Pediatric Pulmonary Milestones 2.0: Development, Lessons Learned, and Future Directions.

Pediatric pulmonology fellowship training programs are required by the Accreditation Council for Graduate Medical Education to report Pediatric Subspecialty Milestones biannually to track fellow progress. However, several issues, such as lack of subspecialty-specific context and ambiguous language, have raised concerns about their validity and applicability to use for fellow assessment and curriculum development. In this Perspective, we briefly share the process of the Pediatric Pulmonology Milestones 2.0 Work Group in creating new specialty-specific Milestones and tailoring information on the Harmonized Milestones to pediatric pulmonologists, with the goal of improving the Milestones' utility for stakeholders, including pulmonology fellows, faculty, program directors, and accrediting bodies. In addition, we created a supplemental guide to better link the Milestones to pulmonary-specific scenarios to create a shared mental model between stakeholders and remove a potential detriment to validity. Through the process, a number of guiding principles were clarified, including: 1) every Milestone should be able to be assessed independently, without overlap with other Milestones; 2) there should be clear developmental progression from one Milestone to the next; 3) Milestones should be based on the unique skills expected of pediatric pulmonologists; and 4) health equity should be a core component to highlight as a top priority to all stakeholders. In this Perspective, we describe these principles that guided formulation of the Pediatric Pulmonary Milestones to help familiarize the pediatric pulmonary community with the new Milestones. In addition, we share lessons learned and challenges in our process to inform other specialties that may soon participate in this process.

Improvement in cystic fibrosis newborn screening program outcomes with genetic counseling via telemedicine.

INTRODUCTION: The Cystic Fibrosis Foundation (CF Foundation) recommends the provision of genetic counseling (GC) to help educate families and decrease anxiety around the cystic fibrosis (CF) newborn screening process. Unfortunately, access to genetic counselors is limited, especially for CF trained genetic counselors. We hypothesized that the GC process for families could be improved by utilizing telemedicine to leverage the availability of two dedicated, CF trained genetic counselors to provide access to GC for several CF centers. In addition, we hoped to demonstrate that use of trained CF genetic counselors, delivering GC via telemedicine at the time of sweat testing, would provide families with understanding of CF genetics as well as result in high satisfaction with the newborn screening process. METHODS: GC was provided by CF trained genetic counselors via telemedicine at the time of sweat testing. Following the counseling session, families were administered an anonymous written survey to evaluate their impression of the services provided. A subset of 50 families was recruited for an assessment of gained knowledge regarding CF genetics using the Ciske knowledge inventory. Using χ2  analysis, Ciske knowledge inventory data from our telemedicine GC families was compared to counseled and uncounseled Ciske historical controls. Lastly, in-depth interviews about the newborn screening process for CF were performed with 10 families and interviews were coded for emerging themes. RESULTS: During the 4 years of the study, 250 patients received GC. Overall comfort with the counseling rated 4.77 out of 5 using a Likert scale. After counseling by telemedicine, parents demonstrated improved understanding of the genetic implications of an abnormal CF newborn screen for their family, with 100% of families understanding that their child was a carrier for CF as compared to 97.2% of counseled (p = .023) and 78.5% of uncounseled (p = .0007) from Ciske historical controls. The study group also showed improvement in understanding of both parents possibly being carriers, with an 87.7% correct response rate compared to a 37.0% correct response rate in the counseled group (p < .0001) and a 35.4% correct response rate in the non-counseled group (p < .0001) from Ciske historical controls. Subgroup analysis at one site showed a significant increase in the number of infants with completed sweat tests from previous years (49% in 2013 vs. 80% in 2017 during the study, p < .0001). CONCLUSIONS: GC by telemedicine was well received by families and demonstrated improved family knowledge acquisition and understanding of CF as it related to risks for their child as well as identification of risks for other family members. Furthermore, in addition to an increase is those receiving GC, a subgroup analysis demonstrated a significant increase in the number of infants receiving sweat tests. This study demonstrates that GC via telemedicine for CF is feasible and demonstrates improvement in parent understanding of CF genetics. Furthermore, this method can be implemented effectively across a wide geographical area with a limited number of CF trained genetic counselors to improve access to care for patients and families.

Implementation of standardized asthma management programs in outpatient settings.

PURPOSE OF REVIEW: This article reviews new approaches, facilitators, barriers, and opportunities to increasing adoption of standardized asthma management programs in the outpatient care setting. RECENT FINDINGS: Primary care clinicians providing asthma care in the outpatient setting are challenged by the complexity of guidelines and want standardization of tools that are easy to use and that can be integrated within their practice's workflow. Programs that integrate clinical decision support tools within a practice's electronic health record and provide support from specialists may enhance uptake of asthma management programs in the outpatient setting and reduce asthma morbidity. Lack of an implementation science framework, consideration for organizational context, and clinician buy-in are recently recognized barriers to adoption of asthma programs and improved asthma outcomes. In addition, many of these interventions are labor intensive, costly, and may not be capable of wide dissemination because of the EHR interoperability problem. CONCLUSION: Programs that simplify the guidelines, integrate clinical decision support within the EHR, and ground their approach with an implementation science framework may improve the quality of asthma care provided in the outpatient setting.

Bacterial Surface Detachment during Nebulization with Contaminated Reusable Home Nebulizers.

Patients with chronic respiratory diseases use home nebulizers that are often contaminated with pathogenic microbes to deliver aerosolized medications. The conditions under which these microbes leave the surface as bioaerosols during nebulization are not well characterized. The objectives of this study were to (i) determine whether different pathogens detach and disperse from the nebulizer surface during aerosolization and (ii) measure the effects of relative humidity and drying times on bacterial surface detachment and aerosolization. Bacteria were cultured from bioaerosols after Pari LC Plus albuterol nebulization using two different sources, as follows: (i) previously used nebulizers donated by anonymous patients with cystic fibrosis (CF) and (ii) nebulizers inoculated with bacteria isolated from the lungs of CF patients. Fractionated bioaerosols were collected with a Next-Generation Impactor. For a subset of bacteria, surface adherence during rewetting was measured with fluorescence microscopy. Bacteria dispersed from the surface of used CF patient nebulizers during albuterol nebulization. Eighty percent (16/20) of clinical isolates inoculated on the nebulizer in the laboratory formed bioaerosols. Detachment from the plastic surface into the chamber solution predicted bioaerosol production. Increased relative humidity and decreased drying times after inoculation favored bacterial dispersion on aerosols during nebulized therapy. Pathogenic bacteria contaminating nebulizer surfaces detached from the surface as bioaerosols during nebulized therapies, especially under environmental conditions when contaminated nebulizers were dried or stored at high relative humidity. This finding emphasizes the need for appropriate nebulizer cleaning, disinfection, and complete drying during storage and informs environmental conditions that favor bacterial surface detachment during nebulization. IMPORTANCE Studies from around the world have demonstrated that many patients use contaminated nebulizers to deliver medication into their lungs. While it is known that using contaminated medications in a nebulizer can lead to a lung infection, whether bacteria on the surface of a contaminated nebulizer detach as bioaerosols capable of reaching the lung has not been studied. This work demonstrates that a subset of clinical bacteria enter solution from the surface during nebulization and are aerosolized. Environmental conditions of high relative humidity during storage favor dispersion from the surface. We also provide results of an in vitro assay conducted to monitor bacterial surface detachment during multiple cycles of rewetting that correlate with the results of nebulizer/bacterial surface interactions. These studies demonstrate for the first time that pathogenic bacteria on the nebulizer surface pose a risk of bacterial inhalation to patients who use contaminated nebulizers.

Nebulizer cleaning and disinfection practices in families with cystic fibrosis: The relationship between attitudes, practice and microbe colonization.

BACKGROUND: The daily burden of health maintenance for children and families with cystic fibrosis (CF) is immense with respect to time and complexity of care. Infection control practices, specifically nebulizer cleaning and disinfection, are a recommended component of home health care for CF families due to colonization of home respiratory equipment with lung pathogens. To better inform education interventions at our center, we were interested in studying how families' views on infection prevention and awareness of CF Foundation infection prevention and control (IP&C) guidelines correlate with actual home nebulizer care and the presence of microorganisms on their nebulizers. METHODS: Twenty families who have children with CF were surveyed to better understand attitudes toward infection prevention, awareness of CFF IP&C guidelines and nebulizer cleaning and disinfection practices in the home. Their nebulizers were also cultured for microbes to correlate recovery with infection control behaviors. RESULTS: A subset of families recognizes the importance of germ avoidance but do not recognize nebulizer cleaning and disinfection as very important for infection control practices. Decreased frequency of disinfection, but not cleaning, was correlated with the recovery of organisms on the nebulizers. CONCLUSIONS: The study questionnaire results identify a gap between recognizing the importance of infection prevention and consistently implementing CFF IP&C guidelines in the home. This demonstrates the need at our center for new educational interventions to promote cleaning and disinfection of home nebulizers after each use as recommended by the CFF.

Repeated hot water and steam disinfection of Pari LC Plus® nebulizers alter nebulizer output.

Currently, cystic fibrosis patients require daily nebulized treatments to achieve optimal lung health. Growth of pathogenic bacteria in patient nebulizers is well known, and disinfection guidelines have been established. In this short communication, we sought to discover what effect, if any, repeated nebulization/disinfection cycles had on nebulizer output. We nebulized saline repeatedly after exposure to boiling water, steam, and alcohol disinfection methods. While alcohol disinfection did not affect nebulizer output, boiling water and steam significantly decreased nebulizer output from baseline, 74.1 ±â€¯5.9% (p = 0.022) and steam 63.6 ±â€¯6.5% (p = 0.0048) after 60 cycles respectively. This decrease in nebulizer output could significantly increase the duration of nebulizer treatment time and negatively impact the burden of care on patients with cystic fibrosis.

Ozone disinfection of home nebulizers effectively kills common cystic fibrosis bacterial pathogens.

OBJECTIVE: The Cystic Fibrosis Foundation (CFF) recommends routine nebulizer disinfection for patients but compliance is challenging due to the heavy burden of home care. SoClean® is a user friendly ozone based home disinfection device currently for home respiratory equipment. The objective of this study was to determine whether SoClean® has potential as a disinfection device for families with CF by killing CF associated bacteria without altering nebulizer output. HYPOTHESIS: Ozone based disinfection effectively kills bacterial pathogens inoculated to home nebulizer equipment without gross changes in nebulizer function. STUDY DESIGN: Common bacterial pathogens associated with CF were inoculated onto the PariLC® jet nebulizer and bacterial recovery compared with or without varied ozone exposure. In separate experiments, nebulizer output was estimated after repeated ozone exposure by weighing the nebulizer. RESULTS: Ozone disinfection was time dependent with a 5 min infusion time and 120 min dwell time effectively killing >99.99% bacteria tested including Pseudomonas aeruginosa and Staphylococcus aureus. Over 250 h of repeat ozone exposure did not alter nebulizer output. This suggests SoClean® has potential as a user-friendly disinfection technique for home respiratory equipment.

Evaluation of Huddles: A Multisite Study.

Regular, interdisciplinary group meetings, "huddles," may be useful in improving communication among disciplines, resolving problems, and sharing information. Daily use of huddles may contribute to the development of a highly reliable health care organization. The purpose of this study was to describe safety huddles in relation to (1) problem type, (2) timeliness of resolution, (3) attendance of representatives from each discipline, (4) amount of information sharing, and (5) attendees' satisfaction with the process. Overall, results demonstrated that the primary function of huddles was the exchange of information that posed or had the potential to pose safety risks to patients. Across seven hospitals, the range of information sharing during huddles was 61.0% to 95.6%. Regarding satisfaction with the huddle process, staff reported that huddles were useful in improving awareness of safety concerns and also improved communication between disciplines. Huddles provide a structured format in which staff can positively impact safety concerns, form a greater sense of medical community, increase sharing of information between disciplines, quickly resolve discipline-based problems, and increase awareness of safety concerns. Given the results of this study, it is recommended that health care administrators and managers develop a huddle process.

An evaluation strategy for potential QTc prolongation with chronic azithromycin therapy in cystic fibrosis.

Chronic azithromycin therapy is recommended for CF patients with persistent Pseudomonas aeruginosa colonization. Other macrolide antibiotics have been reported to cause QT prolongation, but cardiac effects of azithromycin have not been studied in pediatric populations. We analyzed changes in QTc interval after starting chronic azithromycin in a pediatric CF population. Adolescent males showed increased QTc intervals after initiation of therapy. Given the possible effects of azithromycin on the QTc interval, particularly in patients predisposed to cardiac events, we suggest that the QTc interval of CF patients should be monitored throughout the course of chronic azithromycin.

Serial lung magnetic resonance imaging to monitor disease progression in a child with a diffuse alveolar hemorrhage syndrome.

Serial lung magnetic resonance imaging (MRI) was performed in a child with diffuse alveolar hemorrhage (DAH). To minimize radiation exposure with conventional serial chest computerized tomography (CT), serial MRIs of the lungs were used. This effectively monitored her disease process as well as detected acute hemorrhage after 5 years remission.

Blood-based gene expression signatures of infants and toddlers with autism.

OBJECTIVE: Autism spectrum disorders (ASDs) are highly heritable neurodevelopmental disorders that onset clinically during the first years of life. ASD risk biomarkers expressed early in life could significantly impact diagnosis and treatment, but no transcriptome-wide biomarker classifiers derived from fresh blood samples from children with autism have yet emerged. METHOD: Using a community-based, prospective, longitudinal method, we identified 60 infants and toddlers at risk for ASDs (autistic disorder and pervasive developmental disorder), 34 at-risk for language delay, 17 at-risk for global developmental delay, and 68 typically developing comparison children. Diagnoses were confirmed via longitudinal follow-up. Each child's mRNA expression profile in peripheral blood mononuclear cells was determined by microarray. RESULTS: Potential ASD biomarkers were discovered in one-half of the sample and used to build a classifier, with high diagnostic accuracy in the remaining half of the sample. CONCLUSIONS: The mRNA expression abnormalities reliably observed in peripheral blood mononuclear cells, which are safely and easily assayed in infants, offer the first potential peripheral blood-based, early biomarker panel of risk for autism in infants and toddlers. Future work should verify these biomarkers and evaluate whether they may also serve as indirect indices of deviant molecular neural mechanisms in autism.

Clinical features cannot distinguish allergic from non-allergic asthma in children.

OBJECTIVE: Environmental allergens are a major trigger of asthma, but not all asthmatics are allergic. This study was designed to review clinical characteristics in children with allergic and non-allergic asthma, based on responsiveness to allergy skin tests, in order to identify a combination of features that could distinguish allergic from non-allergic asthma in children. METHODS: Medical records of 321 children who had allergy skin testing were reviewed, and demographic and clinical data were compared between allergic and non-allergic patients. RESULTS: Approximately two-thirds of the asthmatic children had at least one positive skin test. These allergic patients were more likely to have a history of eczema or Medicaid insurance, but these findings had poor predictive value. There was no difference between allergic patients and non-allergic patients in terms of family history of atopy or asthma, home tobacco smoke exposure, age of onset of asthma, gender, rate of obesity, or asthma severity. Among the allergic asthma patients, neither the number of positive skin tests nor specific individual allergic sensitivities correlated with age of onset of asthma or asthma severity. CONCLUSIONS: This study failed to identify any combination of features that could reliably distinguish allergic from non-allergic asthma in children. Thus, all children with asthma should undergo allergy testing in order to identify potential allergic triggers in allergic patients and to avoid the institution of unnecessary environmental control measures in non-allergic patients.

Improved pulmonary and growth outcomes in cystic fibrosis by newborn screening.

BACKGROUND: Newborn screening for cystic fibrosis (CF) is effective in improving long-term growth outcomes. However, there is conflicting evidence that early diagnosis maintains normal pulmonary function. Our goal was to determine if newborn screening results in improved longitudinal growth and maintenance of normal pulmonary function. METHODS: A retrospective study of individuals with CF born in Connecticut between 1983 and 1997 was conducted by medical record and CF Foundation Registry review. Growth, pulmonary function and bacterial acquisition/colonization data, from diagnosis through July 1, 2005, were compared in those diagnosed by newborn screen (n = 34) to those diagnosed by sweat test after symptom appearance (n = 21). RESULTS: Screened individuals demonstrated greater weight and height for age at diagnosis (P = 0.01 and 0.01) and through 15 years of age (P = 0.0002 and 0.01). Body mass index was higher in screened individuals (21 vs. 18 kg/m(2)) at 15 years of age (P = 0.01). At 15 years of age, screened individuals had a clinically higher forced expiratory volume in 1 second (FEV(1)) and forced vital capacity (FVC; 90% and 104% predicted) than non-screened individuals (74% and 91% predicted; P = 0.08 and 0.10). Over a 9-year period, from ages 6 to 15, percent predicted FEV(1) and FVC increased by 4% and 13% in screened individuals; and declined by 14% and 5% respectively in non-screened individuals (P = 0.01 and 0.02). Acquisition/colonization of Pseudomonas aeruginosa was similar between groups (P = 0.23). CONCLUSIONS: In this CF cohort, individuals diagnosed by newborn screening have improved growth and preservation of normal pulmonary function without increased risk of Pseudomonas aeruginosa colonization.

How Connecticut primary care physicians view treatments for streptococcal and nonstreptococcal pharyngitis.

BACKGROUND: Inappropriate antibiotic treatment of respiratory infections has been reported to be common; however, the specifics of this inappropriate treatment are not completely defined. OBJECTIVE: The aim of this study was to gather data to determine whether physicians consider the national guidelines of treating group A B-hemolytic streptococci (GABHS) pharyngitis with penicillin (BID or TID) and not using antibiotics to treat nonstreptococcal pharyngitis. METHODS: In this pilot survey, a 1-page questionnaire was sent to a random sample of Connecticut primary care physicians (PCPs) that included emergency physicians, family physicians, internists, and pediatricians. Two short hypothetical scenarios were presented: (1) an untreated male patient aged 18 years (who is not penicillin allergic) was seen the day before with fever and pharyngitis. He returns because he is still symptomatic and his throat culture (TC) is positive for GABHS; and (2) same scenario but this second patient's TC is negative for GABHS. Physicians were asked how they would treat the patients in both scenarios. RESULTS: Of the 642 (representing approximately 15% of all Connecticut PCPs) questionnaires sent, 386 (60%) were returned. Seventy-five of the responding physicians had not seen patients with pharyngitis within the last year and were excluded; the following results were from 311 physicians. Scenario 1: 191 of 311 physicians (61%) indicated a willingness to treat the GABHS with penicillin. Pediatricians were significantly less likely, compared with the other PCPs, to prescribe penicillin (P = 0.01) in this scenario. Seventy-seven of the 191 physicians (40%) indicated a willingness to prescribe the penicillin QID. Seven PCPs who responded to scenario 1 did not respond to scenario 2. Scenario 2: 98 of the 304 physicians (32%) indicated a willingness to prescribe antibiotics for the patient with no: streptococcal pharyngitis. Pediatricians were significantly less likely, compared with the other PCPs, prescribe antibiotics for the patient with nonstreptococcal pharyngitis in the second scenario (P < 0.001). CONCLUSIONS: This pilot survey found that 61% of Connecticut PCPs might treat GABHS with penicillin according to existing guidelines although the penicillin might be prescribed QID instead of the recommended BID or TID. Thirty-two percent of these PCPs reported they would use antibiotics to treat non-GABH pharyngitis.

Endotracheal intubation and pediatric status asthmaticus: site of original care affects treatment.

OBJECTIVES: Status asthmaticus is a common cause of admission to a pediatric intensive care unit (PICU). Children unresponsive to medical therapies may require endotracheal intubation; however, this treatment carries significant risk, and thresholds for intubation vary. Our hypothesis was that children who sought care at community hospitals received less aggressive treatment and more frequent intubation than children who sought care at a children's hospital. DESIGN: Retrospective cohort study. SETTING: A university-affiliated children's hospital PICU. PATIENTS: We retrospectively examined data from all children older than 2 yrs admitted to the PICU with status asthmaticus between April 1997 and July 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 251 children admitted to the PICU with status asthmaticus, 130 initially presented to the emergency department of a children's hospital and 116 presented to the emergency department of a community hospital. Despite similar illness severity, children presenting to a community hospital were significantly more likely to be intubated than those presenting to a children's hospital (17% vs. 5%; p = .004). In addition, those children intubated at community hospitals were intubated sooner after presentation (2.4 +/- 5.2 vs. 7.5 +/- 5.8 hrs; p = .009), had shorter durations of intubation (71 +/- 73 vs. 151 +/- 81 hrs; p = .02), and had shorter PICU length of stays (129 +/- 82 vs. 230 +/- 84 hrs; p = .01). CONCLUSIONS: Children with status asthmaticus are more likely to be intubated, and intubated sooner, at a community hospital. The shorter duration of intubation suggests that some children may not have been intubated had they presented to a children's hospital or received more aggressive therapy at their community hospital.

Nasal polyposis: prevalence of positive food and inhalant skin tests.

OBJECTIVES: To investigate the etiology of nasal polyps and its relationship to allergy. The prevalence of positive food and inhalant skin tests in patients with nasal polyps and nonatopic controls was compared. STUDY DESIGN AND SETTING: Prospective controlled study in tertiary referral rhinology clinic. RESULTS: Seventy percent (70%) of the patients with nasal polyps had positive skin tests to an average of four foodstuffs, compared to 34 percent of controls (P = 0.006). Only 35 percent of the nasal polyp patients also had positive inhalant skin tests. Overall, the prevalence of positive inhalant skin tests was similar in the nasal polyp patients and controls. CONCLUSIONS: These findings suggest that the positive skin tests to foods are not merely a reflection of the general atopic status of patients with nasal polyps. It may be that non-IgE-mediated hypersensitivities, such as to ingested foods, play a role on the basis of a significant number of patients with positive intradermal skin tests to foods. SIGNIFICANCE: Evaluation of the allergic status of patients with polyposis is important. Dietary manipulation may be indicated, though its role needs further investigation.

Effect of using multiple culture media for the diagnosis of noninvasive fungal sinusitis.

BACKGROUND: This study was designed to assess the relative efficiency of three different culture media for isolating fungi in patients suspected of having noninvasive fungal sinusitis. METHODS: A prospective study was performed of 209 operative samples of sinus "fungal-like" mucin from 134 patients on 171 occasions and processed for microscopy and fungal culture in Sabouraud's dextrose agar, potato dextrose agar, and broth media. RESULTS: Ninety-three (69%) of 134 patients had evidence of fungal infection. Two-thirds of patients had negative microscopy samples yet 56% of these went on to positive cultures. Forty-five percent cultured Aspergillus genus. Discrepancy between the fungi cultured in different media and on different occasions was common. With a single culture medium up to 19% of patients and 15% of samples would have been falsely labeled fungal negative. CONCLUSION: Increasing the number and type of fungal culture media used increases the number and range of fungal isolates from mucin in patients with the features of fungal sinusitis. Negative specimen microscopy is unreliable. All specimens should be cultured in multiple media and on multiple occasions when fungal sinusitis is suspected.