|Year : 2016 | Volume
| Issue : 4 | Page : 124-130
The impact of sickle cell disease severity on school performance in affected Yemeni children
Abdul-Wahab M Al-Saqladi
Department of Pediatrics, Faculty of Medicine and Health Sciences, University of Aden, Aden, Yemen
|Date of Web Publication||18-Jan-2017|
Dr. Abdul-Wahab M Al-Saqladi
Department of Pediatrics, Faculty of Medicine and Health Sciences, University of Aden, P.O. Box 6032, Khormaksar, Aden
Source of Support: None, Conflict of Interest: None
Background: School difficulties are common in patients with sickle cell disease (SCD). The study aimed to assess the relationship between severity of SCD in children and their school absenteeism and achievement.
Subjects and Methods: The sample included school-aged children (7–15 years old) with SCD who were enrolled from Hematology Clinic in Al-Sadaqa General Teaching Hospital, Aden, during 2013 through 2014. Data about school absence, academic score achievement, and grade retention were collected. Disease severity was assessed by frequency of clinical events and complications. Differences between groups were assessed by appropriate statistical analysis.
Results: Sixty children were included for the study; their mean age was 11.5 ± 2.4 years and 51.7% were of the female sex. The number of days absent from school in a year ranged from 0 to 112 days, with a median (IQR) of 28 (14–45) days. Absence of more than 20 days in the previous academic year was reported in 60% of the children. Grade retention was reported in 45% of the children. Both school absence and grade retention were significantly correlated with age (r = 0.35, P = 0.006 and r = 0.32, P = 0.01, respectively). During the previous academic year, 48.3% of the children reported a below average final academic score. Severity assessment revealed that 65% of the children scored as severe. School absenteeism was significantly associated with disease severity score (r = 0.44, P < 0.001). Children with low academic achievement and those with grade retention had significantly higher disease severity score (all P < 0.05).
Conclusion: This study suggests that disease severity has important influences on school attendance and performance. Interventions to modify disease severity and school absence might improve academic performance in the affected children.
Keywords: Cognitive function, school absenteeism, school grades, severity, sickle cell
|How to cite this article:|
Al-Saqladi AWM. The impact of sickle cell disease severity on school performance in affected Yemeni children. J Appl Hematol 2016;7:124-30
| Introduction|| |
Sickle cell disease (SCD) is one of the commonest monogenic diseases worldwide, with an estimate of more than 300,000 infants born each year with the majority belonging to developing countries. The basic molecular defect is a single nucleotide change in the 6th codon of the β-globin gene resulting in production of abnormal hemoglobin (HbS). SCD is a heterogeneous group of hereditary hemoglobin disorders (SS, SC, Sβ0-thalassemia, Sβ+-thalassemia, etc.), and the most common and most severe form is the homozygous SS. The sickle hemoglobin is markedly less soluble, polymerizes on deoxygenation, and distorts the erythrocyte into a sickled shape. Sickle red blood cells become less deformable, fragile, and stick easily to the blood vessel wall, impairing normal flow and leading to tissue hypoxia and infarction.
Clinically, SCD is considered a multisystemic disease characterized by protean manifestations and complications including recurrent painful episodes, chronic anemia, acute hand–foot syndrome, splenic sequestration, acute chest syndrome, sepsis, delayed growth and puberty, cerebrovascular disease, and cognitive impairment.
As a chronic illness, SCD has immense health and socioeconomic impacts on families and society as a result of frequent pain episodes, medical visits, hospitalization, blood transfusion, utilization of drugs, and other healthcare services. Furthermore, it is well recognized that children with SCD are at risk of neurocognitive deficits and school performance difficulties, which become greater when the central nervous system (CNS) is involved. Although cognitive impairments were more predominant in those with overt or silent stroke, it was also encountered in those with no evidence of brain abnormalities using conventional magnetic resonance imaging (MRI). This has been attributed to other mechanisms of brain insults, such as hypoxia related to acute exacerbation of chronic anemia, acute chest syndrome, and micronutrient deficiencies related to high metabolic demands.,
School performance in SCD may be compromised by cognitive deficits, but additional factors including various disease-related complications are likely to be relevant, particularly in severely affected individuals. Recurrent clinical events and hospitalization may lead to school disruption, high rate of school absenteeism, and poor academic achievement. Frequent school absences up to 30 days per year on average was reported in children with SCD and was found to be a contributory factor to poor academic performance., Affected children also scored below their peers in school grades, and they have repeated grades consistently with a rate of 23–54%, which is much higher than in the normal child population. Grade retention tends to increase the risk of school dropouts by about 2–11 times.
Studies on the natural history of SCD demonstrate considerable variation in clinical features, complications, and disease severity with wide geographical diversity. Furthermore, determination of severity is a complex issue, particularly in the absence of completely acceptable definition, limited predictors, and lack of definitive biomarkers.
Most studies on the relation between SCD severity and school performance have focused on a single parameter for severity assessment such as frequency of pain crises, hospitalization, hemoglobin, or hematocrit levels., A single indicator may not be sufficient to assess severity; rather, it will be better to include additional factors to identify the wide variation in clinical expression and a combination of indices is expected to classify severity status in a more accurate manner.
Currently, there are limited number of published studies about the influence of SCD on school performance and absenteeism in the Arab World. The aim of this study was to determine the impact of SCD in children without a history of overt neurological involvement on school absenteeism and grades using a comprehensive set of morbidity indicators for the assessment of disease severity and categorization of patients.
| Subjects and Methods|| |
This retrospective cross-sectional study employed a survey of parents and children with SCD, aged 7–15 years, who were enrolled from the Hematology Clinic in Al-Sadaqa General Teaching Hospital, Aden, during 2013 through 2014. The hospital is the main referral health facility providing both general and specialist pediatric care for children in Aden and the adjacent governorates.
The sample included school-going children with SCD, aged below 16 years, who had been diagnosed by clinical manifestations and positive sickling test, and confirmed by hemoglobin electrophoresis. Data were collected through a direct interview according to a prestructured questionnaire. The author himself collected a detailed clinical history and conducted an examination. Information about past medical history and frequency of acute clinical events covering the prior one-year period was collected. Previous hospital admissions, blood transfusion, frequency of crises (vaso-occlusive, splenic sequestration, aplastic, and hyperhemolytic), and other SCD-related complications including acute chest syndrome, avascular bone necrosis, skin ulcer, gall stone, priapism, and urinary disorders were recorded. Definitions of clinical events were those proposed by the SCD cooperative study group. One or both parents were interviewed and were asked to make available all medical documents (doctor prescriptions, previous investigation papers, and notes of hospital discharge). Information related to preceding admissions was cross-checked against the patient’s hospital records.
Clinical severity score was assessed based on frequency of painful crises, hospitalization, blood transfusion, and sickle cell-related complications including acute chest syndrome, leg ulcers, gallstones, enuresis, hematuria, and degree of anemia as previously described. The presence of each of the findings was allocated the corresponding score whereas its absence was scored as zero. A severity index (SI) was calculated for each child using the total score from the previous year. These children were grouped according to their SI; a score of ≤6 disease was considered mild, or severe if >6. This cut-off was used to facilitate discrimination between the two categories and has been validated in previous studies. The severity score is a continuous variable and was used for correlation analyses.
Parents were asked to provide information about the number of days the child was absent from school in the previous academic year. They were asked whether their children faced increased difficulties at school for educational achievement and academic progress than their siblings or other classmates as a result of disease interference. The average academic score for the last year session (in percentage), which was calculated as the overall score for two terms of each academic year, was also collected and was considered below average if it was less than 60% and average or above if it was more than or equal to 60%. This measure had been used previously for the assessment of academic performance of school children with SCD., History of grade retention was obtained by asking the parents if their child had ever repeated a grade during his or her school career. We relied on parental accounts to assess school absence and grade retention, as similar methods had been used in a previous research and were found to be reliable. Those students who had left school for a long period and had no intention of returning were considered school dropouts.
All recruited children attended regular public schools and had no overt neurological dysfunction such as stroke or convulsion, were not on chronic transfusion or hydroxyurea therapy, and had no other chronic comorbidities. The reason for this exclusion was that children with such complications had additional problems that could interfere with their school life.
All parents of eligible children were approached and they agreed to participate; and the study was approved by the Ethics Committee in Al-Sadaqa General Teaching Hospital.
Statistical analysis was performed with Statistical Package for the Social Sciences (SPSS) version 22. Appropriate statistical tests were used to analyze differences between groups. The independent sample t test or Mann–Whitney U for comparison of means or medians, Chi-square (χ2) or Fisher’s exact test for difference between frequencies, and Pearson’s test of correlation between numerical variables were used. The level of significance was considered if P < 0.05.
| Results|| |
A total of 67 children with SCD were recruited; 7 children had left the school (dropouts) and were excluded from the final analysis. Of the 60 children, there were 31 (51.7%) female and 29 (48.3%) male children, with an overall mean age of 11.5 ± 2.4 years (range 7–15 years). The mean age for the female children was 11.4 ± 2.7 years and 11.6 ± 2.2 years for the male children with no significant difference. Children aged more than 10 years were 38 (63.3%) in number.
The number of missing days from school ranged from 0 to 112 days, with a median (IQR) of 28 (14–45) days; absence for more than 20 days was reported for 36 (60%) children. Forty-eight (80%) of the parents reported that their children faced school difficulties more than their other healthy counterparts. Twenty-seven (45%) of the participants reported a history of repeating a grade at some point in their school career. Children with grade retention were found to have higher school absence, with a median (IQR) of 52 (40–110) days, versus those with no history of grade retention, with a median (IQR) of 14 (7–28) days, P < 0.001. School absence and repetition of grade were significantly correlated with age, as (r = 0.35, P = 0.006 and r = 0.32, P = 0.01, respectively). During the previous year, 29 (48.3%) children reported final academic scores below average.
Severity scores distribution is depicted in [Figure 1]. SI categorized 39 children (65%) as severe and 21 (35%) children as mild. Although more girls than boys were in the severe category group (24 vs 15), the differences did not reach statistical significance (P = 0.06). Painful crisis, hospital admission, and blood transfusion were more frequent in the severe category (all P < 0.001) [Table 1]. Haemoglobin level was significantly lower in the severe category than in the mild category (P < 0.05). The main effect of disease severity was observed by school absence, grade retention, and academic score, as shown in [Table 2]. School absenteeism was significantly associated with disease severity score (r = 0.44, P < 0.001). Mean severity score was significantly different between those who had history of grade retention and those who did not (mean 9.9 vs 5.7, 95% CI: 5.43 and 2.9, P < 0.001).
|Table 1: Clinical events, complications, and hemoglobin level as per severity index category|
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|Table 2: School absence, academic score, and grade retention as per severity index category|
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| Discussion|| |
This study examined school absenteeism and performance in children with SCD in relation to severity of their illness using a comprehensive set of morbidity indicators. The primary finding was a higher rate of school difficulties in children with SCD, which was more pronounced in those with severe disease. Specifically, the data demonstrated that increased school absence, low academic achievement, and higher-grade retention rate correlated with disease severity scores.
Numerous studies have consistently reported frequent school absences among children with SCD.,,, Recurrent morbidity was assumed to be a primary cause of such absence, and in particular sickle cell painful episodes that are the most recurrent clinical events. In a previous study, academic performance and school absence were compared in the following two groups of SCD children: those hospitalized for more than 4 times/year for pain crises (high frequency) and those with one or no hospitalization (low frequency). Overall academic achievement for both groups was lower than normative levels by at least one standard deviation, and school absence was almost twice in the high frequency group (35.7 vs 16.8 days). These researchers found substantial number of school absence episodes not related to hospitalization for pain crisis, which is undoubtedly linked to other disease complications that required hospital admission or school disruption like infection episodes, anemic crises, acute chest syndrome, etc., and were not taken in consideration in their analysis. Nonpain-related school absence was reported in about half of the adolescents followed for an average of 10 months. The authors suggested that recurrent school absence may make children lag in their schoolwork, which reinforces their motivation for school avoidance. In pediatric chronic pain conditions in the child, school absenteeism is not a simple or inevitable consequence of pain, and many absences may not be directly related to pain itself.
In a recent study, pain frequency alone was found inadequate in predicting SCD severity and did not correlate with the prevalence of some severe complications. Therefore, the severity scoring method used in this study was preferably selected because it covers almost all relevant items related to SCD severity and incorporates the most important clinical events and complications. This score was confirmed to discriminate degrees of severity correctly into two distinct groups, delineated by genetic and phenotypic characteristics, with a mild disease (Arab-Indian haplotype) in the eastern province and a severe form (African haplotypes) in the south-western regions of Saudi Arabia., The score was validated in other populations, as in Africa and Yemen.
A number of studies have reported differences between SCD children and healthy control groups on full-scale IQ and also on specific neuropsychological domains. There is good evidence suggesting that overall IQ difference between SCD children and normal comparisons was marked and estimated to be about 4.3 standard score points and the difference was increased by age. On specific domain evaluation, most difficulties have been reported with verbal and language abilities, visual motor and spatial attention, and memory as well as executive function skills.,, Deleterious effects on school function are likely to be observed through concentration and attention abilities.
School difficulties in children as a result of disease interference was reported by 80% of the parents in this study; this result is similar though higher than the 60% of the parents who reported in USA. There are many reasons for children with SCD to have school difficulties and academic underachievement; beside school absence, several disease-related factors and psychosocial variables are implicated. One of the most devastating complications of SCD is brain damage caused by cerebral vasculopathy, leading to significant neurocognitive deficits.
Hemoglobin level was significantly lower in the severe category in this analysis. Prior studies demonstrated significant correlation of anemia severity with neurocognitive functioning, which was attributed to persistent cerebral hypoxia.,, Hijmans et al., reported 37 children with SCD, aged 6–18 years, and found a low level of hemoglobin that was used as indicator of disease severity, which was found to be associated with more neurocognitive difficulties, particularly with verbal short-term memory.
In this study, 45% of the patients had been retained in at least one school grade, and this is consistent with reported estimates of grade retention in 19.2–54% of children with SCD.,, When a child failed to make adequate academic progress in school, he or she repeated a grade, to give a chance to reinforce curriculum requirements and to overcome academic deficits. However, grade retention may be not effective in improving students’ learning; on the contrary, it has many negative consequences such as higher school dropout, inattentiveness, anxiety, and increased risk of academic failure.
In this study, school absence and grade retention were significantly correlated with age. This is in agreement with previous studies. The likelihood of increased school difficulties with age seems to be naturally expected, as getting older brings more opportunities to face new and harder experiences to achieve academic goals. Cognitive abilities have been suggested to decrease with age and mean IQ score has been reported to decrease with age. The number of hospital admissions and length of stay were found to increase with age, and a higher frequency of pain crisis was associated with older children.
As expected, relationship between school absence with both academic achievement and grade retention was demonstrated. Some researchers have reported such an association,, whereas others have not., Differences in results may be attributed to sample selection, method of assessment, and existence of educational support. However, children who had school difficulties in our study simply received low academic scores or were not promoted, and received no special educational interventional support, as this type of assistance is currently not available.
The relationship between SCD and school function is complex, and poor school performance may be an expression of wider problems of disease-related morbidities and psychosocioenvironmental factors, which are interrelated; the influence of each factor on educational outcomes is difficult to quantify. School absenteeism is expected to be responsible, in part, for poor school performance, and improved school attendance could enhance academic potential. May be one of the effective strategies to combat excessive school absenteeism is to pay all efforts to get these students back to school as soon as their health condition permits with special emphasis on comprehensive care of SCD. Greater impact is expected on children living in highly disadvantaged conditions, particularly in developing countries where disease management is far from optimal. School intervention program providing accurate information about SCD to children, parents, and teachers was found to decrease absence rate and increase consumer satisfaction.
This study has some of the following limitations: neuroimaging evaluation in SCD such as MRI and transcranial Doppler, which would have provided important information about association of brain damage with impairment of cognitive function, in particular silent cerebral infarction, are not currently available in our locality. Data were collected retrospectively, which could lead to variable degrees of recall bias. This might be randomly distributed between mild and severe cases, although recall may be better for more severe events. We do not have direct access to school transcripts and records and data reported by parents may not be completely accurate. This study focused on disease-related factors; however, the effect of other environmental factors such as parental education and socioeconomic status were not evaluated.
| Conclusions|| |
This study is one of the first to describe the potential impact of SCD severity on school performance in Arab children and adolescent students, using a comprehensive set of severity measures. Children with severe disease missed more school days, achieved lower academic score, and frequently repeated grades even more than those comparatively suffering from a mild disease. We concluded that for assessment of school absenteeism and academic achievement, it would be important to take disease severity in consideration, and measures to modify the impact of disease severity might be helpful in ameliorating academic performance and enhancing the overall educational progress of the affected children.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2]