The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord

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1 The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord Jamie D Olesen MD 1, Darcie A Kiddoo MD FRCSC 2, Peter D Metcalfe MD MSc FRCSC 2 JD Olesen, DA Kiddoo, PD Metcalfe. The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord. Paediatr Child Health 13;18(7):e32-e38. ObJeCTive: To determine the association between urinary continence and quality of life (QoL) in a paediatric spina bifida population. MeTHODs: After appropriate ethics approval, a prospective study was initiated using multiple validated QoL instruments that were distributed to patients as they presented for their annual appointment at the Northern Alberta Spina Bifida Clinic (Edmonton, Alberta). General demographic information was collected and validated questionnaires were used. The survey package included two instruments to assess overall QoL: Global Pediatric QoL (PedsQL 4.) and Health Specific QoL-Spina Bifida (HRQoL-SB). Two instruments were also included to quantify urinary symptoms and assess urinary specific QoL: the Urinary Incontinence Severity Index Pediatric (ISI-P) and Urinary Specific QoL (PinQ). ResulTs: A total of 71 patients were enrolled in the study. The general QoL (PedsQL 4.) and health-specific QoL (HRQoL-SB) scores for the population indicated an overall QoL of 66% (n=69) and 83% (n=67), respectively. Approximately 46% (33 of 71) reported >1 episode of urinary incontinence per week. Urinary continence was associated with a significantly higher urinary-specific QoL (PinQ; P<.1), general QoL (PedsQL 4.; P<.5) and health-specific QoL (HRQoL-SB; P<.5). Furthermore, urinary incontinence and its effect on QoL was not influenced by the presence of a shunt, level of the lesion or manner of dysraphism. COnClusiOn: These data suggest that QoL in patients with spina bifida is related to urinary continence. This effect appears to be independent of the type and level of the spinal dysraphism and the presence or absence of a shunt. Key Words: Incontinence; Quality of life; Spina bifida Spina bifida can be a devastating congenital anomaly and has an annual incidence of approximately 3.5 to 4 per 1, births (1-3). However, contemporary advancements in medical, surgical and rehabilitative care have decreased morbidity and increased life expectancy well into adulthood (4). Therefore, current patient management strategies must now be focused on quality of life (QoL) (5-7). The WHO defines QoL as A state of complete physical, mental and social well-being and not merely the absence of disease or infirmity (8). When dealing with chronic health issues, however, it is important to assess a health-related QoL (HRQoL), which provides a measure of the patient s perception of his or her overall level of functioning (8). This is essential because caregivers tend to ascribe a more significant health burden to the patient than is actually experienced (9). Furthermore, standard of care is trending toward routine administration of standardized health measures to optimize clinical care (6). Patients with spina bifida face many challenges, and the role of Original article l association entre la continence urinaire et la qualité de vie chez les patients d âge pédiatrique ayant un spina-bifida et un syndrome du filum terminal ObJeCTiF : Déterminer l association entre la continence urinaire et la qualité de vie (QdV) au sein de la population d âge pédiatrique ayant un spina-bifida. MÉTHODOlOGie : Après l approbation déontologique pertinente, les chercheurs ont amorcé une étude prospective au moyen de multiples instruments validés relatifs à la QdV, qui ont été distribués aux patients lors de leur rendez-vous annuel à la clinique de spina-bifida du nord de l Alberta située à Edmonton, en Alberta. Ils ont colligé les renseignements démographiques généraux et utilisé des questionnaires validés. Le dossier de l étude se composait de deux instruments pour valider la QdV globale : la QdV pédiatrique globale (PedsQL 4.) et la QdV propre à la santé (HRQoL-SB). Deux instruments ont également été utilisés pour quantifier les symptômes urinaires et évaluer la QdV propre aux symptômes urinaires : l indice de gravité de l incontinence urinaire pédiatrique (ISI-P) et la QdV propre aux symptômes urinaires (PinQ). RÉsulTATs : Au total, 71 patients ont participé à l étude. Les indices de QdV générale (PedsQL 4.) et de QdV propre à la santé (HRQoL-SB) pour cette population ont indiqué une QdV globale de 66 % (n=69) et de 83 % (n=67), respectivement. Environ 46 % (33 sur 71) ont déclaré plus d un épisode d incontinence urinaire par semaine. La continence urinaire s associait à une QdV propre aux symptômes urinaires, à une QdV générale et à une QdV propre à la santé nettement plus élevées (PinQ : P<,1, PedsQL 4. : P<,5 et HRQoL-SB : P<,5, respectivement). Par ailleurs, la présence d un shunt, le niveau de la lésion et la gravité de la dysraphie n avaient pas d influence sur l incontinence urinaire et son effet sur la QdV. COnClusiOn : Selon ces données, la QdV des patients ayant un spina-bifida est liée à la continence urinaire. Cet effet semble indépendant du type et du niveau de dysraphie spinale et de la présence ou de l absence d un shunt. the urologist in providing lifelong surveillance and care is paramount (1). Urinary and bowel symptoms affect almost all children with spina bifida (7) with rates of incontinence ranging from 48% to 76% (11,12). Surprisingly, the impact of incontinence on overall HRQoL remains unclear. It has been shown that the domain of self-concept for continent spina bifida patients is equal to those in the healthy population (13). However, an association between urinary incontinence and poor HRQoL has not yet been demonstrated (9). Furthermore, some studies have shown no improvement in HRQoL despite lower urinary tract reconstruction and an improvement in continence (14). Therefore, the main objective of the present prospective study was to generate a comprehensive databank assessing social, emotional, psychological and HRQoL measures, and to identify how these are influenced by urinary continence. Furthermore, we aimed to evaluate the impact of several disease specific parameters to 1 Faculty of Medicine; 2 Division of Pediatric Surgery, Department of Surgery, University of Alberta, Edmonton, Alberta Correspondence: Dr Peter D Metcalfe, University of Alberta, Stollery Children s Hospital, 2C3.79 WMC, Street, Edmonton, Alberta T6G 2B7. pmetcalf@ualberta.ca Accepted for publication May 29, 13 e32 13 Pulsus Group Inc. All rights reserved Downloaded from on 23 January 18 Paediatr Child Health Vol 18 No 7 August/September 13

2 Urinary continence and QoL in paediatric spina bifida patients determine whether they modulate the impact of incontinence on QoL. We hypothesized that incontinence will adversely impact QoL for children with mild disease, but will have less of an impact for children with more severe disease. MeTHODs After institutional ethics approval, spina bifida patients (five to 17 years of age) were recruited when they presented to the Northern Alberta Spina Bifida Clinic (Edmonton, Alberta) for their annual comprehensive health examination. This clinic serves the vast majority of children with spinal cord pathology from a catchment area that includes approximately two million individuals. Consent was obtained from the patients and/or their parents and they were then provided a questionnaire package to be completed during their time in the clinic. Urinary incontinence was strictly defined as >1 daytime urinary accident per week, determined by one of the questionnaires, the Incontinence Severity Index Pediatric (ISI-P). Other data, such as the level of the lesion, form of dysraphism and presence of a shunt, were collected from a previously established spina bifida database (11) or were extracted from the patient chart. All manners of spinal dysraphism and tethered cord were included. instruments Questionnaires were completed by patients and their parents. It has been shown that children as young as five years of age are equally reliable at determining and expressing their own feelings (15). A measure was, however, included to determine who completed the surveys: this survey was completed by patient/parent/both. isi-p: Degree of urinary symptom severity was determined by the validated ISI-P, which was developed by Nelson et al (16) for healthy children. This 11-question survey was used to quantify stress, urge, insensate and nocturnal symptoms for children 11 to 17 years of age. Patients were defined as incontinent if they answered about once a week or more on any of questions 3 through 6. Furthermore, whole scores expressed as a percentage were used to assess overall symptom severity. Pediatric incontinence Questionnaire: The impact of urinary symptoms on QoL was measured by the instrument developed by Bower et al (17): the definitive Pediatric Incontinence Questionnaire (PinQ). This is a measurement of QoL as directly influenced by urinary symptoms. The questions were scored from to 4, with a maximum possible score of. A higher PinQ score means superior QoL that is unaffected by urinary symptoms (17). This has been validated for otherwise healthy children six years of age and older. Health-Related Quality of life spina bifida: Health-Related Quality of Life Spina Bifida (HRQoL-SB), developed by Parkin et al (18), is a validated QoL instrument taking into consideration all of the challenges faced by the SB patient. There are separate versions for patients five to 12 years of age and 13 to 17 years of age (18). The HRQoL-SB is a 5-point Likert scale containing either 44 (five to 12 years of age) or 47 (13 to 18 years of age) items. The scores range from 44 to 2 and 47 to 235, respectively; however, to enable cross-comparison between the two age groups, these scores were expressed as a percentage. Higher percentages indicate a superior QoL (18). PedsQl 4.: A generic core QoL scale (PedsQL 4.) developed by Varni et al (19) was used to measure issues that would affect QoL for a typical healthy child. There are different yet similar instruments for children five to seven years of age, eight to 12 years of age and 13 to 17 years of age. The scores can be compared, however, because the items assessed render similar concern between the age ranges. Furthermore, the content in the questions is consistent, but is adjusted for cognitive ability (19). Paediatr Child Health Vol 18 No 7 August/September 13 Downloaded from on 23 January 18 Additional instruments To gather additional data not included in the survey instruments, additional questions directly asking about intermittent catheterization status and frequency were included. General feedback and comments were also gathered to assess the degree of participant burden. The PinQ, ISI-P, and HRQoL-SB articles are readily available for dissemination (16-18), and the instruments have been attached as appendixes (Appendixes 1, 2 and 3). The Varni QoL instrument, however, was only available to the authors by special permission. However, the other questionnaires offer merit on their own and can be used without the Varni QoL instrument. statistical analysis Due to unit intervariability among instruments, all scores were expressed as a percentage to facilitate cross-comparison; however, the groups were always considered separately. An exception to this was made with the HRQoL-SB, which has two different measures (total sum of 2 or 235); these scores were expressed as a percentage and considered as one group. Furthermore, the entire package was not voided if a patient failed to complete all included surveys. However, there was no cross-comparison between groups unless the participant had competed both of the instruments being compared. StatPlus (AnalystSoft Inc, USA) for Excel (Microsoft Corporation, USA) was used to calculate descriptive statistics including mean, median, SD, maximum and minimum. StatPlus was also used to calculate linear regression, one-way ANOVA and t tests. ResulTs Demographics A total of 71 patients were enrolled in the present study, with a response rate of 66% (71 of 17). Of those enrolled, 37 were male (mean age 1.1 years, median 11 years) and 34 were female (mean age 12.7 years, median 12 years). A shunt was present in 38% (27 of 71; 12 male, 15 female). The types of dysraphism included were: myelomeningocele (41%; 29 of 71), tethered cord (25%; 18 of 71), lipomeningocele (24%; 17 of 71), meningocele (3%; two of 71), lipomyelomeningocele (1%; one of 71), and spinal cord injury occurring before five years of age (6%; 4 of 71). urinary continence and Qol The overall rate of urinary continence in this population was 46% (33 of 71) and overall HRQoL-SB was 83%. As the degree of urinary symptoms increased (ISI-P) there was a statistically significant decrease in both general paediatric QoL (PedsQL 4.; R=.34; P<.5) and HRQoL scores (R=.38; P<.5) (Figure 1). This trend was also demonstrated comparing the degree of urinary symptoms (ISI-P) with urinary-specific QoL (PinQ) (R=.61; P<.1) (Figure 2). Continence status was associated with a superior urinary-specific QoL (P<.1) (Figure 3). Furthermore, continence extended beyond urinary-specific QoL and influenced general paediatric QoL (P<.5) and also HRQoL-SB (P<.5) (Figure 3). Clean intermittent catheterization Clean intermittent catheterization (CIC) was used in 39% (28 of 71) of the population. The rate of incontinence was comparable between the CIC group (53%; 15 of 28) and the spontaneous void group (42%; 18 of 43). There were not differences on any QoL domains when comparing these two groups. shunts The presence of a ventriculoperitoneal shunt did not have an impact on PedsQL 4. or HRQoL-SB scores (Figure 4). In addition, shunts were not found to affect continence status, degree of e33

3 Olesen et al HRQoL-SB QoL % R =.34, p<.5 4 * * QoL % *** Continent Incontinent 4 PedsQL 4. R =.38, p<.5 *p<.5 ***p< PinQ 1 ISI-P % Figure 1) Global Pediatric Quality of Life (PedsQL 4.) and HealthRelated Quality of Life Spina Bifida (HRQoL-SB) versus Incontinence Severity Index Pediatric (ISI-P). With increasing urinary symptoms (ISI-P scores) there is a corresponding decrease in both PedsQL 4. (R=.34; P<.5) and HRQoL-SB scores (R=.38; P<.5) Figure 3) The effect of continent status on Pediatric Incontinence Questionnaire (PinQ), Global Pediatric Quality of Life (PedsQL 4.), and Health-Related Quality of Life Spina Bifida (HRQoL-SB) scores. The mean scores for continent patients on the PinQ, PedsQL 4. and HRQoL-SB were 84% (n=35), 72% (n=36) and 86% (n=35), respectively. The mean scores for incontinent patients using the same instruments were 66% (n=33), 59% (n=35) and 81% (n=35). There were significant differences in PinQ (P<.1), PedsQL 4. (P<.5) and HRQoL-SB scores (P<.5) R =.61 p<.1 % PinQ % PedsQL 4. HRQoL-SB 4 shunt 4 4 ISI-P % Figure 2) Pediatric Incontinence Questionnaire (PinQ) scores versus Incontinence Severity Index Pediatric (ISI-P) scores. As the degree of urinary symptoms increase, there is a corresponding decrease in urinaryspecific QoL (PinQ) (R=.61; P<.1) urinary symptoms or bother of urinary symptoms (PinQ) (Figure 4). The presence of a shunt did not negate the impact of urinary incontinence on PinQ or HRQoL-SB scores (Figure 5). level and type of lesion When comparing the type of spinal dysraphism (open [myelomeningocele, meningocele or lipomyelomeningocele] versus closed [tethered cord, spinal cord injury] dysraphisms), there were no differences in PedsQL 4., HRQoL-SB or ISI-P scores, continent status or PinQ scores (Figure 6). This was also the case for level of the lesion (lumbar versus sacral) (Figure 7). Feedback The survey package took an average of 15 min to complete (range 1 min to min; median 15 min). Approximately 85% of participants believed they were able to express themselves completely and 86% believed that it contributed to their overall clinic experience. DisCussiOn The importance of measuring QoL in populations experiencing chronic conditions and disability has been well established in the e34 no shunt Downloaded from on 23 January 18 PedsQL 4. HRQoL-SB ISI-P PinQ Incontinent status Figure 4) Impact of a shunt on quality of life and urinary symptoms. Mean scores for shunt or no shunt: Global Pediatric Quality of Life (PedsQL 4.), 66% (n=27) shunt, 65% (n=42 ) no shunt; Health-Related Quality of Life Spina Bifida (HRQoL-SB), 85% (n=25) shunt, 82% (n=42) no shunt; Incontinence Severity Index Pediatric (ISI-P), 33% (n=26) shunt, 26% (n=43) no shunt; Pediatric Incontinence Questionnaire (PinQ), 75% (n=26) shunt, 75% (n=42) no shunt; incontinent status, 52% (n=27) shunt, 43% (n=44) no shunt literature (6,18,). Within specific populations, one can measure generalized HRQoL, as well as a disease-specific HRQoL (5). Disease-specific assessments of QoL lead to reduction in bias and increased accuracy (6). In the paediatric spina bifida population, one must not assume that disease symptoms necessarily correlate with QoL (21). In a single visit, the health professional is unable to take into consideration all potential home and social supports benefitting the patient (9). In fact, it has been shown that children with spina bifida are not typically as bothered by their symptoms as health care providers often believe (9). With this discrepancy of perceived QoL impact, it can often be difficult to make clinical decisions when balancing the benefits and risks of certain therapies especially those with potential for harm. Management of the urinary tract can represent one of the largest challenges for a spina bifida patient. The main goals of treatment are the preservation of renal health and continence (1). The Paediatr Child Health Vol 18 No 7 August/September 13

4 Urinary continence and QoL in paediatric spina bifida patients PinQ % 9 7 * 5 shunt no shunt Figure 5) The impact of shunt and continent status on Pediatric Incontinence Questionnaire (PinQ) scores. The mean PinQ scores were 83% (n=12) if shunted and continent, and 69% (n=14) if shunted and incontinent (P<.5). The mean PinQ scores were 85% (n=23) for continent nonshunted patients and 63% (n=19) for incontinent, nonshunted patients (P<.5) introduction of CIC, anticholinergic medication and judicious use of bladder augmentation have resulted in very low rates of renal insufficiency (11,22). Prevention of incontinence, however, remains very challenging. Management strategies vary from spontaneous voiding to continent surgical reconstruction. It can be very difficult to assess the degree of bother that the patient experiences during a short clinic visit. Therefore, a better appreciation of the effect on QoL should contribute to more effective therapeutic decisionmaking, especially when considering the investment required by the patient and family to achieve continence (,23). No clear inverse linear relationships between urinary incontinence and QoL have been described in the literature. However, certain patterns are beginning to emerge. Lemelle et al (23) have shown that functional improvement in either continence or walking status is not associated with enhanced QoL. This phenomenon has also demonstrated by MacNeily et al (14), who showed that despite improvement of lower urinary tract symptoms after lower urinary tract reconstruction, HRQoL remains the same. MacNeily et al (14) postulate that this may be due to increased burden placed on the patient to attain continence, the imperfect continence success rates and/or the morbidity of the treatment options. Rendeli et al () found that continence is only associated with an increased physical QoL and has no association with emotional QoL. In adolescence, urinary incontinence appears to be the most significant factor to be associated with a decline in both physical and emotional QoL (). Padua et al (21) found that major physical limitations were associated with a decreased physical QoL with no impact on emotional QoL. Conversely, minimal physical limitation was associated with significant impairment on emotional QoL (but not physical QoL). The authors postulate that this was due to sphincter control problems, with urological problems tending to require more support. In our patient population, we found overall HRQoL to be quite high (83%, 183 of 2, n=47; 195 of 235, n=). This indicates that our patients HRQoL is similar to healthy children and adolescents (18). This suggests that the children in our study were well adjusted with their ailments and were generally quite happy. However, our data suggests that urinary incontinence is indeed associated with a decreased QoL (HRQoL-SB; P<.5) and poor global QoL (PedsQL 4.; P<.1). These findings demonstrate the significant amount of bother associated with urinary ** Continent Incontinent *p<.5 **p<.1 Paediatr Child Health Vol 18 No 7 August/September 13 Downloaded from on 23 January 18 % 4 PedsQL 4. HRQoL-SB ISI-P PinQ Incontinent Status Open Closed Figure 6) Impact of open versus closed dysraphism on QoL and urinary symptoms. Mean scores for open or closed dysraphism: Global Pediatric Quality of Life (PedsQL 4.), 65% (n=48) open, 68% (n=21) closed; Health-Related Quality of Life Spina Bifida (HRQoL-SB); Incontinence Severity Index Pediatric (ISI-P), 27% (n=46) open, 33% (n=22) closed; Pediatric Incontinence Questionnaire (PinQ), 76% (n=47) open, 73% (n=21) closed; and incontinent status, 48% (n=48) open and 43% (n=23) closed % 4 PedsQL 4. HRQoL-SB ISI-P PinQ Incontinent Status Lumbar Sacral Figure 7) Impact of lumbar versus sacral dysraphism on quality of life and urinary symptoms. Mean scores for lumbar or sacral dysraphism: Global Pediatric Quality of Life (PedsQL 4.), 64% (n=) lumbar, 66% (n=27) sacral; Health-Related Quality of Life Spina Bifida (HRQoL-SB), 83% (n=) lumbar, 85% (n=27) sacral; Incontinence Severity Index Pediatric (ISI-P), 32% (n=29) lumbar, 23% (n=27) sacral; Pediatric Incontinence Questionnaire (PinQ), 72% (n=19) lumbar, % (n=27) sacral; incontinent status, 44% (n=25) lumbar, 46% (n=28) sacral symptoms, despite all other challenges faced by these children. The association between continence and the PinQ (P<.1) helps to serve as an internal control, demonstrating effectiveness in the spina bifida population. It was surprising that level of lesion, shunt and open versus closed dysraphism did not significantly affect continence or QoL because this phenomenon has been described previously (21). A previous review of our population had also shown that shunts were associated with greater degrees of incontinence (11). Therefore, we would have expected individuals with a ventriculoperitoneal shunt to experience greater urinary symptoms and an associated decrease in QoL. This, however, was not the case. We postulate the reason for this difference to be due to the prospective nature of this data collection and the use of validated questionnaires, as opposed to a retrospective chart review based on clinic notes. The present study also used a more inclusive definition of incontinence: >1 one daytime urinary accident per week as opposed to completely free of any urinary leakage or fecal accidents. Furthermore, our previous e35

5 Olesen et al study only showed association with continence to shunt and level of the lesion; effect on QoL was not investigated. The significant physical impairment associated with spina bifida, hydrocephalus and shunts is often associated with cognitive defects and learning disabilities (24,25). This has been extrapolated to suggest that these patients are less bothered by their incontinence (). However, our data demonstrates that the impact on QoL was significant in our patients, and affected all degrees of disability. Survey burnout was one of the shortfalls of the present study. Of the 71 individuals who agreed to participate, 88% completed the packages in their entirety. We do not believe that this resulted in any bias or misinterpretations because no cross-comparisons were performed if the participant did not complete each instrument. In the future, burnout could be reduced by administering only certain surveys at each visit. Furthermore, computerized assessments may eventually provide instant results that could be used by the physician at the time of the visit, enabling immediate identification of patient concerns or lack thereof. In addition, it would allocate less clinic time to issues that are deemed to be unimportant to the patient. COnClusiOns The results of the present study reveal that not only is urinary-specific QoL dependent on continence, but HRQoL is strongly affected by urinary symptoms. These data also suggest that continence and bother are not affected by the presence of a shunt, level of lesion or open versus closed dysraphism. Collectively, we conclude that continence matters in all of our patients, regardless of the severity of the lesion. Results from our study lead us to recommend routine administration of QoL instruments when caring for the paediatric spina bifida patient. This will not only guide decision making, but will also increase overall patient satisfaction because 85% of our enrolled patients believed it contributed to their overall experience. Furthermore, 86% found they were able to express themselves. ACKnOWeleDGeMenTs: This study would not have been possible without the time and efforts of Rebecca Manuel RN and Leogardo DeLeon RN, care coordinators of the Northern Alberta Spina Bifida Clinic located at the Glenrose Rehabilitation Hospital, Edmonton, Alberta. APPenDiX 1 e36 Downloaded from on 23 January 18 Paediatr Child Health Vol 18 No 7 August/September 13

6 Urinary continence and QoL in paediatric spina bifida patients APPenDiX 2 APPenDiX 3 Pin Q: Impact of bladder function, dysfunction and treatment on the quality of life of children with bladder dysfunction. 1. I get shy because of my bladder problem 2. People in my family treat me in a different way because of my bladder problem 3. I am worried that people might think my clothes smell of pee 4. I think that my bladder problem won't get better 5. Mum and dad worry about me because of my bladder problem 6. I would feel better about myself if I didn't have a bladder problem 7. My bladder problem makes me feel nervous 8. Mum or dad sometimes seem a bit cranky because of my bladder problem 9. My bladder problem stops me going on sleep-overs or holidays 1. My bladder problem makes me feel bad about myself 11. I wake up during my sleep because of my bladder problem Quality of Life in Spina Bifida Questionnaire Age 5 12 Beside each question: please rank on a scale from 1 5 how much you agree. How much do you feel your child: 1 is treated with respect and dignity by others? 2 feels good about her/himself? 3 is able to do some things as independently as possible? 4 is able to get into the houses of his/her friends? 5 accepts his/her physical limitations? 6 will be able to choose a career of his/her own? 1: strongly disagree 2: disagree 3: neutral 4: agree 5: strongly agree 7 has the chance to continue to study the things in which he/she is interested? 8 has the chance to learn to swim? 9 participates in the same recreational activities as other children? 1 has the opportunity to play indoors? 11 has the opportunity to play outdoors? 12 participates in games at recess? 13 feels capable or skillful in some sport or hobby or other activity? 14 is stared at by others? 15 is treated as if he/she were different? is healthy? 17 is integrated in the school system? is able to use public washrooms that are accessible and private? 19 has access to the community via ramps and elevators? is accepted and valued in our society? 21 attends a school that has a positive attitude towards children with disabilities? 22 is in an environment that does not contain a lot of obstacles? 23 has someone to confide in outside of the immediate family? 24 has friends? 25 has a supportive family? 26 feels welcome in other children s homes? 27 receives praise for things that he/she is able to do? 28. feels important? 29 is treated with respect by others? 3 feels that she/he can accomplish her/his plans? 31 expresses her/his emotions? 32 has the opportunity to do everything the other children do in school? 33 is able to learn well in an environment that is favourable to children with disabilities? 34 is motivated to learn? 35 is able to attend a camp for children with disabilities? 36 feels that the examinations and treatments at the hospital or clinic are respectful? 37 feels that the examinations and treatments at the hospital or clinic are private? 38 feels related to as a whole person by the doctor? 39 is able to deal well with being in the hospital? 4 feels in control of the situation in medical appointments and treatments? 41 is learning to deal positively with his/her disability? 42 is becoming appropriately independent in areas of self care, mobility, and self catheterization? 43 will be able to live independently in the future? 44 possesses self confidence? Quality of Life in Spina Bifida Questionnaire Age 13 Beside each question, please rank on a scale from 1 5, how much you agree. How much do you feel: 1 that you are treated the same as everyone else? 2 that you have a supportive family? 3 That you are accepted just as you are 4 That you are able to talk to one or both of your parents? 5 That people enjoy being with you 6 that you are happy with yourself? 7 that you are able to speak up for yourself? 8 that there is hope for the future? 9 positive about yourself? 1 that other people respect you? 11 satisfied with your school programme? 12 able to participate in group activities? 13 that you are able to have a special friend? 14 like you are treated the same as the other kids. 15 that you are able to take care of yourself; for example brushing your hair and teeth? that you are able to feed yourself? 17 that you are able to help with some or all of your catheterization that you are able to participate in some or all of your own bathing 19 that you have a lot of pain that you can stand up for your rights? 21 that you can make your own choices and decisions? 22 that you are as independent as you are able to be? 23 that you can use the telephone? 24 that people listen to your opinions? 1: strongly disagree 2: disagree 3: neutral 4: agree 5: strongly agree 25 that you are treated with respect and dignitiy at your medicial appointments 26 that you have a say in your medical treatment 27 that you understand what your medical condition will be like in the future? 28. that you are getting good care at your spina vifida clinic? 29 that your doctors, nurses and others who treat you know wabout spina bifida? 3 that people see you and not only your disability? 31 that you will have a suitable home in the future? 32 that you have privacy and accessibility in public washrooms? 33 that you are able to use the iitchen at home/? 34 that your present washroom is suitable for you? 35 that you are able to participate in outdoor activities? 36 that you have the physical strength to do sports like swimming, skiing, etc 37 that you are able to go out on dates and to parties 38 challenged and encouraged through sports 39 successful or skilled in some sport or other activity you like? 4 that there will be job opportunities for you in the future? 41 you are able to get an education for a job that interests you 42 that you have a career goal in mind? 43 able to hold down a part-time job? 44 that you will be able to have children in the future? 45 that you will marry? 46 that you have somebody with spina bifida to look up to and to have as a role model? 47 that you have a close friend who is like you in many ways? 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