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Cystic fibrosis is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Novel, highly effective, modulator therapies correcting and potentiating CFTR function are changing the course of the disease. We present an ethical dilemma involving an 11-year-old child with CF and end stage lung disease. Shortly after starting treatment with Elexacaftor-Tezacaftor-Ivacaftor, the family received notification that a matched donor lung had been allocated. Clinical decision-making in this case is challenging as definitive data to medically support one treatment option over the other is limited. A survey of CF center team members was conducted for the purpose of this manuscript. Ethical principles that may guide us in these situations are discussed. Overall, results of the survey present a lack of agreement as to the best approach in this situation. Physicians, when compared to other team members, are more likely to provide a specific recommendation vs. to present the information and let the family decide (odds ratio (95% confidence interval)=4.0 (1.2-12.8), p=0.021). A shared decision-making model, stressing our moral obligation as clinicians to respect autonomy by appreciating family values while offering to participate in the decision-making process and ensuring non-maleficence, is presented. In summary, CFTR modulators affect the outcomes of CF disease and influence clinical decision-making. Current lack of data on long-term outcomes, in young patients with CF receiving effective modulator therapy, should not preclude CF team participation in decision-making. Shared decision-making which is focused on respecting autonomy is our preferred approach in these situations.

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Novel, highly effective, modulator therapies correcting and potentiating CFTR function are changing the course of this disease. We present an ethical dilemma involving an 11-year-old child with CF and end-stage lung disease. Shortly after starting treatment with elexacaftor-tezacaftor-ivacaftor, the family received notification that a matched donor lung had been allocated. Clinical decision-making in this case is challenging as definitive data to medically support one treatment option over the other are limited. A survey of CF center team members was conducted for the purpose of this article. Ethical principles that may guide us in these situations are discussed. Overall, results of the survey present a lack of agreement as to the best approach in this situation. Physicians, when compared with other team members, are more likely to provide a specific recommendation vs presenting the information to the family and letting them decide (OR, 4.0; 95% CI, 1.2-12.8; P = .021). A shared decision-making model, stressing our moral obligation as physicians to respect autonomy by appreciating family values, while offering to participate in the decision-making process and ensuring nonmaleficence, is presented. In summary, CFTR modulators affect the outcomes of CF disease and influence clinical decision-making. The current lack of data on long-term outcomes, in young patients with CF receiving effective modulator therapy, should not preclude CF team participation in decision-making. Shared decision-making, which is focused on respecting autonomy, is our preferred approach in these situations.

Pediatric community-acquired complicated pneumonia (PCACP) is characterized by a prolonged clinical course, but this may be highly variable.A multicenter observational study was conducted to develop and validate a clinical prediction tool for prolonged hospitalizations in PCACP. The derivation and validation cohorts consisted of 144 and 169 patients with PCACP, respectively, hospitalized between the years 1997 and 2017 in three tertiary care hospitals. Logistic regression analyses were used to identify parameters associated with a prolonged hospitalization and to develop and validate a prediction model for constructing a useful clinical tool.Higher levels of lactate dehydrogenase (LDH) (P .026) and lower levels of glucose (P = .018) in pleural fluid were significantly associated with prolonged hospitalization. A predictive stepwise logistic regression model was developed and applied to the validation cohort. The area under the receiver operating characteristic curve (AUROC) constructed indicated that the model retained good predictive value (AUROC for the derivation vs validation data, [0.77 (95% CI, 0.66-0.87) vs 0.82 (95% CI, 0.72-0.91)], respectively). From these data, a clinical tool was derived; the combination of pleural LDH1,000 units/L and pleural glucose levels 1 mmol/L or pleural LDH levels2,000 units/L and pleural glucose levels 2 mmol/L or pleural LDH levels3,000 units/L and pleural glucose 3 mmol/L predict prolonged hospitalization with positive and negative predictive values of 78% (95% CI, 0.71-0.85) and 73% (95% CI, 0.59-0.85), respectively.In children, pleural fluid LDH and glucose levels are useful parameters for assessing the severity of PCACP. The model developed in this study accurately predicts patients who will have prolonged hospitalization.