Case scenarios and decisional factors
Baseline, hypothetical case scenarios were created, describing a female patient with cystic fibrosis (CF) and end-stage lung disease, facing the decision of whether or not to pursue long transplantation. The patient had a Forced Expiratory Volume in the first second (FEV1) of 20 percent predicted, a rapid decline in lung function, and numerous hospitalizations in the preceding year. She had respiratory insufficiency necessitating nocturnal bi-level positive airway pressure (BiPAP) and daytime supplemental oxygen. The patient was developmentally age appropriate. Her disease progression necessitated a decision regarding whether or not to proceed with lung transplantation. The physician in the scenario had been the patient’s primary pulmonologist since infancy, and shared a close relationship with the patient and family.
Four case scenarios were generated by dichotomous systematic manipulation of two factors: patient age and maturity level. These decision factors were chosen given evidence that they are important in pediatric decision-making competency.9-11 They were presented at two levels. The patient’s age was either a pre-adolescent of 11 years old, or an adolescent of 16 years old. Maturity level was represented by whether or not the child performed the majority of her self-cares and asked thoughtful questions regarding her disease. These surrogate measures for maturity level were chosen based on CF R.I.S.E (Responsibility.Independence.Self-care.Education), a toolset widely utilized in assessing developing care independence in patients with CF.15,16 All content other than age and maturity was identical across the four scenarios. The four clinical scenarios were presented in randomized order. An example case scenario is as follows:
Anna is an 11-year-old female with end-stage cystic fibrosis, FEV1 of 20% predicted. She has had numerous hospital admissions over the last year and a rapid decline in her lung function. She is on nocturnal BiPAP and daytime supplemental oxygen. Given her disease progression, it is time to decide whether to proceed with lung transplantation.Anna is responsible for the majority of her self cares and she asks thoughtful questions of her parents and providers regarding her disease. She is developmentally age appropriate. You have been Anna’s primary pulmonologist since infancy. You have a close relationship with her and her family. Anna and her parents have already met with the transplant team, and she is likely a candidate. They are deciding whether to proceed with listing for lung transplantation.