# Trajectory of Cognitive Functioning in Youth with Sickle Cell Disease without Cerebral Infarction

> **NIH NIH K23** · CHILDREN'S RESEARCH INSTITUTE · 2020 · $144,152

## Abstract

PROJECT SUMMARY/ABSTRACT
The overarching goal of this proposal is the development of the candidate into an independent, patient-
oriented clinical investigator capable of leading rigorous research aimed at preserving cognitive health in
youth with sickle cell disease (SCD). As a pediatric psychologist with experience in cognitive assessment
and clinical research with children with SCD, the candidate is uniquely positioned to benefit from this NIH
Mentored Career Development Award. SCD is an understudied, life-limiting disease affecting 100,000
individuals in the United States and over 300,000 newborns globally every year. Youth with SCD perform
lower on tests of cognitive ability, even compared to healthy siblings. Overt stroke, while a substantial
risk, does not affect the majority of patients with SCD yet there are clear indications of non-stroke,
disease effects on the central nervous system. These cognitive deficits impact school achievement,
employment, and disease management. Limitations of previous research have stifled translation of
knowledge about cognitive deficits into clinical applications to preserve cognitive health in the absence of
stroke. The proposed study will address unanswered questions by investigating the following specific
aims: 1) determine the trajectory of cognitive functioning in youth with SCD without overt stroke; and 2)
explore resting-state prefrontal-striatal functional connectivity in a subset of youth with SCD without overt
stroke. In this study, 114 youth ages 8-17 with HbSS without stroke will complete computerized cognitive
tests and blood draws every 6 months for 18 months, as well as transcranial Doppler ultrasonography at
baseline and 12 months later. A subset (n=40) between ages 11-17 will complete resting-state functional
magnetic resonance imaging at baseline. Results will advance the field toward prevention of all SCD-
related cognitive deficits in the absence of stroke by enhancing predictive models that identify patients at
risk for future cognitive declines and informing strategic application of cognitive and disease-modifying
treatments. The candidate has organized an exemplary mentorship team with notable records of NIH
funding and mentorship who are committed to advancing the candidate's career. Mentors have expertise
in longitudinal cognitive assessment (Dr. Kristina Hardy), SCD pathophysiology (Dr. Naomi Luban),
cognitive functioning in SCD (Dr. Jeffrey Schatz), functional neuroimaging (Dr. Chandan Vaidya), SCD
severity measurement models (Dr. Julie Panepinto), and research design and biostatistics (Dr. Jichuan
Wang). The candidate will benefit from an ideal academic environment and extensive resources in
Children's National Health System. A structured training plan will ensure timely progress toward career
development goals involving acquisition of knowledge about: 1) SCD pathophysiology and disease
severity measurement; 2) fMRI design and interpretation; and 3) advanced clinical research met...

## Key facts

- **NIH application ID:** 9869930
- **Project number:** 5K23HL141666-02
- **Recipient organization:** CHILDREN'S RESEARCH INSTITUTE
- **Principal Investigator:** Steven James Hardy
- **Activity code:** K23 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2020
- **Award amount:** $144,152
- **Award type:** 5
- **Project period:** 2019-03-01 → 2024-02-29

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/9869930

## Citation

> US National Institutes of Health, RePORTER application 9869930, Trajectory of Cognitive Functioning in Youth with Sickle Cell Disease without Cerebral Infarction (5K23HL141666-02). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/9869930. Licensed CC0.

---

*[NIH grants dataset](/datasets/nih-grants) · CC0 1.0*