WHAT'S KNOWN ON THIS SUBJECT: Childhood interstitial lung diseases occur in a variety of clinical contexts and are associated with high morbidity and mortality. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. WHAT THIS STUDY ADDS:This study demonstrates that cases of newly described forms of childhood interstitial lung diseases likely occur at all children' s hospitals. With advances in genetic testing and recognition of imaging patterns, a significant portion of cases are identifiable with noninvasive evaluations. abstract OBJECTIVE: Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns. METHODS:Childhood ILD cases seen at Vanderbilt Children' s Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system. RESULTS:A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review.CONCLUSIONS: Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy. Pediatrics 2013;132:684-691

Objective To describe the Trach Safe Initiative and assess its impact on unanticipated tracheostomy-related mortality in outpatient tracheostomy-dependent children (TDC). Methods An interdisciplinary team including parents and providers designed the initiative with quality improvement methods. Three practice changes were prioritized: (1) surveillance airway endoscopy prior to hospital discharge from tracheostomy placement, (2) education for community-based nurses on TDC-focused emergency airway management, and (3) routine assessment of airway events for TDC in clinic. The primary outcome was annual unanticipated mortality after hospital discharge from tracheostomy placement before and after the initiative. Results In the 5 years before and after the initiative, 131 children and 155 children underwent tracheostomy placement, respectively. At the end of the study period, the institution sustained Trach Safe practices: (1) surveillance bronchoscopies increased from 104 to 429 bronchoscopies, (2) the course trained 209 community-based nurses, and (3) the survey was used in 488 home ventilator clinic visits to identify near-miss airway events. Prior to the initiative, 9 deaths were unanticipated. After Trach Safe implementation, 1 death was unanticipated. Control chart analysis demonstrates significant special-cause variation in reduced unanticipated mortality. Discussion We describe a system shift in reduced unanticipated mortality for TDC through 3 major practice changes of the Trach Safe Initiative. Implication for Practice Death in a child with a tracheostomy tube at home may represent modifiable tracheostomy-related airway events. Using Trach Safe practices, we address multiple facets to improve safety of TDC out of the hospital.

OBJECTIVE The National Institutes of Health (NIH) created the Patient Reported Outcomes Measurement Information System (PROMIS®) to allow efficient, online measurement of patient-reported outcomes (PROs), but it remains untested whether PROMIS improves outcomes. Here, we aimed to compare the impact of gastrointestinal (GI) PROMIS measures vs. usual care on patient outcomes. METHODS We performed a pragmatic clinical trial with an off-on study design alternating weekly between intervention (GI PROMIS) and control arms at one Veterans Affairs (VA) and three university-affiliated specialty clinics. Adults with GI symptoms were eligible. Intervention patients completed GI PROMIS symptom questionnaires on an e-portal one week before their visit; PROs were available for review by patients and their providers prior to and during the clinic visit. Usual care patients were managed according to customary practices. Our primary outcome was patient satisfaction as determined by the Consumer Assessment of Healthcare Providers & Systems (CAHPS) questionnaire. Secondary outcomes included provider interpersonal skills (Doctors’ Interpersonal Skills Questionnaire [DISQ]) and shared decision-making (9-item Shared Decision Making Questionnaire [SDM-Q-9]). RESULTS There were 217 and 154 patients in the GI PROMIS and control arms, respectively. Patient satisfaction was similar between groups (p>.05). Intervention patients had similar assessments of their providers’ interpersonal skills (DISQ 89.4±11.7 vs. 89.8±16.0, p=.79) and shared decision-making (SDM-Q-9 79.3±12.4 vs. 79.0±22.0, p=.85) vs. controls. CONCLUSIONS This is the first controlled trial examining the impact of NIH PROMIS in clinical practice. One-time use of GI PROMIS did not improve patient satisfaction or assessment of provider interpersonal skills and shared decision-making. Future studies examining how to optimize PROs in clinical practice are encouraged before widespread adoption.

Objectives/Hypothesis The Seattle Children's Hospital implemented the Trach Safe Initiative to improve airway safety in tracheostomy‐dependent children (TDC). A key tenet of this initiative is surveillance endoscopy. The objectives of this study were to describe the prevalence of abnormal airway changes in TDC, identify risk factors for these changes, and describe the frequency of airway interventions. Study Design Retrospective case series. Methods This is a review of children 0 to 21 years old who underwent tracheostomy and surveillance endoscopy from February 1, 2014 to January 1, 2019. Descriptive statistics were used to report the prevalence of abnormal airway changes and interventions following tracheostomy. Pearson χ2 tests and logistic regression were used to identify risk factors for the development of abnormal changes. Results There were 127 children identified. The median time from tracheostomy to initial surveillance endoscopy was 1.6 months (interquartile range = 1.3–2.4 months). At initial endoscopy, 86.6% of patients had at least one abnormal airway finding. The most common findings were subglottic edema/stenosis (57.3%), glottic edema (37.3%), and suprastomal granulation tissue (31.8%). Prematurity and a history of failed extubations were significantly associated with abnormal findings on endoscopy (odds ratio [OR] = 7.2, P = .01 and OR = 4.1, P = .03, respectively). Of those with abnormal findings, 32.7% underwent an intervention to improve airway patency and safety. The most common interventions performed were suprastomal granuloma excision (44.4%), steroid injection (22.2%), and balloon dilation of the glottis or subglottis (19.4%). Conclusions The prevalence of early abnormal airway changes in TDC is high, particularly in young children with a history of prematurity and failed extubation. Level of Evidence 4 Laryngoscope, 130:1327–1332, 2020

Objective It is important for clinicians to inquire about “alarm features” as it may identify those at risk for organic disease and who require additional diagnostic workup. We developed a computer algorithm called Automated Evaluation of Gastrointestinal Symptoms (AEGIS) that systematically collects patient gastrointestinal (GI) symptoms and alarm features, and then “translates” the information into a history of present illness (HPI). Our study's objective was to compare the number of alarms documented by physicians during usual care vs. that collected by AEGIS. Methods We performed a cross-sectional study with a paired sample design among patients visiting adult GI clinics. Participants first received usual care by their physicians and then completed AEGIS. Each individual thus contributed both a physician-documented and computer-generated HPI. Blinded physician reviewers enumerated the positive alarm features (hematochezia, melena, hematemesis, unintentional weight loss, decreased appetite, and fevers) mentioned in each HPI. We compared the number of documented alarms within patient using the Wilcoxon signed-rank test. Results Seventy-five patients had both physician and AEGIS HPIs. AEGIS identified more patients with positive alarm features compared to physicians (53% vs. 27%; p < .001). AEGIS also documented more positive alarms (median 1, interquartile range [IQR] 0–2) vs. physicians (median 0, IQR 0–1; p < .001). Moreover, clinicians documented only 30% of the positive alarms self-reported by patients through AEGIS. Conclusions Physicians documented less than one-third of red flags reported by patients through a computer algorithm. These data indicate that physicians may under report alarm features and that computerized “checklists” could complement standard HPIs to bolster clinical care.

Objective. Juvenile systemic sclerosis (SSc) is a disabling autoimmune condition that affects multiple organs in addition to skin, notably the gastrointestinal and pulmonary systems. The relationship between esophageal abnormalities and pulmonary disease in juvenile SSc is not well understood. We describe associations between radiologic esophageal abnormalities and pulmonary function.Methods. Clinical and radiographic data of children ages >18 years who fulfilled the 2007 Pediatric Rheumatology Provisional Classification Criteria for juvenile SSc between 1994 and 2016 were reviewed. Fluoroscopic upper gastrointestinal (UGI) studies, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs) within 12 months of presentation to Seattle Children's Hospital were extracted.Results. Twenty-one children with juvenile SSc (67% female, ages 8-17 years) were studied. Esophageal abnormalities, defined as abnormal esophageal peristalsis and/or bolus clearance, were found in 12 patients. Abnormal esophagus on UGI tests was not associated with gastrointestinal or pulmonary symptoms, disease duration, use of medications (proton pump inhibitor or immunosuppressant), or specific autoantibodies. Compared with patients with a normal esophagus on UGI tests, children with an abnormal esophagus had decreased PFTs: mean forced expiratory volume in 1 second 96% versus 78% (P = 0.03), forced vital capacity 94% versus 76% (P = 0.02), and vital capacity 95% versus 76% (P = 0.02). Children with an abnormal esophagus on UGI tests had a larger mean esophageal diameter on HRCT (14.6 mm compared to 8.5 mm; P < 0.01).Conclusion. There was an association between esophageal and pulmonary disease in children with juvenile SSc. Esophageal findings on UGI tests or HRCT, despite lack of symptoms, should raise concern for esophageal dysfunction and prompt heightened surveillance for concurrent lung disease.

Objectives/Hypothesis: Given the costs of healthcare, capitation, and desires for quality improvement (QI), there is a need to better assess healthcare value. Time-driven activity-based costing and the Quadruple Aim have evaluated value by assessing health outcomes and provider experiences relative to costs. The proposed OPPS/Cost method expands on this to examine value for aerodigestive clinic treatment of pediatric persistent pharyngeal dysphagia: O + P 1 + P 2 + S/Cost (O = objective health [video-fluoroscopic swallow study results], P 1 = patient/family experience [Consumer Assessment of Healthcare Providers and Systems], P 2 = provider experience [Copenhagen Burnout Inventory {CBI}], S = subjective health [Feeding/Swallowing-Impact Survey], C = cost [time-driven activity-based costing]).Study Design: Use of QI time data, surveys, and retrospective chart review for 56 patient encounters. Methods: Staff interviews were used to develop process maps, and monetary values were assigned to activities. OPPS/ Cost outcomes were normalized amongst variables, and composite values were calculated. Comparisons were made using a Student t test for pre-and postclinic relocation over a 14-month period.Results: Time reductions were check-in (13 minutes/patient), rooming (21 minutes/patient), and providers (4 minutes/patient). Patient in-room wait time increased (4 minutes/patient). The CBI identified burnout as an area for improvement. OPPS/Cost composite values increased by 14%, with a 1.7% cost reduction, improvement in objective and subjective health outcomes of 47.4% (P < .05) and 7.3%, respectively, and stable patient/family experience.Conclusions: OPPS/Cost is feasible in an interdisciplinary clinic and helped evaluate value during a clinic relocation. The QI opportunities identified are indicative of the potential of OPPS/Cost.

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