Figures
- FIGURE 1
Reference search strategy. CINAHL: Cumulative Index to Nursing and Allied Health Literature.
Tables
- TABLE 1
Respiratory criteria that have been used for initiation of continuous positive airway pressure or noninvasive ventilation [130]
Minimum SpO2 <90% Maximal PtcCO2 >50 mmHg ≥2% of recording time spent with SpO2 <90% ≥2% of recording time spent with PtcCO2 >50 mmHg 3% oxygen desaturation index >1.4 events·h−1 AHI >10 events·h−1 SpO2: oxygen saturation measured by pulse oximetry; PtcCO2: transcutaneous carbon dioxide pressure; AHI: apnoea–hypopnoea index.
- TABLE 2
Interfaces for continuous positive pressure treatment or noninvasive ventilation
Advantages Disadvantages Side-effects Nasal mask Small volume
Large choice of models and sizes
Allows the use of a pacifier
Allows speaking and eating (which provokes mouth leaks)Not usable in cases of mouth leaks (unless a chin strap can be used concurrently) Pressure sores (nasal bridge, face)
Maxillary deformity (retrusion)Oronasal mask Prevents mouth leaks
Less risk of midfacial hypoplasiaLarge volume
Not available for infants
Risk of asphyxia (if can't be removed by the patient)
Difficult to use with a pacifier
Limits speaking and eating
Limits secretion managementPressure sores
Risk of aspirationFull face mask Prevents mouth leaks
Prevents maxillary deformity (retrusion)Large volume
Claustrophobia
Risk of asphyxia (if can't be removed by the patient)
Difficult to use with a pacifier
Limits speaking and eating
Limits secretion managementPressure sores
Risk of aspirationNasal prongs Small, light
No pressure sores
Allows speaking and eating (which provokes mouth leaks)Not available for infants
Not usable in cases of mouth leaksNasal irritation and/or pain Mouthpiece Small, light, can be used on demand while awake Cannot be used during sleep
Difficulty of use by young childrenNone - TABLE 3
Respiratory criteria that have been used to allow discontinuation of continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV): all four major criteria should be fulfilled with at least two minor criteria [168]
Major criteria • Resolution of nocturnal and daytime symptoms of sleep disordered breathing after several nights sleeping without CPAP/NIV, such as snoring, sweating, arousals, laboured breathing, change in behaviour or attention • <2% of recording time spent with SpO2 ≤90% • <2% of recording time spent with PtcCO2 ≥50 mmHg • Obstructive apnoea–hypopnoea index <10 events·h−1 on poly(somno)graphy Minor criteria • Minimal SpO2 >90% • Maximal PtcCO2 <50 mmHg • 3% oxygen desaturation index ≤1.4 events·h−1 SpO2: oxygen saturation measured by pulse oximetry; PtcCO2: transcutaneous carbon dioxide pressure.
- TABLE 4
Summary of input and comments of the caregivers and patients panel
Section Patient and caregiver input General remarks Harmonisation of practices among different countries The harmonisation of the management of CPAP/NIV failure and intolerance is a priority to keep tracheostomy as a last treatment option Initiation Acclimatisation to CPAP/NIV should be adapted to the individual patient, i.e. initial settings to facilitate acclimatisation may differ between patients, as well as the time delay to obtain optimal compliance “Optimal compliance”, e.g. during the entire sleep time, should be the goal Equipment Regular check-ups including read-outs of the ventilator and the possibility of current ventilators (i.e. volume guarantee modes) to correct for changes in the patient's clinical conditions is reassuring for the caregivers Need for easy and immediate access to supplies when needed The availability of a back-up interface and circuit at home, prompt intervention in case of ventilator malfunctioning or breakdown, and a back-up ventilator in case of high ventilator dependency (patients with NMD) Follow-up More research is needed on patient-reported outcomes on the effects of CPAP/NIV It is critical that the patients and parents have understood and accepted the need for CPAP/NIV and get acquainted with the equipment, especially the interface, beforehand Definition/goals for subjective sleep quality under CPAP/NIV, as this might differ according to the underlying condition and comorbidities Importance of 24/7 access to medical and technical back-up Side-effects: who to manage gastric/abdominal distension?
how to prevent the risk of vomiting/aspiration in patients who are unable to remove their mask?
how to prevent/manage CPAP/NIV craniofacial complications?
Weaning Harmonisation of weaning criteria and procedures Quality of life Patient and caregiver QoL will increase after observing the positive effects of CPAP/NIV Importance of open and transparent communication with between caregivers and the medical team The effect of CPAP/NIV on neurocognitive functioning in children with underlying syndromes requires further study Therapeutic education Importance of continuous, age-appropriate education on the underlying condition, its evolution and consequences and the importance of CPAP/NIV as they grow older Importance of technical education on the different aspects and troubleshooting with machine and interface malfunctioning Education should include a patient and caregiver booklet which includes all practical information Tips and tricks for the cleaning and maintenance of the equipment In patients with neuromuscular diseases, education should also integrate airway clearance (which is not the scope of this statement) Transition What are the goals in the transition procedure for our patients? The transition process should be prepared/started early (during adolescence) Patient's self-management should be promoted with psychological support addressing potential barriers Paediatric and adult teams should work closely together CPAP: continuous positive airway pressure; NIV: noninvasive ventilation; NMD: neuromuscular disease; QoL: quality of life.
- TABLE 5
Future clinical and research priorities
Population • Harmonisation of the management of paediatric NIV patients across countries Initiation of CPAP/NIV • The efficacy, benefit and outcome of CPAP/NIV should be assessed according to the initiation criteria • The most pertinent criteria to initiate CPAP/NIV according to the underlying disease or age should be identified • Comparison of the efficacy and benefits according to the location of CPAP/NIV initiation. Definition of criteria for hospital or home initiation • Comparison of the efficacy of “complex” CPAP modes versus constant CPAP • Evaluation of the usefulness of additional settings (ramp for CPAP, humidification) • Larger-scale studies on patients with BPAP to have better idea of settings used in a more comprehensive cohort of NIV patients Equipment • Comparative data of interfaces with regard to tolerance and side-effects and the usefulness of alternating different types on interfaces in a single child • The long-term facial effects in older children • Lack of data on the reversibility of the adverse effects after CPAP/NIV weaning or change of interface • Lack of information on the importance of the headgear (suitability, skull deformity induced by the headgear) • Evaluation of the interest of complementary technologies (three-dimensional printing, pressure measurements) to guide the choice and positioning of the interface • Development and validation of built-in software data for children, especially those weighing <30 kg • Usefulness and benefit of new NIV modes (AVAPS, iVAPS) Follow-up Follow-up procedures: • Evaluation of the optimal follow-up strategy in terms of timing and protocols • Evaluation of the usefulness and limitations of telemonitoring for follow-up Adherence: • Usefulness of new technologies to improve adherence (telemedicine, mobile phone applications) • Investigating the link between adherence and relevant end-organ morbidity Benefits: • Benefits of CPAP on academic function and behaviour in children with “complex” OSA • Efficacy of NIV should also be assessed on the child's neurocognitive outcome, behaviour and academic performance Weaning • Development and validation of weaning criteria and protocols for CPAP and NIV CPAP/NIV failure • Multicentre randomised controlled trials on alternative ventilation strategies Palliative care • Effects of NIV in palliative care (improvement in dyspnoea, sleep quality and QoL) Special populations Infants: • Multicentre studies investigating factors predicting greater benefit from long-term CPAP/NIV use with a focus on long-term outcome data. Studies looking at technical aspects concerning interfaces and ventilation modes are also warranted Obesity: • Studies assessing the long-term follow-up of obese children treated with CPAP/NIV and differences comparing CPAP to NIV in obese children including differences in required pressures, adherence and health outcomes • Additional sleep problems are common in children with obesity and may impact adherence to therapy; this has not been explored Severe neurodisability: • Prospective data collection focusing on QoL and changes in health outcomes in patient with severe neurodisability that is attributable to CPAP/NIV • Prospective studies to assess the clinical benefit of CPAP/NIV in this patient group, comparing to alternative treatments, such as oxygen or nasopharyngeal airway, are needed QoL • Longitudinal study investigating fluctuations and factors influencing the QoL of children on CPAP/NIV and their parents/caregivers, in conjunction with evolution of the underlying conditions, family functioning/coping strategies • To examine the interaction between adherence and QoL outcomes for the patients and families Therapeutic education • Development of therapeutic education tools and programs for CPAP/NIV with studies investigating their efficacy • Which healthcare professionals should be involved in therapeutic education, and should they receive specific training? Transition • The efficacy of different transition programmes evaluated on loss of follow-up, optimal data, effect of the underlying disease, cognitive dysfunction or physical dependence, control of the disease and patient satisfaction Costs • Evaluation of healthcare cost savings thanks to CPAP/NIV (reduction of hospitalisations, healthcare use, etc.) CPAP: continuous positive airway pressure; NIV: noninvasive ventilation; QoL: quality of life; BPAP: bilevel positive airway pressure; AVAPS: average volume-assured pressure support; iVAPS: intelligent volume-assured pressure support; OSA: obstructive sleep apnoea.
Supplementary Material
Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.
Supplementary table S1: Final search strategy: paediatric CPAP and NIV ERJ-01404-2021.Table_S1
Supplementary table S2.1: Patients (pathologies) who may benefit from CPAP ERJ-01404-2021.Table_S2.1
Supplementary table S2.2: Patients (pathologies) who may benefit from NIV ERJ-01404-2021.Table_S2.2
Supplementary table S2.3: Longitudinal (local/regional/national) surveys ERJ-01404-2021.Table_S2.3
Supplementary table S3.1: Initiation criteria and location for CPAP or NIV initiation ERJ-01404-2021.Table_S3.1
Supplementary table S3.2: Initial and follow up settings for CPAP ERJ-01404-2021.Table_S3.2
Supplementary table S3.3: Initial and follow up settings for NIV ERJ-01404-2021.Table_S3.3
Supplementary table S4.1: Description of interfaces ERJ-01404-2021.Table_S4.1
Supplementary table S4.2: Side effects of interfaces ERJ-01404-2021.Table_S4.2
Supplementary table S4.3: Ventilators for CPAP and NIV ERJ-01404-2021.Table_S4.3
Supplementary table S5.1: Follow up of CPAP and NIV ERJ-01404-2021.Table_S5.1
Supplementary table S5.2: CPAP/NIV adherence ERJ-01404-2021.Table_S5.2
Supplementary table S5.3: Benefits of CPAP (except decrease in AHI) ERJ-01404-2021.Table_S5.3
Supplementary table S5.4: Benefits of NIV (except decrease in AHI) ERJ-01404-2021.Table_S5.4
Supplementary table S6.1: Which patients may benefit from a weaning trial? ERJ-01404-2021.Table_S6.1
Supplementary table S6.2: Weaning from CPAP or NIV: optimal timing and requirements for a weaning trial and follow-up? ERJ-01404-2021.Table_S6.2
Supplementary table S7: Which options when CPAP or NIV fails? ERJ-01404-2021.Table_S7
Supplementary table S8: Role of CPAP and NIV in palliative care ERJ-01404-2021.Table_S8
Supplementary table S9.1: Special populations: CPAP and NIV in infants ERJ-01404-2021.Table_S9.1
Supplementary table S9.2: Special population: obese children ERJ-01404-2021.Table_S9.2
Supplementary table S9.3: Special population: CPAP or NIV in children with neurodisability ERJ-01404-2021.Table_S9.3
Supplementary table S10: CPAP and NIV and quality of life for patients and families/caregivers ERJ-01404-2021.Table_S10
Supplementary table S11: Therapeutic education programmes for CPAP and NIV ERJ-01404-2021.Table_S11
Supplementary table S12: Transition ERJ-01404-2021.Table_S12
Supplementary table S13. Cost and resource use of CPAP or NIV ERJ-01404-2021.Table_S13
Supplementary Material
This one-page PDF can be shared freely online.
Shareable PDF ERJ-01404-2021.Shareable
Jump To
- Article
- Abstract
- Abstract
- Introduction
- Methods
- Disorders that may benefit from CPAP/NIV
- Initiation criteria, initiation location and recommended/optimal settings
- Equipment
- Follow-up
- Weaning
- CPAP/NIV failure
- Role of CPAP/NIV in palliative care
- CPAP/NIV in special populations
- CPAP/NIV and quality of life in children and parents
- Therapeutic education
- Transition
- Cost and resource use considerations of CPAP/NIV
- Conclusion
- Supplementary material
- Shareable PDF
- Footnotes
- References
- Figures & Data
- Info & Metrics