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Journal of Pediatric Psychology, Vol. 26, No. 5, 2001, pp. 309-319
© 2001 Society of Pediatric Psychology

Social Information Processing and Magnetic Resonance Imaging in Children With Sickle Cell Disease

LeAnn C. Boni, PhD1, Ronald T. Brown, PhD2, Patricia C. Davis, MD3, Lewis Hsu, MD, PhD3 and Katharine Hopkins, MD3

1 Emory University, 2 Medical University of South Carolina, 3 Emory University School of Medicine

All correspondence should be sent to Ronald T. Brown, Department of Pediatrics, Medical University of South Carolina, Hagood Avenue, P.O. Box 250822, Charleston, South Carolina 29425. E-mail: brownron{at}musc.edu .


    Abstract
 Top
 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
Objective: To examine social information processing, social skills, and adjustment difficulties in children with sickle cell disease (SCD) as rated by caregivers, teachers, and the children themselves. Children were classified in two groups: cerebral vascular accidents (CVA) (n = 21) or without central nervous system (CNS) pathology (n = 20) on magnetic resonance imaging (MRI). Both groups had HbSS SCD. We compared these two groups and a third group of 11 children who had a milder type of SCD (HbSC).

Methods: Participants referred for evaluation of learning and behavior problems were administered MRIs to ascertain the presence of pathology and a series of measures designed to assess nonverbal emotional decoding abilities and ratings of social emotional functioning.

Results: Children with CVA displayed more errors on tasks of facial and vocal emotional decoding than did comparison controls without CVA.

Conclusions: Acquired neurological impairments in children with SCD seemed to be associated with difficulties in the decoding of emotions of other children and adults. We recommend that future research integrate neuropsychological and psychosocial research programs for pediatric chronic illness groups.

Key words: pediatric; sickle cell disease; cerebral vascular accident; social information processing.


    Introduction
 Top
 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
Recent research has indicated that up to 20% of children with sickle cell disease (SCD) (HbSS type, the homozygous condition known as sickle cell anemia) sustain either overt or silent cerebral vascular accidents (CVA or stroke) (Armstrong et al., 1996Go; Bonner, Gustafson, Schumacher, & Thompson, 1999Go; Brown et al., 2000Go). Brown et al. found that children with overt CVA and silent infarcts differed from their peers without central nervous system (CNS) pathology on measures of cognitive functioning, including attention and executive functioning. Similarly, Rodgers, Clark, and Kessler (1984Go) provided evidence of altered metabolism in the frontal lobe area, measured by position emission tomography (PET). Taken together, the studies suggest deficits in cognitive functioning in patients with SCD, with both overt and silent CVA (Brown et al., 2000Go; DeBaun et al., 1998Go; Rodgers et al., 1984Go; Schatz et al., in pressGo).

SCD is a life-long chronic illness that poses major stressors on children and places them at significant risk for problems with psychosocial adjustment For example, the results of a meta-analysis indicated the prevalence of depressive symptoms in youths with SCD (Bennett, 1994Go). As rated by parents and teachers, children with SCD exhibited more internalizing and externalizing problems and were nominated less frequently for social-desirability roles than their typically developing peers (Noll et al., 1996Go; Short, Vannatta, Kalinyak, & Noll, 1999Go), although not all investigators have corroborated these findings (Lemanek, Horwitz, & Ohme-Frempong, 1994; Noll, Ris, Davies, Bukowski, & Koontz, 1992Go). However, there is minimal research directed at identification of factors that contribute to the psychosocial difficulties of children with SCD.

Another line of inquiry suggests that the difficulties in emotional and social adjustment characteristic of children with SCD may be related to neurocognitive dysfunction from acquired damage to structures of the brain associated with social and emotional processing skills. For example, as shown in both adult psychopathology (Adolphs, Demasio, Tranel, & Demasio, 1996Go) and clinical child literature (Tramontana & Hooper, 1997Go), individuals who have sustained acquired brain injuries frequently suffer from concomitant emotional and psychiatric disorders. Thus, the difficulties in emotional and social adjustment experienced by children with SCD may be associated with infarcts to structures of the brain related to these functions (Noll et al., 1996Go). In support of this notion, neurological deficits have been demonstrated to play a significant role in the social and emotional processing of children with various acquired brain injuries (Tramontana & Hooper, 1997Go), as well as for children with certain types of learning disabilities (Bryan & Bryan, 1990Go) and low incidence psychiatric disorders, such as autism and schizophrenia (Tramontana & Hooper, 1997Go).

Perception of emotion by means of facial recognition and prosody (i.e., the understanding of an individual's intended verbal communication by frequency, pitch, intensity of loudness, duration, rhythm, and timing of speech) as it relates to cerebral damage is receiving considerable research focus (Adolphs et al., 1996Go; Adolphs, Tranel, & Demasio, 1994Go; Adolphs, Tranel, Demasio, & Demasio, 1995Go). In a study with pediatric populations, Cohen, Branch, and Hynd (1994Go) provided compelling evidence that the right hemisphere was dominant for receptive prosody in children. Similarly, in an investigation of children who had sustained early unilateral brain damage, only participants with right hemisphere damage showed impairments on an affective comprehension task (Trauner, Ballantyne, Friedland, & Chase, 1996Go). Nonverbal learning disabilities also have been associated with right hemisphere dysfunction in children, based on neuropsychological profiles (Rourke & Del Dotto, 1994Go), neurological examinations (Denkla, 1983Go), and neuroimaging studies (Grace & Malloy, 1992Go; Manoach, Sandson, & Weintraub, 1995Go). Depressive symptoms in children with right hemisphere dysfunction were also revealed.

We designed our investigation to extend the psychosocial and neuropsychological literatures related to SCD by examining the association of social information-processing deficits with acquired cerebral injuries in children with SCD. Few researchers in SCD have examined psychosocial and neurological functioning simultaneously. Our study included a group of children with HbSS SCD with documented (MRI) cerebral infarcts and two comparison control groups, including a group with HbSS SCD with no evidence (MRI) of cerebral vascular deficits and a group with HbSC disease, a milder variant of SCD, with no documented neurological insults. Our hypotheses proposed that participants with CNS pathology would demonstrate less accuracy in social information processing (as evidenced on a task of nonverbal emotional decoding abilities) and greater deficits in social skills than their peers without CNS pathology, as rated by both caregivers and teachers.


    Method
 Top
 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
Participants
Participants were 52 children and adolescents with SCD receiving their treatment at a comprehensive National Institutes of Health sickle cell center. This center is located at a major university-affiliated teaching hospital that serves primarily individuals of lower socioeconomic status (SES). Thus, our sample was composed primarily of a fairly low SES group, and this is characteristic of other investigations of children and adolescents with sickle cell syndromes (Hurtig, Koepke, & Park, 1988Go; Ievers, Brown, Lambert, Hsu, & Eckman, 1998Go). Children were required to be at least 6 years of age and not older than 17 years. Approximately 500 children with sickle cell syndromes were followed at this center at the time that the data were collected (from 1996 to 1998). Participants were referred to us by pediatric hematologists to investigate their cognitive, academic, and emotional functioning (Brown, Armstrong, & Eckman, 1993Go; Brown et al., 2000Go; Brown, Doepke, & Kaslow, 1993Go; Devine, Brown, Lambert, Donegan, & Eckman, 1999Go; Ievers et al., 1998Go). Although the children were referred to the investigators because of possible learning problems and adjustment difficulties, only three of the children were receiving special education services at the time of evaluation; none of the participants was receiving psychotropic medication at the time of the evaluation. All caregivers of children and adolescents who were approached to participate in the study agreed to do so.

Informed consent was obtained from the participants' caregivers, and assent was obtained from children and adolescents capable of understanding the nature of the study. Demographic information was collected with questionnaires, and medical information was obtained by chart reviews and the center's computer database. Caregivers were paid $25 for participation in the study. MRI examinations of the brain were conducted for clinical purposes within 3 months of the assessment. All of the children were right-handed. The length of the assessment was approximately 2 hours. Forty-one of the children had HbSS SCD, and 11 had HbSC SCD. The 41 children were divided into two groups, 21 with CNS pathology and 20 without.

Measures
Illness Variables
Disease severity was quantified in a manner similar to other investigations of children with SCD (Brown et al., 2000Go; Brown et al., 1993Go; Devine et al., 1999Go). The severity was measured by several variables that included number of hospital contacts over the past 12 months, mean white blood cell count over the past three clinic visits, and transfusion history. Currently receiving transfusion therapy was coded as 3, a past history of transfusions was coded as 2, and no recorded history was coded as 1. Because a high hemoglobin level is associated with less disease severity for some symptoms of SCD (e.g., stroke) (Charache, Lubin, & Reed, 1989Go), reverse scoring was used for hemoglobin levels averaged over the past three clinic visits.

Intellectual Functioning
To ensure equivalence of the groups for intellectual functioning, all participants were administered an abbreviated form of the Wechsler Intelligence Scale for Children-Revised (Information, Vocabulary, Block Design, Object Assembly). These four subtests have been highly correlated with the Full Scale intelligence score (FSIQ) (Noll et al., 1996Go; Wechsler, 1991Go). Scaled scores were computed for each of the subtests and were subsequently converted into a composite deviation quotient using Sattler's (1992Go) formula.

Assessment of Nonverbal Emotional Decoding Abilities
The Diagnostic Analysis of Nonverbal Accuracy (DANVA) (Nowicki & Duke, 1994Go) was used to assess affective decoding abilities of all participants. This test is similar to the one developed by Ekman and Friesen (1976Go) that has been used in an impressive body of research with adults (Ekman, 1972Go). Two subtests measuring nonverbal receptive abilities for child facial and prosodic expressions of emotion (Child Faces and Child Voices), and three subtests measuring nonverbal receptive abilities for adult facial and adult prosodic expressions of emotion (African American Adult Faces, Adult Faces, and Adult Voices) were used. Each of the subtests has 24 pictorial or audio scenes of actors displaying four emotions (happy, sad, angry, fearful) at two levels of intensities. Most of the actors displaying emotions were Caucasian and some were African or Asian American. The African American Faces subtest consisted of all African American actors. The Voices subtest had a neutral content ("I am going out of the room now"). For all subtests, approximately half of the actors were female. Studies using the DANVA with children have provided support for the construct validity of the test (Baum & Nowicki, 1996Go; Nowicki & Carton, 1993Go; Nowicki & Duke, 1993Go, 1994Go; Nowicki, Glanville, & Demertzis, 1998Go; Nowicki & Mitchell, 1998Go). All photographs and voices received at least 80% agreement on type and intensity of emotion among elementary and high school students and adults. The subtests have been found to have adequate internal reliability (coefficient {alpha}s =.64-.81) and test retest reliability (rs range from.74 to.81) for children and adolescents 4 to 16 years.

For each of the DANVA subtests, responses were scored by a computerized program that generated an error profile for each participant on each subtest. The error analysis included total number of errors, number of errors for each emotion at each intensity, type of error for each target emotion category (e.g., number of instances in which a participant endorsed sad when the stimulus was angry), and type of error for each target emotion category at each intensity (e.g., number of instances in which a participant endorsed low intensity sad when the designated stimulus was actually high intensity angry). Intensity of emotion refers to the degree of complexity or ambiguity of the various stimuli in communicating a particular emotion. For example, low intensity emotional stimuli indicate a great deal of complexity or ambiguity; high intensity emotional stimuli suggest little complexity or ambiguity in communicating an emotion. Stimuli on the DANVA are graded for intensity of emotional voice prosody and facial affect across adults, children, and African American faces. An example of a high intensity facial affect is a large smile on a face, depicting a happy adult; an example of a low intensity adult voice is a command without affect requesting that a person cease talking. Test-retest reliability for intensity of emotion has been found to range from.70 to.81 (Nowicki & Carton, 1993Go).

Ratings of Social-Emotional Functioning
Social Skills Rating System (SSRS; Gresham & Elliott, 1990Go). Caregiver and teacher ratings are obtained in the areas of social skills competence, problem behaviors, and academic competence (the latter is only in the teacher version). The SSRS was standardized nationally with African American and Caucasian children, with these groups slightly more overrepresented than other groups. Adequate to excellent internal consistency and test-retest reliabilities have been demonstrated for the SSRS. Criterion-related validity using other rating forms of self-competence, problem behaviors, and self-concept also has been demonstrated (Gresham & Elliott, 1990Go).

Children's Depression Inventory (CDI; Kovacs, 1992Go). The CDI, a frequently used self-report measure of depressive symptoms in children and adolescents, was used to assess the extent to which symptoms of depression might be associated with neurological impairment. Extensive data have been reported supporting its reliability and validity, and several investigations have supported its construct validity (Kovacs, 1992Go) and its cross-cultural sensitivity in studies of African American children (Politano, Nelson, Evans, Sorenson, & Zeman, 1986Go). We used standardized T-scores in our analyses.

Magnetic Resonance Imaging Studies
MRI studies of the brain without contrast were performed in accordance with standard practices. Children who were younger and those who needed assistance were sedated in accordance with the guidelines outlined by the American Academy of Pediatrics (Committee on Drugs, 1992Go). Our procedures were similar to those of the Cooperative Study of Sickle Cell Disease (Armstrong et al., 1996Go) and other studies (Brown et al., 2000Go; DeBaun et al., 1998Go; Schatz et al., in pressGo). Each child's MRI was reviewed independently by a pediatric neuroradiologist and a pediatric radiologist. Any disagreements between the two radiologists were resolved by consensus. MRI readings without CNS pathology were classified as normal (no CNS pathology). Those with CNS pathology were classified as cerebral infarction, atrophy, or cerebral infarction and atrophy. If no pathology was found in the medical record or the MRI, the child was classified as normal, without CNS pathology (n = 20). Children who had an MRI indicating an infarct were classified in the positive CVA group (n = 21). A comparison control group consisted of children and adolescents with a milder variant of SCD (HbSC), none of whom were found to have CVA according to medical records (n = 11). Individuals with HbSC disease have much lower risk for CVA than those with HbSS (Ohene-Frempong et al., 1998Go). Thus, our investigation compared three groups of children: (1) 21 children with SCD (HbSS) with no evidence of CVA as documented by MRI (negative group), (2) 20 with SCD (HbSS) with documented evidence of CVA as revealed by MRI, and (3) 11 with a milder variant of SCD (HbSC) who served as comparison controls.

Data Analyses
A series of one-way multivariate analyses of covariance (MANCOVA) was performed for each domain of measures (DANVA total error scores, DANVA intensity error scores, and social-emotional functioning ratings as provided by caregivers, children, and teachers), and the classification of CNS pathology (positive MRI, negative MRI, comparison controls) served as the independent variable. No statistically significant differences between the three groups were found, but significant associations were found for FSIQ and the various DANVA subtests (rs ranged from -.04 to -.32, three of five correlation coefficients were significant, p <.01), indicating that children who scored lower on the FSIQ measure obtained higher error scores on the DANVA subtests. For these reasons, FSIQ was used as a covariate. Although no differences were found for the FSIQ, separate one-way analyses of variance (ANOVAs) were performed for each of the WISC-III subtest scores (Information, Vocabulary, Block Design, Object Assembly). Findings revealed significant differences for the Block Design, F(2, 49) = 8.79, p <.05, and the Object Assembly, F(2, 49) = 4.83, p <.05, subtests. The results of a Tukey post-hoc HSD test revealed that the two groups with HbSS disease (CNS pathology, no CNS pathology) differed significantly from the comparison controls (p <.05). To determine whether performance on the various WISC-III subtest scores was associated with performance on the DANVA scores, a series of Pearson-product moment correlation coefficients was performed among the measures. Correlations ranged from r =.04 to -.34. Six of the 20 correlation coefficients were significant (p <.05), again indicating that children who scored lower on the various WISC-III subtests obtained higher error scores on the DANVA. The pattern of associations for both FSIQ and DANVA scores and the individual WISC-III subtests was similar, and for this reason FSIQ was used as the covariate in each of the analyses. Chronological age was not a covariate because no differences were found for chronological age for the three groups. In addition, chronological age was not found to be associated with any of the dependent variables (rs ranged from.02 to -.16, all ns). Thus, three MANCOVAs were performed; significant multivariate tests were followed by analyses of covariance (ANCOVAs); and Tukey HSD post hoc analyses were performed on any dependent measure for which there was a significant ANCOVA.


    Results
 Top
 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
Of the 52 participants with SCD, 58% were male (n = 30) and 42% were female (n = 22), with a mean age of 11 years and 3 months (SD = 2 years, 10 months, range = 6 years, 4 months to 16 years). Grades in school ranged from first to tenth, with a mean of fifth grade (M = 5.33, SD = 2.76). Children were primarily in regular classrooms (94.2%; n = 49), although 3.8% (n = 2) were receiving special education resource services (part-time resource help in certain subjects) at the time of evaluation. One child (1.9%) was in a self-contained special education classroom at the time of assessment. Forty-one children (78.9%) had HbSS disease, and the 11 comparison control children (21.1%) had HbSC (the milder heterozygous condition for hemoglobin S and hemoglobin C).

Most caregivers in our sample were mothers (84.6%, n = 44). Other caregivers were fathers (9.6%, n = 5), grandmothers (1.9%, n = 1), and aunts (3.9%, n = 2). The marital status of caregivers was 28.9% (n = 15) currently married, 48% (n = 25) single, 21.2% (n = 11) separated or divorced, and 1.9% (n = 1) widowed. Although most caregivers had graduated from high school (n = 40, 76.9%), approximately a fourth (n = 12, 23.1%) had not completed high school and had not been granted an equivalent diploma through testing. The mean for years of education was 12.56 (SD = 1.61, range = 9-16 years). Most of the families had annual incomes of less than $10,000 (below poverty level) (n = 37, 67.3%). The remainder of the sample had incomes ranging from $10,000 to $19,000 (lower middle class, n = 13, 25%), $20,000 to $30,000 (middle class, n = 1, 1.9%), and above $31,000 (upper middle class, n = 1, 1.9%).

Table I presents the participants' chronological age and gender, children's FSIQ, and severity of disease (mean hemoglobin of the past three visits, white blood cell count, and number of hospital contacts over the past year) for each of the three groups. No significant differences were found for any of the demographic variables, although the two groups of children with HbSS (MRI positive and negative groups) had a higher frequency of receiving transfusion therapy (past and present), had lower hemoglobin levels, and higher white blood cell counts than their peers in the HbSC control group, thereby reflecting greater disease severity in the two HbSS groups.


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Table I. Group Membership, FSIQ, and Severity of Disease
 

The first MANCOVA, conducted to determine whether there were any group differences when decoding facial and prosodic emotions, yielded a difference that only approached statistical significance. FSIQ scores were a covariate in each of the analyses. Given the exploratory nature of the investigation, coupled with the low power, we performed separate ANCOVAs on each of the dependent measures, and a statistically significant effect was revealed for the African American Faces total error score, F(2, 48) = 3.47, p <.04, and the Adult Voices total error score, F(2, 48) = 3.69, p <.03. To determine the source of statistical significance, we performed post hoc analyses on the African American Faces and the Adult Voices total error scores. The results of these Tukey HSD post hoc tests indicated that, for both measures, the group designated with CNS pathology made more errors than the HbSC comparison control group (p <.05; see Table II for these measures).


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Table II. Means and Standard Deviations for Dependent Measures
 

A one-way MANCOVA with FSIQ as a covariate was performed on the high and low intensity DANVA scores and demonstrated a significant main effect, F(20, 78) = 1.89, p <.03. Subsequent ANCOVAs revealed significant effects for the African American Faces, F(2, 48) = 7.46, p <.002; Adult Voices, F(2, 48) = 4.81, p <.01; and Child Voices, F(2, 48) = 5.45, p <.007, low intensity error scores. Tukey HSD post hoc tests were performed for each of the measures to determine the source of significance. Participants in the positive MRI group made significantly more low intensity errors than did participants in either the negative MRI group or the HbSC comparison control group (p <.05; see Table II).

Finally, a third MANCOVA was used to determine whether there were any group differences for either caregiver (SSRS Social Skills, Problem Behaviors) or child ratings (CDI). No significant main effects were revealed, F(6, 88) =.89, ns, indicating that caregiver and child ratings did not differ as a function of MRI pathology. Because an insufficient number of teacher ratings were returned to include these scores in the MANCOVA, separate one-way ANCOVAs with FSIQs as covariates were performed for each of the teacher measures yielded on the SSRS, and none was significant (all Fs < 1.25; see Table II).


    Discussion
 Top
 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
We examined the hypothesis that the social processing skills and social and emotional difficulties experienced by youths with SCD are associated with cerebral abnormalities, as identified on MRI. Findings revealed that children with HbSS SCD who had sustained cerebral injuries evidenced a greater frequency of errors on tasks associated with social information processing than did their peers with a milder variant of SCD (HbSC). We found errors in facial recognition for African American adults in children with CNS pathology, relative to their peers with HbSC disease. Children with documented cerebral abnormalities also evidenced a greater frequency of errors associated with tasks involving low intensity of adult and child facial and vocal recognition of emotions than their peers without CNS pathology and comparison controls with HbSC disease. Finally, participants with CNS pathology also demonstrated a greater frequency of errors for low intensity facial affect of African American adults in comparison to their peers with no CNS pathology and to comparison controls with HbSC disease.

The study supported our hypothesis that children with documented CNS pathology are less successful in correctly decoding emotions associated with voices or emotional prosody than are their peers without such documented pathology who suffer from a nearly identical chronic illness. Participants with CNS pathology were less accurate at decoding low intensity adult facial affect of African American individuals and low intensity prosodic expressions in adults and children compared to children without CNS pathology and those with HbSC disease. Children with CNS pathology made more errors as the intensity of the emotional prosody stimuli decreased and became more subtle. Children with CNS abnormalities showed difficulty in the identification of emotion in adult voices, low intensity child voices, and when decoding facial expressions of same-race adults, compared to their peers in the other two groups. These data are important because they suggest that children with SCD who suffer from documented CNS pathology may encounter difficulty decoding or interpreting certain social situations that are particularly complex or ambiguous. Because intelligence test scores were controlled statistically, our findings do not appear to be attributable to general cognitive functioning.

The finding that children with SCD who have acquired CNS abnormalities may have difficulty reading subtle nonverbal and verbal social cues is in accord with the learning disability literature, which suggests that children with learning disabilities are at risk for social difficulties because they are less proficient in processing subtle social messages (Bryan & Bryan, 1990Go). Taken together with our data, the results suggest that children with neurocognitive impairments experience greater difficulty than their normally developing peers in comprehending social situations in which the verbal and nonverbal cues are ambiguous or less explicit, but they do not differ from their peers in routine or well-known situations (Pearl & Cosden, 1982Go). These observations are in accord with those of Lemanek and colleagues (1994Go), who found that children with SCD and healthy comparison controls did not differ on global teacher and parent ratings of social competence or on self-perceived social competence. Several investigators (Fletcher & Ewing-Cobbs, 1991Go; Perrott, Taylor, & Montes, 1991Go) have suggested that behavior rating scales designed for use in the general population have had mixed success in identifying the more subtle sequelae of childhood brain disease.

For our sample, caregiver, teacher, and children's ratings reflected adequate social and emotional functioning for all three groups of children with SCD, including those with documented acquired brain injuries. However, some studies of children and adolescents who have sustained acquired neurological insults, as well as children with functional brain disturbances such as learning disabilities (Shaffer et al., 1985Go; Taylor & Alden, 1997Go; Tramontana & Hooper, 1997Go), clearly indicated increased vulnerability for adjustment difficulties.

At first examination, our results may seem discrepant from other studies suggesting psychological difficulties in youths with SCD (Brown, Mulhern, & Simonian, in pressGo), but four factors may account for discrepancies. First, all of the children in our sample were diagnosed with some type of SCD, thereby mitigating differences among the groups. All of the children were receiving some type of medical treatment (e.g., transfusion therapies) throughout the course of the investigation. In addition, the mean age of participants in our investigation was younger than that in previous studies where difficulties in psychosocial adjustment have been reported. Most studies identifying psychosocial problems in youths with SCD have focused either on adolescents (Hurtig & Park, 1989Go) or have provided compelling evidence that these difficulties emerge at adolescence (Bennett, 1994Go; Brown, Doepke, et al., 1993Go). Most participants in our study were elementary school students. Thus, we suggest that social processing difficulties may result in differential developmental trajectories of social development that may not actually become apparent to caregivers and teachers until well into adolescence. Therefore, children with SCD must receive ongoing evaluation, particularly as they transition to the stages of early and late adolescence. The task of social processing, such as that used in this investigation, in addition to ratings of global behavior as completed by teachers and caregivers, will be important in the monitoring of these children during early and middle childhood, as well as during adolescence. Finally, there has been considerable diversity across studies in the assessment of psychosocial functioning. For example, many investigators have differed in their choice of informants (e.g., child, caregiver, or teacher) and in their assessment of psychopathology (e.g., internalizing, externalizing). As White and DeBaun (1998Go) cogently observed, results in the SCD literature have varied across studies as a function of the methodology used.

The finding that children who have SCD with documented neurological impairments demonstrated deficits only on specific measures of social information processing (i.e., DANVA), and not on ratings of social skills and adjustment as reported by caregivers, teachers, and the children themselves, underscores the importance of multiple measurement sources in pediatric populations. Thus, although significant others in their environment are not always able to detect subtle difficulties in social cognition among children with SCD who have sustained neurological insults, our findings in part suggest that these deficits may be detected from psychometric assessment. The difficulties that these children experience in decoding of emotions may be detected in peer relationships and in relationships with adults in informal settings (e.g., with camp counselors). Future research will need to examine the association between peer sociometric ratings and SCD children's capacity for decoding emotions.

The contribution of our investigation must be considered within the limitations of the study design, including the small sample size that may have limited power. In addition, significant variability within the three groups increased error variance and may have diminished significant effects. Further, the children were part of a clinical sample, all of whom were initially referred for a history of school difficulties. Both of the groups designated as normal may have been more accurately designated as low functioning. Additional research will need to include multisite collaborative clinical samples and larger cohorts of children with SCD. Finally, volume of cerebral injury was not assessed in our investigation, so future examination of both volume and location of injury will be profitable (Schatz et al., in pressGo).

Some evidence suggests that premorbid functioning and rehabilitation services provided immediately after injury may predict functioning shortly after the injury (Kolb & Fantie, 1989Go; Taylor & Alden, 1997Go). Future research efforts with this population should carefully examine premorbid functioning and rehabilitation services provided to patients immediately after injury. Environmental factors such as family functioning are robust predictors of children's functioning following brain injury (Rivara et al., 1996Go; Taylor et al., 1995Go; Yeates et al., 1997Go), and family functioning has been demonstrated to be an important mediator for children's adjustment to SCD (Ievers et al., 1998Go). Future research will need to demonstrate whether family functioning interacts with these children's neurological status to predict emotional adjustment.

Notwithstanding these limitations, ours is one of the first investigations to examine neurological and psychosocial functioning simultaneously in a pediatric chronic illness population. In part, our findings may explain the etiology of these children's adjustment difficulties that have been noted in other studies (Brown et al., in pressGo). Our data may show promise for the identification of the functional impairments associated with CVA in children with SCD and may eventually lead to rehabilitation efforts.


    Acknowledgments
 
This research was supported in full by a grant awarded from the National Institute of Mental Health, National Research Service Award, 5F31 MH 12071, and in part by a grant awarded from the National Institutes of Health, Heart, Lung, and Blood Branch, HLB P0-HL48-482. We thank David Freides, PhD, for his assistance with the design and conceptualization of this research.

Received June 29, 2000; revision received November 4, 2000; accepted January 3, 2001


    References
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 Abstract
 Introduction
 Method
 Results
 Discussion
 References
 
Adolphs, R., Demasio, H., Tranel, D., & Demasio, A. R. (1996). Cortical systems for the recognition of emotion in facial expressions. Journal of Neuroscience, 16, 7678-7687.[Abstract/Free Full Text]

Adolphs, R., Tranel, D., & Demasio, A. R. (1994). Impaired recognition of emotion in facial expressions following bilateral damage to the human amygdala. Nature, 6507, 669-672.

Adolphs, R., Tranel, D., Demasio, H., & Demasio, A. R. (1995). Fear and the human amygdala. Journal of Neuroscience, 15, 5879-5892.[Abstract]

Armstrong, F. D., Thompson, R. J., Wang, W., Zimmerman, R., Pegelow, C. H., Miller, S., Moser, F., Bello, J., Hurtig, A., & Vass, K. (1996). Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease. Pediatrics, 97, 864-870.[Abstract/Free Full Text]

Baum, K. M., & Nowicki, S. (1996). A measure of receptive prosody for adults: The Diagnostic Analysis of Nonverbal Accuracy-Adult Voices. Paper presented at the annual meeting of the Society for Research in Personality, Atlanta, GA.

Bennett, D. S. (1994). Depression among children with chronic medical problems: A meta-analysis. Journal of Pediatric Psychology, 19, 149-169.[Abstract/Free Full Text]

Bonner, M. J., Gustafson, K. E., Schumacher, E., & Thompson, R. J., Jr. (1999). The impact of sickle cell disease on cognitive functioning and learning. School Psychology Review, 28, 182-193.

Brown, R. T., Armstrong, F. D., & Eckman, J. R. (1993). Neurocognitive aspects of pediatric sickle cell disease. Journal of Learning Disabilities, 26, 33-45.

Brown, R. T., Buchanan, I., Doepke, K., Eckman, J. R., Baldwin, K., Goonan, B., & Schoenherr, S. (1993). Cognitive and academic functioning in children with sickle cell disease. Journal of Clinical Child Psychology, 22, 207-218.[ISI]

Brown, R. T., Davis, P. C., Lambert, R., Hsu, L., Hopkins, K., & Eckman, J. R. (2000). Neurocognitive functioning and magnetic resonance imaging in children with sickle cell disease. Journal of Pediatric Psychology, 25, 503-513.[Abstract/Free Full Text]

Brown, R. T., Doepke, K. J., & Kaslow, N. J. (1993). Risk-resistance-adaptation model for pediatric chronic illness: Sickle cell syndrome as an example. Clinical Psychology Review, 13, 119-132.

Brown, R. T., Mulhern, R., & Simonian, S. (in press). Disorders of the blood. In T. Boll (Ed.), Behavioral applications to medical disorders (Vol. 3). Washington, DC: American Psychological Association.

Bryan, T., & Bryan, J. (1990). Social factors in learning disabilities: An overview. In H. L. Swanson & B. Keogh (Eds.), Learning disabilities: Theoretical and research issues (pp. 131-138). Mahwah, NJ: Lawrence Erlbaum.

Charache, S., Lubin, B., & Reed, C. D. (1989). Management and therapy of sickle cell disease. (NIH Publication No. 89-2117). Washington, DC: National Institutes of Health.

Cohen, M. J., Branch, W. B., & Hynd, G. W. (1994). Receptive prosody in children with left or right hemisphere dysfunction. Brain and Language, 47, 171-181.[ISI][Medline]

Committee on Drugs. (1992). Guidelines for monitoring and management of pediatric patients during and after sedation for diagnostic and therapeutic procedures. Pediatrics, 93, 1110-1115.

DeBaun, M. R., Schatz, J., Siegel, M. J., Koby, M., Craft, S., Resar, L., Chu, J. Y., Launius, G., Dedash-Zadel, M., Lee, R. B., & Noetzel, M. (1998). Cognitive screening examinations for silent cerebral infarcts in sickle cell disease. Neurology, 50, 1678-1682.[Abstract]

Denkla, M. B. (1983). The neuropsychology of social-emotional learning disabilities. Archives of Neurology, 40, 461-462.[ISI][Medline]

Devine, D., Brown, R. T., Lambert, R., Donegan, J., & Eckman, J. R. (1999). Predictors of psychosocial and cognitive adaptation in children with sickle cell syndromes. Journal of Clinical Psychology in Medical Settings, 5, 295-313.

Ekman, P. (1972). Emotion in the human face: Guidelines for research and an integration of findings. New York: Pergamon Press.

Ekman, P., & Friesen, W. V. (1976). Pictures of facial affect. Palo Alto, CA: Consulting Psychologists Press.

Fletcher, J. M., & Ewing-Cobbs, L. (1991). Head injury in children. Brain Injury, 5, 337-338.[Medline]

Grace, J., & Malloy, P. (1992). Neuropsychiatric aspects of right hemisphere learning disability. Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 5, 194-204.

Gresham, F. M., & Elliott, S. N. (1990). Social Skills Rating System. Circle Pines, MN: American Guidance Service.

Hurtig, A. L., Koepke, D., & Park, K. B. (1988). Relationship between severity of chronic illness and adjustment in children and adolescents with sickle cell disease. Journal of Pediatric Psychology, 14, 117-132.[Abstract/Free Full Text]

Hurtig, A. L., & Park, K. B. (1989). Adjustment and coping in adolescents with sickle cell disease. Annals of the New York Academy of Sciences, 565, 172-182.[ISI][Medline]

Ievers, C. E., Brown, R. T., Lambert, R. G., Hsu, L., & Eckman, J. R. (1998). Family functioning and social support in the adaptation of caregivers of children with sickle cell syndromes. Journal of Pediatric Psychology, 23, 377-388.[Abstract/Free Full Text]

Kolb, B., & Fantie, B. (1989). Development of the child's brain and behavior. In C. R. Reynolds & E. Fletcher-Janzen (Eds.), Handbook of clinical child neuropsychology (pp. 17-39). New York: Plenum.

Kovacs, M. (1992). Children's Depression Inventory Manual. North Tonawanda, NY: Multi-Health Systems.

Lemanek, K. L., Horwitz, W., & Ohne-Frempong, K. (1994). A multiperspective investigation of social competence in children with sickle cell disease. Journal of Pediatric Psychology, 19, 443-456.[Abstract/Free Full Text]

Manoach, D. S., Sandson, T. A., & Weintraub, S. (1995). The developmental social-emotional processing disorder is associated with right hemisphere abnormalities. Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 8, 99-105.

Noll, R. B., Ris, M. D., Davies, W. H., Bukowski, W. M., & Koontz, K. (1992). Social interactions between children with cancer or sickle cell disease and their peers: Teacher ratings. Journal of Developmental and Behavioral Pediatrics, 13, 187-193.[ISI][Medline]

Noll, R. B., Vannatta, K., Koontz, K., Kalinyak, K., Bukowski, W. M., & Davies, W. H. (1996). Peer relationships and emotional well-being of youngsters with sickle cell disease. Child Development, 67, 423-436.[ISI][Medline]

Nowicki, S., & Carton, J. (1993). The measurement of emotional intensity from facial expressions: The DANVA FACES 2. Journal of Social Psychology, 133, 749-750.

Nowicki, S., & Duke, M. (1993). The association of children's nonverbal decoding abilities with their popularity, locus of control, and academic achievement. Journal of Genetic Psychology, 153, 385-394.

Nowicki, S., & Duke, M. (1994). Individual differences in the nonverbal communication of affect: The Diagnostic Analysis of Nonverbal Accuracy Scale. Journal of Nonverbal Behavior, 18, 9-35.

Nowicki, S., Glanville, D., & Demertzis, A. (1998). A test of the ability to recognize emotion in the facial expressions of African American adults. Journal of Black Psychology, 3, 335-350.

Nowicki, S., & Mitchell, J. (1998). Accuracy in identifying affect in child and adult faces and voices and social competence in preschool children. Genetic, Social and General Psychology Monographs, 124, 39-59.[ISI][Medline]

Ohene-Frempong, K., Weiner, S. J., Sleeper, L. A., Miller, S. T., Embury, S., Moohr, J. W., Wethers, D. L., Pegelow, C. H., & Gill, F. M. (1998). Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood, 91, 288-294.[Abstract/Free Full Text]

Pearl, R., & Cosden, M. (1982). Sizing up a situation: LD children's understanding of social interactions. Learning Disabilities Quarterly, 3, 3-9.

Perrott, S., Taylor, H., & Montes, J. (1991). Neuropsychological sequelae, family stress, and environmental adaptation following pediatric head injury. Developmental Neuropsychology, 7, 69-86.

Politano, P. M., Nelson, W. M., Evans, H. E., Sorenson, S. B., & Zeman, D. J. (1986). Factor analytic evaluation of the differences between Black and Caucasian emotionally disturbed children on the CDI. Journal of Psychopathology and Behavioral Assessment, 8, 1-7.

Rivara, J. B., Jaffe, K. M., Polissar, N. L., Fay, G. C., Liao, S., & Martin, K. M. (1996). Predictors of family functioning and change 3 years after traumatic brain injury in children. Archives of Physical Medicine and Rehabilitation, 77, 754-764.[ISI][Medline]

Rodgers, G. P., Clark, C. M., & Kessler, R. M. (1984). Regional alterations in brain metabolism in neurologically normal sickle cell patients. Journal of the American Medical Association, 256, 1692-1700.

Rourke, B. P., & Del Dotto, J. E. (1994). Learning disabilities: A neuropsychological perspective. Thousand Oaks, CA: Sage.

Sattler, J. M. (1992). Assessment of children (Revised and updated, 3rd ed.). San Diego, CA: Jerome M. Sattler.

Schatz, J., Craft, S., Kobny, M., Siegel, M. J., Resar, L., Lee, R., Chu, Y. F., Launius, G., Dadash, M., Zadeh, M., & DeBaun, M. R. (in press). Neuropsychologic deficits in children with sickle cell disease and cerebral infarction: The role of lesion location and volume. Child Neuropsychology.

Shaffer, D., Schonfeld, I., O'Connor, P. A., Stokman, C., Trautman, P., Shafer, S., & Ng, S. (1985). Neurological soft signs. Archives of General Psychiatry, 42, 342-351.[Abstract]

Short, A., Vannatta, K., Kalinyak, K., & Noll, R. B. (1999). Peer relationships of children with sickle cell diseases: A replication study. Paper presented at the 23rd annual meeting of the National Sickle Cell Disease Program, San Francisco, CA.

Taylor, H. G., & Alden, J. (1997). Age-related differences in outcomes following childhood brain insults: An introduction and overview. Journal of the International Neuropsychological Society, 3, 555-567.

Taylor, H. G., Drotar, D., Wade, S., Yeates, K., Stancin, T., & Klein, S. (1995). Recovery from traumatic brain injury in children: The importance of the family. In S. H. Broman & M. E. Michel (Eds.), Traumatic head injury in children (pp. 188-216). New York: Oxford University Press.

Tramontana, M. G., & Hooper, S. R. (1997). Neuropsychology of child psychopathology. In C. R. Reynolds & E. Fletcher-Janzen (Eds.), Handbook of clinical child neuropsychology (2nd ed., pp. 120-139). New York: Plenum.

Trauner, D. A., Ballantyne, A., Friedland, S., & Chase, X. (1996). Disorders of affective and linguistic prosody in children after early unilateral damage. Annals of Neurology, 39, 361-367.[ISI][Medline]

Wechsler, D. (1991). Wechsler Intelligence Scale for Children (3rd ed.). San Antonio, TX: Psychological Corporation.

White, D. A., & DeBaun, M. (1998). Cognitive and behavioral function in children with sickle cell disease: A review and discussion of methodological issues. Journal of Pediatric Hematology/Oncology, 20, 458-462.

Yeates, K. O., Taylor, H. G., Drotar, D., Wade, S. L., Klein, S., Stancin, T., & Schatschneider, C. (1997). Pre-injury family environment as a determinant of recovery from traumatic brain injuries in school-age children. Journal of the International Neuropsychological Society, 3, 617-630.


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