key: cord-0918702-dtxikcvn
authors: Raithatha, Dhvani; Sahu, Jitendra Kumar; Bhanudeep, Singanamalla; Saini, Lokesh; Prinja, Shankar; Bharti, Bhavneet; Madaan, Priyanka; Rana, Saroj Kumar; Negi, Sandeep
title: Financial Burden in Families of Children with West Syndrome
date: 2021-05-26
journal: Indian J Pediatr
DOI: 10.1007/s12098-021-03761-1
sha: 493c6f98ac9825cd579e0fec10d84989f8cdc31f
doc_id: 918702
cord_uid: dtxikcvn

OBJECTIVE: To evaluate the illness-related expenditure by families of children with West syndrome (WS) during the first year of illness and to explore the potential determinants of the financial drain. METHODS: This cross-sectional study was conducted at a tertiary care hospital between July 2018 and June 2020. Eighty-five children with WS who presented within one year from the onset of epileptic spasms were included. The details of the treatment costs (direct medical and nonmedical) incurred during the first year from the onset of epileptic spasms were noted from a parental interview and case record review. Unit cost was fixed for drugs and specific services. Total cost was estimated by multiplying the unit cost by the number of times a drug or service was availed. The determinants of the financial burden were also explored. RESULTS: The median monthly per-capita income of the enrolled families (n = 85) was INR 3000 (Q1, Q3, 2000, 6000). The median cost of treatment over one year was INR 27035 (Q1, Q3, 17,894, 39,591). Median direct medical and nonmedical expenses amounted to INR 18802 (Q1, Q3, 12,179, 25,580) and INR 6550 (Q1, Q3, 3500, 15,000), respectively. Seven families had catastrophic healthcare expenditure. Parental education and choice of first-line treatment were important determinants driving healthcare expenses. The age at onset of epileptic spasms, etiology, treatment lag, the initial response to treatment, and relapse following initial response did not significantly influence the illness-related expenditure by the families. CONCLUSION: WS imposes a substantial financial burden on the families and indirectly on the healthcare system. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12098-021-03761-1.

West syndrome (WS) is characterized by clustered epileptic spasms in infancy and hypsarrhythmia on electroencephalogram (EEG) [1] . It is the most common type of infantile-onset developmental and epileptic encephalopathy. A recent study from the United Kingdom suggested that the incidence of WS is 6.7 (95% CI 1.2-5.7)/10,000 live births [2] . It is expected to be higher in developing countries with a higher incidence of perinatally acquired brain injuries, e.g., hypoxic-ischemic and hypoglycemic brain injury [3] . These children often have comorbidities, including developmental delay, locomotor, visual, and hearing impairment necessitating multidisciplinary care for holistic management. The first-line treatment options include adrenocorticotropic hormone (ACTH), oral steroids, and vigabatrin [4] . The short-term management goals are enduring cessation of epileptic spasms and resolution of hypsarrhythmia, which are achieved in nearly one-third of patients after first-line therapy [5] . The long-term neurodevelopmental outcome is often poor and depends on the initial therapeutic response [6] . Unlike the developed countries, the management of WS in India and developing countries has its own intricacies. These include a long treatment lag, preponderance of structural etiology, lack of awareness, and out-of-pocket expenditure for healthcare utilization [5, 7] . Besides, two of the three first-line therapeutic options (ACTH and vigabatrin) are quite expensive, with ACTH often requiring hospitalization for the initiation and monitoring of adverse effects. Vigabatrin is not readily available in India. Considering the comorbidities, cost of drugs, and long-term treatment, illness-expenditure might be catastrophic for a lowincome family. The financial burden borne by the families having children with WS has not been systematically studied in India.

The financial burden is usually quantified in terms of direct, indirect, and intangible costs due to a specific disease [8] . The direct costs can be classified into medical costs (related to prevention, diagnosis, treatment, and rehabilitation) and nonmedical costs (related to travel for hospital visits, food and accommodation, etc.). The indirect costs are estimated in terms of loss of earnings, time, and attributed value of the lost household work. Furthermore, the intangible costs include stress, suffering, or anxiety due to the illness or its treatment [9] . The majority of the studies in adults with epilepsy have focussed primarily on the direct costs because of the inherent difficulty in computing the indirect and intangible costs [8, 9] . WS poses a distinct challenge because of infantile-onset, costly investigations and treatment, and comorbidities management. A lack of universal health insurance in India further contributes to the financial burden handled by the families. Therefore, the present study was performed to evaluate financial spending (direct costs) during the first year of illness in children with WS. Besides, an attempt was made to explore the possible determinants of the financial drain.

This cross-sectional, descriptive, observational study was conducted between July 2018 and June 2020 in the Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, a tertiary-care government children hospital in North India. The study was approved by the Institute Ethics Committee. Children with WS (epileptic spasms and hypsarrhythmia in EEG) attending outdoor clinics of the Pediatric Neurology unit were screened. All consecutive children who presented within one year from the onset of epileptic spasms were considered eligible for inclusion. Detailed informed consent was obtained from the parents at the time of enrollment. Those who declined consent were excluded from the study.

The parents/guardians of the enrolled children were interviewed for the sociodemographic aspects and details of the treatment costs incurred. The clinical and investigational details were retrieved from the medical records. The data on demographic characteristics, age at onset of epileptic spasms, presence of any specific perinatal insult, parental educational status, monthly family income, delay in seeking treatment, diagnosis, and the start of appropriate first-line treatment were collected. It was noted whether the children were beneficiaries under the poor free scheme for below poverty line (BPL) families. The criterion for inclusion in the BPL facility was a provision of yellow ration cards to the families by the Government of India. It was also noted whether infants were benefitted from Janani Shishu Suraksha Karyakram (JSSK).

The costs incurred in a year were determined by interviewing the caregivers. The bottom-up approach of cost analysis was used wherein, every single area of expenditure was calculated. Costs were categorized as: a) direct medical costs including the cost of outpatient consultation, hospital admission, drugs, investigations, and ancillary services; b) direct nonmedical costs involving an expenditure of travel to and from the health setup, the cost of food, and accommodation while being stationed at the hospital with the child (two caregivers per patient).

Unit cost (under the direct medical costs) was fixed for each commodity based on the price at the index institute (Supplementary Table 1 ). Total cost was calculated by multiplying the unit cost by the number of times a service or product was availed. Direct nonmedical costs were noted at first admission (or at first contact for those who were never admitted) at the index institute, and it was assumed that the pattern of expenditure on these components remained the same throughout the year. Parents were interviewed at the first visit and asked to keep records of the purchases being made for drugs and investigations. They were followed-up 3-monthly to gather data of expenditure made in the past 3 mo. The expenses made on drugs and investigations were crosschecked from the medical records. Children belonging to BPL families and under the JSSK scheme were exempted from expenses made on investigations and hospital admission charges. The expenditure was calculated in Indian rupee (INR) and the United States dollar (USD) as on August 8, 2020. Figure 1 shows an outline of potential cost-driving factors considered during the collection of data. Annual direct medical and nonmedical costs were calculated, and their relationship with sociodemographic and disease-specific variables was analyzed.

Statistical Analysis Descriptive statistics like mean, median, interquartile range (IQR), and 95% confidence interval were used for various heads of total expenses and their determinants. The sign test was employed to study the significance of the median value of total cost among its determinants. All calculations were done with p value level of ≤ 0.05 as statistically significance. The analysis was done by the Statistical Package for the Social Sciences (SPSS) 22·0 version (IBM Corporation, USA).

A total of 138 children were initially enrolled. However, there was significant attrition of 53 children (38%) due to follow-up difficulties and inability to provide accurate details on financial expenditure incurred over one year. Hence, 85 children The median age at onset of epileptic spasms was 7.5 (IQR 5-11) mo. A structural etiology was observed in 71 (83.5%) children, whilst 14 (16.5%) had presumed genetic etiology. Structural causes were as follows: hypoxic-ischemic brain injury (n = 34, 48%), neonatal hypoglycemic brain injury (n = 18, 25%), postmeningitic hydrocephalus (n = 5, 7%), intracranial hemorrhage sequelae (n = 4, 6%) and others (n = 10, 14.0%). Of 14 children with presumed genetic etiology, two were tested with clinical exome sequencing. Constipation (n = 18, 21.1%), visual impairment (n = 13, 15.2%), gastroesophageal reflux (n = 6, 7.0%), hearing impairment (n = 5, 5.8%), hemiparesis (n = 3, 3.5%), infantile tremor syndrome (n = 2, 2.3%), congenital heart disease (CHD; n = 2, 2.3%) and hypothyroidism (n = 1, 1.2%) were notable comorbidities apart from global developmental delay. Three children had Down syndrome (one had comorbid CHD and hypothyroidism, and one had CHD alone). The median diagnostic lag was 7 wk (Q1, Q3, 3, 16 wk), and the median treatment lag was 10 wk (Q1, Q3, 4.7, 18.2 wk). Initial therapeutic choices were ACTH (n = 58, 68.2%), prednisolone (n = 20, 23.5%), and vigabatrin (n = 7, 8.2%). Of 85 children, 49 received only one of the first-line drugs. Crossover of first-line drugs was seen in 36 (42.3%) children. Of 58 children with initial ACTH therapy, 4 subsequently received high-dose prednisolone, 11 vigabatrin, and 6 a combination of prednisolone and vigabatrin. Of 20 children with initial high-dose oral prednisolone therapy, 3 were subsequently received ACTH, 6 vigabatrin, and 1 a combination of ACTH and vigabatrin. Of seven with initial vigabatrin therapy, 3 subsequently received ACTH, and 2 oral prednisolone. Complete response, as defined by enduring cessation of epileptic spasms for 4 wk, to any of the first-line therapies was seen in 40% of children (n = 34). Of those with complete response, 20.5% (n = 7) had a relapse requiring additional antiseizure medications. Relapse was defined by a recurrence of epileptic spasms after achieving a complete initial response.

The Seven patients in the study had catastrophic healthcare expenditure (CHE), i.e., healthcare expenditure was more than 30% of total family income during the study period. depict the distribution of expenditure made over 1 y from the onset of epileptic spasms Fig. 2 .

The families coming from nearby states of Punjab, Haryana, and Chandigarh spent significantly less than those coming from far-off states ( Table 2 ). There was no significant difference in the median expenditure based on the gender of the child, locality of residence (rural versus urban), and occupation of parents. However, the cost borne by parents with higher educational status was significantly more. Looking at the disease-specific parameters, age at onset, etiology, the initial response to treatment, and relapse following initial response did not significantly influence the costs borne by the families ( Table 3 ). The cost of treatment was slightly lower in those with a longer treatment lag. Children initiated on expensive drugs like ACTH and vigabatrin had higher medical costs as compared with those who started on prednisolone. It was seen that choice of primary therapy (ACTH vs. steroids) was driven by the affordability of the families.

With India's public health expenditure being less than 2% of GDP [10], it is important to evaluate the out-of-pocket expenditure and understand the distribution of costs for healthcare utilization for different chronic disorders. WS, often associated with multiple comorbidities, merits attention in this regard. This will not only assist in the identification of cost-effective management strategies but also guide administrative authorities in reducing the burden on families by recognizing important issues related to licensing and availability of drugs, and increasing government aid and expenditure on healthcare infrastructure.

The current study evaluated the expenditure on children with WS from diverse economic backgrounds and different states. The median age at onset of epileptic spasms in the present study population and a preponderance of the male gender was in agreement with the reported literature from India [6] . The most common etiology was structural, with adverse perinatal insults such as hypoxic-ischemic encephalopathy, neonatal hypoglycemia, and neonatal meningitis being the predominant causes. This was concordant with previously published literature [5, 6, [11] [12] [13] . The median diagnostic and treatment lag were significant and were comparable to that reported in different Indian studies [5, 6, [11] [12] [13] .

The median direct medical cost in the first year of illness exceeded the direct nonmedical costs. Like other studies evaluating the costs involved in the management of epilepsy and refractory epilepsy syndromes, the cost of medications formed the major bulk of direct medical as well as total costs [14, 15] . Hence, the choice of first-line therapy significantly affected the expenses borne by the family. The choice of first-line therapy, in turn, is affected by several factors such as affordability, the feasibility of daily injections, and monitoring, parental understanding and beliefs. Expensive first-line therapy (ACTH) had been used mostly by families with higher income. Despite receiving the cheapest first-line option, families [16, 17] . This strategy can also prove beneficial from an economic point of view, especially for developing countries, including India. With the marked contraction of the Indian economy due to the pandemic containment measures, using oral steroids as the preferred first-line option may reduce the initial financial burden on families of affected children during this crisis. Regardless of the implementation of JSSK since 2014, many families in this study did not access free transport to public health institutions. Hence, there is a need to facilitate patient transport services under JSSK once the halted transport services commence.

Although the number of girls in this cohort was less, the fact that parents of girl-child spent equally on treatment as parents of boys was reassuring. The expenses borne by families were associated with parental education. This might be due to the relatively higher income of these families and due importance of health for educated parents. However, the expenses were not affected by the occupation, which is also an important determinant of income. Government assistance under the BPL scheme protected poor families from CHE and further impoverishment. It was interesting to note that none of the disease-related factors, except the choice of first-line therapy, significantly affected the total costs incurred.

Like the present study, the studies on cost-of-illness evaluation in various chronic childhood disorders like epilepsies, leukemia, and thalassemia have also concluded that the direct medical costs exceed the nonmedical costs [14, 15, [18] [19] [20] . Cost of drugs has been reported as the predominant part of the direct medical cost, which is again in concordance with previous studies [14, 15, [18] [19] [20] .

The present study assessing the financial spending on WS treatment is the first of its kind from the Indian subcontinent. Besides the epilepsy syndrome, the expenditure made on the treatment of comorbidities including cerebral palsy, constipation, visits made to ophthalmology and otorhinolaryngology specialists for assessment, and follow-up of disabilities was also taken into account because these comorbidities are an indispensable part of the care of such children. Unit cost was fixed for drugs and most services, thereby reducing the recall bias.

However, the present study is a single-center study. The subsidized rates from a public setup hospital may not apply to the hospitals in the private sector, which cater to India's significant healthcare needs [21] . The sample size was limited because of which, many potential factors affecting financial burden could not be assessed. The treatment expenditure was relatively lower in those with a long treatment lag, as expenditure assessment was performed until 1 y from the onset of 

The present study provides a realistic magnitude of financial burden due to WS in the first year of illness. WS imposes substantial financial burden on the families and indirectly on the health system. The selection of ACTH therapy requiring initial inpatient care was a significant cost determinant. Further studies are warranted to quantify the indirect costs arising from the parents' loss of workdays, government expenditure, etc. Furthermore, cost-effectiveness analysis for different therapies, especially ACTH and oral steroids, may further help in identifying cost-effective strategies.

The online version contains supplementary material available at https://doi.org/10.1007/s12098-021-03761-1.

Authors' Contribution DR: Study design, data acquisition, data interpretation, data analysis, prepared the initial draft of the manuscript and subsequent revision. SB, PM, SKR and SN: Data interpretation, data analysis, manuscript writing and final approval of the manuscript. LS, BB, and SP: Study design, interpretation of the data, data analysis and final approval of the manuscript. JKS: Concept, design, interpretation of the data, critical revision for intellectual content, and final approval of the manuscript. All authors approved the final version of the manuscript.

Conflict of Interest None

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