Road map to developing a fellowship program in a tertiary care hospital.

BACKGROUND: The American Academy of Pediatrics has identified the need for more subspecialists. Beginning a pediatric pulmonary fellowship program in a tertiary care hospital can be a challenging process. Persistence, perseverance, and working through barriers to education are important strides to take toward achieving this goal. We share our experience with this endeavor. The objective of this study was to describe our experience developing a pediatric pulmonary fellowship program, the challenges we faced, and the methods we used to meet and overcome those challenges. METHODS: Self-reflection by the program director, associate director, and coordinator involved in the development and implementation of a successful fellowship program. This includes the step by step process, effort, and time commitment needed for planning and implementing a fellowship program. Our goals are to share this information with the medical community. RESULTS/OUTCOMES/IMPROVEMENTS: Planning, preparing, and implementing a pediatric pulmonary fellowship program can be challenging in the current economic climate, especially considering budget constraints and increasingly demanding clinical mandates. Department chairs and administrators view work relative value units as a marker for remuneration; educational efforts are more often an unfunded mandate. Major difficulties included imposing new educational expectations on the clinical staff and expanding the budget to include fellows' costs. Developing a program information form (PIF) was a work-in-progress over a two-year period with the dedicated staff meeting regularly during this time.

Cough in asthma triggered by reflux episodes.

With combined pH and impedance monitoring, non-acid, as well as acid reflux episodes, are more commonly detected immediately prior to cough in asthma in children. Gastroesophageal reflux should be evaluated as a trigger for cough in difficult childhood asthma.

Cystic fibrosis infant care challenges in diagnosis and management in the era of newborn screening.

With newborn screening for cystic fibrosis (CF) now accessible in every state in the US, more infants are being diagnosed. Currently, no large-scale randomized studies are available to assist clinicians in providing evidence-based medical care for the youngest CF patients. We review the standard of diagnostic evaluation, the sweat test, showing a slightly altered range for infants less than 6 months of age. Cystic fibrosis transmembrane conductance regulator (CFTR) metabolic syndrome is an entity that can be clinically challenging for parents and caregivers. Simply indentifying infants with CF can be challenging. This review tries to clarify the CFTR gene and its expressions and the additional benefits that may be drawn from newborn screening. We searched literature to review guidelines for care of infants with CF, and we reviewed newborn screening methods and diagnosis. We focused on early nutritional intervention and lung protective strategies to improve growth and lung function outcomes.

Association between practice patterns and body mass index percentile in infants and young children with cystic fibrosis.

BACKGROUND: Few guidelines focus on the preventive and maintenance care of infants with cystic fibrosis (CF). We explored how practice variability at accredited CF centers affected nutritional outcomes. METHODS: A retrospective cohort study using Cystic Fibrosis Foundation registry data (1993-2004) from three CF centers compared the initial management with respiratory, antimicrobial, and nutritional agents in infants. Further, we examined the association between dornase alpha use prior to two years of age and BMI percentile over time accounting for several possible factors including gender, race, CF center, presentation, age at diagnosis, sweat value, F508del status, first Pseudomonas aeruginosa infection age, second-year weight percentile, supplemental feedings use, and pancreatic enzymes use. RESULTS: Patient characteristics and prescribed therapies were similar at all sites for 165 patients who met inclusion criteria. However, one CF center prescribed dornase alpha significantly more frequently, 82% vs. 10% (p<0.001), and supplemental feeds significantly less frequently, 56% vs. 78% (p=0.04). Dornase alpha prescription prior to age two was associated with a 10-percentile increase in BMI through age six compared to infants not prescribed dornase alpha. CONCLUSIONS: Treating infants less than two years old with dornase alpha may improve nutritional outcome through age six.

Parapneumonic effusion and empyema in children: retrospective review of the duPont experience.

Management of pediatric parapneumonic effusions and empyema remains controversial. Treatment includes antibiotics, chest tube, fibrinolytic therapy, video-assisted thoracoscopy and debridement, and open thoracotomy and decortication. A retrospective 10-year study was done to identify patient selection variables for specific therapies. Charts (n = 101) with diagnoses of empyema without comorbidity were reviewed, a database was developed, and variables between patients who did and did not receive thoracoscopic debridement were compared at admission and during hospitalization. The difference in positive culture reports with video-assisted thoracoscopy compared with medical management was significant (P < .018). Postsurgical patients used the intensive care unit and had 2 or more chest tubes with greater frequency than medically managed patients (P < .014, P < .002). Antibiotics, video-assisted thoracoscopy, and chest tube within 48 hours of admission shortened hospitalization by 4 days (P < .001) compared with delayed video-assisted thoracoscopy done after 48 hours of admission.

Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function.

OBJECTIVE: Previous analyses of the Epidemiologic Study of Cystic Fibrosis database revealed that sites with the highest average patient lung function monitor patients and treat with antibiotics more aggressively than those where average lung function is lowest. The aim of this study was to assess whether patterns of care for infants at cystic fibrosis sites with superior average lung function in 6- to 12-year-old children showed any differences from those at the lowest outcome sites. METHODS: We divided cystic fibrosis sites with > or = 20 patients who were 6 to 12 years of age into quartiles on the basis of median forced expiratory volume in 1 second of that age group in 2003 and compared demographic and clinical characteristics and treatment patterns during the first year of enrollment for patients who were aged 0 to 3 years at those sites in 1994 to 1999. The analysis included 755 infants from 12 upper quartile sites and 743 infants from 12 lower quartile sites. RESULTS: Upper quartile sites had more infants whose disease was diagnosed by family history or newborn screening, fewer infants with symptoms at diagnosis, higher weight for age at enrollment, more white patients, and more deltaF508 homozygotes. Medical conditions and respiratory tract microbiology differed between sites. Infants at upper quartile sites had more office and sick visits; more respiratory tract cultures; and more frequent use of intravenous antibiotics, oral corticosteroids, mast cell stabilizers, and mucolytics; but they received less chest physiotherapy, inhaled bronchodilators, oral nutritional supplements, and pancreatic enzymes. CONCLUSIONS: Both enrollment characteristics and infant care patterns are associated with lung function outcomes in later childhood. Our analysis suggests that pulmonary function of older children may be improved through specific interventions during the first 3 years of life.

The use of bilevel positive airway pressure for the treatment of acute chest syndrome of sickle cell disease.

OBJECTIVE: To assess the outcome of bilevel positive airway pressure (BPAP) use for pediatric patients with sickle cell disease and acute chest syndrome. DESIGN: Retrospective chart review of 25 occurrences of acute chest syndrome in nine children from 1994 to 2000. SETTING: A tertiary care children's hospital. PATIENTS: Seven boys and two girls (average age, 11.8 years; range, four to 20 years). Prior to admission, 80% had chest pain, 48% had back pain, 48% had extremity pain, 24% had fever, and 20% had cough. INTERVENTIONS: Bilevel positive airway pressure therapy. MEASUREMENTS AND MAIN RESULTS: Patients' clinical values before and after BPAP were as follows: oxygen support (L), 4.1 +/- 3.2 and 1.4 +/- 1.7 (p < 0.001); oxygen saturation (%), 96.3 +/- 2.8 and 97.9 +/- 1.6 (p < 0.05); respiratory rate (per minute), 28.5 +/- 8.6 and 25.1 +/- 6.6 (p < 0.05); heart rate (per minute), 109 +/- 18 and 92 +/- 13 (p < 0.001). Patients' average highest intermittent positive airway pressure was 12 cm H2O, and the average highest expiratory positive airway pressure was 6 cm H2O. Patients spent an average of 3.1 days receiving BPAP. Of the patients, 4% suffered skin irritation over their nasal bridge, and 56% were admitted to the intensive care unit. The BPAP therapy was ineffective for only one patient. Most patients (96%) received BPAP in response to respiratory distress; 4% received it in response to increasing oxygen requirements after administration of narcotics and inability to perform incentive spirometry. CONCLUSION: Data suggest that BPAP therapy can be used to improve oxygenation and decrease work of breathing among patients with acute chest syndrome. We believe that BPAP may prevent progression to acute hypoxic respiratory failure requiring intubation and ventilation. It may reduce costs, especially if intensive care unit admission can be avoided by beginning therapy early. This therapy may become a standard of care for children with acute chest syndrome. The study design (a retrospective chart review) was subject to limitations and bias. A multicenter, prospective, randomized trial is recommended.

Respiratory management of pediatric patients with spinal cord injuries: retrospective review of the duPont experience.

Pulmonary complications contribute to morbidity and mortality in spinal cord injuries (SCIs). A retrospective review of 20 years of experience with tracheostomy- and ventilator-dependent SCI children is presented. The authors developed and analyzed a database of 47 children (average age = 11.4 years). Of the patients, 27% had concomitant brain injuries, 6% had prior histories of reactive airway disease, and 2% had thoracic fractures. Injuries were caused by motor vehicle accidents (53%); gunshot wounds (19%); sports-related accidents (19%); and vascular injuries, transverse myelitis, or spinal tumors (8%). Of the injuries, 52% were high level (C1 to C2) and 48% were mid- or low level (C3 to C5). Two groups were analyzed for demographic information. Complications included tracheitis, atelectasis, and pneumonia. Mean tidal volume was 14 cm2/kg (maximum = 22 cm2/kg). Bedside lung function parameters were attempted to assess readiness and the rapidity of weans. T-piece sprints were used to successfully wean 63% of patients. Successfully weaned patients were compared with those not weaned. No deaths or readmissions for late-onset respiratory failure postwean occurred. The authors' clinical impression favors higher tidal volumes and aggressive bronchial hygiene to minimize pulmonary complications and enhance weaning. Successfully weaned patients had fewer complications. A critical pathway for respiratory management of SCI children is presented.

The pediatric use of bilevel positive airway pressure therapy for obstructive sleep apnea syndrome: a retrospective review with analysis of respiratory parameters.

The purpose of this study was objective documentation of clinical benefits of bilevel positive airway pressure in pediatric patients with obstructive sleep apnea. We performed a retrospective chart review and data collection/analysis in a suburban tertiary care children's hospital. The study consisted of 10 pediatric patients (age range: 3 to 18 years); 3 patients had craniofacial abnormalities, 1 patient had neuromuscular disease, and 6 patients were obese. Eleven-channel polysomnography and support with bilevel positive airway pressure were used. We measured obstructive sleep apnea indices, lowest oxygen saturation rate, and average breath lengths before and after bilevel positive airway pressure use. We found that the apnea index decreased from 19.7 +/- 26.46 to 0.82 +/- 1.01, the lowest oxygen saturation increased from 75.60% +/- 14.93% to 89.50% +/- 5.50%, and breath length increased from 3.22 +/- 0.95 to 3.68 +/- 0.82. Bilevel positive airway pressure may be considered as a treatment modality for pediatric patients with obstructive sleep apnea.