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

Overview

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

• 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 traini
• 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.

Primary outcome measures

• Oxygen Consumption [Time frame: First Day]

Secondary outcome measures (12)

• Pulmonary function (Forced vital capacity (FVC)) [Time frame: Second Day]
• Pulmonary function (Forced expiratory volume in the first second (FEV1)) [Time frame: Second Day]
• Pulmonary function (FEV1 / FVC) [Time frame: Second Day]
• Pulmonary function (Flow rate 25-75% of forced expiratory volume (FEF 25-75%)) [Time frame: Second Day]
• Pulmonary function (Peak flow rate (PEF)) [Time frame: Second Day]
• Respiratory Muscle Strength [Time frame: Second Day]
• Respiratory Muscle Endurance [Time frame: Second Day]
• Peripheral Muscle Strength [Time frame: Second Day]
• Functional exercise capacity [Time frame: Second Day]
• Quality of Life (For Children) [Time frame: First Day]
• Dyspnea [Time frame: First and Second Day]
• Fatigue [Time frame: First and Second Day]

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

Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.

Healthy volunteers: No

Study design

Allocation

Randomized

Model

Parallel assignment

Masking

Triple blind

Primary purpose

Supportive care

Study locations

Turkey (Türkiye) · 1 center

• Gazi University, Faculty of Health Sciences, Department of Physiotherapy and Rehabilitatio — Ankara

Publications

• American Thoracic Society; American College of Chest Physicians. ATS/ACCP Statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2003 Jan 15;167(2):211-77. doi: 10.1164/rccm.167.2.211. No abstract available. PMID 12524257
• ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002 Jul 1;166(1):111-7. doi: 10.1164/ajrccm.166.1.at1102. No abstract available. PMID 12091180
• Li AM, Yin J, Au JT, So HK, Tsang T, Wong E, Fok TF, Ng PC. Standard reference for the six-minute-walk test in healthy children aged 7 to 16 years. Am J Respir Crit Care Med. 2007 Jul 15;176(2):174-80. doi: 10.1164/rccm.200607-883OC. Epub 2007 Apr 26. PMID 17463419
• Crum EM, O'Connor WJ, Van Loo L, Valckx M, Stannard SR. Validity and reliability of the Moxy oxygen monitor during incremental cycling exercise. Eur J Sport Sci. 2017 Sep;17(8):1037-1043. doi: 10.1080/17461391.2017.1330899. Epub 2017 May 30. PMID 28557670
• Lee JA, Laurson KR. Validity of the SenseWear armband step count measure during controlled and free-living conditions. J Exerc Sci Fit. 2015 Jun;13(1):16-23. doi: 10.1016/j.jesf.2014.11.002. Epub 2015 Jan 29. PMID 29541094
• Patel SA, Benzo RP, Slivka WA, Sciurba FC. Activity monitoring and energy expenditure in COPD patients: a validation study. COPD. 2007 Jun;4(2):107-12. doi: 10.1080/15412550701246658. PMID 17530503
• Ridgers ND, Hnatiuk JA, Vincent GE, Timperio A, Barnett LM, Salmon J. How many days of monitoring are needed to reliably assess SenseWear Armband outcomes in primary school-aged children? J Sci Med Sport. 2016 Dec;19(12):999-1003. doi: 10.1016/j.jsams.2016.02.009. Epub 2016 Mar 3. PMID 26971298
• American Thoracic Society/European Respiratory Society. ATS/ERS Statement on respiratory muscle testing. Am J Respir Crit Care Med. 2002 Aug 15;166(4):518-624. doi: 10.1164/rccm.166.4.518. No abstract available. PMID 12186831

Identifiers

NCT: NCT06092528 · Gazi University 73

Primary sources (government registries)

• ClinicalTrials.gov (US NIH)

View this study on ClinicalTrials.gov ↗