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Perception and communication of life expectancy and prognosis in sickle cell disease: a multi-center study of adolescents with sickle cell disease, their parents and pediatric hematologists

key information

source: The American Society of Pediatric Hematology/Oncology

year: 2016

authors: Lydia H. Pecker, Nicholas Whipple, Julie Krystal, Vera Osafo, Barbara Speller-Brown, Lisa Thaniel, Banu Aygun, Jane S. Hankins, Ellen Silver, Michael Roth, Deepa Manwani

summary/abstract:

Background: Sickle cell disease (SCD) is a heterogenous condition causing substantial morbidity and early mortality, but life expectancy has improved in recent decades. Research shows that affected adolescents/young adults (AYAs) and their parents understand the effect of SCD on their future differently than physicians. How prognosis is addressed in clinical practice is unknown and important for shared, informed decision-making.

Objectives: To compare perceptions of SCD-related prognosis among AYAs, parents and pediatric hematologists.

Design/Method: We conducted surveys to English-speaking AYAs (ages 11 – 21) with SCD, their parents, and pediatric hematologists about prognosis in SCD. AYAs and parents were recruited from four centers during health-maintenance visits. Questions were grouped by theme and answers compared using Chi-square or Fisher’s exact tests.

Results: 101 adolescents, 98 parents and 115 pediatric hematologists completed surveys. Adolescents were 51.5% female, 85% Black, with a mean age of 16.44 +/- 2.6 years; 72.3% had HbSS, 63.5% were taking hydroxyurea. Parents were 77.4% female, 84.4% Black; 73.5% of their children had HbSS, 56.1% were taking hydroxyurea. Parents (67.3%) and AYAs (53.4%) rated discussions of life expectancy as “very important”, however 68% of parents and 57.3% of AYAs reported that their doctors did “not often” discuss how long patients with SCD live. Consistent with this, 37% of doctors reported discussing life expectancy with AYAs; 39% did with parents. Most doctors (77%) reported patients initiating prognostic discussions. AYAs (63%) and parents (50%) want doctors to discuss life expectancy. Most hematologists thought AYAs (98.2%) and parents (89.8%) were somewhat to very afraid of SCD. Parents reported more fear of SCD than adolescents (29.2% vs. 13% “very afraid”, 39.8% vs. 36% “a little afraid”, 30.2 vs. 51% “not afraid,” p<.01). AYAs (50%) and parents (71%) did not believe that SCD would change life expectancy.

Conclusion: Prognostic discussions that include life expectancy are infrequent among AYAs with SCD; AYAs and their parents report wanting this information. Pediatric hematologists perceive that patients and families have more fear of SCD than patients and parents report. This study provides novel information to design interventions to guide conversations regarding disease prognosis and possibly treatment decisions with patients with SCD and their families.

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