Difficult conversations: Discussing prognosis with children with cystic fibrosis

Abstract

Objectives * Describe how and when individuals with cystic fibrosis learn about prognosis with a life-limiting disease, specifically its progressive nature and the median life expectancy. * Describe the emotional impact of initial communication about prognosis with cystic fibrosis, a life-limiting genetic disease. * Acquire new language for initiating conversation about prognosis with individuals with a lifelimiting disease as well as a general age-based timeline for when to initiate prognosis conversations. Original Research Background. No published studies address how patients with cystic fibrosis (CF) receive and perceive education about disease prognosis. Related research suggests a need for more information and strategies for coping with this burdensome disease and its limited life expectancy. Research Objectives. To describe patient, parent, and provider experiences with communication about prognosis and the emotional impact of initial communication and to elicit recommendations for improving communication. Methods. Semi-structured interviews were conducted with 18-25-year-olds with CF, parents of the 18-25-yearolds, and CF care providers at two CF centers. Thematic analysis of responses was conducted by the research team. Results. Thirteen patients, eight parents, and seven CF care providers (five physicians, one nurse, and one social worker) participated. More than half of patients (54%) learned about prognosis independently, not from a parent or provider. Fear and sadness were common emotional responses. All groups recommended in-person, individualized communication, describing this discussion as a “milestone” in disease self-management. Most patients (77%) and parents (86%) felt a provider should initiate communication about prognosis. Providers described uncertainty about when to address prognosis and how to instill hope while sharing information. Limited time, lack of confidence in addressing this topic, waiting for patient cues, and protecting parents were the top barriers to communication. While providers expressed concern about affecting mood and adherence, most patients reported that learning about prognosis did not negatively affect their outlook (79%) and that effects on adherence were often positive. Conclusion. Patients with CF and their parents and providers acknowledge challenges communicating about prognosis. Patients desire earlier, individualized, in-person communication. Standardized provider prompts to initiate communication about prognosis would ensure all patients are appropriately aware of prognosis. Age-appropriate educational materials could be used to facilitate conversations between patients, families, and providers. Implications for Research, Policy, and Practice. Though CF-focused, the themes elicited from this research can be applied to communicating prognosis in other life-limiting childhood illnesses.