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

NCT ID: NCT06092528

Last Updated: 2023-12-05

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

NA

Total Enrollment

40 participants

Study Classification

INTERVENTIONAL

Study Start Date

2023-11-01

Study Completion Date

2026-09-20

Brief Summary

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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

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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)).

Conditions

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Primary Immunodeficiency

Keywords

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primary immunodeficiency pulmonary rehabilitation exercise capacity muscle oxygenation physical activity

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Parallel Assignment
Primary Study Purpose

SUPPORTIVE_CARE

Blinding Strategy

TRIPLE

Participants Investigators Outcome Assessors
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.

Study Groups

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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.

Group Type EXPERIMENTAL

Inspiratory muscle training

Intervention Type OTHER

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.

Upper extremity aerobic exercise training

Intervention Type OTHER

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.

Lower extremity aerobic exercise training

Intervention Type OTHER

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.

Resistant exercise training

Intervention Type OTHER

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.

Control Group

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

Group Type SHAM_COMPARATOR

Thoracic expansion exercises

Intervention Type OTHER

The control group will be asked to do thoracic expansion exercises seven days/week and 120 pieces/day for six weeks.

Interventions

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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.

Intervention Type 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.

Intervention Type 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.

Intervention Type 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.

Intervention Type 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.

Intervention Type OTHER

Eligibility Criteria

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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
Minimum Eligible Age

6 Years

Maximum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Gazi University

OTHER

Sponsor Role lead

Responsible Party

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Meral Boşnak Güçlü

Study director, PT, PhD, Prof.Dr. Faculty of Health Sciences, Department of Physiotherapy and Rehabilitation, Head of Cardiopulmonary Rehabilitation Clinic

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Betül Yoleri, Pt. MsC

Role: PRINCIPAL_INVESTIGATOR

Gazi University

Caner Aytekin, Assoc. Prof.

Role: PRINCIPAL_INVESTIGATOR

MH. Dr. Sami Ulus Children's Health and Diseases Training and Research Hospital

Meral Boşnak Güçlü, Prof. Dr.

Role: STUDY_DIRECTOR

Gazi University

Locations

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

Ankara, Çankaya, Turkey (Türkiye)

Site Status RECRUITING

Countries

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Turkey (Türkiye)

Central Contacts

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Meral Boşnak Güçlü, Prof. Dr.

Role: CONTACT

Phone: 03122162647

Email: [email protected]

Betül Yoleri, Pt. MsC

Role: CONTACT

Email: [email protected]

Facility Contacts

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Meral BOŞNAK GÜÇLÜ, Prof.Dr.

Role: primary

Betül Yoleri, Pt. MsC

Role: backup

References

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Other Identifiers

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Gazi University 73

Identifier Type: -

Identifier Source: org_study_id