Neurocognitive testing and functioning in adults sickle cell disease.

The impact of cerebrovascular events (CVE) on cognitive functioning in adults with sickle cell disease is not well understood. Sequelae of CVEs can adversely impact their quality of life. We reviewed neurocognitive presentation and testing in adults with sickle cell disease. We evaluated the frequency of complaints of memory disturbance using survey methodology in a sample of 109 adult patients with sickle cell disease (48 males, 61 females). A subsample of 24 patients also received a memory questionnaire where specific cognitive functions were assessed. Overall, we found that males and females did not differ in the frequency of experienced memory disturbance during painful crises. However, the frequency of men reporting that their ability to remember where they place common objects such as keys (p = 0.017) and remembering the item they intended to buy in a grocery store or pharmacy (p = 0.048) was worse now compared to when they were in high school and was greater when compared to women. The frequency of men who reported their memory was worse now than when they were in high school (p = 0.051) was also greater than in women. We concluded that memory dysfunction predicts global monthly presentation of pain. We suggest incorporating cost-effective neurocognitive screening measures as a standard of practice in sickle cell disease.

Opioid management and dependency among adult patients with sickle cell disease.

While pain is one of the most debilitating symptoms of sickle cell disease, narcotics remain an effective although controversial widely practiced intervention. Vaso-occlusive crises are the most common cause for seeking pharmacological treatment. The influence of stigmatization and pseudo addiction in emergency departments and outpatient clinics was reviewed. We analyzed patterns of narcotic utilization in a sample of 63 adult patients with sickle cell disease to determine if their psychological functioning and reports of pain differed as a function of the primary narcotics they were taking for oral pain management. Fifty-one percent of patients reported treatment of Oxycodone, 35% OxyContin, 24% methadone and 11% morphine. Patients who were treated with Oxycodone reported greater sensory reactions to pain (p = 0.001), visual analog scale (VAS) (p = 0.02), and averaged weekly pain intensity ratings than patients who did not use this medication. There were no differences in pain or affective response in patients treated with OxyContin, methadone or morphine. We suggest there are clear differences between the reports of pain in patients with sickle cell disease taking short-acting narcotics for pain management as compared to those who are not, a pattern that does not distinguish patients who are managed with long-acting preparations. We discuss the relevance of addressing narcotic management in the context of the perception of health care providers and patients with sickle cell disease.

Somatization in the conceptualization of sickle cell disease.

The unpredictable nature of sickle cell disease (SCD) and its social and environmental consequences can produce an unhealthy and almost exclusive focus on physical functioning. At the upper range of this focus on health concerns is somatization. In the current study, using 156 adult patients (55.13% female, 86) with SCD, mean age 35.59 +/- 12.73, we explored the relationship of somatization to pain. We found somatization to be predictive of pain severity and current pain intensity as well as a range of averaged indices of pain over time (p < .0001). We further found somatization to be predictive of a range of negative psychological experiences to include depression, anxiety, and hostility (p < .0001). We interpret these data to suggest that patients with SCD who have a propensity to focus exclusively on their health or are more sensitive to minor changes in their health status (somatization) may also be more likely to report greater concerns about their health and higher ratings of pain.

Psychosocial treatments in pain management of sickle cell disease.

The principal symptom of sickle cell disease (SCD) is pain. Many studies have been conducted on pain management strategies for this illness. There is recognition that psychosocial factors influence clinical disease outcomes; therefore, more attention is being provided to behavioral interventions that address psychosocial problems. This review examines the psychosocial interventions that have been researched for children and adults with SCD, the limitations of these studies, and barriers to implementing the treatments. The intervention receiving the most empirical support was cognitive-behavioral therapy. Additional research is needed to define the efficacy and effectiveness of the other psychosocial treatments. Suggestions for future investigations include conducting research that has better methodology, and providing more education for health care providers about psychosocial treatments and the importance of considering cultural factors in health care delivery. In addition, individuals with SCD need to have more information about their illness and better access to psychosocial interventions.

Depression, suicidal ideation, and attempts in black patients with sickle cell disease.

There is a strong relationship between suicidal ideation, suicide attempts, and depression. Rates of successful suicides are relatively high among the chronically ill compared to other populations but are reduced with treatment. Depression and suicide rates also often differ among blacks as compared to other populations. Using survey methods, we evaluated self-reported rates of depression, suicidal ideation, and suicide attempts in 30 male and 37 female black patients with sickle cell disease (SCD). SCD is a condition characterized by chronic, unpredictable pains and psychosocial distress. Thirty-six percent of the sample self-reported depression in the past 30 days, while 22 percent of the sample exhibited scores on the Beck Depression Inventory indicative of mild or greater depression (mean BDI, 8.31 +/- 7.79). Twenty-nine percent of patients indicated an episode of suicidal ideation and 8%, a suicidal attempt in their lifetime. Thirty-three percent reported treatment by a mental health professional. We conclude that there is a continuing need for mental health services in the management of depressed affect and risk for suicide among patients with SCD. Standards of clinical care must remain flexible to accommodate the mental health needs of this population of patients.

Neuropsychological effects and attitudes in patients following electroconvulsive therapy.

The current study examined the effects of electroconvulsive therapy (ECT) on neuropsychological test performance. Forty-six patients completed brief neuropsychological and psychological testing before and after receiving ECT for the treatment of recalcitrant and severe depression. Neuropsychological testing consisted of the Levin Selective Reminding Test (Levin) and Wechsler Memory Scale-Revised Edition (WMS-R). Self-report measures included the Beck Depression Inventory (BDI), the Short-Term Memory Questionnaire (STMQ), and several other measures of emotional functioning and patient attitudes toward ECT. The mean number of days between pre-ECT and post-ECT testing was 24. T-test revealed a significant decrease in subjective ratings of depression as rated by the BDI, t(45) = 9.82, P < 0.0001 (Pre-BDI = 27.9 +/- 20.2; post-BDI = 13.5 +/- 9.7). Objective ratings of memory appeared impaired following treatment, and patients' self-report measures of memory confirmed this decline. More specifically, repeated measures MANOVA [Wilks Lambda F(11,30) = 4.3, p < 0.001] indicated significant decreases for measures of immediate recognition memory (p < 0.005), long-term storage (p < 0.05), delayed prose passage recall (p < 0.0001), percent retained of prose passages (p < 0.0001), and percent retained of visual designs (p < 0.0001). In addition, the number of double mentions on the Levin increased (p < 0.02). This study suggests that there may be a greater need to discuss the intermittent cognitive risks associated with ECT when obtaining informed consent prior to treatment. Further that self-reports of cognitive difficulties may persist even when depression has remitted. However, patients may not acknowledge or be aware of changes in their memory functioning, and post-ECT self-reports may not be reliable.

Fear of movement (kinesiophobia), pain, and psychopathology in patients with sickle cell disease.

OBJECTIVES: Fear of movement (ie, kinesiophobia) has emerged as a significant predictor of pain-related outcomes including disability and psychologic distress across various types of pain (eg, back pain, headache, fibromyalgia, complex regional pain syndrome). However, no research has examined the prevalence of kinesiophobia in adults with sickle cell disease (SCD). The purpose of this study was to assess the degree of kinesiophobia reported by African American men and women with SCD and to determine whether kinesiophobia is related to pain and psychologic distress in this population. METHODS: Sixty-seven men and women with SCD recruited from a comprehensive sickle cell treatment program in a large academic medical center completed questionnaires that assess fear of movement, pain and pain interference, and psychologic distress. RESULTS: Participants reported levels of kinesiophobia (M=30.48, SD=7.55) that were comparable to those obtained for patients with low back pain and fibromyalgia. Although pain levels did not differ by sex, men reported greater kinesiophobia than women (P=0.02). As hypothesized, higher levels of kinesiophobia were associated with greater psychologic distress, particularly Phobic Anxiety (r=0.35), Psychoticism (r=0.29), Somatization (r=0.45), Anxiety (r=0.35), Obsessive-compulsive (r=0.34), Interpersonal Sensitivity (r=0.25), Depression (r=0.29), and all 3 summary indices of the SCL-90-R (all Ps<0.05). DISCUSSION: Although and historically, pain associated with SCD has not been considered in the context of fear of movement, findings suggest that both kinesiophobia and sex are relevant constructs for consideration in understanding pain-related outcomes in SCD. Though our results require replication, this study suggests that greater kinesiophobia is associated with greater pain and psychologic distress.

Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD).

Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD) have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE) may account for an alarming number of deficits in this population. In the current brief review, we critically evaluated the pediatric and adult literatures on the neurocognitive effects of SCD. We highlighted the studies that have been published on this topic and posit that early detection of CVE via neurocognitive testing, neuropsychiatric evaluations, and neuroimaging may significantly reduce adult cognitive and functional morbidities.