Chronic tracheostomy care of ventilator-dependent and -independent children: Clinical practice patterns of pediatric respirologists in a publicly funded (Canadian) healthcare system.

OBJECTIVES: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation. METHODS: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up. RESULTS: The response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re-use, and cleaning practices. Most centers require two trained caregivers (14/17) and recommend 24/7 eyes on care and oxygen saturation monitoring. Discharge with an emergency tracheostomy kit was universal (17/17). Considerable heterogeneity was observed in the timing and use of speaking valves and speech-language assessment. Inhaled anti-pseudomonal antibiotics are employed by most centers (16/17) though the indication, agent, and protocol varied by center. Though decannulation practices varied considerably, the requirement of upper airway patency was universally required to proceed with decannulation (17/17) independent of ongoing ventilatory support requirements. CONCLUSION: Considerable variability in pediatric tracheostomy tube care practice exists across Canada. These results will serve as a starting point to standardize and evaluate tracheostomy tube care nationally.

Granulomatosis with polyangiitis in an adolescent male with chronic cough and pulmonary nodules.

Granulomatosis with polyangiitis (GPA) is a small to medium vessel vasculitis that is uncommon in paediatrics. However, with chronic cough often being the initial symptom, a common complaint and a median age of diagnosis of 14 years, it is nevertheless an important condition for paediatricians to consider as it can otherwise go undiagnosed for a long period of time. In this case report, we discuss a paediatric patient with GPA that presented with non-specific respiratory symptoms for several months and was then found to have pulmonary nodules on chest imaging once a broader differential diagnosis was considered. We will review the common presentation of GPA, the classification criteria and its management. This will ultimately assist any providers in identifying and managing GPA cases.

Assessing air travel safety in neuromuscular disease: standard versus prolonged hypoxic challenge tests.

PURPOSE: The hypoxic challenge test (HCT) is used to evaluate safety for air travel in individuals with respiratory disease by breathing in 15% oxygen for 20 min. Our aim was to determine if a prolonged HCT, lasting 120 min, identified more individuals with neuromuscular disease at potential risk than the standard HCT lasting 20 min. METHODS: This was a cross-sectional study. All of the clinical testing took place at SickKids, Toronto, Canada. Patients were included in the study if they had a diagnosis of NMD, greater than 6 years of age, resting oxygen saturation ≥ 94%, and partial pressure of carbon dioxide (pCO2) ≤ 45 mmHg. Notable exclusion criteria were left ventricular ejection fraction < 30%, presence of a tracheostomy, and use of non-invasive ventilation for more than 12 h daily. Participants underwent a standard HCT as well as the prolonged HCT on the same day. RESULTS: Twenty-three patients consented to the study. One patient was withdrawn because he was unable to follow the study procedures. The 22 study participants had a mean age of 14.9 years (standard deviation (SD) of 5 years). Seventeen (77%) participants were male. Two participants were withdrawn on the day of testing due to hypercapnia. Twenty participants completed the standard and prolonged HCTs. None of the participants had a positive standard or prolonged HCT. CONCLUSION: Our results suggest that performing a standard or prolonged HCT may, in fact, not be of clinical utility in individuals with less severe NMD.

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.

Respiratory Diagnostic Tools in Neuromuscular Disease.

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.

Pulmonary Alveolar Microlithiasis.

Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive condition that is often asymptomatic despite significant changes in chest imaging. Diagnosis is often made when patients become symptomatic in adulthood. There are still no proven treatments, but earlier diagnosis may allow for evaluation of preventative strategies that could improve outcome. It is an important diagnosis to consider in children who have marked radiographic findings with no or very mild symptoms or physical findings. Diagnosis can be made with imaging alone but may necessitate lung biopsy for definitive diagnosis.