Primary Immunodeficiency
Conditions
Keywords
primary immunodeficiency, pulmonary rehabilitation, exercise capacity, muscle oxygenation, physical activity
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)).
Interventions
OTHERInspiratory 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.
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 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.
OTHERResistant 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.
The control group will be asked to do thoracic expansion exercises seven days/week and 120 pieces/day for six weeks.
Sponsors
Gazi University
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
SUPPORTIVE_CARE
Masking
TRIPLE (Subject, Investigator, Outcomes 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.
Intervention model description
Parallel Assignment
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Oxygen Consumption | First Day | Cardiopulmonary Exercises Test |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary function (Forced expiratory volume in the first second (FEV1)) | Second Day | 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) | Second Day | 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%)) | Second Day | 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)) | Second Day | 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 | Second Day | 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 | Second Day | Incremental threshold loading test |
| Peripheral Muscle Strength | Second Day | Hand held dynamometer |
| Functional exercise capacity | Second Day | 6 minute walking test |
| Quality of Life (For Children) | First Day | 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. |
| Pulmonary function (Forced vital capacity (FVC)) | Second Day | 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. |
| Muscle oxygenation | First and Second Day | Moxy® monitor |
| Dyspnea | First and Second Day | 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. |
| Physical Activity Level (Total energy expenditure) | Second 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. 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)) | Second 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)) | Second 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)) | Second 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)) | Second 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)) | Second 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. 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)) | Second 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. |
| Fatigue | First and Second Day | 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. |
Countries
Turkey (Türkiye)
Contacts
Primary ContactMeral Boşnak Güçlü, Prof. Dr.
Backup ContactBetül Yoleri, Pt. MsC
Outcome results
None listed