Sickle Cell-Related Neurocognitive Symptoms May Predict Daily Living Skills

A new study among patients with sickle cell disease found that patient-reported outcomes of neurocognitive symptoms predicted poor skills related to essential daily activities. These skills included employment, post-secondary education, independence with daily tasks, ability to engage in leisure activities, and medication adherence.

With many neurocognitive effects of sickle cell disease persisting into adulthood, patients may suffer from reduced intellectual functioning and cognitive deficits. However, little is known about this relationship between self-perceived cognitive difficulties and challenges performing instrumental activities of daily living.

Jennifer Longoria, PhD, of St. Jude Children’s Research Hospital, and colleagues thus evaluated patients from the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) to elucidate this relationship more fully.

The Study

Upon enrollment in the registry, patients (n = 2436) had completed patient-reported outcomes (PRO) measures of attention, executive functioning, processing speed, learning, and comprehension. Several of the surveys that were utilized were developed by the National Institute of Health (NIH), which included the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) and Quality of Life in Neurological Disorders (Neuro-QoL).

The Neruo-QoL Item Bank v2.0, Cognitive Function Short-Form assessed cognitive symptoms and difficulties performing tasks, such as reading, following complex instructions, planning and keeping appointments, managing time, and so on.

The ASCQ-Me v2.0 Social Functioning Short-Form evaluated the impact of health on the patient’s independence and engagement in social activities

In addition to the cognitive and health assessments, information on employment and educational status and daily behaviors were collected by Longoria’s team. Medication adherence was assessed as a separate outcome measure.

The Results

Overall, 65.1% of participants reported some degree of difficulty (“sometimes” or “often” responses) with reading comprehension, and 56.5% indicated challenges with mental processing, As many as 55.2% of participants reported having difficulty with attention in the past week.

Current problems included time management (50.7%), planning (43.3%), learning (32.1%), and following complex instructions (25.7%). Furthermore, within the past month, many participants experienced some degree of interruption to social activities (68.9%) and relied on others for care (53.7%) because of their health problems.

After controlling for age (P < .0001), pain (P < .0001), and hydroxyurea use (P = .0001), Longoria and colleagues found that executive dysfunction (P < .0001), and "sometimes" or "often" experiencing learning difficulties (P < .0001 and P = .0397, respectively) and poor comprehension (P = .0002 and P = .0005, respectively) were associated with poor instrumental activities of daily living skills. 

When the investigators removed participants who were both disabled and still engaged in work from their analysis (n = 10), they saw that history of stroke became a significant predictor (P = .0441) in their univariate modeling. Nevertheless, this association did not remain in their final multivariable model.

Although executive dysfunction (P < .001) and attention difficulties (P = .0225 for “sometimes,” and P < .0001 for “often.”) predicted instrumental activity of daily living skills among this subpopulation, self-reported challenges with learning and comprehension were no longer significant.

Excluding patients who were disabled and not working (n = 556) meant that history of hydroxyurea, education, executive functioning, learning, and comprehension were no longer significant in their univariate model and final model.

However, difficulties with attention control (P = .0089 for “sometimes” and P ≤ .0001 for “often”) was considered a significant predictor of essential daily skills.

Among the total population, executive functioning difficulties (P = .0212) predicted hydroxyurea non-adherence—when controlling for age (P = .0129 for ages 25-34), genotype (P = .0006), and hemoglobin (P =.0039).

Conclusion

“In the largest PRO study of individuals with SCD in the United States, more than half of adolescent and adult participants reported contending with cognitive difficulties on a regular basis, including inattention, poor executive functioning, reduced processing speed, and difficulty with comprehension,” Longoria and colleagues wrote.

They suggested that cost- and time-sensitive targeted interventions aimed at improving executive functioning skills and medication adherence should be prioritized among these patients, a population particularly vulnerable to poor daily living skills.

“Further, programming should be developed to support employment and educational pursuits among individuals with risk factors for disengagement,” the investigators wrote.

The study, “Patient-reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease,” was published online in American Journal of Hematology.