DG Taşkın, A Gülseren

Abstract

Purpose: In our study, we aimed to measure the quality of life (QoL) of patients and parents using the PedsQL questionnaire in our celiac disease (CD) group. Methods: The study was prospectively designed between 1/06/2020 and 01/09/2020. Paediatric Quality of Life Inventory 4.0 scales for children and parents were used as the QoL scales. Findings: Children diagnosed in the first two years and their parents were more adversely affected in terms of physical, social, and psychosocial health. There was also a weak positive correlation between body mass index at the last follow-up and parental emotional functional score and total scale score (TSS). The Total Physical Health Score, Social Functioning Score and TSS of the parents of children with constipation were lower. There was a strong correlation between children's and parents' responses to the same questions regarding School Functioning Score, Total Psychosocial Health Score, and TSS. Conclusion: CD has a serious impact on the QoL of children and parents.

Introduction

Celiac disease (CD) is a chronic enteropathy with a variety of presentation patterns that manifests after gluten ingestion in genetically susceptible individuals.¹ The prevalence of the disease in paediatric patients varies between 1/300 and 1/80 in Europe and America; however, it is approximately 1% worldwide.² As awareness of this disease increases, its incidence is on the rise. The treatment for this disease is a lifelong gluten-free diet.³ CD presents with a wide range of multisystemic manifestations, such as malnutrition, growth retardation, precocious puberty, osteoporosis, and particularly villus destruction triggered by gluten intake. It is also common for CD patients to suffer from neuropsychiatric symptoms, such as perception impairments, irritability, behavioural problems, anxiety, depression, and fatigue.⁴ Furthermore, CD patients are adversely affected psychologically, physically, and socially by lifelong dieting, in addition to all these complications.⁵ Numerous studies have shown that children with chronic diseases have a decreased Quality of life (QoL) because of chronic disease.^6.7 QoL is a multidimensional state of physical, emotional, mental, and social well-being perceived due to the impact of the illness, its complications and treatment.⁸ The measurement of health-related QoL (HRQoL) is becoming increasingly important, particularly among chronically ill individuals. Children in particular are affected by the disease itself, as well as disease-related factors and many family-related factors. The assessment of QoL in children is, however, challenging.⁹

In Turkey, numerous surveys have been conducted regarding the measurement of QoL related to CD, but no comparison has been made with a healthy control group. Patients and their parents with lifelong diet therapy were administered the Paediatric Quality of Life Inventory (PedsQL) inventory in our study to measure their QoL.

Methods

Our study was prospectively designed in Paediatric Gastroenterology Outpatient Clinic of Adana City Hospital between 1.06.2020 and 01.09.2020. Totally 142 patients between 8-18 years (all had motor and mental competence to answer the survey questions) diagnosed with CD and 50 children as a control group (in the same age as the patient group and had no chronic disease) and their parents were included.¹⁰ The PedsQL inventory was administered to the children in both groups to measure QoL, and the results were compared. The same questionnaire was also administered to the parents of the children in both groups to assess the children's QoL from the parent's perspective. Children with psychiatric disorders or severe neurological findings and their parents were excluded from the study. For children and parents whose native language was not Turkish, the questionnaire was translated into their native language by an interpreter. The questionnaire was administered only to the mothers of the children to reduce differences between parents. Written and verbal informed consent was obtained from the parents.

Ethical approval was obtained from Adana City Hospital (date: 20.05.2020, number: 867/57) Ethic Committee. We adhered to the Declaration of Helsinki principles.

The Paediatric Quality of Life Inventory (PedsQL)
The PedsQL is a general QoL scale developed by Varni et al¹¹ in 1999 and can be used in children aged 2-18 years with chronic illness. In the Turkish version of the PedsQL 4.0, the child and parent scales include the same questions for ages 8-12 and 13-18. The reliability and validation study of the Turkish version of the questionnaire was conducted in 2007.¹² It consists of 23 items and 4 subscales, 8 of which question functionality in physical health score, 5 in emotional functioning score, 5 in social functioning score, and 5 in school functioning score, total physical health score, total psychosocial health score, and total scale score are used in the scale as determined by the World Health Organization. PedsQL 4.0 includes a self-administered child questionnaire for children aged 5-18 and a parent questionnaire for families of children aged 2-18.

Parents and children participating in the study were asked to indicate how much of a problem mentioned in the items had been in the last month. The questionnaire is scored on a 5-point Likert scale, with 0=never, 1=rarely, 2=sometimes, 3=often, and 4=always. The scoring of the items is reversed: 100 points if the answer is marked as never, 75 points if it is marked as rarely, 50 points if it is marked as sometimes, 25 points if it is marked as often, and 0 points if it is marked as almost always. The scores are summed and divided by the number of items completed to obtain a total score, with a median score between 0 and 100,11 and high total score means better HRQoL.¹³

The scale has separate forms for parents and children, with separate scales of 5-7 years, 8-12 years, and 13-18 years for children and separate scales for 2-4 years, 5-7 years, 8-12 years and 13-18 years for parents. All forms, including the parent forms, have exactly the same items. Since this inventory only has Turkish versions for the 8-12 and 13-18 age groups and validity and reliability studies were conducted only in these age groups, patients in this age group were included in our study.

In the statistical analysis categorical variables were expressed as numbers and percentages, whereas continuous variables were summarised as the mean and standard deviation and median and inter quantile range when appropriate. We compared categorical variables between groups by using Chi-square test and the comparison of continuous variables between two groups were analysed by using Student's t test or Mann‒Whitney U test. The normality of distribution for continuous variables was confirmed with the Shapiro‒Wilk test. For, depending on whether the statistical hypotheses were fulfilled. For the comparison of more than two groups, one-way ANOVA or the Kruskal‒Wallis test was used depending on whether the statistical hypotheses were fulfilled. For normally distributed data, Tukey or Games & Howell tests were used for multiple comparisons of groups with respect to homogeneity of variances. For non-normally distributed data, the Bonferroni-adjusted Mann‒Whitney U test was used for multiple comparisons between groups. The Pearson correlation coefficient or Spearman rank correlation coefficient was used to evaluate correlations between measurements. SPSS Statistics version 20.0 (SPSS reference: IBM Corp. Released 2011. IBM SPSS Statistics for Windows, version 20.0. Armonk, NY: IBM Corp.) were performed in the analyses. The statistical significance level was set at 0.05.

Results

A total of 142 patients with CD (138 from Turkey, 4 from other ethnicities) were included in the study. The demographic data of the patients are presented in Table 1.

The children diagnosed in the first two years of life were found to be significantly impaired in physical, social, and psychosocial health. Parents of children diagnosed in the first two years of life were also more affected than other age groups, although the results were not significant. There was no difference between scores in the first year after diagnosis and scores one year after diagnosis. There was also no gender difference. There was no difference for parents from same patients. Regarding ethnicity, there was no difference between the patients and their parents. The scores of the patients and their parents were not correlated with the presence of growth retardation at the time of initial diagnosis. There was a weak positive correlation (r=0.178, p=0.047 for total physical health score, r=0.178, p=0.046 for total scale score) between patients' weight at the last follow-up and parental total physical health score and total scale score. Parental total physical health score and total scale score decreased in patients with weight gain at the last follow-up, and the correlation was positive, albeit weakly, influenced. There was also a weak positive correlation between Body Mass İndex (BMI) at the last follow-up and parental emotional functioning score and total scale score (r=0.176, p=0.050 for emotional functioning score and r=0.193, p=0.031 for total scale score). The parental emotional functioning score and total scale score of patients with increased BMI in the control group decreased, and the correlation was positively affected, although weakly. Detailed analysis of complaints found that the total physical health score, social functioning score, total psychosocial health score, and total scale score of parents of children who complained only of constipation were lower. However, a similar relationship was not found in patients with diarrhoea. Children with comorbidities associated with celiac disease had lower social functioning score. Children who had a comorbidity in addition to CD were found to be more socially impaired in Table 2.

There was a positive correlation between the responses of children and parents to the same questions. The correlation was found to be strong, especially in terms of school functioning score, total psychosocial health score, and total scale score in Table 3.

When the data of both groups were compared, all scores between the answers given by the children and the answers given by the parents were significantly different in Table 4.

Discussion

In our study, children diagnosed in the first two years of life were found to be significantly impaired in terms of their physical, social, and psychosocial health. Parents of children diagnosed in the first two years of life were also more affected than other age groups, although the results were not significant.

As reported in the literature,¹⁴ age at diagnosis, duration of diagnosis, clinical presentation, and dietary adherence have an impact on QoL. In a study, in contrast to our study,⁸ showed that the younger age at diagnosis (<2 years) had better QoL than patients diagnosed later (more than 8 years ago) in life. The fact that the QoL was more impaired in patients diagnosed at 2 years of age in our study was an important finding, but those patients are small (n=5, 4%) in numbers. We suggested that we should investigate this question with a larger number of patients.

Although some studies have found a difference between genders,6 there are also publications in the literature showing that there is no difference between the scores of males and females and between the parental scores of the same patients when evaluated by gender, which is consistent with our study.⁸ Overall, females are expected to be more emotionally affected. However, this difference is generally more pronounced during the post-adolescent period. The lack of a gender difference in our study may be attributed to the fact that nearly half of the patients are children aged 12 years or younger. No difference was found between patients and their parents in terms of scale scores when ethnicity was evaluated. In our patient group, we did not found any association between QoL and dietary compliance. Likewise, QoL in patients with autoimmune hepatitis under treatment was measured to be worse than that in the general population despite remission shows how much the treatment and diet decrease the QoL, even if the disease is under treatment.¹⁵ On the other hand, social and economic problems such as not being able to eat out can develop with the introduction of a gluten-free diet, which in turn affects the QoL differently.¹⁶ The same study also showed that 63% of children with CD stay away from social activities, 54% stay away from eating out most of the time, and 28% have difficulty in finding gluten-free products.¹⁷

There is no difference between the scores when newly diagnosed (with the first year of diagnosis) and those after it are analysed. Some studies found no correlation between disease duration and QoL.¹⁸ In the study by Zarkadas et al, it was found that the QoL score was decrease in females and newly diagnosed CDs.¹⁹ In the study by Casthilos et al,²⁰ when patients diagnosed within the first 1 year after diagnosis and for more than 4 years were compared, but there is no statistical significance in QoL. However, in the same study, when patients with a diagnosis of more than 10 years and who were on a diet were evaluated, the QoL was found to be much better in these patients. It has been suggested that QoL may increase with adaptation to the diet over time. There are also studies in the literature in which no relationship was found between disease occurrence and QoL.¹⁵ However, in some studies, unlike my work, the QoL increases with the time elapsed after diagnosis (after 8 years).⁸

There was a weak positive correlation (r=0.178, p=0.047 for the total physical health score, r=0.178, p=0.046 for the total scale score) between the weight of the patients at the last follow-up and the parental total physical health score and total scale score. Parental total physical health score and total scale score decreased in patients with weight gain at the last follow-up, and the correlation was positive, albeit weakly, affected. There was also a weak positive correlation between BMI at the last follow-up visit and parental emotional functioning score and total scale score (r=0.176, p=0.050 for emotional functioning score and r=0.193, p=0.031 for total scale score). Parental emotional functioning score and total scale score of patients with increased BMI in the control were decreased, and the correlation was positively affected, albeit weak.

Similarly, in our patient group, there was no difference in the questionnaire evaluations of patients who had complaints or were diagnosed incidentally without complaints, in line with the literature.^8,21 However, some studies have shown that patients presenting with severe complaints have a considerably poor QoL, which improves with diet, whereas the improvement in QoL in patients without complaints is not as significant.²² In studies conducted according to existing complaints, the PCS (physical health composite scale score) is lower when compared to other complaints in patients with complaints such as diarrhoea, mouth sores, and weakness. In the same study, the MCS (mental health composite scale score) was found to be lower in patients with complaints such as dyspepsia, mood changes, and depression than in patients with other complaints.²³ In the study, the relationship was found between the number of complaints and the QoL was found to be lower in patients with more than 6 complaints.²⁴ The lack of correlation between complaints and QoL assessment in our study may be because a questionnaire was conducted on patients who were started on diet therapy during the course of the disease. In the study by Fuchs et al, it was shown that a delay of 3 years in the diagnosis of patients affected the QoL very much.²⁵

When the complaints were analysed in detail, the total physical health score, social functioning score, total psychosocial health score, and total scale score of the parents of children with only constipation complaints were found to be lower. However, a similar relationship was not found in patients presenting with diarrhoea. In studies conducted in different chronic patient groups in the literature, Gulati et al²⁶ showed that the most important complaint in patients with autoimmune hepatitis is abdominal pain. In the survey conducted on these patients, it was found that the physical, social, and school success and total scores of the patients presenting with abdominal pain were more affected than those of the patients without abdominal pain. However, it has been shown that disease severity does not affect patient and parent scores. This suggests that different symptoms specific to the disease affect the QoL differently.

Children with comorbidities accompanying CD had lower social functioning score. It was found that children with comorbidities in addition to CD were more negatively affected socially.

There was a positive correlation between the responses of children and parents to the same questions. A correlation was found to be strong, especially in terms of school functioning score, total psychosocial health score, and total scale score. While there are recent studies that showed a significant correlation between child and parent total scores,²⁷ in some studies conducted in different regions, the perceived QoL assessment of their children by the parents was much worse than the children themselves thought.²⁸ The PedsQL questionnaire by Bozzini et al²⁹ was administered to different chronic disease groups, and the physical, emotional, school, and total scores of the child's self-perception were found to be significantly lower than the control group, as in patients with OİH (p<0.05). However, in this study, only the physical and total scores were significantly lower (p<0.05). As a result of the surveys conducted on many chronic diseases, QoL assessment of their children from the perspective of the parents is worse than self-perception of children in the QoL assessments between parents and children.³⁰ The scores between the answers given by the children and the answers given by the parents were significantly different when the data from the patient and control groups were compared. We note the significant differences between the control and patient groups to demonstrate how much chronic diseases impact the QoL of individuals. In QoL studies, especially in celiac patients, it has been shown that the total scale is significantly lower in CD than controls.6 There are numerous QoL studies in the literature on CD.^20,31 According to studies, improving QoL is the most important factor in achieving compliance with treatment. Unfortunately, fewer than 25% of paediatricians assess the impact of QoL.³²

There was a positive correlation between the responses of children and parents to the same questions. A correlation was found to be strong, especially in terms of school functioning score, total psychosocial health score, and total scale score. While there are recent studies that showed a significant correlation between child and parent total scores,²⁷ in some studies conducted in different regions, the perceived QoL assessment of their children by the parents was much worse than the children themselves thought.²⁸ The PedsQL questionnaire by Bozzini et al²⁹ was administered to different chronic disease groups, and the physical, emotional, school, and total scores of the child's self-perception were found to be significantly lower than the control group, as in patients with OİH (p<0.05). However, in this study, only the physical and total scores were significantly lower (p<0.05). As a result of the surveys conducted on many chronic diseases, QoL assessment of their children from the perspective of the parents is worse than self-perception of children in the QoL assessments between parents and children.30 The scores between the answers given by the children and the answers given by the parents were significantly different when the data from the patient and control groups were compared. We note the significant differences between the control and patient groups to demonstrate how much chronic diseases impact the QoL of individuals. In QoL studies, especially in celiac patients, it has been shown that the total scale is significantly lower in CD than controls.⁶ There are numerous QoL studies in the literature on CD.^20,31 According to studies, improving QoL is the most important factor in achieving compliance with treatment. Unfortunately, fewer than 25% of paediatricians assess the impact of QoL.³²

Considering that my study included patients from all segments of society with different ethnic identities, economic statuses, and socioeconomic statuses and compared them with the control group, our study was highly significant. Our study had some limitations. The limitations include the inability to examine in detail factors related to family life, such as divorce, education level, and maternal age, that impact QoL.

In conclusion, CD is a disease that seriously affects the QoL and their families. As part of our study, we identified factors affecting the QoL in CD and suggested that being aware of these factors might have a positive effect on QoL for patients during the treatment. It is particularly interesting to note that we have seen how important it is for parents to play a part in this process, as well as how they evaluate the QoL that their child is experiencing for themselves, providing an important step in the treatment process. Additionally, the assessment of the parent's QoL, similar to that of the patient, may prevent the future deterioration of their QoL. There is a need for further studies to evaluate the factors affecting patients' QoL with chronic diseases and to also consider the involvement of more patients as well as their parents in these studies.

Declaration of Interest

The authors declare that they have no conflict of interest.

Funding

The authors did not receive any financial support from any public or private source. The authors have no financial or proprietary interest in a product, method, or material described here.

Acknowledgements

None

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