Quality of Life in Children with Spina Bifida: A Cross-Sectional Evaluation of 102 Patients and their Parents

Zegers SHJ^1*, Houterman S², Uiterwaal CSPM³, Winkler-Seinstra PLH⁴, Kimpen JLL⁵ and de Jong-de Vos van Steenwijk CCE⁶

¹Pediatrician, Department of Pediatrics, Máxima Medical Center, Veldhoven, Netherlands

²Epidemiologist, Catharina Hospital, Eindhoven, Netherlands

³Clinical Epidemiologist, Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Netherlands

⁴Pediatric Researcher, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Netherlands

⁵Professor in Pediatrics, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Netherlands

⁶Pediatrician, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Netherlands

*Corresponding Author:

Zegers SHJ
Máxima Medical Center
Department of Pediatrics
P.O. Box 7777, 5500 MB
Veldhoven, Netherlands
Tel: 0031 40 8888270
Fax: 0031 40 8889609
E-mail: b.zegers@mmc.nl

Received Date: June 01, 2015; Accepted Date: July 21, 2015; Published Date: July 28, 2015

Citation: Zegers SHJ, Houterman S, Uiterwaal CSPM, Winkler-Seinstra PLH, Kimpen JLL, et al. (2015) Quality of Life in Children with Spina Bifida: A Cross-Sectional Evaluation of 102 Patients and their Parents. J Child Adolesc Behav 3:225. doi: 10.4172/2375-4494.1000225

Copyright: © 2015 Zegers SHJ, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Abstract

Background: In the so-called SPIN UTI study on 176 patients with spina bifida and clean intermittent cathererization (CIC) we have studied the influence of antibiotic prophylaxis (AP) on the number of yearly urinary tract infections (UTIs) and bacterial resistance to commonly used antibiotics. We have proven that stopping AP increased the number of yearly UTIs only slightly and clinically irrelevant patients would have to take AP for two years to prevent one extra non-febrile, non-scarring UTI, We have also shown that bacterial resistance improved over time when AP was stopped. In this article we study the influence of the recommended discontinuation of AP on Health Related Quality of Life (HRQL) in spina bifida. Methods: Participating children aged 4-16 years and their parents were asked to fill out the validated KINDL-R questionnaire, combined with specific spina bifida questions. Scores in study participants were compared with healthy controls. Also, the impact of AP, gender, level of spinal lesion, hydrocephalus, methods of catheterization and defecation, mobility and level of schooling on HRQL was evaluated. Results: One hundred and two of the 133 eligible children (77%) filled out the KINDL-R. Patients and their parents had significantly lower HRQL scores than healthy controls (p<0.001). Parents had significantly lower scores than their children, especially in the psychological domains. Severity of co-morbidity was not associated with differences in HRQL. There were no differences between children who continued and stopped AP. Conclusions: HRQL scores are lower in spina bifida patients compared to healthy children. Parents have even lower scores, especially in the psychological domains. Stopping antibiotic prophylaxis does not adversely affect HRQL, and should therefore be pursued to improve bacterial resistance patterns in spina bifida patients.

Keywords

Health related quality of life; Spina bifida; Antibiotic prophylaxis

Introduction

Despite periconceptional administration of folic acid and prenatal ultrasound screening, spina bifida is the second most common congenital birth defect [1]. Due to genetic and environmental factors, prevalence varies throughout the world, with approximately one in every two thousand live births in the Western World [2]. After the innate postnatal surgical morbidity, there is a lifelong burden of neurological, urological and orthopedic consequences and severely disabling emotional and behavioral problems [3-6].

In many studies the Health Related Quality of Life (HRQL) was assessed in children with spina bifida [3,6-12]. A wide variety of HRQL questionnaires has been developed, addressing the psychological and physiological aspects of various domains of everyday life activities, either with or without specific spina bifida related questions. Most studies show that parental expectancy, patient mobility and urinary continence are the most important factors to predict HRQL in patients with spina bifida [6,9,11].

We recently conducted the so-called SPIN UTI randomized controlled trial to assess whether stopping antibiotic prophylaxis (AP) in 176 patients with spina bifida on clean intermittent catheterization (CIC) influenced the number of urinary tract infections (UTIs) [13]. Stopping prophylaxis increased the number of yearly UTIs only slightly.Patients would have to take prophylaxis for two years to prevent one extra non-febrile, non-scarring UTI. Therefore it was concluded that most spina bifida patients on CIC can safely discontinue AP. Bacterial resistance is common in every day AP use (REF). In our study on bacterial resistance patterns in continuing and stopping AP, bacterial susceptibility for commonly used antibiotics improved when AP was stopped. Discontinuation of AP should therefore be pursued to improve bacterial susceptibility for antibiotics as long as this has no negative effect on the number of UTIs and perceived HRQL in spina bifida patients on CIC. Our aim in this study is to investigate the HRQL in our cohort of 133 eligible children, and whether stopping AP either increased HRQL due to less daily medication or decreased HRQL due to loss of perceived antibiotic protection.

Method

Study population

From 2005 through 2008, 176 patients with spina bifida on CIC were included in the SPIN UTI study and randomized to either continue or stop daily AP; primary outcome was the number of urinary tract infections over an 18 month study period. Details and results of the study are described elsewhere [13]. Out of the 176 participants, 133 were between age 4 and 16 years old and therefore eligible for the HRQL sub-study.

Procedure

At the end of the study period, the 133 eligible patients and their parents were asked to fill out the validated KINDL-R questionnaire [12]. KINDL-R consists of two separate lists: one list with general questions on six domains (physical well-being, emotional well-being, self-esteem, family, friends and school), each domain containing four specific questions, and one chronic disease module with extra questions for the parents. We also added the KINDL-R list of 37 specific spina bifida related questions for parents [12]. The first two lists were validated for both chronic disease and children with spina bifida, with 1501 healthy German primary school children as historical controls. The added specific spina bifida questionnaire has not been validated yet. The results were compared with the results of previous studies of HRQL observations in both healthy controls and spina bifida children and their parents [5,11,14,15]. The answers to the questions were calculated into a combined total HRQL score and scores per domain.

Demographic data and current functional status

To determine the influence of gender and severity of disability in the participating patients, gender, level of spina bifida lesion, manner of micturation and defecation, mobility and level of schooling were also evaluated, using regression analysis.

Statistical analysis

First, general characteristics of the patients were tabulated by intervention (stop or continue antibiotic prophylaxis). Only a subgroup of children in the trial was age-eligible for KINDL-R (133 of 176) of whom 77% did participate in this quality of life study. Therefore, the randomization principle was violated and we tested for group differences in general characteristics using Chi-square tests or Fisher’s exact tests where appropriate. Subsequently, differences in HRQL scores between the intervention groups were tested for both patients and parents with independent samples T-tests. Univariable linear regression analyses were used to correct for the effect of differences in general characteristics that might confound the association between intervention and HRQL. Additionally, we intended to test for differences between patients’ and parents’ perceived quality of life. This was done in the total patient group, and we intended to use paired t-tests in case of no treatment effect or general linear models in case of a treatment effect on HRQL. As all analyses of subdomains of HRQL are part of one overriding hypothesis, we did not account for multiple testing. All statistical analyses were performed using SPSS, version 19.0 (IBM Company).

Results

Subject responses and baseline characteristics

One hundred and two (77%) of the 133 eligible children and their parents filled out the KINDL-R questionnaire at the end of the trial period between 2006 and 2010. Patient characteristics are described in Table 1. There were no significant differences in the relevant variables between the stopping and continuing group, with the exception of the division of children over 8 years of age in the stop and continuing group (p = 0.03).

Table 1: Patient characteristics of evaluated children with spina bifida on clean intermittent catheterization.

Quality of life scores

Compared to healthy controls, both spina bifida children and their parents have significantly lower HRQL scores on every domain except disease, as well as in the total score (Table 2). Patients generally had higher HRQL scores than their parents (62.0 vs. 56.8, p<0.001), specifically in the domains of disease (p<0.001), emotional well-being (p = 0.005), self-esteem (p<0.001), friends (p = 0.002) and school (p = 0.02). Regression analyses revealed no significant influence of gender, level of spinal lesion, presence of hydrocephalus, route of catheterization, manner of defecation or level of mobility and schooling on HRQL.

Table 2: HRQL scores in spina bifida children, their parents and healthy controls.

There was also no difference between the sub-domain HRQL scores of the group stopping daily AP and the group that continued AP in any specific domain (Table 3). Also, the total HRQL score, both in patients (62.0 in stop group vs. 62.3 in continuing group, p = 0.92) and parents (57.2 vs 54.8, p = 0.16) did not differ significantly between stopping or continuing prophylaxis (Table 3).

Table 3: Differences in HRQL scores in continuing and stopping antibiotic prophylaxis in spina bifida patients and their parents.

Discussion

Spina bifida patients and their parents have lower HRQL scores than healthy controls, and parents have lower HRQL scores than their children. Severity of the spinal lesion and stopping daily AP for urinary tract infections in children with spina bifida on CIC has no significant effect on HRQL.

This is in accordance with prior studies [11,14], and may be explained by the fact that children appreciate life as they know it: their answers reflect current and short term social functioning, while they disregard potential future threats. Parents however tend to incorporate future outcome in their responses, and therefore have a less optimistic view of social status, relationships and employment possibilities of their offspring. Another explanation could be that children want to protect their parents by concealing their true feelings. Janse et al. showed that pediatricians tend to misunderstand perception of HRQL in patients’ parents [15]. Therefore, systematic assessment of HRQL is needed in patients as well as their parents, as this may differ from the assumed HRQL by the health care professional. Proper assessment of HRQL thus contributes to a tailored psychosocial and somatic counseling by health care professionals treating spina bifida patients.

The level of the spinal lesion, presence of hydrocephalus, manner of both micturation and defecation, gender and level of mobility and schooling does not influence the HRQL scores in either patients or their parents. This is in accordance with previous studies [5]. Padua et al. found that patients with high disability have lower scores in physical domains, whilst patients with milder lesions have more emotional problems, due to their incontinence problems. Spina bifida itself is therefore the main factor contributing to perception of HRQL, and not so much the severity of the morbidity.

Conclusion

Spina bifida patients have lower HRQL scores than their healthy peers. Parents have even lower scores, especially in the psychological domains. Stopping daily antibiotic prophylaxis in children with spina bifida on clean intermittent catheterization can be done safely to improve bacterial resistance for antibiotics without influencing their quality of life.

References

1. Shaer CM, Chescheir N, Schulkin J (2007) Myelomeningocele: A review of the epidemiology, genetics, risk factors for conception, prenatal diagnosis, and prognosis for affected individuals. Obstet Gynecol Surv 62: 471-479.
2. Williams LJ, Rasmussen SA, Flores A, Kirby RS, Edmonds LD (2005) Decline in the prevalence of spina bifida and anencephaly by race/ethnicity. Pediatrics 116: 580-586.
3. Bier JA, Prince A, Tremont M, and Msall M (2005) Medical, functional, and social determinants of health-related quality of life in individuals with myelomeningocele. Dev Med Child Neurol 47: 609-612.
4. Hetherington R, Dennis M, Barnes M, Drake J, Gentili F (2006) Functional outcome in young adults with spina bifida and hydrocephalus.  Childs Nerv Syst 22: 117-124.
5. Padua L, Rendeli C, Rabini A, Girardi E, Tonali P, et al. (2002) Health-related quality of life and disability in young patients with spina bifida.  Arch Phys Med Rehabil 83: 1384-1388.
6. Schoenmakers MA, Uiterwaal CS, Gulmans VA, Gooskens RH, Helders PJ (2005) Determinants of functional independence and quality of life in children with spina bifida.  Clin Rehabil 19: 677-685.
7. Buran CF, Sawin KJ, Brei TJ, Fastenau PS (2004) Adolescents with myelomeningocele: activities, beliefs, expectations, and perceptions.  Dev Med Child Neurol 46: 244-252.
8. van den Berg-Emons HJ, Bussmann JB, Meyerink HJ, Roebroeck ME, Stam HJ (2003) Body fat, fitness and level of everyday physical activity in adolescents and young adults with meningomyelocele. J Rehabil Med 35: 271-275.
9. Gladh G, Eldh M, Mattsson S (2006) Quality of life in neurologically healthy children with urinary incontinence. Acta Paediatr 95: 1648-1652.
10. Parkin PC, Kirpalani HM, Rosenbaum PL, Fehlings DL, Van Nie A (1997) Development of a health-related quality of life instrument for use in children with spina bifida. Qual Life Res 6: 123-132.
11. Kirpalani HM, Parkin PC, Willan AR, Fehlings DL, Rosenbaum PL, et al. (2000) Quality of life in spina bifida: Importance of parental hope. Arch Dis Child 83: 293-297.
12. Körner I, Schlüter C, Lax H, Rübben H, Radmayr C (2006) Health-related quality of life in children with spina bifida.  Urologe A 45: 620-625.
13. Zegers B, Uiterwaal C, Kimpen J, van Gool J, de Jong T, et al. (2011) Antibiotic prophylaxis for urinary tract infections in children with spina bifida on intermittent catheterization.  J Urol 186: 2365-2370.
14. Vermaes IP, Janssens JM, Bosman AM, Gerris JR (2005) Parents' psychological adjustment in families of children with spina bifida: A meta-analysis.  BMC Pediatr 5: 32.
15. Janse AJ, Sinnema G, Uiterwaal CS, Kimpen JL, Gemke RJ (2005) Quality of life in chronic illness: Perceptions of parents and paediatricians.  Arch Dis Child 90: 486-491.