Chronic Respiratory Support Research Articles

A Longitudinal Study of Escherichia coli Clinical Isolates from the Tracheal Aspirates of a Paediatric Patient-Strain Type Similar to Pandemic ST131.

Escherichia coli is a Gram-negative bacterium and part of the intestinal microbiota. However, it can cause various diarrhoeal illnesses, i.e., traveller's diarrhoea, dysentery, and extraintestinal infections when the bacteria are translocated from the intestine to other organs, such as urinary tract infections, abdominal and pelvic infections, pneumonia, bacteraemia, and meningitis. It is also an important pathogen in intensive care units where cross-infection may cause intrahospital spread with serious consequences. Within this study, four E. coli isolates from the tracheal aspirates of a tracheotomised paediatric patient on chronic respiratory support were analysed and compared for antibiotic resistance and virulence potential. Genomes of all four isolates (5381a, 5381b, 5681, 5848) were sequenced using Oxford Nanopore Technology. According to PFGE analysis, the clones of isolates 5681 and 5848 were highly similar, and differ from 5381a and 5381b which were isolated first chronologically. All four E. coli isolates belonged to an unknown sequence type, related to the E. coli ST131, a pandemic clone that is evolving rapidly with increasing levels of antimicrobial resistance. All four E. coli isolates in this study exhibited a multidrug-resistant phenotype as, according to MIC data, they were resistant to ceftriaxone, ciprofloxacin, doxycycline, minocycline, and tetracycline. In addition, principal component analyses revealed that isolates 5681 and 5848, which were recovered later than 5381a and 5381b (two weeks and three weeks, respectively) possessed more complex antibiotic resistance genes and virulence profiles, which is concerning considering the short time period during which the strains were isolated.

A breakthrough effect of gene replacement therapy on respiratory outcomes in children with spinal muscular atrophy.

Spinal muscular atrophy (SMA) is an inherited progressive neuromuscular disorder characterized by generalized hypotonia, respiratory failure and early death. The introduction of gene replacement therapy (GRT) modified the natural history of the disease. However, more data is needed to understand the long-term effect of GRT on measurable respiratory outcomes. We report the respiratory outcomes in our cohort of patients with SMA post-GRT in 2-year period. A retrospective chart-review of genetically confirmed children with SMA who received GRT between 2019 and 2021 in Qatar. The evaluated respiratory outcomes were chronic respiratory support, respiratory hospitalizations, escalation of respiratory support and polysomnography results before and after GRT. Nonrespiratory outcomes; nutritional status, swallowing, and motor functions; were also assessed. A total of 11 patients (9 patients with SMA-1 and 2 patients with SMA-2) received GRT at a median age of 12 months and 22 months in patients with SMA-1 and SMA-2, respectively. All patients were successfully weaned off Noninvasive ventilation (NIV) except one patient who remained on mechanical ventilation through tracheostomy tube. The annualized hospitalization rate dropped by half after GRT. The average length of stay (LOS) in intensive care unit (ICU) decreased by 17.32 days/patient/year after GRT. Duration of required escalation of respiratory support during acute hospitalizations has dropped by 18.56 days/patient/year post-GRT. We report favorable respiratory outcomes of GRT in our cohort. GRT resulted in discontinuation of chronic respiratory support in majority of ventilated patients. GRT also resulted in decreased respiratory hospitalization rate, hospital-LOS, ICU-LOS, and need for escalation of ventilatory support.

“New Trach Mom Here…”: A qualitative study of internet‐based resources by caregivers of children with tracheostomy

Decision-making around tracheostomy placement and chronic respiratory support in children is complicated. Families often seek support and advice from outside the medical care team, including from social media. We undertook this study to characterize the content and nature of online resources created and managed primarily by caregivers of children living with tracheostomy and chronic mechanical ventilation. We used a "grey literature" search methodology to identify internet resources created by caregivers of children with tracheostomy. We included only publicly available, nonindustry associated, English language, North American websites updated at least once in 2019. We then applied inductive content analysis to establish central themes, patterns and associations. We identified six blogs/forums that met our search criteria. We identified four main themes: (1) Uncertainty, (2) Lived experience-wants, needs, and emotions, (3) Seeking context and meaning, and (4) Advice/information sharing/support. Two patterns of coping were identified on the basis of the relationships between codes. The "Acceptance pathway" is associated with a sense of self-actualization, mastery, satisfaction, return to normalcy, and ultimately acceptance. The "Resignation pathway" is associated with a sense of lack of control, frustration, burnout and stress, persistent lack of normalcy, and resignation to the tracheostomy as a negative but necessary outcome. Caregivers often come to see themselves as experts in the care of children with tracheostomy, though many still express ambivalence about their knowledge and skills. Those early in the experience express a desire for community and can potentially benefit from online resources.

Pneumonia and Cardiac Function in Patients with Muscular Dystrophy

Pneumonia and Cardiac Function in Patients with Muscular Dystrophy Background: Patients with Duchenne Muscular Dystrophy (DMD) suffer from progressive muscle degeneration and weakness. Weakness of the respiratory muscles leads to decline in respiratory function and recurrent pneumonia. With chronic respiratory support, more patients may survive pulmonary complications and succumb to heart failure and ventricular arrhythmias associated with their cardiomyopathy. The relationship between pneumonia and its impact on cardiomyopathy has not been well explored. We hypothesized that pneumonia in a patient with …

Prognostic relevance and inter-observer reliability of chest-imaging in pediatric ARDS: a pediatric acute respiratory distress incidence and epidemiology (PARDIE) study

PurposeDefinitions of acute respiratory distress syndrome (ARDS) include radiographic criteria, but there are concerns about reliability and prognostic relevance. This study aimed to evaluate the independent relationship between chest imaging and mortality and examine the inter-rater variability of interpretations of chest radiographs (CXR) in pediatric ARDS (PARDS).MethodsProspective, international observational study in children meeting Pediatric Acute Lung Injury Consensus Conference (PALICC) criteria for PARDS, which requires new infiltrate(s) consistent with pulmonary parenchymal disease, without mandating bilateral infiltrates. Mortality analysis focused on the entire cohort, whereas inter-observer variability used a subset of patients with blinded, simultaneous interpretation of CXRs by intensivists and radiologists.ResultsBilateral infiltrates and four quadrants of alveolar consolidation were associated with mortality on a univariable basis, using CXRs from 708 patients with PARDS. For patients on either invasive (IMV) or non-invasive ventilation (NIV) with PaO2/FiO2 (PF) ratios (or SpO2/FiO2 (SF) ratio equivalent) > 100, neither bilateral infiltrates (OR 1.3 (95% CI 0.68, 2.5), p = 0.43), nor 4 quadrants of alveolar consolidation (OR 1.6 (0.85, 3), p = 0.14) were associated with mortality. For patients with PF ≤ 100, bilateral infiltrates (OR 3.6 (1.4, 9.4), p = 0.01) and four quadrants of consolidation (OR 2.0 (1.14, 3.5), p = 0.02) were associated with higher mortality. A subset of 702 CXRs from 233 patients had simultaneous interpretations. Interobserver agreement for bilateral infiltrates and quadrants was “slight” (kappa 0.31 and 0.33). Subgroup analysis showed agreement did not differ when stratified by PARDS severity but was slightly higher for children with chronic respiratory support (kappa 0.62), NIV at PARDS diagnosis (kappa 0.53), age > 10 years (kappa 0.43) and fluid balance > 40 ml/kg (kappa 0.48).ConclusionBilateral infiltrates and quadrants of alveolar consolidation are associated with mortality only for those with PF ratio ≤ 100, although there is high- inter-rater variability in these chest-x ray parameters.Electronic supplementary materialThe online version of this article (10.1007/s00134-020-06074-7) contains supplementary material, which is available to authorized users.

A Membrane Lung Design Based on Circular Blood Flow Paths.

Current hollow fiber membrane lungs feature a predominantly straight blood path length across the fiber bundle, resulting in limited O2 transfer efficiency because of the diffusion boundary layer effect. Using computational fluid dynamics and optical flow visualization methods, a hollow fiber membrane lung was designed comprising unique concentric circular blood flow paths connected by gates. The prototype lung, comprising a fiber surface area of 0.28 m, has a rated flow of 2 L/min, and the oxygenation efficiency is 357 ml/min/m. The CO2 clearance of the lung is 200 ml/min at the rated blood flow. Given its high gas transfer efficiency, as well as its compact size, low priming volume, and propensity for minimal thrombogenicity, this lung design has the potential to be used in a range of acute and chronic respiratory support applications, including providing total respiratory support for infants and small children and CO2 clearance in adults.

Retrospective Analysis of Factors Leading to Pediatric Tracheostomy Decannulation Failure. A Single-Institution Experience.

There is a lack of evidence regarding factors associated with failure of tracheostomy decannulation. We aimed to identify characteristics of pediatric patients who fail a tracheostomy decannulation challenge Methods: A retrospective review was performed on all patients who had a decannulation challenge at a tertiary care center from June 2006 to October 2013. Tracheostomy decannulation failure was defined as reinsertion of the tracheostomy tube within 6 months of the challenge. Data on demographics, indications for tracheostomy, home mechanical ventilation, and comorbidities were collected. Data were also collected on specific airway endoscopic findings during the predecannulation bronchoscopy and airway surgical procedures before decannulation. We attempted to predict the decannulation outcome by analyzing associations. 147 of 189 (77.8%) patients were successfully decannulated on the first attempt. Tracheostomy performed due to chronic respiratory failure decreased odds for decannulation failure (odds ratio = 0.34, 95% confidence interval = 0.15-0.77). Genetic abnormalities (45%) and feeding dysfunction (93%) were increased in the population of patients failing their first attempt. The presence of one comorbidity increased the odds of failure by 68% (odds ratio = 1.68, 95% confidence interval = 1.23-2.29). Decannulation pursuit based on parental expectation of success, rather than medically determined readiness, was associated with a higher chance of failure (P = 0.01). Our study highlights the role of genetic abnormalities, feeding dysfunction, and multiple comorbidities in patients who fail decannulation. Our findings also demonstrate that the outcome of decannulation may be predicted by the indication for tracheostomy. Patients who had tracheostomy placed for chronic respiratory support had a higher likelihood of success. Absence of a surgically treatable airway obstruction abnormality on the predecannulation bronchoscopy increased the chances of success.

Description of Respiratory Microbiology of Children With Long-Term Tracheostomies.

There is little evidence in the medical literature to guide empiric treatment of pediatric patients with long-term tracheostomies who present with signs and symptoms of a bacterial respiratory infection. The overall goal of this study was to describe the respiratory microbiology in this study population at our institution. This study was a retrospective chart review of all subjects with tracheostomies currently receiving care at the Arkansas Center for Respiratory Technology Dependent Children. Descriptive statistics were used to describe the respiratory microbiology of the full study group. Several subgroup analyses were conducted, including description of microbiology according to time with tracheostomy, mean time to isolation of specific organisms after the tracheostomy tube was placed, association between Pseudomonas aeruginosa or methicillin-resistant Staphylococcus aureus isolation and prescribed antibiotic courses, and description of microbiology according to level of chronic respiratory support. Available respiratory culture results up to July 2011 were collected for all eligible subjects. Descriptive statistics were used to describe subject characteristics, and chi-square analysis was used to analyze associations between categorical data. P < .05 was considered statistically significant. A total of 93 subjects met inclusion criteria for the study. The median (interquartile range) age at time of tracheotomy was 0.84 (0.36-3.25) y, and the median (interquartile range) time with tracheostomy was 4.29 (2.77-9.49) y. The most common organism isolated was P. aeruginosa (90.3%), with Gram-negative organisms predominating. However, 55.9% of the study population had a respiratory culture positive for methicillin-resistant S. aureus. The first organism isolated after tracheostomy placement was Methiciliin-sensitive S. aureus was isolated the soonest after tracheostomy placement. Specific organisms were not related to level of chronic respiratory support or likelihood of receiving antibiotics. This study provides an updated overview of the variety of potential pathogens isolated from respiratory cultures of pediatric subjects with long-term tracheostomies.

Congenital Myasthenic Syndromes in childhood: Diagnostic and management challenges

The Congenital Myasthenic Syndromes (CMS), a group of heterogeneous genetic disorders of neuromuscular transmission, are often misdiagnosed as congenital muscular dystrophy (CMD) or myopathies and present particular management problems. We present our experience of 46 children with CMS, referred to us between 1992–2007 with provisional diagnoses of congenital myopathy (22/46), CMS or limb-girdle myasthenia (9/46), central hypotonia or neurometabolic disease (5/46), myasthenia gravis (4/46), limb–girdle or congenital muscular dystrophy (4/46) and SMA (2/46). Diagnosis was often considerably delayed (up to 18y4 m), despite the early symptoms in most cases. Diagnostic clues in the neonates were feeding difficulties (29/46), hypotonia with or without limb weakness (21/46), ptosis (19/46), respiratory insufficiency (12/46), contractures (4/46) and stridor (6/46). Twenty–five children had delayed motor milestones. Fatigability developed in 43 and a variable degree of ptosis was eventually present in 40. Over the period of the study, the mainstay of EMG diagnosis evolved from repetitive nerve stimulation to stimulation single fibre EMG. The patients were studied by several different operators. 66 EMGs were performed in 40 children, 29 showed a neuromuscular junction abnormality, 7 were myopathic, 2 had possible neurogenic changes and 28 were normal or inconclusive. A repetitive CMAP was detected in only one of seven children with a COLQ mutation and neither of the two children with Slow Channel Syndrome mutations. Mutations have been identified so far in 32/46 children: 10 RAPSN, 7 COLQ, 6 CHRNE, 7 DOK7, 1 CHRNA1 and 1 CHAT. 24 of 25 muscle biopsies showed myopathic changes with fibre size variation; 14 had type-1 fibre predominance. Three cases showed small type-1 fibres resembling fibre type disproportion, and four showed core-like lesions. No specific myopathic features were associated with any of the genes. Twenty children responded to Pyridostigmine treatment alone, 11 to Pyridostigmine with either 3, 4 DAP or Ephedrine and five to Ephedrine alone. Twenty one children required acute or chronic respiratory support, with tracheostomy in 4 and nocturnal or emergency non-invasive ventilation in 9. Eight children had gastrostomy. Another 11 were underweight for height indicative of failure to thrive and required dietetic input. A high index of clinical suspicion, repeat EMG by an experienced electromyographer and, if necessary, a therapeutic trial of Pyridostigmine facilitates the diagnosis of CMS with subsequent molecular genetic confirmation. This guides rational therapy and multidisciplinary management, which may be crucial for survival, particularly in pedigrees where previous deaths have occurred in infancy.

Included but isolated: early intervention programmes provision for children and families with chronic respiratory support needs

To measure enrollment of children with mechanical respiratory support needs within the Massachusetts early intervention programmes (EIP) and describe challenges in execution of individual family service plans (IFSPs). Mixed methods provided a complementary assessment. Quantitative data were collected in 2005 from the Commonwealth of Massachusetts EIP administrative database as part of a cross-sectional state census. Qualitative data were retrieved from EIP regional coordinators in a key informant focus group. Descriptive statistics were used for quantitative survey data. Audio-recordings were transcribed verbatim and a qualitative, thematic analysis was undertaken. Four hundred and eighty children requiring mechanical respiratory support at the time of EIP enrollment were identified between 1 July 1997 and 1 January 2005. Focus group analysis revealed themes including: (1) barriers to community transition; (2) community expertise and training; (3) interface with medical providers; and (4) the role of web-based resources. Isolation of families emerged as a recurrent and resounding concept, relating to all of the themes. Findings support the assumption that implementation of IFSPs for the cohort of children with chronic mechanical respiratory support needs in EIPs is challenging. Barriers reflect inconsistent care coordination and practical encumbrances, contributing to the physical and social isolation of these children and their families.

Chronic Ventilator Need in the Community: A 2005 Pediatric Census of Massachusetts

The purpose of this study was to describe the population of children with chronic mechanical ventilation in Massachusetts and their patterns of medical care. Investigators surveyed all of the Massachusetts home ventilator clinics, pediatric pulmonary services, hospital-based pediatric services for special health care needs, insurers, home care vendors, nursing agencies, the Massachusetts Department of Public Health, selected individual providers, and rehabilitation and long-term care facilities providing services to children with chronic respiratory support needs. Support was defined as daily use of noninvasive, negative-pressure, or invasive/transtracheal ventilators. Subsequent matching of demographic data, including date of birth, zip code, and gender supported maximal census yield without duplications. Geographic information systems were used to create distribution maps and estimate distances between children with chronic mechanical ventilator needs and key resources. A total of 197 children were identified as requiring chronic mechanical respiratory support in Massachusetts in 2005, which was a nearly threefold increase in this population in the 15-year interval since the last census. Congenital or perinatal-acquired neurologic or neuromuscular disorders constituted the majority of primary diagnoses (n = 107 [54%]). Chronic lung disease attributed to prematurity represented only 7% of the sample. Children receiving chronic mechanical respiratory support are a growing population. The shift in underlying diagnoses from pulmonary disease to neurogenic respiratory insufficiency has implications for hospital and community-based providers from all disciplines in extending services to the home setting. Barriers encountered when performing this study, however, reflect an overall lack of coordination among the many individuals and agencies involved in their care. Coordinated and centralized care efforts require a clear and managed flow of information; census reports such as this one are only the beginning. Direct needs assessments and quality-of-life surveys from families are needed to design and implement programmatic changes and advocacy efforts.

Psychosocial problems arising from home ventilation.

To study psychosocial questions and problems of patients, who are chronically dependent on artificial ventilation, and their families. A total of 38 patients and family members (n = 43) were randomly selected. Several patients (n = 12) received respiratory support by nasal mask; the majority (n = 26) received respiratory support via a tracheotomy tube. Semistructured interviews were conducted. The Visual Analog Scale was also used. The extent to which the illness and chronic respiratory support were experienced as emotionally stressful, both by the patients and family members, was marked, on a scale ranging from 0 (light) to 10 (severe). Differences and similarities in the problems experienced were found among the patients and their families. Particularly in the case of patients receiving respiratory support by tracheostomy ventilation 24 hr/day, emotional stress on the family caregivers increased during the course of years. That the patient should urgently require bronchial suctioning at any moment made caregivers very anxious. However, several patients demonstrated increased skills in survival coping strategies. Despite diverse problems, the patients and their caregivers would elect to receive respiratory support, if the opportunity arose. In the case of chronically ill patients who receive continuous respiratory support via a tracheotomy tube, there is greater need for psychosocial support. Family members, who must be available 24 hr a day, need psychosocial support, professional home care, and voluntary assistance that have not been previously offered.

Pediatric noninvasive nasal ventilation.

Noninvasive nasal ventilation is an effective but underutilized method of chronic respiratory support for patients with respiratory insufficiency due to neuromuscular disease. Noninvasive nasal ventilation corrects nocturnal hypoxia and hypercapnia, resolving symptoms of chronic alveolar hypoventilation. Noninvasive nasal ventilation can allow selected patients with acute respiratory failure to avoid intubation and it can facilitate endotracheal extubation. Practical guidelines and the rationale for pediatric noninvasive nasal ventilation therapy will be discussed in this review.