Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy

Pascoe, John E.; Sawnani, Hemant; Hater, Brooke; Sketch, M.; Modi, Avani C.

doi:10.1002/ppul.24484

Cited by 21 publications

(19 citation statements)

References 39 publications

(127 reference statements)

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“…Adherence is assessed on objective criteria (built-in software data) because children and caregivers tend to overestimate real adherence [148]. Adherence reported in the literature usually not covers the entire night and represents the greatest challenge for long term CPAP/NIV [14,148,[181][182][183][184][185][186][187][188]. Numerous predictors of adherence have been identified: greater self-perceived improvement in SDB symptoms [180], developmental delay (lower compliance in children with Down syndrome) [144,189] and better adherence in children with other causes of developmental delay [190,191]), gender [190], rapid acclimatization to treatment [180], technical issues [180], NIV vs CPAP [192], side effects [180], familiarity with medical treatments, understanding of the disease and its consequences [180], greater improvement in the AHI [188,193], age [184], ethnicity [184], maternal education [184], family social support [180,184], family structure [185], perception of CPAP benefits [185], family member using CPAP [14], caregiver self-reported efficacy [194], and internalizing problems [188].…”

Section: Adherencementioning

confidence: 99%

ERS statement on paediatric long-term noninvasive respiratory support

et al. 2021

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Long term noninvasive respiratory support, comprising continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV), in children is expanding worldwide, with increasing complexities of children being considered for this type of ventilator support and expanding indications such as palliative care. There have been improvements in equipment and interfaces. Despite growing experience, there are still gaps in a significant number of areas: there is a lack of validated criteria for CPAP/NIV initiation, optimal follow-up and monitoring; weaning and long term benefits have not been evaluated. Therapeutic education of the caregivers and the patient is of paramount importance, as well as continuous support and assistance, in order to achieve optimal adherence. The preservation or improvement of the quality of life of the patient and caregivers should be a concern for all children treated with long term CPAP/NIV. As NIV is a highly specialised treatment, patients are usually managed by an experienced pediatric multidisciplinary team. This Statement written by experts in the field of pediatric long term CPAP/NIV aims to emphasize on the most recent scientific input and should open up to new perspectives and research areas.

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Section: Adherencementioning

confidence: 99%

ERS statement on paediatric long-term noninvasive respiratory support

et al. 2021

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“…A common indication for home CPAP in children is obstructive sleep apnea refractory to adenotonsillectomy, 18 where CPAP functions as a "splint" to maintain patency of the pharynx and upper airway, to improve airway obstruction, decrease respiratory muscle load, and reduce the work of breathing. Home NIV may also be prescribed for patients with chronic hypercapnic respiratory failure, specifically in patients with neuromuscular and chest wall disease such as children with Duchenne's muscular dystrophy 19 …”

Section: Implementation Of Niv In the Intensive Care Vs Home Nivmentioning

confidence: 99%

“…Home NIV may also be prescribed for patients with chronic hypercapnic respiratory failure, specifically in patients with neuromuscular and chest wall disease such as children with Duchenne's muscular dystrophy. 19 Clinicians caring for children on home NIV devices in the perioperative period should be comfortable using these devices, with special attention to the specific capabilities for maximum inspired fraction of oxygen (usually limited). Most devices need to be connected to a power source, although in some countries portable battery-powered devices may be available.…”

Section: Implementati On Of Niv In the Inten S Ive C Are Vs Home Nivmentioning

confidence: 99%

Noninvasive ventilation in children: A review for the pediatric anesthesiologist

Sequera‐Ramos

Garcia‐Marcinkiewicz

Riva

et al. 2021

Pediatric Anesthesia

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Preserving adequate respiratory function is essential in the perioperative period.Mechanical ventilation with endotracheal intubation is widely used for this purpose.In select patients, noninvasive ventilation (NIV) may be an alternative to invasive ventilation or may complement respiratory management. NIV is used to provide ventilatory support and increase gas exchange at the alveolar level without the use of an invasive artificial airway such as an endotracheal tube or tracheostomy. NIV includes both continuous positive airway pressure (CPAP) and noninvasive positive pressure ventilation. Indications for NIV range from acute hypoxic respiratory failure in the intensive care unit or the emergency department, to chronic respiratory failure in patients with neuromuscular disease with nocturnal hypoventilation. In the perioperative setting, NIV is commonly applied as CPAP, and bilevel positive airway pressure (BPAP). There are limited data on the role of NIV in children in the perioperative setting, and there are no clear guidelines regarding optimal timing of use and pressure settings of perioperative NIV. Contraindications to the use of NIV include reduced level of consciousness, apnea, severe respiratory distress, and inability to maintain upper airway patency or airway protective reflexes. Common problems encountered during NIV involve airway leaks and asynchrony with auto-triggering. High-flow nasal oxygen (HFNO) has emerged as an alternative to NIV when trying to decrease the work of breathing and improve oxygenation in children. HFNO delivers humidified and heated oxygen at rates between 2 and 70 L/min using specific nasal cannulas, and flows are determined by the patient's weight and clinical needs. HFNO can be useful as a method for preoxygenation in infants and children by prolonging apnea time before desaturation, yet in children with decreased minute ventilation or apnea HFNO does not improve alveolar gas exchange. Clinicians experienced with these devices, such as pediatric intensivists and pulmonary medicine specialists, can be useful resources for the pediatric anesthesiologist caring for complex patients on NIV.

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“…Another study evaluating NIV adherence, adherence barriers in patients with DMD using nocturnal NIV found that mask discomfort was the most commonly reported adherence barrier. The assessment of the psychosocial functioning of patients and their caregivers revealed that caregivers' barriers and child internalizing symptoms predicted lower NIV adherence, and authors recommended multidisciplinary approach to improve adherence in children ( 112 ).…”

Section: Complications and Adherencementioning

confidence: 99%

Non-invasive Ventilation for Children With Chronic Lung Disease

2020

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Advances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.

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Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy

Cited by 21 publications

References 39 publications

ERS statement on paediatric long-term noninvasive respiratory support

ERS statement on paediatric long-term noninvasive respiratory support

Noninvasive ventilation in children: A review for the pediatric anesthesiologist

Non-invasive Ventilation for Children With Chronic Lung Disease

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