Objective To create a functional status outcome measure for large outcome studies that is well defined, quantitative, sufficiently rapid, reliable, minimally dependent on subjective assessments, and applicable to hospitalized pediatric patients across a wide spectrum of ages and inpatient environments. Patients and Methods The Functional Status Scale (FSS) was developed by a multidisciplinary consensus process. Domains of functioning included mental status, sensory, communication, motor, feeding, and respiratory categorized from normal (1) to very severe dysfunction (5). The Adaptive Behavior Assessment System (ABAS) II established construct validity and calibration within domains. Seven institutions provided pediatric intensive care unit (PICU) patients within 24 hours of PICU discharge, high-risk non-PICU patients within 24 hours of admission, and technology-dependent children. Primary care nurses completed the ABAS II based on patient’s functioning when the FSS was completed. Patients from 10% of the study days were used to evaluate inter-rater reliability. Data were randomly split into estimation and validation sets. Statistical analyses included Pearson correlations, construct validity, linear regression analysis, receiver operating characteristic (ROC) curve analysis for discriminant validity, and the intraclass correlation for inter-rater reliability. Results A total of 836 children with a mean FSS of 10.3 (standard deviation 4.4) were studied. Eighteen percent had the minimum possible FSS = 6, 44% had FSS ≥ 10, 14% had a FSS ≥ 15, and 6% had FSS scores ≥ 20. Each FSS domain was associated with mean ABAS II (p<.0001). Cells in each domain were collapsed and reweighted, which improved correlations with ABAS II from −0.58 to −0.62 in the estimation sample, and −0.60 to −0.63 in the validation sample (p<0.001 for improvements). Discrimination was very good for moderate and severe dysfunction (ABAS II categories) and improved with FSS weighting (area under the ROC curve > 0.8). Intraclass correlations of original and weighted total FSS were 0.95 and 0.94 respectively. Conclusions The FSS met our objectives and is well suited for large outcome studies.

Survival among this cohort of adults with severe respiratory failure supported with ECMO was 50%. Advanced patient age, increased pre-ECMO ventilation duration, diagnosis category and complications while on ECMO were associated with mortality. Prospective studies are needed to evaluate the role of this complex support mode.

Plastic bronchitis is characterized by marked obstruction of the large airways by bronchial casts. We reviewed our experience and the literature to determine whether mortality rates are determined by underlying disease or cast type. We present 3 children with obstructive bronchial casts. One 3-year-old patient with Noonan's syndrome developed respiratory failure following surgery for tetralogy of Fallot requiring support with extracorporeal membrane oxygenation (ECMO) the first such case. There were 42 cases in the literature of children with plastic bronchitis. Casts may be divided into two types. Type I casts are inflammatory, consisting mainly of fibrin with cellular infiltrates, and occur in inflammatory diseases of the lung. Type II, or acellular casts, consist mainly of mucin with a few cells, and usually occur following surgery for congenital cardiac defects. Patients categorized by underlying disease included 31% with asthma or allergic disease, 40% with underlying cardiac defects, and 29% with other diseases. Mortality was 16%, but increased to 29% in patients with cardiac defects. Deaths occurred as long as 1 year after surgical repair for underlying defects. There were no deaths in patients with asthma. Life-threatening events were statistically higher in patients with cardiac defects (41%) than in those with asthma (0%, P = 0.02). Higher mortality in patients with type II casts compared to type I casts did not reach statistical significance (28% vs. 6%; P = 0.06). In conclusion, patients presenting with plastic bronchitis are at high risk for serious complications, especially with underlying cardiac disease.