Patient-reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease

Details

• Alternative Title:
  Am J Hematol
• Personal Author:
  Longoria, Jennifer N. ; Pugh, Norma L. ; Gordeuk, Victor ; Hsu, Lewis L. ; Treadwell, Marsha ; King, Allison A. ; Gibson, Robert ; Kayle, Mariam ; Crego, Nancy ; Glassberg, Jeffrey ; Melvin, Cathy L. ; Hankins, Jane S. ; Porter, Jerlym
• Description:
  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.
• Subjects:
  Activities Of Daily Living Adolescent Adult Anemia, Sickle Cell Antisickling Agents Cognitive Dysfunction Female Humans Hydroxyurea Male Medication Adherence Middle Aged Young Adult

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• Keywords:
  Alzheimer's Disease Alzheimer's Disease Sequencing Project Antiviral Agents Herpes Simplex Human Papillomavirus Instrumental Activities Of Daily Living Medication Adherence Neurocognitive Patient-reported Sickle Cell Disease Torque Teno Viruses Whole Exome Sequencing Whole Genome Sequencing

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• Source:
  Am J Hematol. 96(11):1396-1406
• Pubmed ID:
  34350622
• Pubmed Central ID:
  PMC8855994
• Document Type:
  Journal Article
• Funding:
  U24 HL133948/HL/NHLBI NIH HHSUnited States/ ; U01 HL134042/HL/NHLBI NIH HHSUnited States/ ; U01 HL133994/HL/NHLBI NIH HHSUnited States/ ; U01 HL133964/HL/NHLBI NIH HHSUnited States/ ; U01 HL134007/HL/NHLBI NIH HHSUnited States/ ; U01 HL134004/HL/NHLBI NIH HHSUnited States/ ; U01 HL133990/HL/NHLBI NIH HHSUnited States/ ; U01 HL133997/HL/NHLBI NIH HHSUnited States/ ; U18 DD000015/DD/NCBDD CDC HHSUnited States/ ; U01 HL133996/HL/NHLBI NIH HHSUnited States/ ; K01 HL125495/HL/NHLBI NIH HHSUnited States/
• Volume:
  96
• Issue:
  11
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha-512:9ce02b961616e515187840a44e2b369323655d5c57078a5f65c5ccc13f85ea03cb67aac3e9343cd91afd6290a7311a435eb8bd66e8a5b8c0cde4c3743c917acb
• Download URL:
  https://stacks.cdc.gov/view/cdc/114600/cdc_114600_DS1.pdf
• File Type:
  [PDF - 456.14 KB ]