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Burden of care in families of patients with early onset scoliosis

Campbell, Megana; Matsumoto, Hirokob; St. Hilaire, Triciac; Roye, Benjamin D.b; Roye, David P.b; Vitale, Michael G.b

Author Information
Journal of Pediatric Orthopaedics B: November 2020 - Volume 29 - Issue 6 - p 567-571
doi: 10.1097/BPB.0000000000000711
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Abstract

Introduction

Children with early onset scoliosis (EOS) suffer from progressive three-dimensional spinal deformity as a result of variable etiologies. Though this population is heterogeneous, many patients have multiple complex medical concerns associated with their diagnoses which may or may not be related to their spinal deformity [1]. These include but are not limited to pulmonary impairments, skeletal dysplasias, developmental delays, and neurologic sequelae. Often such ailments require many medical and surgical interventions, iterative trips to specialists, and sometimes critical care.

Currently, literature exists which examines the health-related quality of life (HRQoL) of these patients; however, the degree to which this disease impacts the caregivers of these patients has yet to be documented. Though caregivers have long been used as proxies to estimate the status of patients in pediatric studies, investigations into the perspective of caregivers themselves occur far less frequently. In fact, few studies have explored the experience of caregivers of children with scoliosis [2–8]. Most of these focus on experiences following treatment, mainly post-operative caregiver experience for specific diagnoses, and none of these use EOSQ-24 as a measure of HRQoL. However, careful examination of the burden imposed upon caregivers of patients with EOS is important in order to understand the holistic effects of this disease and of clinical intervention in order to attempt to better address the disease course and those caring for patients with this disease. The results of this study will contextualize how we approach treatment for these patients.

This study examined the burden on caregivers of children with varying etiologies of EOS in comparison to age-matched healthy peers.

Materials and methods

Design and setting

This is a multicenter retrospective cohort study using data collected from the Children’s Spine Study Group (CSSG). The registry receives national data contribution from many hospitals, with 11 sites contributing data for this study before beginning their prescribed intervention from January 2012 to November 2015. IRB Approval/Research Ethics Committee: This work is approved by the Institutional Review Boards at all sites, including Columbia University.

Study participants

The CSSG registry was queried for data on patients with an EOS diagnosis and a completed EOSQ-24 Patients with prior operative treatment were excluded from the study. Patients were divided into groups by their prescribed treatment – observation, conservative treatment such as bracing or casting, and surgery – as well as by the etiology of their scoliosis: idiopathic etiology and non-idiopathic etiology, including congenital, syndromic, and neuromuscular etiologies.

Intervention

Patients with EOS were compared to national norms, comprised of otherwise healthy patients without EOS.

Outcome

Caregiver and financial burden (FB) were assessed using the EOSQ-24 [9]. Completed pre-treatment EOSQ-24 responses to questions 17–22 of the questionnaire were used to assess burden. These domains ask caregivers to reflect on the previous 4 weeks and answer the following questions regarding their child:

  • 17. How often have you felt anxious/nervous about his/her health condition?
  • 18. How often has your child’s health condition interfered with family activities?
  • 19. How much has your child’s health condition affected your energy level?
  • 20. How often have you missed or have you been late for work or social events due to your child’s health condition?
  • 21. Have you been able to spend enough time with your family/partner/spouse despite your child’s health condition?
  • 22. How much of a FB has your child’s diagnosis of Early Onset Scoliosis been?

Based on published instruction, caregiver burden (CB) and FB sub-domains were calculated (questions 17–21 and question 22, respectively) [9]. Higher scores are indicative of a ‘better’ HRQoL and lesser burden. Published EOSQ-24 scores for those respective sub-domains were identified from age-matched healthy norms.

Data analysis

Comparisons of EOSQ-24 mean scores between pre-treatment and aged-matched norms were conducted using t-tests. Data were stratified based on etiology (idiopathic and non-idiopathic) and compared using independent t-tests.

A power analysis was conducted to confirm statistical power to detect the minimal clinically important difference of outcomes – mean differences of 10 in EOSQ-24 scores between aged-matched norms and patients at pre-treatment. The post-hoc power analysis revealed that on the basis of the mean difference of 10 and standard deviations of 15 and at 5% significance level, effect measure of 10 could be detected with above 80% power when there are more than 35 patients in each group. Thus some subgroup analyses or analyses of follow-up assessments did not have sufficient power.

Results

Patient characteristics

Results of the multicenter registry query returned 503 patients eligible for participation in the study. Of these patients, 53.5% were male (269) and 46.5% were female (234). The mean age at EOSQ-24 completion was 5.6 ± 3.7 years, but varied from 4.3 ± 3.4 years (casting/bracing) to 6.3 ± 3.1 years (surgery) depending on treatment plan. One hundred eighty-five patients had EOS of idiopathic etiology (36.8%) and 318 (63.2%) had EOS of a non-idiopathic etiology, including 175 with congenital (34.8%), 99 with neuromuscular (19.7%), and 44 with syndromic (8.7%) etiologies. When reviewed after their respective treatments, 310 (61.6%) went on to be observed, 118 (23.5%) were eventually casted or braced, and 75 (14.9%) had surgery.

Comparison of burden to age-matched peers

Pre-treatment EOSQ-24 data was available for all 503 patients. Overall, CB and FB domains of EOQ-24 completed by caregivers of patients with EOS resulted in significantly lower scores than those domain scores completed by caregivers of age-matched healthy peers. Caregivers of patients with EOS had average CB scores of 75.9 ± 23.7 (compared to average normative scores of 91.6 ± 4.7, P < 0.001) and average FB scores of 81.9 ± 25.9 (compared to average normative scores of 97.5 ± 2.2, P < 0.001).

Comparison of burden between etiologies

Burdens reported by caregivers of patients with EOS differ by etiology (Table 1). While caregivers of both idiopathic and non-idiopathic patients report significantly lower scores of CB and FB compared to caregivers of healthy children, caregivers of idiopathic patients report burdens merely 5% and 10% greater than caregivers of healthy peers (CB and FB, respectively, P < 0.001). This difference, while significant, falls short of the minimal clinically important difference for EOSQ-24 scores of 10%. In contrast, caregivers of non-idiopathic patients reported burdens 22% and 19% greater than caregivers of healthy children (CB and FB, respectively, P < 0.001). Caregivers of neuromuscular patients experienced the greatest burdens of the non-idiopathic subtypes, reporting burdens 38 and 25% greater than normative age-matched scores for CB and FB, respectively (Table 1).

Table 1 - Average caregiver burden and financial burden EOSQ-24 domain scores reported by caregivers of patients with early onset scoliosis compared to those scores reported by caregivers of healthy age-matched peers
Caregiver burden Norms % difference P value Financial burden Norms % difference P value
Idiopathic 86.0 91.2 5.1 <0.01 87.4 97.2 9.8 <0.01
Non-Idiopathic 70.0 92.1 22.1a <0.01 78.7 97.6 19.0a <0.01
Congenital 79.8 91.4 11.6a <0.01 83.3 97.3 14.0a <0.01
Neuromuscular 55.6 93.2 37.6a <0.01 73.6 98.2 24.5a <0.01
Syndromic 63.8 92.5 287.7a <0.01 71.0 98.0 27.0a <0.01
aGreater than the calculated minimal clinically significant difference between EOSQ-24 domain scores.

Comparison of burden between treatment types

Patients who went on to be treated nonoperatively exhibited least reported burden (Table 2). These patients had mean domain scores of 81.3 and 80.9 as compared to 90.4 (9% difference, P < 0.001) and 97.1 (16% difference, P < 0.001) for caregiver and FB, respectively. Patients who eventually underwent surgical intervention reported the lowest scores, with means of 60.2 and 71.6, respectively. This is compared to mean age-matched norm scores of 92.8 and 98.1 (35 and 27% difference, both P < 0.001). Those patients who would eventually be treated only by observation still scored lower than norms, with mean scores of 77.9 and 84.8, compared to age-matched norm scores of 92 and 97.4, respectively (P < 0.001). Burdens reported by caregivers of all EOS patients were significantly greater than those reported by caregivers of age-matched peers by ≥10% in all pre-treatment subgroups (P < 0.001).

Table 2 - Patient characteristics as stratified by treatment group
Observation (N = 310) Casting/bracing (N = 118) Surgery (N = 75) P value
Age (years) 6.0 ± 3.8 4.3 ± 3.4 6.3 ± 3.1 <0.001a
Sex
 Male 152 (49.0%) 43 (36.4%) 39 (52.0%) 0.04a
 Female 158 (51.0%) 75 (63.6%) 36 (48.0%)
Etiology
 Idiopathic 95 (30.6%) 85 (72.1%) 5 (6.7%) <0.001a
 Non-idiopathic 215 (69.4%) 33 (27.9%) 70 (93.3%)
  Congenital 142 (45.8%) 11 (9.2%) 22 (29.3%) <0.001a
  Neuromuscular 45 (14.5%) 14 (11.9%) 40 (53.3%)
  Syndromic 28 (9.1%) 8 (6.8%) 8 (10.7%)
aIndicates statistically significant values P = 0.05.

Caregivers of non-idiopathic patients reported experiencing greater burden than caregivers of age-matched peers by ≥16% on average, regardless of eventual treatment (P < 0.001) (Table 3). Non-idiopathic patients destined for surgery scored the worst, averaging CB and FB scores 37 and 27% lower than their peers. Casting/bracing-bound and observed non-idiopathic patients also exhibited higher caregiver and FB, with casting/bracing patients reporting burdens 18 and 23% greater than norms and observation patients reporting burdens 21 and 16% greater than norms, respectively (all P < 0.001).

Table 3 - Pre-treatment EOSQ-24 scores for caregiver burden and financial burden
Observation
All pts Norms Idiopathic Norms Non-idiopathic Norms Idio vs. non
Caregiver burden 77.9 92 88.9 92.2 72.9 92
95% confidence interval 75.781 91.592.6 86.4–91.3 91.2–93.1 70.1–77.1 91.3–92.7
% difference 15 4 21 18
P value <0.001 0.016 <0.001
Financial burden 84.8 97.4 91.7 97.4 81.7 97.5
95% confidence interval 82.187.7 97.297.7 88.5–94.8 96.9–97.8 78.2–85.5 97.2–97.8
% difference 13 6 16 11
P value <0.001 0.001 <0.001
Casting/bracing
All Pts Norms Idiopathic Norms Non-idiopathic Norms Idio vs. Non
Caregiver burden 81.3 90.4 83.8 89.9 74.7 91.4
95% confidence interval 78.2–84.5 89.6–91.2 80.7–87.3 89–90.9 67.6–81.9 90–92.8
% difference 10 7 18 11
P value <0.001 <0.001 <0.001
Financial burden 80.9 97.1 83.2 96.8 75 97.9
95% confidence interval 76.1–85.6 96.8–97.5 78.1–88.3 96.4–97.3 64.1–85.9 97.2–98.5
% difference 17 14 23 10
P value <0.001 <0.001 <0.001
Surgery
All Pts Norms Idiopathic Norms Non-idiopathic Norms Idio vs. Non
Caregiver burden 60.2 92.8 69 94.2 58.9 92.8
95% confidence interval 54.2–66.3 92–93.6 40.8–97.2 90.6–97.8 53.3–65.9 91.9–93.5
% difference 35 27 37 15
P value <0.001 0.059 <0.001
Financial burden 71.6 98.1 75 99.4 71.3 98
95% confidence interval 64.5–78.7 97.6–98.5 44–100 97.9–100 63.8–78.8 97.5–98.5
% difference 27 25 27 5
P value <0.001 0.094 <0.001
Italicized values indicate statistical significance P = 0.05.

However, caregiver and FB for idiopathic EOS patients eventually treated by observation or casting/bracing differed no more than 14% from healthy peers’ burden. Scores for idiopathic patients going to be observed were similar to national norms, differing only by 4 and 6% for caregiver and FB, respectively, as compared to their peers (P < 0.01). For patients going onto casting/bracing, FB scores were 14% worse than norms (P < 0.001), while CB scores were 7% lower (P < 0.001). Pre-surgical patients experienced the greatest difference from their peers among the idiopathic patients, having 25% lower mean FB scores and 27% lower mean CB scores than norms (P = 0.094 and 0.059, respectively); however, these differences did not reach statistical significance as a result of the small number of idiopathic patients who warranted surgical treatment (5).

Discussion

To our knowledge, this is the largest study to investigate burden of care of parents with EOS. This study demonstrated that caregivers of children with EOS carry greater personal and FBs than caregivers of age-matched healthy peers. Because these burdens are assessed before any treatment, it is safe to assume that the disease itself has an impact on caregivers despite the burden imposed later by individual management/treatment types. Because the onset of progressive spinal deformity at a young age distorts both the spine and the thoracic cavity, growing lungs are increasingly affected as the deformity gets larger. Pulmonary decline is a common consequence, which takes a toll on exercise fatigue and HRQoL, and when untreated is the source of significant morbidity and mortality. In addition, progressive spinal curvature can affect coronal and sagittal balance leading to difficulty with ambulation, balance, sitting, and other functional abilities as well as self-perception and appearance during a vulnerable age range. Any and or all of these factors may contribute to a caregiver’s burden when caring for a patient facing these issues, especially when caregivers are often more aware of the situation than patients themselves.

This study demonstrated that caregivers of children with non-idiopathic etiologies of EOS had greater caregiver and FBs compared to caregivers of idiopathic patients. Idiopathic EOS patients are often healthy aside from their scoliosis, whereas children with non-idiopathic EOS more commonly have underlying diagnoses associated with concurrent medical concerns [1,10–12]. The significantly higher burden reported by these caregivers may reflect the cumulative stress and increasing cost incurred by the total effect of the patient’s medical condition [13–22]. This could be a result of consulting with multiple medical and surgical specialists, travel burden and cost associated with spinal deformity care, a higher propensity for ER visits for pulmonary problems or other medical needs, and a higher degree of dependency exhibited in patients with chronic illness such as many of those with EOS.

Interestingly, this study demonstrated that caregivers of patients who eventually required surgery reported a higher burden of care even before the treatment was initiated compared to caregivers of patients who went on to be observed or later received non-operative. By the time a treatment assessment was made, caregivers of patients who were indicated to undergo future surgery were already at increased level of burden. This likely is a reflection of an increased level of severity in these children. Though surgical intervention for patients with EOS is sometimes inevitable as spinal curves progress, indications are generally a question of timing which take into account a combination of the consideration of spinal curve characteristics, age, etiology, likelihood of progression, functional capabilities, co-existing comorbidities, and other patient-specific factors [10,12,23–32]. Larger spinal curvature is associated with losing ambulation, sitting and increased severity of comorbidities including decline of pulmonary function [12,16,33,34]. It is possible that these all impact the caring for these involved children that their parents and caregivers provide daily.

Limitations of this study include the small number of patients in some subgroups resulting in some underpowered analyses. This study also offers no insight into the burden inflicted by treatment of EOS in general or the burden associated with any particular treatment. Further work into this area with larger, better-powered studies and longitudinal follow-up may yield additional valuable insight into the best mechanisms for the management of EOS while maintaining an optimal HRQoL. However, we believe the insight provided by this study into the disease-specific burden of EOS is valuable to enlighten surgeons, primary care providers, and other practitioners as well as validate patients and families.

In summary, this study paints a broad picture of the significantly greater caregiver and FB imposed on parents and caregivers of patients with EOS than the burden placed on caregivers of their healthy peers. Specifically, it indicates the particular challenges faced by parents of children with non-idiopathic EOS etiologies, and highlights the even greater burden on those caregivers of non-idiopathic patients who eventually require surgery. This research can serve to highlight the need for additional interventions for caregiver support which further research may help identify adjunct resources which could indirectly benefit these patients by helping their caregivers, done by either further interview work or by examination of these patients’ needs and outcomes. In addition to informing parents and providers, this work serves as a framework from which further research can build to better quantify changes in burden as it is affected by different treatments.

Acknowledgements

This work was conducted with the support of a grant from the Children’s Spine Foundation.

Conflicts of interest

There are no conflicts of interest.

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