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.	Second 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.	Second Day
Respiratory Muscle Strength	Maximal inspiratory (MIP) and maximal expiratory (MEP) pressures expressing respiratory muscle strength were measured using a portable mouth pressure measuring device according to American Thoracic Society and European Respiratory Society criteria	Second Day
Respiratory Muscle Endurance	Incremental threshold loading test	Second Day
Peripheral Muscle Strength	Hand held dynamometer	Second Day
Functional exercise capacity	6 minute walking test	Second Day
Quality of Life (For Children)	The Pediatric Quality of Life Inventory (PedsQL): It includes 8 items that measure physical functionality, 5 items that measure emotional functionality, 5 items that measure social functionality, and 5 items that assess functionality at school. There are separate forms for parents and children. There are separate scales for children 5-7 years old, 8-12 years old and 13-18 years old. For parents, there are separate forms for 2-4 years, 5-7 years, 8-12 years and 13-18 years. Items are scored between 0-100. The higher the total PedsQL score, the better the health-related quality of life is perceived.	First Day
Dyspnea	Modified Borg Scale: The Modified Borg scale is a subjective scale that scores 0-10 for breathlessness and fatigue at rest and/or during activity. The lowest 0 points "not at all" the highest 10 points "very severe" means shortness of breath.	First and Second Day
Fatigue	Modified Borg Scale: The Modified Borg scale is a subjective scale that scores 0-10 for breathlessness and fatigue at rest and/or during activity. The lowest 0 points "not at all" the highest 10 points "very severe" means shortness of breath.	First and Second Day
Muscle oxygenation	Moxy® monitor	First and Second Day
Physical Activity Level (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 4 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 4 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 4 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 4 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 4 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 4 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 4 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

Collaborators and Investigators

• Sponsor: Gazi University
• Investigators: Principal Investigator: Betül Yoleri, Pt. MsC, Gazi University; Study Director: Meral BOŞNAK GÜÇLÜ, Prof. Dr., Gazi University; Principal Investigator: Caner Aytekin, Assoc. Prof., MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital

Publications and helpful links

General Publications

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Study record dates

Study Major Dates

• Study Start (Actual): November 1, 2023
• Primary Completion (Estimated): September 20, 2025
• Study Completion (Estimated): September 20, 2026

Study Registration Dates

• First Submitted: September 28, 2023
• First Submitted That Met QC Criteria: October 14, 2023
• First Posted (Actual): October 23, 2023

Study Record Updates

• Last Update Posted (Actual): December 5, 2023
• Last Update Submitted That Met QC Criteria: December 3, 2023
• Last Verified: December 1, 2023

More Information

Terms related to this study

• Keywords: physical activity; exercise capacity; pulmonary rehabilitation; primary immunodeficiency; muscle oxygenation
• Additional Relevant MeSH Terms: Immune System Diseases; Genetic Diseases, Inborn; Immunologic Deficiency Syndromes; Primary Immunodeficiency Diseases
• Other Study ID Numbers: Gazi University 73

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No