Pain and fatigue did not appear to be linked with neurocognitive function in a recent study of young patients with sickle cell disease (SCD). The study’s findings were reported in the journal Pediatric Blood & Cancer.

Because patients with SCD frequently experience chronic pain and fatigue, in addition to possible neurocognitive impacts, the study investigators aimed to explore whether they could identify a relationship between these symptoms using performance-based measures of neurocognitive function.

Participants in the Sickle Cell Clinical Research Intervention Program (SCCRIP) were included in this study. As part of the SCCRIP, patients receive neurocognitive assessments occurring every 4 or 6 years based on the patient’s age. Participants included in this analysis were either in late adolescence, ages 16 through 18 years, or early adulthood, 20 through 25 years.

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The Wechsler Abbreviated Scale of Intelligence–Second Edition was used to identify intelligence quotient (IQ) scores, and other neurocognitive assessments were also performed. The Pediatric Quality of Life Inventory (PedsQL) was utilized to evaluate fatigue and pain, specifically using the PedsQL Multidimensional Fatigue Scale and the PedsQL SCD Module: Pain and Hurt Scale, respectively.

There were 106 patients included in this analysis who had a mean age of 19 years (SD, 3.3). Slightly over half (53.8%) of the patients had an SCD genotype of HbSS or Hbb0-thalassemia, while 46.2% had a genotype of HbSC, HbSb+-thalassemia, or Hb other.

The mean full-scale IQ in this study population was 86 (SD, 13). Patients also had a mean PedsQL Fatigue score of 64 (SD, 18.1), on a scale of 0 to 100, with higher numbers reflecting less fatigue. They also had a mean PedsQL Pain and Hurt score of 62 (SD, 22.2), on a scale of 0 to 100, with higher scores reflecting less pain.

Across various statistical analyses, including analyses controlled for genotype and age, there appeared to be no significant association between self-reported fatigue and IQ, working memory, or processing speed in this patient population. Across analyses, there also appeared to be no significant association between self-reported pain and IQ, working memory, or processing speed.

“From a clinical perspective, our data suggest that clinicians may not need to weigh chronic pain and fatigue as significant contributory factors when interpreting neurocognitive test data,” the study investigators wrote in their report.

Disclosures: Some study authors declared affiliations with biotech, pharmaceutical, or device companies. Please see the original reference for a full list of disclosures.


Semko JH, Longoria J, Porter J, et al. Examining the influence of pain and fatigue on neurocognitive functioning in adolescents and young adults with sickle cell disease. Pediatr Blood Cancer. Published online August 10, 2023. doi:10.1002/pbc.30621