Virtual mask fitting in pediatric patients during COVID-19: A case series.

INTRODUCTION: The COVID-19 pandemic has been an unprecedented threat to our health care system. Clinicians had to pivot and develop creative and timely "virtual" solutions to provide clinical care. Our aim was to develop a standardized approach to virtual "mask fitting" for children who are either being initiated or are already on existing long-term ventilation (LTV) at a pediatric hospital. CASE AND OUTCOMES: We present three cases involving the care of children who required mask fitting for noninvasive ventilation (NIV). LTV team consultations were delivered via videoconference or phone. With the guidance of the respiratory therapist (RT), the family caregiver (FC) took measurements on their child using a standardized clinical approach (developed by the LTV RTs). Based on the measurements, an appropriate mask was selected. Successful mask fit was based on patient/FC reports, as well as objective leak data obtained from the NIV download data. DISCUSSION: Virtual clinics used for managing patients in our LTV program were feasible and efficient resulting in improved workflow for the RTs and convenience for patients and FCs. Patients and FCs had significantly less pressure to attend in-person clinics and expressed high satisfaction in terms of their experience and importantly, meeting respiratory care needs. Within the context of COVID-19, remote patient education and intervention can be delivered effectively, while reducing the risk of exposure from in-person visits to hospital. CONCLUSION: A virtual/telemedicine program to manage pediatric patients requiring mask fitting for LTV was a feasible option during COVID-19.

Tough transitions: Family caregiver experiences with a pediatric long-term ventilation discharge pathway.

OBJECTIVES: Discharging a child home on long-term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, there has been a paucity of data with respect to the experiences of families transitioning a child home on LTV. Our objective was to explore the perceptions of family caregivers (FCs) who have completed a newly developed LTV discharge pathway as they transitioned home. METHODS: We conducted 11 semi-structured interviews with FCs. Interviews focused on FC's experience with the training process, perception of competency from a knowledge and skill perspective, and opportunities for improvement. Interviews were audiotaped, transcribed verbatim, coded, and analyzed using an inductive thematic analysis approach. RESULTS: Eight mothers and three fathers of ten children participated. Six primary themes were identified: (1) making an informed decision, (2) transitioning to rehabilitation, (3) building capacity for self-care, (4) coordinating case management, (5) readying for discharge home, and (6) experiencing home care. CONCLUSION: Overall, FCs felt that the preparation and transition support obtained through the application of a standardized LTV discharge pathway allowed successful attainment of new knowledge and skills necessary to care for their child with LTV at home.

Volume-assured pressure support mode for noninvasive ventilation: can it improve overnight adherence in children with neuromuscular disease?

PURPOSE: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD). METHODS: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes. RESULTS: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes. CONCLUSION: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD.

A Cross-Sectional Study of Nemaline Myopathy.

OBJECTIVE: Nemaline myopathy (NM) is a rare neuromuscular condition with clinical and genetic heterogeneity. To establish disease natural history, we performed a cross-sectional study of NM, complemented by longitudinal assessment and exploration of pilot outcome measures. METHODS: Fifty-seven individuals with NM were recruited at 2 family workshops, including 16 examined at both time points. Participants were evaluated by clinical history and physical examination. Functional outcome measures included the Motor Function Measure (MFM), pulmonary function tests (PFTs), myometry, goniometry, and bulbar assessments. RESULTS: The most common clinical classification was typical congenital (54%), whereas 42% had more severe presentations. Fifty-eight percent of individuals needed mechanical support, with 26% requiring wheelchair, tracheostomy, and feeding tube. The MFM scale was performed in 44 of 57 participants and showed reduced scores in most with little floor/ceiling effect. Of the 27 individuals completing PFTs, abnormal values were observed in 65%. Last, bulbar function was abnormal in all patients examined, as determined with a novel outcome measure. Genotypes included mutations in ACTA1 (18), NEB (20), and TPM2 (2). Seventeen individuals were genetically unresolved. Patients with pathogenic ACTA1 and NEB variants were largely similar in clinical phenotype. Patients without genetic resolution had more severe disease. CONCLUSION: We present a comprehensive cross-sectional study of NM. Our data identify significant disabilities and support a relatively stable disease course. We identify a need for further diagnostic investigation for the genetically unresolved group. MFM, PFTs, and the slurp test were identified as promising outcome measures for future clinical trials.

Assessment of factors affecting the sleep hygiene of medical students in Bahawalpur, Pakistan: a cross-sectional study.

OBJECTIVE: To identify the factors which have a statistically significant association with sleep hygiene of medical students. MATERIAL AND METHODS: This study was done on 100 medical students. The questionnaire that was used for the collection of data had two parts. First was related to demographic variables and second was a validated Pittsburgh sleep quality index (PSQI). All participants were from the first to fifth years of Bachelor of Medicine and Bachelor of Surgery (MBBS). Data were analysed using SPSS 23. The association was found by applying chi-square test. RESULTS: Our study revealed that out of 100 students, 61% had poor sleep hygiene (PSQI ≥ 5). Global PSQI scores among women were slightly higher than males. A statistical association was found between the residence and habit of studying at night of medical students with their sleep hygiene. DISCUSSION: Medical students should ensure good sleep hygiene in order to maintain their academic performance, physical health, and mental health.

How to use noninvasive positive airway pressure device data reports to guide clinical care.

There has been a significant increase in the past few decades in the number of children receiving noninvasive positive airway pressure (PAP) therapy at home. At present, PAP therapy can be successfully used in children of all ages, for a variety of indications. Data acquired from PAP devices is clinically useful, providing objective information regarding adherence, leak, and efficacy of PAP therapy. However, guidelines outlining a standardized approach to interpretation of PAP device data in pediatrics is currently lacking. Given the rapidly expanding use of PAP therapy in pediatric practice, we aim to provide an overview of the interpretation of data reports, otherwise called "data downloads," from PAP devices and illustrate how they can be used to guide clinical care.

Caregiver knowledge and skills to safely care for pediatric tracheostomy ventilation at home.

OBJECTIVE: Caregivers of children using home mechanical ventilation (HMV) via tracheostomy require appropriate knowledge and skills. Existing training curricula are locally developed and content variable. We sought to develop a competency checklist to inform initial training and subsequent assessment of knowledge and skills of family caregivers. METHODS: We used a 2-step process. Candidate items were generated by synthesis of a scoping review, existing checklists, with additional items suggested by an eight member inter-professional group representing pediatric HMV programs across Canada. Following removal of duplicate items, we conducted a three-round Delphi to gain consensus on items for the KidsVent Checklist. RESULTS: The scoping review and checklists from five HMV programs identified 18 domains and 172 items; one additional domain and 83 additional items were identified by our expert group who also classified domains as mandatory or optional. We recruited 95 clinicians representing 12 Canadian paediatric HMV programs to participate in Delphi round 1 (response rate 72%; 84%, and 100% for subsequent rounds). Importance rating of the 255 items reduced them to 246 items. In the final checklist, the 19 domains comprised 14 mandatory (189 mandatory items) and 5 optional domains (57 optional items). CONCLUSIONS: We have developed the KidsVent checklist using rigorous consensus building methods, informed by participants with diverse geographic and inter-professional representation. This checklist represents knowledge and skills required to safely care for children using tracheostomy ventilation at home. Further study is required to explore the impact of this checklist on outcomes of this growing group of technology-dependent children.

The Use of Speaking Valves in Children With Tracheostomy Tubes.

One-way speaking valves have been successfully used to restore audible meaningful speech in adult patients after tracheostomy tube placement. One-way speaking valves have also been used in pediatric patients after tracheostomy tube placement with promising results. We conducted a scoping review to synthesize and summarize the current evidence on the use of one-way tracheostomy tube speaking valves in the pediatric population to identify knowledge gaps that could inform future research programs and facilitate evidence-based clinical decision making. The Arksey and O'Malley 5-step methodological framework was used for this scoping review. We searched OVID MEDLINE, EMBASE, PubMed, Web of Science, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and Google Scholar to locate articles published between January 1, 1946 and May 26, 2016. Our search resulted in a total of 524 articles. After removing 270 duplicates, we screened 254 abstracts, and 50 articles were identified for full text review. We excluded 38 references. A total of 12 articles met our inclusion criteria. Details of all studies were charted. Application of the Sackett levels of evidence to evaluate the qualitative strength of the evidence provided by the 12 articles selected for study found that 6 studies were level 5, 4 were level 4, and 2 studies were categorized as level 3 evidence. Eligibility criteria for trials of speaking valves were inconsistent across all studies and included a combination of clinical assessment coupled with published indications. Much of the literature has focused on tolerance/successful use of speaking valves in children with a tracheostomy with limited evidence on its impact on verbal communication. Current evidence on the use of speaking valves in children with a tracheostomy, its indication, and its impact on verbal communication is inadequate, mandating further research in this area.

What families have in the emergency tracheostomy kits: Identifying gaps to improve patient safety.

OBJECTIVE: To evaluate the contents of parent-created emergency tracheostomy kits and identify deficiencies. METHODS: This was an observational study. Data on emergency tracheostomy kits were abstracted for 30 consecutive children who had a tracheostomy tube in situ during an outpatient clinic visit with the Division of Respiratory Medicine and/or the Department of Otolaryngology-Head and Neck Surgery at the Hospital for Sick Children between February 1 and October 30, 2016. A checklist of 12 essential items based on expert consensus was used to evaluate each tracheostomy kit. RESULTS: Emergency tracheostomy kits from all children were missing at least one item from the 12-item checklist. Nineteen (63%) kits had three or more critical items missing. All kits had the same size tracheotomy tube. Twenty-two (73%) kits did not have a half size smaller tracheostomy tube. Fifteen (50%) were missing a manual resuscitation bag and four (13.3%) were missing a suction machine. Children who had tracheostomy tube in situ for ≥4 years were more likely to have ≥3 missing items in their kit (43.4%) compared to those who had tracheostomy tube for <4 years (20%), (χ2 (1) = 9.85, P = 0.0017). CONCLUSION: Maintenance of a fully stocked emergency tracheostomy kit can save a child's life. It is incumbent upon healthcare providers to ensure ongoing reassessment of knowledge and skills required to care for a child with a tracheostomy tube and to regularly review the components of a child's emergency tracheostomy kit.

Intelligent volume-assured pressured support (iVAPS) for the treatment of congenital central hypoventilation syndrome.

PURPOSE: Congenital central hypoventilation syndrome (CCHS) is characterized by ventilatory insensitivity to hypercapnia and hypoxemia during sleep and/or wakefulness. Management of CCHS includes a long-term ventilation. However, ventilation can be challenging given differences in the control of breathing during different sleep stages. Intelligent volume-assured pressure support (iVAPS) is a mode of Bi-level positive airway pressure (BPAP) ventilation in which the pressure support is modulated to ensure a constant alveolar ventilation. The aim of this study was to determine if BPAP with iVAPS mode is more effective at controlling hypercapnia than BPAP with spontaneous/timed (S/T) mode. METHODS: A retrospective chart review of CCHS patients who underwent both a titration polysomnogram (PSG) with standard BPAP S/T mode and a consecutive follow-up study with BPAP iVAPS mode at The Hospital for Sick Children, Toronto, Canada, between January 1, 2013 and September 30, 2015 were included. Comparisons were made between S/T mode and iVAPS mode. RESULTS: Eight (four males) children with CCHS were included. The median (IQR) age at the time of PSG using Bi-level ventilation with S/T mode for study participants was 10.0 (IQR 8.4, 11.6) years followed by PSGs with iVAPS mode, median age 10.6 (IQR 9.1, 12.5) years. The non-rapid eye movement (NREM) peak transcutaneous CO2 (tcCO2) median (IQR) for iVAPS was 43.0 (40.0-46.0-) mmHg versus 46.5 (45.0-48.0) mmHg for S/T mode, (p value <0.05). CONCLUSION: iVAPS was associated with a reduction in the maximum tcCO2 during NREM sleep as compared to traditional S/T mode. Prospective, longitudinal studies are needed to evaluate the benefits of BPAP therapy iVAPS mode for the treatment of pediatric CCHS.

Factors Associated with Changes in Invasive and Noninvasive Positive Airway Pressure Therapy Settings during Pediatric Polysomnograms.

STUDY OBJECTIVES: Our aim was to identify clinical predictors associated with changes in settings for pediatric invasive and noninvasive positive airway pressure therapy, which could help inform the allocation of limited polysomnogram (PSG) resources. METHODS: A retrospective review was conducted in children who underwent one or more PSGs for technology titration. Children were included if they were using continuous positive airway pressure (CPAP) therapy, bilevel positive airway pressure (BPAP) therapy, or invasive positive pressure ventilation (IPPV) the night of the PSG. The primary outcome measure for the study were predictors of change in settings during IPPV, CPAP, and BPAP titration studies. RESULTS: During the study period, 274 children using CPAP, BPAP, or IPPV underwent one or more titration PSGs. The mean (standard deviation [SD]) age of the children at the time of the first titration PSG was 10.52 (5.11) y. Fifty percent (n = 136) of the study participants were male. Most patients underwent BPAP titration studies (n = 166), followed by CPAP (n = 83) and then IPPV (n = 25). A total of 623 technology titration PSGs were completed. Reason for respiratory technology, type of respiratory technology, and time between ventilation initiation and the PSG were significant predictors of a change in settings in the multivariable regression model. CONCLUSIONS: Children were more likely to have a change in their technology settings during a PSG if there was a shorter period of time from the original technology initiation, if they were using BPAP (as compared to CPAP or IPPV) and/or if they had a primary central nervous system or musculoskeletal diagnosis.

Special Considerations in the Nursing Care of Mechanically Ventilated Children.

Mechanical ventilation is often required to support the recovery of critically ill children. Critical care nurses must understand the unique needs of the children and design supportive care that is sensitive to their changing physiology, developmental stage, and socioemotional needs. This article describes the unique considerations in providing care for mechanically ventilated children. It addresses invasive and noninvasive ventilation and the needs of long-term ventilated children and family in critical care. Supportive nursing care that is aligned with the unique needs of the critically ill child is paramount to ensuring best outcomes for these vulnerable patients.

How long does it take to initiate a child on long-term invasive ventilation? Results from a Canadian pediatric home ventilation program.

OBJECTIVE: To assess the length of stay required to initiate long-term invasive ventilation at the authors' institution, which would inform future interventional strategies to streamline the in-hospital stay for these families. METHODS: A retrospective chart review of children initiated on invasive long-term ventilation via tracheostomy at the authors' acute care centre between January 2005 and December 2013 was performed. RESULTS: Thirty-five children were initiated on long-term invasive ventilation via tracheostomy at the acute care hospital; 19 (54%) were male. The median age at time of admission was 0.52 years (interquartile range [IQR] 0.06 to 9.58 years) . Musculoskeletal disease (n=11 [31%]) was the most common reason for tracheostomy insertion. Two children died during the hospital admission. Fifteen children were discharged home directly from the acute care hospital and 18 were moved to the rehabilitation hospital. Six are current inpatients of the rehabilitation centre and were never discharged home. Combining the length of stay at the acute care and rehabilitation hospitals for the entire cohort, the median length of stay was 162.0 days (IQR 98.0 to 275.0 days) and 97.0 days (IQR 69.0 to 210.0 days), respectively, from the time of tracheostomy insertion. CONCLUSIONS: The median length of stay from the initiation of invasive long-term ventilation to discharge home from the rehabilitation hospital was somewhat long compared with other ventilation programs worldwide. Additionally, approximately 20% of the cohort never transitioned home. There is a timely need to benchmark across the country and internationally, to identify and implement strategies for cohesive, coordinated care for these children to decrease overall length of stay.

Pediatric long-term home mechanical ventilation: twenty years of follow-up from one Canadian center.

BACKGROUND: Canadian longitudinal data from a pediatric domiciliary long-term mechanical ventilation (LTMV) program is lacking. OBJECTIVE: Our aim was to report on the clinical characteristics and trends of children followed in one of Canada's pediatric home ventilation programs over the past 20 years. METHODS: A retrospective chart review was conducted on patients receiving long-term domociliary mechanical ventilation between January 1, 1991 and December 31, 2011 in a single center. Domiciliary long-term mechanical ventilation was defined as the daily use of invasive mechanical ventilation (IMV) or noninvasive positive pressure ventilation (NiPPV) for at least 3 months, in the users' home or in a long-term residential facility. RESULTS: Between 1991 and 2011, a total of 379 children were identified (313 [83%] with noninvasive ventilation). The median age at initiation was 9.6 years (interquartile range [IQR] 2.9-13.9), the median duration of ventilation was 2.2 years (IQR 0.8-4.9) and 53% were male. Ninety-nine percent of children were cared for at home. The reason for ventilation was "musculoskeletal" in origin for the majority of children. The number of children receiving long-term mechanical ventilation at home increased from 2 in 1991 to 156 children as of December 2011. There was a twofold increase in the number of invasive ventilation initiations in the second 10 years, n = 45 (2001-2011) as compared to the first 10 years, n = 21 (1991-2000). However, there was more than a fivefold increase in the number of noninvasive initiations in the first 10 years, n = 50 (1991-2000) as compared to the second 10 years, n = 263 (2001-2011). The largest growth was in the 13-18 years age group. There were 55 (15%) mortalities over the study period. CONCLUSIONS: In summary, our 20-year retrospective study has shown that there has been an exponential growth in the number of children receiving domiciliary LTMV with the majority of children having favorable outcomes. Our study represents a step towards developing a Canadian registry to design and implement programmatic change for this medically complex population to ensure best practice for these children as well as their families.