A longitudinal examination of health-related quality of life in children and adolescents with spina bifida.

Murray CB, Holmbeck GN, Ros AM, Flores DM, Mir SA, Varni JW. (2015) A longitudinal examination of health-related quality of life in children and adolescents with spina bifida.

J Pediatr Psychol. 2015 May;40(4):419-30. doi: 10.1093/jpepsy/jsu098

Abstract

OBJECTIVE:

The current study examined (1) spina bifida (SB) youths' health-related quality of life (HRQOL) compared with nonclinical and chronic health condition (CHC) samples, (2) parent-child agreement regarding HRQOL, and (3) prospective changes in HRQOL. 

METHODS:

Child and parent-proxy reports of Pediatric Quality of Life were collected at two time waves (Time 1: N = 134, ages 8-15 years; Time 2: N = 109, ages 10-17 years) as part of a larger longitudinal study. 

RESULTS:

SB youth had statistically and clinically reduced physical HRQOL compared with the nonclinical and CHC samples at both time points. There were significant discrepancies between youth and parent-proxy reports of HRQOL; youth reported higher levels of physical and social HRQOL than parents. The majority of parent- and child-reported HRQOL domains remained stable, yet youth-reported social HRQOL increased over time. 

CONCLUSIONS:

Youth with SB are at risk for poor HRQOL. Examining modifiable condition and social-environmental predictors of youth HRQOL will be important in informing future interventions.
© The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningocele.

Buffart LM, Berg-Emons RJ, Meeteren JV, Stam HJ, Roebroeck ME. (2009) Lifestyle, participation, and health-related quality of life in adolescents and young adults with myelomeningocele. Developmental Medicine and Child Neurology. 2009 Mar 20.

Department of Rehabilitation Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.

This study aimed to describe participation and health-related quality of life (HRQoL) in adolescents and young adults with myelomeningocele and to explore their relationships with lifestyle-related factors. Fifty-one individuals with a mean age of 21 years 1 month (SD 4y 6mo) years participated (26 males, 25 females; 82% hydrocephalus, 55% wheelchair-dependent). Participation was assessed using the Life Habits Questionnaire, and HRQoL was assessed using the Medical Outcomes Study 36-item Short-form Health Survey. Physical activity was measured using an accelerometry-based activity monitor, fitness (peak oxygen uptake) was measured during a maximal exercise test, and the sum of four skin-folds was assessed to indicate body fat. Relationships were studied using logistic regression analyses. Of the participants, 63% had difficulties in daily activities and 59% in social roles. Participants perceived lower physical HRQoL than a Dutch reference population. Participants with higher levels of physical activity and fitness had fewer difficulties in participating in daily activities (odds ratio [OR]=8.8, p=0.02 and OR=29.7, p=0.02 respectively) and a higher physical HRQoL (OR=4.8, p=0.02 and OR=30.2, p=0.006 respectively), but not mental HRQoL. Body fat was not related to participation or HRQoL. In conclusion, a large proportion of individuals with myelomeningocele had difficulties in participation and perceived low physical HRQoL. Higher levels of physical activity and fitness were related to fewer difficulties in participation and higher physical HRQoL.

PMID: 19416327

Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele.

Danielsson AJ, Bartonek A, Levey E, McHale K, Sponseller P, Saraste H.Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele. Journal of Children's Orthopedics. 2008 Feb;2(1):45-54.

Department of Orthopaedics, Sahlgrenska University Hospital, 413 45, Göteborg, Sweden, danielsson.aina@telia.com.

PURPOSE: Modern principles for treatment of patients with myelomeningocele include early closure of the neural tube defect, neurosurgical treatment of hydrocephalus and treatment aimed at minimizing contractures and joint dislocations. The aim is to achieve a better survival rate and a better quality of life (QOL). Better ambulatory function is thought to improve the management of activities of daily living. This study focused on evaluating which factors might affect ambulation, function and health-related QOL in children with myelomeningocele.

METHODS: Thirty-eight patients with neurological deficit from myelomeningocele were examined in an unbiased follow-up. This included a physical examination using validated methods for ambulatory function and neuromuscular status, chart reviews and evaluation of radiographs in terms of hip dislocation and spine deformity. The Pediatric Evaluation of Disability Inventory (PEDI) was used to measure mobility, self-care and social function, and the Child Health Questionnaire (CHQ-PF50) was used to measure QOL.

RESULTS: Muscle function class, quadriceps strength, spasticity in hip and/or knee joint muscles and hip flexion contracture as well as the ambulatory level all affected functional mobility as well as self-care/PEDI. Patients with hip dislocation, spinal deformity or those who were mentally retarded also had significantly worse functional mobility. Besides being affected by the severity of the neurological lesion, self-care/PEDI was significantly impaired by hip flexion contracture and absence of functional ambulation. General health-related QOL was significantly lower in this patient group than for US norms. Nonambulatory and mentally retarded patients had a significantly lower physical function of their QOL (CHQ).

CONCLUSIONS: The severity of the disease, i.e. reduced muscle strength and occurrence of spasticity around hip/knee, affected ambulation, functional mobility and self-care. Acquired deformities (hip dislocation and spine deformity) affected functional ambulation only. Patients with reduced functional mobility and self-care experienced lower physical QOL. Children with myelomeningocele had significantly reduced QOL compared to healthy individuals.

PMID: 19308602

Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida

Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.

Department of Urology, University of California-Davis, Davis Children's Hospital, California, USA.

PURPOSE: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers. MATERIALS AND METHODS: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks. RESULTS: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all 7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the reliability of all FIC QOL questions. CONCLUSIONS: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.

PMID: 18721959

Life satisfaction of young adults with spina bifida

Barf HA, Post MW, Verhoef M, Jennekens-Schinkel A, Gooskens RH, Prevo AJ. Life satisfaction of young adults with spina bifida. Developmental Medicine and Child Neurology. 2007 Jun;49(6):458-63.

This study concerns life satisfaction and its determinants in Dutch young adults with spina bifida (SB). Data on life satisfaction (Life Satisfaction Questionnaire [LiSat-9]) were related to hydrocephalus, lesion level, disabilities, and demographic variables. In total, 179 young adults with SB participated (41% male, age range 16-25y; 79% SB aperta, 67% hydrocephalus [HC], 39% wheelchair-dependent). Most were satisfied with their life as a whole (24% dissatisfied). No difference was found from a population reference group (28% dissatisfied). Highest proportions of dissatisfaction were found for financial situation (44%), partnership relations (49%), and sex life (55%). Least dissatisfaction was found for contact with friends (17%) and families (15%). Young adults with SB and HC were more satisfied with their financial situation and family life but were less satisfied with self-care ability and partnership relations than those without HC and the reference group. However, except for self-care ability, relationships between life satisfaction and having SB were weak. In conclusion, self-care ability and partnership relations were rated least favourable and may need more attention from care providers. Overall, SB does not seem to be an important determinant of life satisfaction.

PMID: 17518933

Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edit

Dodson JL, Furth SL, Hsiao CJ, Diener-West M, Levey EB, Wu AW, Gearhart JP. Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edition.
Journal of Urology. 2008 Oct;180(4 Suppl):1846-51; discussion 1851.

PURPOSE: We studied the impact of abnormal bladder function due to congenital urological disorders on health related quality of life in children. A reliable patient based method is needed to assess the impact of these conditions in children and the interventions used to treat them.

MATERIALS AND METHODS: Participants 11 to 17 years old with bladder exstrophy-epispadias complex, spina bifida or other causes of abnormal bladder function self-administered the Child Health and Illness Profile-Adolescent Edition, a generic health related quality of life instrument. They also responded to questions about incontinence, catheterization status and bother level. Mean scores on the profile were compared to population based norms.

RESULTS: Mean age of the 50 participants was 14.9 years, 62% were male and 82% were white. Diagnoses included bladder exstrophy-epispadias complex in 37 patients, spina bifida in 10 and other in 3. The mean +/- SD score on the disorders domain of 14.2 +/- 6.3 was significantly worse than the population norm of 20. Mean scores on the satisfaction, discomfort, resilience, risks and achievement domains were comparable to or better than the population based norm of 20. A total of 29 participants reported incontinence and 31 performed catheterization.

CONCLUSIONS: In this study of adolescents with congenital causes of abnormal bladder function Child Health and Illness Profile-Adolescent Edition generic health related quality of life scores were significantly worse in the disorders domain but largely comparable to or better than those of the general population in other domains. This suggests that the profile may discern between adolescents with structural urological disease and norms but it may not be sensitive enough to fully detect the impact of the condition. Alternatively adolescents may adapt well to the challenges of urological disease.



Key Words: urinary bladder, quality of life, questionnaires, abnormalities, adolescent

PMID: 18721969

Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida

Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.

Purpose: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most
disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers.

Materials and Methods: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks.

Results: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all
7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the
reliability of all FIC QOL questions.

Conclusions: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.

Key Words: abnormalities, spinal dysraphism, quality of life, questionnaires, fecal incontinence

PMID: 18721959

Prospective, longitudinal evaluation of health related quality of life in the pediatric spina bifida population undergoing reconstructive urological s

Parekh AD, Trusler LA, Pietsch JB, Byrne DW, DeMarco RT, Pope JC 4th, Adams MC, Deshpande JK, Brock JW 3rd. Prospective, longitudinal evaluation of health related quality of life in the pediatric spina bifida population undergoing reconstructive urological surgery. Journal of Urology. 2006 Oct;176(4 Pt 2):1878-82.

General Clinical Research Center, Vanderbilt Children's Hospital, 2200 Children's Way, Nashville, TN 37232, USA.

PURPOSE: Spina bifida, the most frequent permanently debilitating birth defect, results in major urological problems of voluntary bladder control and bowel function, which may impair quality of life. We prospectively assessed quality of life in patients with spina bifida using child and parent reports simultaneously. This study had 3 goals, that is to 1) document baseline health related quality of life in patients with spina bifida preoperatively, 2) study health related quality of life, reporting differences between parents and children, and 3) study changes in health related quality of life prospectively at preoperative and postoperative intervals.

MATERIALS AND METHODS: Patients with spina bifida who were 2 to 18 years old and required reconstructive urological surgery in 2004 were included in the study. Demographic survey and the validated PedsQL 4.0 health related quality of life questionnaire were used preoperatively and postoperatively. A clinical outcomes data set was completed after the clinician saw the patient. PedsQL 4.0 subscales were scored using the algorithms provided.

RESULTS: The response rate was 100%. Mean participant age was 10.3 years. Preoperatively child physical and psychosocial health and school functioning were significantly higher than parent reports (p <0.001). Overall health related quality of life in patients with spina bifida was lower than in healthy children (62.4 vs 85, p <0.001). Six weeks postoperatively significant differences in health related quality of life reporting between parents and children had lowered. Six months postoperatively child emotional and social functioning scores were higher than parent scores (p <0.001). No correlation was found between health related quality of life, and clinical and demographic factors due to insufficient sample size.

CONCLUSIONS: Children with spina bifida recorded higher health related quality of life scores than parents/guardians. This health related quality of life study addresses concerns that impact daily quality of life in patients with spina bifida. Future health related quality of life studies in patients with spina bifida should use child self-reports.

PMID: 16945679

An assessment of health related quality of life

Dodson JL, Diener-West M, Gerson AC, Kaskel FJ, Furth SL. An assessment of health related quality of life using the child health and illness profile-adolescent edition in adolescents with chronic kidney disease due to underlying urological disorders. Journal of Urology. 2007 Aug;178(2):660-5; discussion 665. Epub 2007 Jun 15.

PURPOSE: We examined health related quality of life in adolescents with congenital urological disease causing kidney disease using a generic health related quality of life instrument. We then compared the results to those in adolescents with medical kidney disease and to population based norms.

MATERIALS AND METHODS: The Child Health and Illness Profile-Adolescent Edition was administered to 113 patients 10 to 18 years old with chronic kidney disease. Mean domain and subdomain scores for adolescents with urological disease were compared to those of adolescents with medical kidney disease and to population based norms.

RESULTS: The cohort included adolescents with an underlying diagnosis of congenital urological anomaly (37 patients) or other causes of kidney disease (76). Compared to adolescents with kidney disease caused by other factors, those with congenital urological disease scored statistically significantly better in the Child Health and Illness Profile-Adolescent Edition subdomain of Limitations of Activity (mean 22.3 [SD 2.5] vs 20.4 [SD 5.0], p = 0.04). Compared to population norms, adolescents with congenital disorders scored lower in the Disorders domain (mean 16.5, 95% CI 14.2 to 18.9) but better in the Risks domain (mean 25.9, 95% CI 25.1 to 26.6) and in the Home Safety and Health subdomain (mean 25.2, 95% CI 23.7 to 26.6).

CONCLUSIONS: As assessed by the Child Health and Illness Profile-Adolescent Edition generic health status questionnaire, adolescents with kidney disease due to underlying congenital urological disease had fewer limitations of activity compared to those with underlying medical kidney disease. Except for low scores in the Disorders domain, children with underlying urological disease did not have significant impairments in any other domain compared to population based norms.

PMID: 17574620

Impact of spinal cord dysfunction and obesity on the health-related quality of life of children and adolescents.

Abresch RT, McDonald DA, Widman LM, McGinnis K, Hickey KJ. Impact of spinal cord dysfunction and obesity on the health-related quality of life of children and adolescents. Journal of Spinal Cord Medicine. 2007;30 Suppl 1:S112-8.

OBJECTIVES: The objectives of this study were: (1) to compare the health-related quality of life (HRQOL) of children and adolescents with mobility impairments due to spinal cord injury (SCI) and spina bifida (SB) to the HRQOL of children and adolescent controls without mobility impairments (CTRL); and (2) to examine the impact of of obesity on the HRQOL of these subjects.

METHODS: The Pediatric Quality of Life Inventory (PedsQL) was administered to 42 SB, 71 SCI and 60 able-bodied subjects who were 8-20 years of age. Subjects were categorized as obese if their BMI exceeded the 95th percentile for age. Twenty-one CTRL, 26 SB and 26 SCI subjects were obese.

RESULTS: The SCI and SB subjects had significantly lower subscores than the control subjects on the physical (p < 0.001), emotional (p < .01), social (p < .001), and school (p < .001) domains of the PedsQL. The obese (CTRL) group had lower subscores on the physical (p < 0.001), social (p < 0.001), and psychosocial (p < 0.001) domains of the PedsQL as compared to the non-obese CTRL group, while there were no significant differences in subscores from the emotional and school domains. In contrast to the subjects without mobility impairment, there were no significant differences between the sub-scores of the obese and non-obese subjects with spinal cord dysfunction secondary to SCI or SB. The mean total PedsQL score of the non-obese control group (87.7 +/- 2.1) was significantly higher than the obese control group (75.2 +/- 3.4, p < 0.02), which in turn was significantly higher than the SCI group (63.7 +/- 2.2, p < 0.02), and the SB group (63.0 +/- 2.2, p < 0.02).

CONCLUSION: Patients with SCI and SB have significantly lower HRQOL than children and adolescents without mobility impairments. Whereas obesity significantly reduces the quality of life scores of adolescents without mobility impairments, it has no significant incremental effect on subjects with SCI or SB.

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