A Multidisciplinary Health Care Team’s Efforts to ImproveEducational Attainment in Children With Sickle-CellAnemia and Cerebral InfarctsAllison King, Sonya Herron, Robert McKinstry, Stephen Bacak,Melissa Armstrong, Desiree White, Michael DeBaun

ABSTRACT: The primary objective of this study was to improve the educational success of children with sickle-cell disease (SCD) andcerebral infarcts. A prospective intervention trial was conducted; a multidisciplinary team was created to maximize educationalresources for children with SCD and cerebral infarcts. Students were evaluated systematically before and after the intervention. Abaseline evaluation was completed assessing the presence of an Individualized Education Plan (IEP), grade retention in school, anddays absent from school in the year preceding the intervention. A postintervention assessment occurred 2 years later for these samemeasurements. At baseline, 74% (17 of 23) of the students were receiving IEPs. Two years later, 87% (20 of 23) students received IEPs(p ¼ .34). Despite the intervention, the rate of children retained in their school grade increased from 0.6 per 100 years in school atbaseline to 1.7 per 100 years, 95% CI (�3.86, 1.49). The school absenteeism rate did not significantly change after the intervention;the average days absent per student rose from 15.5 to 22.5, (p ¼ .05). The multidisciplinary team effort alone was insufficient todecrease grade retention and absenteeism rate. Further support, from either the parents or school administration, is needed toincrease education attainment of students with cerebral infarcts. (J Sch Health. 2006;76(1):33-37)

Children with sickle-cell disease (SCD) are at risk formultiorgan disease including cerebral infarcts.1 At

least 30% of children with sickle-cell anemia have cerebralinfarcts before 18 years of age.2 Children with either overtstrokes or silent cerebral infarcts typically have significantcognitive deficits and poor academic achievement,3,4 withalmost 74% of students with cerebral infarcts having beenretained a grade or placed in a special education classroom.5

All students with cerebral infarcts should be evaluatedfor an Individualized Education Plan (IEP). An IEP is awritten, legal document that describes the special educa-tion and related services that an eligible student receives.Students with SCD qualify for an IEP because they havea chronic disease resulting in limited alertness with re-spect to the educational environment.6 Developing and im-plementing an IEP is one of the key steps taken by parents,teachers, and school counselors to assist a student withspecial needs in obtaining educational services. In a cross-sectional study in students with SCD and cerebral infarcts,only 70% had an IEP when uniform adoption of the IEPwas advocated.7

Based on the poor academic performance of studentswith SCD and cerebral infarcts, a multidisciplinary pro-gram was developed to ensure appropriate use of additionaleducation resources. The investigators hypothesized that 2years after starting the program, there would be an overallincrease in the proportion of children receiving an IEP foreligible children, a decrease in the proportion of studentsretained additional grades, and a decrease in the schoolabsenteeism rate when compared to baseline statistics.

METHODSIn 1999, the Stay in School Program was created with

the purpose of increasing the use of educational resourcesin the community by all the children with SCD and cere-bral infarcts. The Stay in School program was designedto increase IEP development and lead to a more support-ive educational approach for each student by targetingspecific accommodations, including home tutoring to com-pensate for missed school days. By substituting tutoreddays at home for days absent, the team hoped to reduceschool absenteeism and reduce grade retention as morestudents obtained IEPs. The Stay in School Program wasinstituted as a pilot intervention to determine if familieswould accept more support to maximize the students’ aca-demic experience.

The multidisciplinary team included hematologists, anurse practitioner, a social worker, a neuropsychologist, aneurologist, and a neuroradiologist. A social worker andnurse practitioner were hired to focus on improving thequality of medical care for this high-risk population. Thesocial worker provided support and guidance to the fami-lies of each student. Each student (with or without a his-tory of cerebral infarct) in the hematology office had anannual educational inventory as part of routine practice.The team independently verified whether the child receivedspecial education or resource classes, whether the childhad an IEP, the child’s current courses, and the child’smost recent grades by receiving copies of the report cardsand IEPs from the children’s schools. For children whowere frequently admitted to the hospital or had a historyof missing more than a week of school, the social workeralso arranged for intermittent homebound tutoring at thebeginning of each school year. A tutor was provided bythe local special school districts, and the tutor would visitthe student in the home or hospital to cover material thatwould be covered in the classroom. On average, for everyschool day missed, the school district provided 2 to 3hours of tutoring in the home. The nurse practitioner pro-vided a continuity of medical care for the children andfamilies on an inpatient and outpatient basis.

Allison King, MD, MPH, Instructor of Pediatrics, (king_a@kids.wustl.edu);Sonya Herron, MSW; Stephen Bacak, MPH; Melissa Armstrong, BA;Michael DeBaun, MD, MPH; Associate Professor of Pediatrics, PediatricPediatric Hematology and Oncology, 660 S. Euclid Ave, Campus Box8116, St. Louis, MO 63110; Robert McKinstry, MD, PhD, Assistant Pro-fessor of Neuroradiology, Neuroradiology, St. Louis Children’s Hospital,St. Louis, MO; and Desiree White, PhD, Associate Professor of Psychol-ogy, Department of Psychology, Washington University, St. Louis, MO.This study was funded by the American Heart Association (AK) and theDoris Duke Foundation, (MD).

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Institutional Review Board approval was obtained fromWashington University School of Medicine, but consentwas not obtained from the patients or their families toparticipate in this study because all information obtainedwas identified as part of routine care. Data were collectedfor the academic years 1999-2000 (baseline), 2000-2001,and 2001-2002. The multidisciplinary team was involvedduring the 3 years.

Inclusion and Exclusion CriteriaBased on a previous study,7 students were considered at

high risk for school failure if they had cerebral infarcts,either overt or silent. Overt cerebral infarcts were definedas an acute neurologic event lasting for greater than 24hours. Silent cerebral infarcts were defined as children withcerebral infarcts on magnetic resonance imaging (MRI)and no focal neurologic finding or history of a neurologicevent.4 MRIs were obtained as standard care with neuro-logic findings to evaluate for an overt infarct or if parentshad raised concerns regarding changes in the child’s behav-ior or school performance to screen for silent infarcts.

Inclusion criteria were the following: children withSCD as documented by hemoglobin analysis, childrenwith a documented infarct on MRI, children currentlyenrolled in school, and parental consent to release schoolrecords to the team. Children were excluded if parentsrefused to participate.

RecruitmentThe social worker from the team approached consecu-

tive families in clinic each week. Twenty-three school-agedstudents with a history of a cerebral infarct were offeredthe chance to participate in the intervention, and all ofthe students and families agreed to have the multidisci-plinary team become involved. Three students were ini-tially identified as having no history of cerebral infarctbut had a cerebral infarct within the first school year ofthe study. Twenty students with cerebral infarcts and SCDwere identified at baseline.

Cognitive EvaluationEach child was offered neuropsychological testing to

assess his or her cognitive domains and academicachievement by a neuropsychologist. These data wereused to identify general levels of intelligence, domain-specific strengths and weaknesses, and academic achieve-ment that could assist in the development of an IEP. Thefollowing tests were utilized: the Wechsler AbbreviatedScale of Intelligence (WASI),8 the Wechsler IndividualAchievement Test (WIAT),9 the NEPSY Verbal Fluencytest,10 the Children’s Memory Scale,11 the CVLT_C,12 theNEPSY Visual Attention,10 the TOVA,10 the Stroop Colorand Word Test,13 and the NEPSY Tower.10 Results of thetesting were provided to each child’s school to incorporateinto the IEP or teaching plan.

After the school records, neuropsychological testing,and academic testing results were received, the socialworker and pediatric hematologist met with each familyto review the results. The team sent a letter to the child’sschool to explain the nature of the student’s SCD and theresults of the cognitive tests at the parent’s request. Thechild’s parent and/or the social worker contacted each

child’s school to request an IEP. When deemed necessary,the social worker attended the IEP meeting to serve asan advocate for the child and to maintain record of themeeting for the child’s records. The importance of regularattendance at school was emphasized to each parent andstudent. To facilitate school attendance, each student wasencouraged to have an intermittent homebound educationplan within the IEP. Such plans allow a student to have atutor employed by the special school district go to thehome when the student is ill and cannot attend school. Thechild is still able to perform some schoolwork at homeand, therefore, should receive credit for attending school.

Outcome measures for the intervention included com-pletion of an IEP for students who needed an IEP basedon the assessment of the parents, teachers, and the SCDteam; the number of school days missed, and the numberof students retained in their current grade. If a homeboundtutor arranged by the school saw the students at home,then the student was not considered absent. Statisticalanalysis was performed with the Statistical Package forthe Social Sciences (SPSS, Chicago, Ill), version 11.0. At test was used for parametric comparisons and 95% confi-dence intervals (95% CI) were provided. A Wilcoxonsigned ranks test or the Mann-Whitney were used for non-parametric comparisons. A p value , .05 was consideredstatistically significant. The 95% CIs were not provided forthe nonparametric statistics because of the small, skeweddistributions.

RESULTSDemographics at Baseline

Twenty-three students with cerebral infarcts and SCDwere identified (Table 1). All students were AfricanAmerican and 11 were male. More than 60% of the stu-dents were under the age of 14 years, with the averageage being 12 years old. A total of 87% (19 of 23) of stu-dents had Medicaid for health insurance; the other 4 hadprivate insurance. Ten students were of age to graduatefrom high school within the study period. Over 40% (10of 23) of the students were retained a grade at some pointprior to the Stay in School Program. All of the studentswere enrolled in public schools.

None of the students were on chronic medicationsother than daily folic acid. Students may have takenopioids intermittently for pain, but none of the studentswere admitted more than 3 times per year to the hospital.In a separate study, a higher disease severity was de-scribed as 3 or more hospitalizations per year for paincontrol.7

Intervention Increases Proportion of Students Withan IEP. An increase in the proportion of students with anIEP was observed after the start of the Stay in SchoolProgram. After the intervention, the proportion of studentswith cerebral infarcts who received IEPs increased, from74% (17 of 23) to 87% (20 of 23), (Wilcoxon, p ¼ .34).

Three students did not receive an IEP after the inter-vention had silent cerebral infarcts because they were per-forming above average in all subjects, and their parentsand teachers jointly decided not to request an IEP. Theiraverage global IQ was 97.

Intervention Did Not Reduce Missed School Days. Nodifference in school days missed was observed after the

34 d Journal ofSchool Health d January 2006, Vol. 76, No.1 d � 2006, American School Health Association

intervention. During the 1999-2000 school year, the stu-dents missed an average of 15.5 school days, and duringthe 2000-2001 school year the students missed an averageof 22.5 school days (Wilcoxon, p ¼ .05) (Table 2).

Intervention Program Was Unsuccessful in Decreasingthe Proportion of Grade Retention for Students. The rateof grade retention increased (Table 2). In the 1999-2000school year, the retention rate was 0.6 per 100 years inschool (1 failure per 175 years in school). After the inter-vention in 2001, the retention rate was 1.5 retentions per100 years in school (2 failures per 135 years in school).The students were followed for an additional year, andthe retention rate remained approximately the same, 1.8retentions per 100 years in school (2 retentions per 114years in school), 95% CI (�3.86, 1.49).

The proportion of students graduating from highschool was low. Overall, only 20% (2 of 10) of the stu-dents with cerebral infarcts graduated or were on courseto graduate from high school in 4 years with a highschool diploma. Three of 24 students were not in tradi-tional educational courses and were enrolled in specialeducation curricula without the structure of passing orfailing a grade. These curricula provide educational ortraining programs until a student reaches 21 years of age.Two of the students turned 21 years of age during the2000-2001 school year, while the third received a highschool certificate and not a diploma at age 19. (In St.

Louis, Missouri, the majority of students receive theirhigh school diploma by 18 years of age.) Additionally, 1student dropped out of school, and 2 graduated during the2001-2002 school year. Generally, the students with themost diffuse lesions on the MRIs were the most impairedand completed special education coursework or droppedout of school.

Global IQ Was a Significant Factor in Grade Reten-tion. Neuropsychological testing was offered to all of thestudents with cerebral infarcts (Table 3). A total of 13%(3 of 23) students were physically unable to complete thefull battery of tests, and 4% (1 of 23) were unable tocomplete the battery due to behavioral issues at initialdiagnosis but did complete the testing in 2003. The stu-dents with cerebral infarcts and grade retention (n ¼ 10)had a significantly lower full-scale IQ than students withcerebral infarcts and no grade failures (n ¼ 8), 75 versus93, respectively (Mann-Whitney, p ¼ .002). Statisticallysignificant differences existed for verbal (79 vs 94) andperformance (75 vs 93) IQ as well, (p ¼ .016 and .001,respectively).

DISCUSSIONCerebral infarcts represent one of the most feared com-

plications in children with SCD. For children with SCD,late effects relating to cognitive function are devastating,particularly for students who have multiple years of for-mal education prior to anticipated high school graduation.At the beginning of our study, only 71% of students hadIEPs and over 40% of students had been retained a grade.After the intervention, 100% of the students whose pa-rents and teachers believed needed an IEP received one.However, the rate of grade retention and average days ofmissed school actually increased. The increase in schooldays was unexpected, as we postulated that our interven-tion would demonstrate improvement in grade promotionand decrease in school absenteeism.

Among children with cerebral infarcts that includeboth silent and overt strokes, this is the first descriptionof the factors associated with poor academic attainment.The common feature among the students with cerebral in-farcts who failed was a low IQ, an average of 75 versus93 in the group that did not fail. The small number ofstudents who failed prevents any meaningful inferences;however, these data coupled with the poor high school

Table 1Baseline Demographics of Students With Sickle-

Cell Disease and Cerebral Infarcts, St. LouisChildren’s Hospital, 1999-2000

Characteristic N = 23

Age in years5-9 30.4%10-14 30.4%15 and older 39.1%

GenderMale 47.8%

Retained a grade in past (% Yes) 43.5%

Table 2IEP Attainment, School Days Missed, and Grade Retention Rates for Students With

Sickle-Cell Disease and Cerebral Infarcts at St. Louis Children’s Hospital*

Baseline(1999-2000)

Intervention(2000-2002)

p Value or 95%Confidence Intervals

IEP (%) 74 87 .34School days missed 15.5 22.5 (2000-2001) .05Rate of grade failure(failures per 100 school years)

1/175 ¼ 0.6 2/114 ¼ 1.8 �3.86, 1.49

* One student was retained 2 years in a row before dropping out of school in the 2000-2001 academic year.

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graduation rate of 20% would strongly suggest that alter-native approaches to educating this population be consid-ered. Prior studies comparing children with SCD andcerebral infarcts with children with SCD and no infarctsdemonstrated that those with infarcts have significantlylower IQs (77 vs 90).14

Children with infarcts in the frontal lobes tend to havedeficits in memory and attention, while those with diffuseinfarcts that include the frontal and parietal lobes tend tohave memory, attention, and visual spatial deficits.3,15-17

Accommodations in an IEP for a student with SCD and aninfarct would depend on the results of the cognitive

Table 3Neuropsychological Testing and Postintervention Educational Status in Children With

Sickle-Cell Disease and Cerebral Infarcts, St. Louis Children’s Hospital

Type ofCerebralInfarct

Locationof

Infarct

Age atCerebral Infarct

(in Years)

Age at StudyEntry

(in Years)Full-Scale

IQVerbalIQ

PerformanceIQ

Educational Statusat Completion

of Study

1 Overt Thalamus 2.5 5 89 94 88 Passing IEP classes2* Overt Frontal 2.5 16 57 66 55 Certificate for completion

of high school3 Overt Frontal, parietal,

temporal3 17 89 83 99 Not on track, failed grade,

and dropped out of school4 Overt Frontal 3.1 14 82 89 77 Not on track, failed grade5 Overt Frontal, parietal,

temporal4 14 78 86 75 Not on track, failed grade

6 Overt Frontal 4.6 17 69 80 58 Did not graduate fromhigh school, failed grade

7 Overt Frontal, parietal,temporal

5.2 5.2 Unable tocomplete

Certificate track specialeducation in grade school

8yz Silent Frontal 6.2 5 98 106 89 Passing grade school9 Silent Basal ganglia 6.5 13 59 63 62 Failed a grade, passing

special education classes10§ Silent Thalamus 7 7 97 92 101 Passing grade school11 Silent Basal ganglia 7.6 8 66 66 72 Failed a grade, passing

special education classes12 Overt Frontal 7.7 8 89 94 88 Passing grade school13z Silent Frontal, parietal,

occipital8.3 7 95 86 105 Passing grade school

14* Overt Frontal, parietal,occipital

8.4 13 71 87 60 Passing specialeducation classes

15 Silent Temporal 8.8 9 86 88 86 Passing grade school16 Silent Caudate 9.4 16 87 91 86 Failed in past, passing

special education classes17 Overt Frontal 10.8 12 59 64 61 Failed in past, passing

special education classes18§ Overt Frontal, parietal 11.7 17 106 108 103 Graduated19 Overt Thalamus 13.4 13.5 88 96 84 Failed in past, passing

IEP classes20 Silent Frontal 13.7 15 72 73 77 Not on track, failed grade21 Overt Caudate, basal

ganglia14.1 19 Unable to

completeCertificate

22y§ Silent Caudate, basalganglia

14.1 17 83 86 85 Graduated

23 Overt Frontal, parietal,temporal,basal ganglia

16.7 19 Unable tocomplete

Certificate

* These 2 patients completed cognitive testing in 1995 and 1998, respectively.y This patient originally failed to complete testing during the 1999-2000 school year due to behavioral issues but was able to sitthrough testing in 2003.

z These patients were diagnosed with a silent infarct during the first school year of the study.§ These patients completed the cognitive testing during the 2000-2001 school year.

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evaluation. For example, if a student with a frontal infarcthad poor memory function, the teacher may need to givewritten as well as verbal instructions for assignments. Astudent with a poor processing speed may need individual-ized instruction as well as longer time periods to completetests than other students in the same classroom. A moreintensive educational intervention such as a remediationprogram that focuses on specific deficits may benefit thesestudents, or a shift in the focus of traditional curriculum tovocational education may be of greater value.

As with any intervention study, there are limitations tothis study’s design. The socioeconomic status (SES) foreach student and the amount of time that parents spentwith each child in the home on schoolwork was not con-sidered. Although SES may influence academic perfor-mance, we could not evaluate SES given the highproportion of the families in the study on Medicaid,approximately 87%. In addition, evaluation for passing orfailing a grade has a subjective component. However,after the intervention, the rate of retention unexpectedlyincreased in the students with cerebral infarcts.

Several plausible reasons exist as to the lack of signifi-cant findings. Possibly, the students need more than theStay in School Program provides, or the sample size wastoo small to distinguish a true difference when one actu-ally existed. The study was designed to assess the impactof a comprehensive approach to improve educationalattainment. A power calculation prior to the start of thestudy was not appropriate because there was no alterna-tive. Further, after completion of the study, the directionof the postulated outcome was opposite from what weanticipated, strongly suggesting that the intervention wasnot effective, as opposed to the sample size being toosmall. The more likely reason for the lack of improve-ment in grade promotion and days of school attendance isthat the intervention was insufficient to overcome eithercognitive or environmental factors. Alternatively, childrenmay have developed a progressive decline in cognitivefunction with time as other investigators have docu-mented.18 Five of the 23 students in the study were retestedwithin 2 years and had stable scores, but this again repre-sents only a sample. Future studies with a longer period oftime, a more rigorous schedule of cognitive measures, andtighter evaluations of the content of the IEPs may be ableto address these variables.

This is the first prospective study to document thepotential benefit of an intensive school intervention pro-gram in students with SCD and cerebral infarcts. Despiteindividualized effort allocated by the social worker, neu-ropsychological evaluations to facilitate formal interven-tion by the school, and an increase in access to providingIEPs, students with SCD and cerebral infarcts did notimprove their educational attainment after 2 years of

a multidisciplinary effort. Additional modifiable factorscontributing to poor academic attainment such as thequality of remediation or improving support for the stu-dent at home must be explored in students with cerebralinfarcts. j

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