TY - JOUR
T1 - Patient-reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease
AU - Longoria, Jennifer N.
AU - Pugh, Norma L.
AU - Gordeuk, Victor
AU - Hsu, Lewis L.
AU - Treadwell, Marsha
AU - King, Allison A.
AU - Gibson, Robert
AU - Kayle, Mariam
AU - Crego, Nancy
AU - Glassberg, Jeffrey
AU - Melvin, Cathy L.
AU - Hankins, Jane S.
AU - Porter, Jerlym
N1 - Funding Information:
The Sickle Cell Disease Implementation Consortium has been supported by US Federal Government cooperative agreements U24HL133948, U01HL133964, U01HL133990, U01HL133996, U01HL133994, U01HL133997, U01HL134004, U01HL134007, and U01HL134042 from the National Heart Lung and Blood Institute and the National Institute on Minority Health and Health Disparities (Bethesda, MD). The content is solely the responsibility of the authors and does not represent the policy of the National Institutes of Health or the Department of Health and Human Services.
Funding Information:
JSH receives research funding from Global Blood Therapeutics, and consultancy fees from Global Blood Therapeutics, bluebird bio, UptoDate and Vindico Education. AAK receives research funding from Global Blood Therapeutics. JP received funding from the National Heart Lung and Blood Institute K01 HL125495 during the development of this work. MK received funding from NU58DD000015‐01‐00 during development of this work.
Funding Information:
The Sickle Cell Disease Implementation Consortium has been supported by US Federal Government cooperative agreements U24HL133948, U01HL133964, U01HL133990, U01HL133996, U01HL133994, U01HL133997, U01HL134004, U01HL134007, and U01HL134042 from the National Heart Lung and Blood Institute and the National Institute on Minority Health and Health Disparities (Bethesda, MD). The content is solely the responsibility of the authors and does not represent the policy of the National Institutes of Health or the Department of Health and Human Services.
Publisher Copyright:
© 2021 Wiley Periodicals LLC.
PY - 2021/11/1
Y1 - 2021/11/1
N2 - Individuals with sickle cell disease (SCD) experience neurocognitive decline, low medication adherence, increased unemployment, and difficulty with instrumental activities of daily living (IADL). The relationship between self-perceived cognitive difficulties and IADLs, including employment, school enrollment, independence, engagement in leisure activities, and medication adherence is unknown. We hypothesized that self-reported difficulties across neurocognitive areas would predict lower IADL skills. Adolescent and adult participants of the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) (n = 2436) completed patient-reported outcome (PRO) measures of attention, executive functioning, processing speed, learning, and comprehension. Cognitive symptoms were analyzed as predictors in multivariable modeling. Outcome variables included 1) an IADL composite that consisted of employment, participation in school, reliance on others, and leisure pursuits, and 2) hydroxyurea adherence. Participants reported cognitive difficulty across areas of attention (55%), executive functioning (51%), processing speed (57%), and reading comprehension (65%). Executive dysfunction (p < 0.001) and sometimes or often experiencing learning difficulties (p < 0.001 and p = 0.04) and poor comprehension (p = 0.000 and p = 0.001), controlled for age (p < 0.001), pain (p < 0.001), and hydroxyurea use (p = 0.001), were associated with poor IADL skills. Executive functioning difficulties (p = 0.021), controlled for age (p = 0.013 for ages 25–34), genotype (p = 0.001), and hemoglobin (p = 0.004), predicted hydroxyurea non-adherence. Analysis of PRO measures indicated that cognitive dysfunction is prevalent in adolescents and adults with SCD. Cognitive dysfunction translated into clinically meaningful outcomes. PRO of cognitive symptoms can be used as an important adjunct clinical tool to monitor symptoms that impact functional skills, including engagement in societal activities and medication adherence.
AB - Individuals with sickle cell disease (SCD) experience neurocognitive decline, low medication adherence, increased unemployment, and difficulty with instrumental activities of daily living (IADL). The relationship between self-perceived cognitive difficulties and IADLs, including employment, school enrollment, independence, engagement in leisure activities, and medication adherence is unknown. We hypothesized that self-reported difficulties across neurocognitive areas would predict lower IADL skills. Adolescent and adult participants of the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) (n = 2436) completed patient-reported outcome (PRO) measures of attention, executive functioning, processing speed, learning, and comprehension. Cognitive symptoms were analyzed as predictors in multivariable modeling. Outcome variables included 1) an IADL composite that consisted of employment, participation in school, reliance on others, and leisure pursuits, and 2) hydroxyurea adherence. Participants reported cognitive difficulty across areas of attention (55%), executive functioning (51%), processing speed (57%), and reading comprehension (65%). Executive dysfunction (p < 0.001) and sometimes or often experiencing learning difficulties (p < 0.001 and p = 0.04) and poor comprehension (p = 0.000 and p = 0.001), controlled for age (p < 0.001), pain (p < 0.001), and hydroxyurea use (p = 0.001), were associated with poor IADL skills. Executive functioning difficulties (p = 0.021), controlled for age (p = 0.013 for ages 25–34), genotype (p = 0.001), and hemoglobin (p = 0.004), predicted hydroxyurea non-adherence. Analysis of PRO measures indicated that cognitive dysfunction is prevalent in adolescents and adults with SCD. Cognitive dysfunction translated into clinically meaningful outcomes. PRO of cognitive symptoms can be used as an important adjunct clinical tool to monitor symptoms that impact functional skills, including engagement in societal activities and medication adherence.
UR - http://www.scopus.com/inward/record.url?scp=85113731943&partnerID=8YFLogxK
U2 - 10.1002/ajh.26315
DO - 10.1002/ajh.26315
M3 - Article
C2 - 34350622
AN - SCOPUS:85113731943
SN - 0361-8609
VL - 96
SP - 1396
EP - 1406
JO - American journal of hematology
JF - American journal of hematology
IS - 11
ER -