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

Primary Purpose

Primary Immunodeficiency

Status
Not yet recruiting
Phase
Not Applicable
Locations
Turkey
Study Type
Interventional
Intervention
Inspiratory muscle training
Upper extremity aerobic exercise training
Lower extremity aerobic exercise training
Resistant exercise training
Thoracic expansion exercises
Sponsored by
Gazi University
About
Eligibility
Locations
Arms
Outcomes
Full info

About this trial

This is an interventional supportive care trial for Primary Immunodeficiency focused on measuring primary immunodeficiency, pulmonary rehabilitation, exercise capacity, muscle oxygenation, physical activity

Eligibility Criteria

6 Years - 18 Years (Child, Adult)All SexesDoes not accept healthy volunteers

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

Sites / Locations

  • Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit

Arms of the Study

Arm 1

Arm 2

Arm Type

Experimental

Sham Comparator

Arm Label

Pulmonary Rehabilitation Group

Control Group

Arm Description

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.

Control group will be given breathing exercises as a home program for 6 weeks.

Outcomes

Primary Outcome Measures

Oxygen Consumption
Cardiopulmonary Exercises Test

Secondary Outcome Measures

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.
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.
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.
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.
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.
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
Respiratory Muscle Endurance
Incremental threshold loading test
Peripheral Muscle Strength
Hand held dynamometer
Functional exercise capacity
6 minute walking test
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.
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.
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.
Muscle oxygenation
Moxy® monitor
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.
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.
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.
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.
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.
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.
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.

Full Information

First Posted
September 28, 2023
Last Updated
October 14, 2023
Sponsor
Gazi University
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1. Study Identification

Unique Protocol Identification Number
NCT06092528
Brief Title
Investigation of the Effects of Pulmonary Rehabilitation in Children With Primary Immunodeficiency
Official Title
Investigation of the Effects of Pulmonary Rehabilitation on Exercise Capacity, Muscle Oxygenation and Physical Activity Level in Children With Primary Immunodeficiency
Study Type
Interventional

2. Study Status

Record Verification Date
October 2023
Overall Recruitment Status
Not yet recruiting
Study Start Date
November 1, 2023 (Anticipated)
Primary Completion Date
September 20, 2025 (Anticipated)
Study Completion Date
September 20, 2026 (Anticipated)

3. Sponsor/Collaborators

Responsible Party, by Official Title
Principal Investigator
Name of the Sponsor
Gazi University

4. Oversight

Studies a U.S. FDA-regulated Drug Product
No
Studies a U.S. FDA-regulated Device Product
No
Data Monitoring Committee
No

5. Study Description

Brief Summary
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.
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)).

6. Conditions and Keywords

Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Primary Immunodeficiency
Keywords
primary immunodeficiency, pulmonary rehabilitation, exercise capacity, muscle oxygenation, physical activity

7. Study Design

Primary Purpose
Supportive Care
Study Phase
Not Applicable
Interventional Study Model
Parallel Assignment
Model Description
Parallel Assignment
Masking
ParticipantInvestigatorOutcomes Assessor
Masking Description
Triple-blind study; the patients will not be informed about training group or control group and they will be evaluated and trained at different places and times. Evaluations and interventions will be performed different physiotherapist. In addition, before statistical analysis patients' groups will be coded.
Allocation
Randomized
Enrollment
40 (Anticipated)

8. Arms, Groups, and Interventions

Arm Title
Pulmonary Rehabilitation Group
Arm Type
Experimental
Arm Description
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.
Arm Title
Control Group
Arm Type
Sham Comparator
Arm Description
Control group will be given breathing exercises as a home program for 6 weeks.
Intervention Type
Other
Intervention Name(s)
Inspiratory muscle training
Intervention Description
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.
Intervention Type
Other
Intervention Name(s)
Upper extremity aerobic exercise training
Intervention Description
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.
Intervention Type
Other
Intervention Name(s)
Lower extremity aerobic exercise training
Intervention Description
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.
Intervention Type
Other
Intervention Name(s)
Resistant exercise training
Intervention Description
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.
Intervention Type
Other
Intervention Name(s)
Thoracic expansion exercises
Intervention Description
The control group will be asked to do thoracic expansion exercises seven days/week and 120 pieces/day for six weeks.
Primary Outcome Measure Information:
Title
Oxygen Consumption
Description
Cardiopulmonary Exercises Test
Time Frame
First Day
Secondary Outcome Measure Information:
Title
Pulmonary function (Forced vital capacity (FVC))
Description
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.
Time Frame
Second Day
Title
Pulmonary function (Forced expiratory volume in the first second (FEV1))
Description
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.
Time Frame
Second Day
Title
Pulmonary function (FEV1 / FVC)
Description
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.
Time Frame
Second Day
Title
Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%))
Description
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.
Time Frame
Second Day
Title
Pulmonary function (Peak flow rate (PEF))
Description
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.
Time Frame
Second Day
Title
Respiratory Muscle Strength
Description
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
Time Frame
Second Day
Title
Respiratory Muscle Endurance
Description
Incremental threshold loading test
Time Frame
Second Day
Title
Peripheral Muscle Strength
Description
Hand held dynamometer
Time Frame
Second Day
Title
Functional exercise capacity
Description
6 minute walking test
Time Frame
Second Day
Title
Quality of Life (For Children)
Description
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.
Time Frame
First Day
Title
Dyspnea
Description
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.
Time Frame
First and Second Day
Title
Fatigue
Description
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.
Time Frame
First and Second Day
Title
Muscle oxygenation
Description
Moxy® monitor
Time Frame
First and Second Day
Title
Physical Activity Level (Total energy expenditure)
Description
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.
Time Frame
Second Day
Title
Physical activity (Active energy expenditure (joule / day))
Description
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.
Time Frame
Second Day
Title
Physical activity (Physical activity time (min / day))
Description
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.
Time Frame
Second Day
Title
Physical activity (Average metabolic equivalent (MET / day))
Description
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.
Time Frame
Second Day
Title
Physical activity (Number of steps (steps / day))
Description
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.
Time Frame
Second Day
Title
Physical activity (Time spent lying down (min / day) days))
Description
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.
Time Frame
Second Day
Title
Physical activity (Sleep time (min / day))
Description
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.
Time Frame
Second Day

10. Eligibility

Sex
All
Minimum Age & Unit of Time
6 Years
Maximum Age & Unit of Time
18 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Meral Boşnak Güçlü, Prof. Dr.
Phone
03122162647
Email
meralbosnak@gazi.edu.tr
First Name & Middle Initial & Last Name or Official Title & Degree
Betül Yoleri, Pt. MsC
Email
betulyoleri@gazi.edu.tr
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Betül Yoleri, Pt. MsC
Organizational Affiliation
Gazi University
Official's Role
Principal Investigator
First Name & Middle Initial & Last Name & Degree
Caner Aytekin, Assoc. Prof.
Organizational Affiliation
MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital
Official's Role
Principal Investigator
First Name & Middle Initial & Last Name & Degree
Meral Boşnak Güçlü, Prof. Dr.
Organizational Affiliation
Gazi University
Official's Role
Study Director
Facility Information:
Facility Name
Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Cardiopulmonary Rehabilitation Unit
City
Ankara
State/Province
Çankaya
ZIP/Postal Code
06490
Country
Turkey

12. IPD Sharing Statement

Plan to Share IPD
No
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Investigation of the Effects of Pulmonary Rehabilitation in Children With Primary Immunodeficiency

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