A study of psychosocial burden of multi-transfused thalassemia children in a tertiary care hospital in North Bengal

INTRODUCTION

Thalassemia is a hereditary condition characterized by impaired hemoglobin production. Though the disease is also common in Africa, the Middle East, and Asia, the term "thalassa" comes from the Greek phrase "the sea" because it was initially identified in communities around the Mediterranean Sea.¹ Thalassemia is a collection of conditions that can range from a blood anomaly that is hardly noticeable to severe or fatal anemia. Two alpha (α) and two beta (β) polypeptide chains make up adult hemoglobin. The hemoglobin alpha gene (HBA1 and HBA2) is found on chromosome 16 and is found in two copies. Both copies encode an α-chain. The β-chain is encoded by the hemoglobin beta gene (HBB), which is found on chromosome 11.

The production of β-chains is absent in β-thalassemia. Furthermore, the extra α-chains inside red blood cells form insoluble clumps. Severe anemia results from the early death of red blood cells and their progenitors caused by these aggregates. It is a persistent hemolytic anemia condition.

Previous research has indicated that the general prevalence of β-thalassemia in India is 3.4–4%, with an estimated 8,000–10,000 newborns having serious disease annually.² The prevalence of β-thalassemia varied from 0 to 10.5% among India's various ethnic groups, according to a big multi-centric study carried out in six distinct towns. The primary treatment for these kids is regular packed cell transfusions to keep their hemoglobin levels at the necessary level. Every three to four weeks, they need to be admitted to the hospital for a blood transfusion. These children are particularly vulnerable to behavioral and psychosocial issues such as opposition, passivity, anxiety, phobias, and depression for a variety of reasons.

These issues can undermine their self-esteem and cause feelings and thoughts that impair their quality of life and therapy compliance. Many of the thalassemic children experience fear related to intravenous line insertion and subcutaneous infusion pumps. Children with thalassemia in the pre-school and latency age groups are usually anxious and excessively dependent on their parents.

They display psychosomatic symptoms and are frequently absent from school. Thalassemic children have more of negative self-concept when compared to their normal counterparts.³ The aim of the study was to evaluate the co-morbid psychiatric disorders of children with thalassemia who require regular blood transfusions.

METHODS

Study duration

Necessary data for the study were collected from February 2020 to July 2021.

Study type and design

An observational, descriptive study with cross sectional design was conducted. Cross‑sectional design was preferred over prospective design due to logistic constraints as subjects visited once in 4–8 weeks and stayed only for a few hours, that is, for the duration of the blood transfusions.

Study place

The study was conducted in the Paediatric ward, Department of Paediatric Medicine, North Bengal Medical College and Hospital, Darjeeling West Bengal.

Study population

The study population consisted of beta thalassemia major patients admitted in paediatric ward aged between 6-12 years and fulfilling the inclusion criteria.

Inclusion criteria

Established diagnosis of thalassemia, age between 6-12 years, children having received regular blood transfusions for more than one year. Absence of any other current or previous chronic physical disease. Both parents alive and are living together.

Exclusion criteria

Children with any other chronic physical disease. Children with impaired intellectual behavior. Children with associated neurodevelopmental disorder were excluded from the study.

Sample size

A total of 60 samples have been included in this study.

Statistical analysis

For statistical analysis, data were initially entered into a Microsoft Excel spreadsheet and then analyzed using SPSS (version 27.0; SPSS Inc., Chicago, IL, USA) and GraphPad Prism (version 5). Numerical variables were summarized using means and standard deviations, while categorical variables were described with counts and percentages.

Two-sample t-tests, which compare the means of independent or unpaired samples, were used to assess differences between groups. Paired t-tests, which account for the correlation between paired observations, offer greater power than unpaired tests. Chi-square tests (χ² tests) were employed to evaluate hypotheses where the sampling distribution of the test statistic follows a chi-squared distribution under the null hypothesis; Pearson's chi-squared test is often referred to simply as the chi-squared test.

For comparisons of unpaired proportions, either the chi-square test or Fisher’s exact test was used, depending on the context. To perform t-tests, the relevant formulae for test statistics, which either exactly follow or closely approximate a t-distribution under the null hypothesis, were applied, with specific degrees of freedom indicated for each test. P values were determined from Student's t-distribution tables. A p value ≤0.05 was considered statistically significant, leading to the rejection of the null hypothesis in favour of the alternative hypothesis.

RESULTS

The distribution of CBCL internalization problem scores among the children were 28.3% above 63 and 21.7% in between 60-63; whereas, 50% of the children were having score of less than 60. The distribution of CBCL externalization problem scores among the children were 23.3% above 63 and 20% in between 60-63; whereas, 56.9% of the children were having score of less than 60.

The distribution of CBCL externalization problem scores among the children were 24.1% above 63 and 20% in between 60-63; whereas, 56.7% of the children were having score of less than 60. The distribution of CBCL total problem scores among the children were 23.3% above 63 and 15% in between 60-63; whereas, 61.7% of the children were having score of less than 60. The proportion of children with CBCL total problem scores of more than 63 was more among ≥9 years of age, Girls and from lower middle and lower socio-economic status. The sex of the subjects and socio-economic status were statistically significantly associated with CBCL Total problem scores of the children.

DISCUSSION

Early-life internalization and externalization difficulties consistently exhibit persistence of these issues in later life if treatment is not received. The child's mental health is impacted by the behavioral issues. While aggressive behavior, worry, tension, restlessness, and low self-esteem are all symptoms of mental health issues, good behavior is a sign of good mental health. A child's learning, social, and cognitive development are all directly impacted by their mental health. Here, we talked about the study's results in relation to another pertinent research. The majority (61.7%) of the children in the current study were in the 9–10 age range, with a mean (SD) age of 9.23 (±1.26) years. Males made up 56.7% of the study participants, while girls made up 43.3%, and the majority of the kids (50%) came from lower socioeconomic backgrounds. The children in the study by Kumar et al had an average age of nine years. The majority of 48 (48%) were from low socioeconomic backgrounds, which supports the results of our study.⁴

Similar findings were made by Hongally et al's study, which indicated that 56% of the participants were children. The majority of the study group, 58% of whom were from low socioeconomic backgrounds, had children with an average age of 7 years.⁵

The present study observing the clinically significant abnormal CBCL Internalization problem scores show that, among the children 28.3% above 63 and 21.7% in between 60-63; whereas, 50% of the children were having score of less than 60. Kumar et al reported that the clinically significant abnormal CBCL Internalization problem scores were seen in 41% children, indicating symptoms of social withdrawal, somatic complaints, and anxiety/depression. Hongally et al reported that the clinically significant abnormal CBCL Internalization problem scores were seen in 34% children. CBCL externalization problem scores among the children were 24.1% above 63 and 20% in between 60-63; whereas, 56.7% of the children were having score of less than 60. According to Aydin B et al., a total of 24% of the patients had a mental diagnosis, which included tic disorder, major depression, anxiety disorder, and nocturnal enuresis.⁶

In this present study, we observed the CBCL total problem scores among the children were 23.3% above 63 and15% in between 60-63; whereas. And the mean (SD) and median (Inter quartile range) of the CBCL total problem score is 61.35 (10.46) and 61 (52, 72). We found lesser but very appropriately significant children with CBCL total problem scores of more than 63 mostly among children above ≥9 years of age, among Girls and children with lower middle and lower socio-economic status. The sex of the subjects and socio-economic status were statistically significantly associated with CBCL total problem scores of the children.

Studies over the past 25 years have shown that prevalence of behavioral disorders among thalassemic children ranged from 23 to 80%, and these psychological disturbances adversely affect compliance to treatment in thalassemia.⁷

Study by Valen et al in Turkey had also revealed that older age (>12 years), higher education of mothers and poor school performance were associated with higher risk of behavioral problems.⁸ A study in South Turkey by Canatan et al reported 31% of thalassemic children had anxiety disorders and 60% had poor school performance.⁹

According to Khurana et al, thalassemic children's psychological issues as children were comparable to those of other chronic medical disorders but had not been identified or addressed.⁹ Using CBCL, we attempted to determine whether multitransfused thalassemic youngsters, particularly those in the pre-adolescent age range, had behavioral issues.

CONCLUSION

On the basis of salient findings of the study it can be concluded that the present study found that prevalence of high CBCL total score (behavioural problems) among the multi-transfused thalassaemic children was significant. Behavioral problems associated were more commonly seen among girls with of nine years of age and amongst children belonging to lower middle and lower socio-economic status. The gender of the subjects and socio-economic status were statistically significantly associated with CBCL total problem scores.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: The study was approved by the Institutional Ethics Committee

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