Pediatric hematologists report infrequent prognosis discussions in the routine care of children with sickle cell disease

Lydia H. Pecker, Ellen Johnson Silver, Michael Roth, Deepa Manwani

Research output: Contribution to journalArticle


A large proportion of the morbidity and almost all of the mortality of sickle cell disease (SCD) now occurs in adulthood. Children with SCD underuse disease-modifying and curative treatments, in part because of how patients/parents understand SCD morbidity and mortality. Whether practitioners provide prognostic information to families is unknown. We emailed a 31-item survey to 1,149 pediatric hematologist-oncologists and analyzed 96 responses. Most said discussing prognosis would change patient/parent willingness to start hydroxyurea, but fewer actually discuss prognosis when they want to start hydroxyurea (91% vs. 75%, p=.001). Similarly, most said discussing prognosis would change adherence to therapy, but fewer actually discuss prognosis to motivate adherence (78% vs 31%, p<.001). Most (77%) addressed prognosis when the “patient or their parent bring it up.” Respondents reporting frequent life expectancy discussions were more likely to report a pathway for such discussions (p=.017). Pediatric hematologists may not conduct prognostic discussions without prompting, although these conversations may be important for shared, informed decision-making.

Original languageEnglish (US)
Pages (from-to)398-423
Number of pages26
JournalJournal of Health Care for the Poor and Underserved
Issue number1
StatePublished - Feb 2020



  • Life expectancy
  • Prognosis
  • Sickle cell anemia
  • Sickle cell disease

ASJC Scopus subject areas

  • Public Health, Environmental and Occupational Health

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