Behavioral and Emotional Problems in Children with Sickle Cell Disease
Authors: Hijmans Channa , Grootenhuis Martha , Oosterlaan Jaap , van Oirschot Marit , Hendriks Carla , Heijboer Harriët , Peters Marjolein , Last Bob , Fijnvandraat Karin 
Published: April 2020

Abstract
Background: One of the most devastating complications of sickle cell disease (SCD) is cerebral infarction. Cerebral infarcts are present on MRI scans in one third of SCD patients at the age of 18 years, although most of them are not accompanied by focal neurological deficits. These so-called silent infarcts appear to be associated with diminished neurocognitive functioning. Besides the medical problems, most families with a child with SCD have to cope with financial and social problems, as the majority of these families belong to immigrant communities with a lower socio-economic status (SES) and many are single parented. The combined effects of neuropsychological dysfunction, stress caused by the disease and socio-demographic factors may lead to behavioral and emotional problems in children with SCD. When behavioral and emotional problems arise, it may be difficult for parents and professionals involved in the care of these patients to distinguish between the causes of these problems. The aim of this ongoing study is first to compare behavioral and emotional problems of children with SCD with a healthy Dutch norm population of children. Our second aim is to make a comparison with a group of healthy children, matched for ethnicity and SES.
Patients and Methods: The Child Behavior Checklist (CBCL) and the Teacher Report Form (TRF) were distributed among the parents and teachers of 124 children with SCD aged 6 to 18 years. The CBCL was also distributed among parents of 36 healthy siblings of children with SCD in the same age range. The TRF was distributed among 22 teachers of these siblings. These questionnaires provide scores on two broad-band scales: Internalizing (Anxious/Depressed, Withdrawn/Depressed and Somatic Complaints) and Externalizing (Rulebreaking Behavior and Aggressive Behavior) and a score on Total Problems. Problem scores above the 90th percentile represent the clinical range and indicate that a child needs professional help. First, the proportion of children with SCD with a score in the clinical range was compared to the proportion in the norm population. Second, we compared the group of children with SCD with a control group of healthy siblings with SCD. Differences in the proportion of children with a score in the clinical range between both groups were statistically tested using the Chi-square test.
Results: The CBCL was returned by parents of 80 children with SCD (response rate 65%). Of these children (48 boys, 32 girls, mean age 11,6 years, SD 3,5) 67 (84%) have homozygous SCD. The TRF was returned by 90 teachers of children with SCD (response rate 73%). Of these children (58 boys, 32 girls, mean age 11,5 years, SD 3,5) 66 (73%) have homozygous SCD. Parents of 30 healthy siblings returned the CBCL (response rate 83%). This group consisted of 13 boys and 17 girls, mean age 10,5 years, SD 3,2. The TRF was returned by 18 of their teachers (response rate 82%). This group consisted of 9 boys and 9 girls, mean age 10,3 years, SD 3,2. In Table 1 the proportion of behavioral and emotional problem scores in the clinical range on CBCL and TRF is given for children with SCD and healthy siblings.
Conclusions: These preliminary results indicate that children with SCD have more behavioral and emotional problems in the clinical range than the norm population. Parents and teachers report more internalizing problems. Teachers also report more externalizing problems. In comparison to healthy siblings, there is a trend that children with SCD have more internalizing problems, although this difference is not statistically significant (p&lt;0.10). The lack of statistical significance may be due to the small sample size of the control group. Alternatively, behavioral and emotional problems of children with SCD may truly be of similar magnitude as those in a group of healthy children that is matched for ethnicity and SES. When data collection is complete, more insight will be gained into this matter.
Table 1. Proportion of children with behavioral and emotional problem scores in the clinical range.
Children with SCD Healthy siblings n % 95%CI n % 95%CI Note: In the norm population 9% of the children has a score within the clinical range. * Trend at p&lt;0.10 for comparison between children with SCD and healthy siblings CBCL 80 30 Internalizing* 20 25 16–36 3 10 2–27 Externalizing 3 4 1–11 0 0 Total Problems 10 13 2–22 2 7 1–22 TRF 90 18 Internalizing 18 20 12–30 1 6 0–27 Externalizing 16 18 11–27 4 22 6–48 Total Problems 13 14 8–23 3 17 4–41