Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

Amin, Reshma; MacLusky, Ian; Zielinski, David; Adderley, Robert J.; Carnevale, Franco A.; Chiang, Jackie; Côté, Aurore; Daniels, Carolyn; Daigneault, Patrick; Harrison, Christine; Katz, Sherri; Keilty, Krista; Majaesic, Carina; Moraes, Theo J.; Price, April; Radhakrishnan, Dhenuka; Rapoport, Adam; Spier, Sheldon; Thavagnanam, Surendran; Witmans, Manisha; Society, Canadian Thoracic

doi:10.1080/24745332.2017.1300463

Cited by 57 publications

(166 citation statements)

References 39 publications

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“…The decision to withdraw life-sustaining ventilator support later in life is often in response to an acute event or significant cumulative morbidities, such as severe hypoxic-ischaemic insults. Practice and ethical considerations of long-term home ventilation in neuromuscular diseases are evolving and will likely continue with the advent of treatments that reduce morbidity and improve quality of life and survival for these patients 33 34…”

Section: Discussionmentioning

confidence: 99%

Mortality and respiratory support in X-linked myotubular myopathy: a RECENSUS retrospective analysis

et al. 2019

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Purpose Individuals with X-linked myotubular myopathy (XLMTM) who survive infancy require extensive supportive care, including ventilator assistance, wheelchairs and feeding tubes. Half die before 18 months of age. We explored respiratory support and associated mortality risk in RECENSUS, particularly among patients ≤5 years old who received respiratory support at birth; this subgroup closely matches patients in the ASPIRO trial of gene therapy for XLMTM. Design RECENSUS is an international, retrospective study of patients with XLMTM. Descriptive and timeto-event analyses examined survival on the basis of age, respiratory support, tracheostomy use, predicted mutational effects and life-sustaining care. Results Outcomes for 145 patients were evaluated. Among 126 patients with respiratory support at birth, mortality was 47% overall and 59% among those ≤5 years old. Median survival time was shorter for patients ≤5 years old than for those >5 years old (2.2 years (IQR 0.7-5.6) vs 30.2 years (IQR 19.4-30.2)). The most common cause of death was respiratory failure (66.7%). Median survival time was longer for patients with a tracheostomy than for those without (22.8 years (IQR 8.7-30.2) vs 1.8 years (IQR 0.2-not estimable)). The proportion of patients living without a tracheostomy was 50% at age 6 months and 28% at age 2 years. Median survival time was longer with provision of life-sustaining care than without (19.4 years (IQR 3.1-not estimable) vs 0.2 years (IQR 0.1-2.1)). Conclusions High mortality, principally due to respiratory failure, among patients with XLMTM ≤5 years old despite respiratory support underscores the need for early diagnosis, informed decision-making and diseasemodifying therapies. Trial registration number NCT02231697

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Section: Discussionmentioning

confidence: 99%

Mortality and respiratory support in X-linked myotubular myopathy: a RECENSUS retrospective analysis

et al. 2019

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“…Delivery of home LT-NIV necessitates the existence of a home ventilation program, whose characteristics have been described (33,34). Close collaboration is mandatory between the hospital multidisciplinary team (who initiates LT-NIV and supervises the medical follow-up and complex care of the patient), the personnel from the home-ventilation program (who ensures equipment delivery; sets up, monitors, and adjusts LT-NIV as prescribed by the hospital team; and answers emergency calls 24/7), and the family caregivers.…”

Section: Indications For Long-term Non-invasive Ventilation General Cmentioning

confidence: 99%

“…Chronic lung diseases can also benefit from LT-NIV. These include severe cystic fibrosis in adolescents, in whom LT-NIV has been attempted not only to improve quality of life and prevent deterioration, but also as a bridge to lung transplantation, albeit with mixed evidence (33,52,53). LT-NIV has also been utilized for some inherited metabolic disorders, which can variably involve the various components of the respiratory system from the brain to the lungs (54).…”

Section: Medical Conditions Treated By Long-term Non-invasive Ventilamentioning

confidence: 99%

“…LT-NIV has also been utilized for some inherited metabolic disorders, which can variably involve the various components of the respiratory system from the brain to the lungs (54). Conversely, if needed, home ventilation is usually provided via tracheostomy in infants with severe bronchopulmonary dysplasia, most often with pulmonary arterial hypertension (33,34,55). And while bronchopulmonary dysplasia is listed as a potential indication, including as a bridge from invasive ventilation to spontaneous ventilation without respiratory support, extensive review of the literature finds very few infants with this diagnosis in reports on home LT-NIV (17,56,57).…”

Section: Medical Conditions Treated By Long-term Non-invasive Ventilamentioning

confidence: 99%

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Long-Term Non-invasive Ventilation in Children: Current Use, Indications, and Contraindications

Praud

2020

Front. Pediatr.

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This review focuses on the delivery of non-invasive ventilation-i.e., intermittent positive-pressure ventilation-in children lasting more than 3 months. Several recent reviews have brought to light a dramatic escalation in the use of long-term non-invasive ventilation in children over the last 30 years. This is due both to the growing number of children receiving care for complex and severe diseases necessitating respiratory support and to the availability of LT-NIV equipment that can be used at home. While significant gaps in availability persist for smaller children and especially infants, home LT-NIV for children with chronic respiratory insufficiency has improved their quality of life and decreased the overall cost of care. While long-term NIV is usually delivered during sleep, it can also be delivered 24 h a day in selected patients. Close collaboration between the hospital complex-care team, the home LT-NIV program, and family caregivers is of the utmost importance for successful home LT-NIV. Long-term NIV is indicated for respiratory disorders responsible for chronic alveolar hypoventilation, with the aim to increase life expectancy and maximize quality of life. LT-NIV is considered for conditions that affect respiratory-muscle performance (alterations in central respiratory drive or neuromuscular function) and/or impose an excessive respiratory load (airway obstruction, lung disease, or chest-wall anomalies). Relative contraindications for LT-NIV include the inability of the local medical infrastructure to support home LT-NIV and poor motivation or inability of the patient/caregivers to cooperate or understand recommendations. Anatomic abnormalities that interfere with interface fitting, inability to protect the lower airways due to excessive airway secretions and/or severely impaired swallowing, or failure of LT-NIV to support respiration can lead to considering invasive ventilation via tracheostomy. Of note, providing home LT-NIV during the COVID 19 pandemic has become more challenging. This is due both to the disruption of medical systems and the fear of contaminating care providers and family with aerosols generated by a patient positive for SARS-CoV-2 during NIV. Delay in initiating LT-NIV, decreased frequency of home visits by the home ventilation program, and decreased availability of polysomnography and oximetry/transcutaneous PCO 2 monitoring are observed. Teleconsultations and telemonitoring are being developed to mitigate these challenges.

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“…The British Thoracic Society guidelines recommend sleep assessments in patients with clinical symptoms suggestive of SDB who have lost ambulation, who will never gain ambulation, or who are infants with NMD [ 3 ]. Canadian Thoracic Society guidelines recommend evaluation for SDB if the FVC in spirometry is less than 60% predicted, in those who are symptomatic, or in those who have become non-ambulant because of muscle weakness on at least a yearly basis [ 41 ]. The recommendations for which test to use are often contentious but typically take a pragmatic approach, with PSG being the ideal but oximetry or oxycapnography being reasonable alternatives for initial testing if PSG is unavailable.…”

Section: Evaluation Of Breathing During Sleepmentioning

confidence: 99%

Respiratory Diagnostic Tools in Neuromuscular Disease

2018

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Children with neuromuscular disease (NMD) are at risk of acquiring respiratory complications. Both clinical assessments and respiratory diagnostic tests are important to optimize the respiratory health and care of such children. The following respiratory diagnostic tools and their utility for evaluating children with NMD are discussed in this article: lung function testing (spirometry and lung volumes), peak cough flow (PCF), respiratory muscle strength testing, oximetry, capnography, and polysomnography.

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Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

Cited by 57 publications

References 39 publications

Mortality and respiratory support in X-linked myotubular myopathy: a RECENSUS retrospective analysis

Mortality and respiratory support in X-linked myotubular myopathy: a RECENSUS retrospective analysis

Long-Term Non-invasive Ventilation in Children: Current Use, Indications, and Contraindications

Respiratory Diagnostic Tools in Neuromuscular Disease

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