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Assessing health-related quality of life in childrens with Spina Bifida in Lithuania

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Lithuanian University of Health Sciences

Assessing health-related quality of life in

childrens with Spina Bifida in Lithuania

By

Faris Magdi Genawi Mohamed Ali

A thesis submitted in part fulfillment for the

Degree of Master of Medicine

In the

Faculty of Medicine

Department of Pediatrics Rehabilitation

Kaunas, 2016/2017

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TABLE OF CONTENTS

1. TITLE PAGE 1 2. TABLE OF CONTENTS 2 3. SUMMARY 3 4. ACKNOWLEDGEMENTS 4 5. CONFLICT OF INTEREST 4

6. ETHICS COMMITTEE CLEARANCE 4

7. ABBREVIATIONS LIST 6

8. INTRODUCTION 7

9. AIM AND OBJECTIVES OF THE THESIS 9

10. LITERATURE REVIEW 10

11. RESEARCH METHODOLOGY AND METHODS 15

12. RESULTS 17

13. DISCUSSION OF THE RESULTS 24

14. CONCLUSION 26

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Summary:

Author Name: Faris Magdi Genawi Mohamed Ali

Aim: To estimate the health related quality of life (HRQOL) in Lithuanian children with Spina

Bifida (SB) in relation to comorbidities, level of lesions and mobility.

Objectives:

1. To investigate the HRQOL of Lithuanian children born between 1999 and 2012 with SB.

2. To analyse the relation between HRQOL and it„s comorbidities, including hydrocephalus, Chiari II malformation, incontinence and epilepsy.

3. To determine the relationship of health variables, level of lesions and mobility to HRQOL.

Methods:

This was a quantitative cross-sectional study on children with spina bfida across Lithuania to assess the HRQOL. Subjects were chosen and interviewed from various cities including: Kaunas, Vilnius, Marijampole, Gargzdai, Birzai, Panevezys, Palanga and Alytus. The Parkins et al. (1997) questionnaire was used as an instrument to measure HRQOL. The level of lesion, comorbidities and other health variables were obtained from the medical files and directly from patient's history.

Results:

Regarding HRQOL, our study population showed the highest scores in the emotional, medical, intellectual and social domains. The lowest sub-scores were on recreational, vocational, environmental and then physical domains. We also found that certain co-morbidities including hydrocephalus, epilepsy and incontinence negatively affected the QoL (p=0.00). In our study group, we also found that the ambulatory group scored significantly higher in the overall QoL (p=0.002). However, when comparing the level of lesions to the HRQOL we found no statistical signficance difference.

Conclusion:

1. Positive results were obtained regarding the medical aspects of patients with SB in Lithuania as they scored high in this domain. However, the environment and vocational domains scored low, suggesting further examination needs to be carried in these domains.

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have negative impacts on the QOL. Patients whom suffered from epilespy had a statistically significant lower QOL.

3. No significant difference was found in the association between the level of lesion and QOL in our study.

Acknowledgement:

I would like to give thanks to my lovely family and friends in both Ireland and Lithuania. I am very appreciative of the support they have given me through this journey.

Special thanks to my supervisor, Prof. Audrone Prasauskiene, who always came up with amazing ideas and was always able to get the best out of me. I would also like to thank Dr. Indre Bakaniene for helping me gather the data needed for my research and Ms. Edita Sakyte for assisting me with the statistical analysis of the results.

In addition, I would like to thank the staff in the Pediatrics Clinic in Klinkos Kaunas.

Conflict of Interest:

I declare no conflict of interest.

Ethics Committee Clearance:

Title of thesis: Assessing health-related quality of life in children with spina bifida in Lithuania. Number: BEC- MF- 453

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Abbreviations:

• SB - Spina Bifida • QoL – Quality of life

• HRQOL- health-related quality of life • PedsQL - Pediatric Quality of Life Inventory

• SEIQol-DW- Schedule for the Evaluation of Individual Quality of Life-Direct Weight • HUI3- Health Utilities Index Mark 3

• SBH- Spina Bfida and Hydrocephalus • NGO- Non-Governmental Organisation • EEA – Eurpean Economic Area

• NBDPN - National Birth Defects Prevention Net-work • CNS – Central Nervous System

• CSF- Cerebrospinal fluid

• CM-II - Chiari Malformation Type II • MCQ - Multiple Choice Question

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Introduction:

In recent years, more emphasis have been placed on improving the health-related quality of life (HRQOL) in children with Spina Bifida (SB). Chronic disability is understood to impact various aspects of the person‟s life, family and social functioning, in addition to the disease specific needs. HRQOL is done to assess the patient‟s quality of life in various domains including physical and mental. Back in the 1900s few children survived SB, whereas today they almost have normal life expectancy. By understanding the contributing factors on quality of life (QOL) more targeted interventions can be put in place in order to maximise the psychological and social well being of these patients.

In Lithuania no study has been conducted on the HRQOL of children with SB. Despite SB being the second most common congenital disorder after Down Syndrome by their prevalence in Lithuania.(1) Very few people know about the disease and its long-term effects; SB is caused by both genetic and external harmful factors which disrupts the normal development of the spinal development. Symptoms will vary depending on the level of lesion.

In the literature there are only a few studies performed regarding the long term prognosis of children with SB. In general, survival and the degree of neurological impairment depends on the level of the spinal segment involved, the severity of the lesion, and the extent of associated abnormalities.Long-term survival into adulthood and advanced age is now common with aggressive treatment and an interdisciplinary clinical approach. The average survival rate before the 1960's for all forms of SB was only about 10% to 12%.(2) The National Birth Defects Prevention Net-work (NBDPN)and other multi-states had found the mortality rates declined withfolic acid fortification from U.S grain supply in the 1990's.(3) Furthermore, the survival at 1 yearof age increased significantly from 20% to 80% following neurosurgical intervention, despite limited knowledge regarding long-term effects.(4) The followingmain systems are involved in causing mortality in children with SB: renal, cardio-respiratory, CNS infection, hydrocephalus and, thrombocytopenic purpura.(4) Many of these could possibly be avoided if a timely and adequate treatment is provided.

The majority of the affected children require a complex and long-term multidisciplinary care input. Recently in 2016, a conference had been organised in Norway; with an aim of bringing both the

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Lithuanian and Norwegien Spina Bifida and Hydrocephalus (SBH) Association together to form a partnership.(1) This was successfully done with the help of the Non- Governmental organization (NGO) and the European Economic Area (EEA) grant fundings. Centres have made special training courses available for Lithuanian nurses and social worker, which will in turn help to improve training of Lithuanian families with SB.(1) The Scandinavian countries are considered to have one of the best health care systems in the world and hopefully this new partnernship will improve our understanding of SB in Lithuania and encourage more local studies to be carried out.

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Aim and Objective of Final Thesis:

Aim:

To estimate the health related quality of life (HRQOL) in Lithuanian children with Spina Bifida (SB) in relation to comorbidities, level of lesion and mobility.

Objectives:

1. To investigate the HRQOL of Lithuanian children born between 1999 and 2012 with SB.

2. To analyse the relation between HRQOL and it„s comorbidities (hydrocephalus, Chiari II malformation, incontinence, epilepsy).

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Literature Review:

1.Spina Bifida Classifications

SB is a congenital birth defect characteristized by incomplete closure of one or more vertebrae leaving a gap or split. It is the most common birth defect affecting the CNS. The incidence varies between countries, with no clear figures specific to Lithuania obtained.In general, a higher incidence is noted in Asian countries, for e.g. cases per live births is 1 in 1,000 in the UK compared with 6 in 1,000 in China. (4) Annually, more than 4,500 pregnancies within European Union alone are affected by neural tube defect. This condition has different effects on the child‟s function depending on the level of lesion and the nerve damage involved. Secondary impairments include orthopedic deformities, osteoporosis, obesity, pressure sores and pain. (5)

SB can be divided into spina bfida aperta and spina bifida occulta. SB occulta also known as 'hidden' SB since only little or no changes are observed. In many cases patients can be asymptomatic and unaware of the diagnosis, unless an X-Ray is taken or symptoms develop later on. In SB aperta the symptoms and defects are visible immediately and diagnosed at birth or even in utero with ultrasound. Myelomeningocele is the most serious and common form of SB aperta and is characteristized by bulge of the meninges, neural structures and cerebrospinal fluid through a dural sac.(6) In many cases the sac can rupture and fluid leaks out causing high risk of infection until the sac is operatively closed. This affects the brain with phenotypic features that involve cognition, behavior and adaptation. The brain is often dysplastic together with the spine causing other pathologies such as Chiari II Malformation, where the cerebellum part of the brain protudes out of the foramen magnum and into the upper spinal canal. Hydrocephalus develops in 80-90% of cases with Myelomeningocele and causes excessive accumulation of cerebrospinal fluid which results in widening of the brain ventricles.(6) SB malformation causes other neurological disorders such as fecal incontinence, paralysis of extremities and neurocognitive retardation.(6)

SB by many, is viewed as an orthopaedic disorder, especially in schools in which it is learnt to be as an orthopaedic impairment with learning difficulties, even though the majority of children with Myelomeningocele have congenital malformation of the brain and hydrocephalus.(7)

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2.Instruments

Various types of questionnaires were used to assess the HRQOL in children with SB compared to healthy children. Examining the literature, the questionnaires that have been used in these studies include the following: Parkins et al. (1997), the Pediatric Quality of Life Inventory (PedsQL), Schedule for the Evaluation of Individual Quality of Life-Direct Weight (SEIQoL-DW) and the Health Utilities Index Mark 3 (HUI3) (8-12).The SEIQol-DW survey varied greatly from the others in that it used open questions and therefore translates more easily across cultures. (9-12)

In general these surveys were divided into 3 phases within phase 1, the parent and child identify the five most important things in a patient's life. In phase two, those 5 things are scored using a visual analogue scale from 0-100 and in the final phase all 5 areas are ranked and weighed according to specific colors and were made equal in such a way that they all add up to 100%.The weakness of this type of questionnaire was that the various answers given could possibily increase the difficulty of grouping the answers into appropriate clusters. However, this weakness does not apply to the other two types of questionnaires.

Both the Peds-QL and HUI3 are strictly based on answering MCQ type of questions relating to various domains. The most frequently used questionnaire seen from PubMed literature reviews is the PedsQL (4.0) (8)(13)(14). It is used to assess the HRQOL in the physical, emotional, social, and school functioning domains. However, its popularity doesn‟t stop it from having limitations; In several studies the results varied depending on the selected population, age group and the different questions presented. In addition, the PedsQL is a general measure of HRQOL and was not developed to assess physical problems for individuals who use wheelchairs as the primary mode of mobility. Some questions mentioned in the physical function scale are not an appropriate measure of the physical function of children who depend on wheelchairs for their mobility. For this reason different results can be observed depending on the selected patients. Furthermore, it may not be possible to make accurate comparisons on the effect of physical domain on QOL compared to other conditions. Another limitation from many questionnaires published on this topic, is the use of parental responses on behalf of the child‟s QOL which may not be fully reflective of the child‟s subjective experience.

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In 1997, Parkin et al. aimed to develop a HRQOL instrument specific for patients with SB. (15)Its concept was to develop a discriminative measure from the viewpoint of the affected individuals and their parents in order to make it as relevant as possible in predictinglong term factors which may influence HRQOL in children and adolescents with SB. From a cohort of 553 patients, a heterogeneous group representing the broad spectrum of severity of SB, semi-structured interviews were performed. These generated itemswere reduced by frequency importance product ranking. The reproducibility and validity of this instrument was very good. A larger random sample was then selected for field testing of the questionnaire,questions with poor correlation were then eliminated and internal conisistency and validity were confirmed.From the 600+ items generated, it was reduced to questionnaire with 44 and 47 questions for children and adolescents with SB respectively. Additionally this questionnaire had positive feedback from the participants with the final questions being easily understood and the majority of children were able to answer the form without the help from the parents.

3. QoL

In general it is noted that in children with SB, the lowest sub-scores are usually in the physical health domain as seen with a large study done in Northern California which also noted highest sub-scores in the Emotional Functioning domain.(8) This differed greatly from another study that was done in Belgium showing lowest sub-scores in social functioning domain and highest sub-scores in the Physical domain.(14)

In one study, the effects of QOL wereassessed using six separate domains; (i) Health and Disease (complications of SB, symptoms in general and urination issues); (ii) Development (able to walk and grow); (iii) Resources (health insurance, financial status); (iv) Basic Needs (food, clothes, shelter, sleep); (v) Self-Actualization(religion, interests and hobbies, independence); and (vi) Social environment (bullying, school, friends, family, children).(6) In this study, the most important domain was "development" and next in line came “Self-actualization”. However, “Health and disease” received the lowest score, which unlike in other studies, suggests that the degree of defect in SB does not significantly influence the QOL in these patients.

There are also studies which have demonstrated an association between factors other than disability such as the availability of resources as influential to the QOL of children with conditions such as SB. It

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is these findings that reiterate the importance of easy and accessible help and support to families caring for children with disability.

Not many studies directly compared the level of lesion in SB with the quality of life. However, we identified one large studywhich compared the HRQOL according to the level of lesion using the HUI3 index. (16) It consisted of 125 patients with myelomeningocele ( 86% had CSF shunts in place and 11% had undergone Chiari malformation Type II (CM-II)), 25 with lipomyelomeningocele, and 9 with other dysraphisms). As expected the scores of patients with open myelomeningocele were significantly lower in mutlipe domains than those with closed neural tube defects especially in the ambulation and cognition domains. The score was also lower than those with closed dysraphism. Patients with myelomeningocele that had a history of CSF shunting had a lower HRQOL compared to those without. The 11% that had undergone CM-II decompression had a lower QOL compared to those without a history of CM-II decompression.

These findings however were not consistent with another study that involved 102 children with SB in Kenya; which concluded that there was no statistically significant difference in the overall QOL depending on the various level of lesion in patients with SB. (6)The setting of this study was important as a comparative also; having been in a lower socioeconomic area is expected to have some influence on QoL, which may not be apparent in the studies based in Western world, where the majority of patients are from higher social backgrounds.

A study from Sweden focused on children with myelomeningocele and the HRQOL and ambulation. It concluded that ambulatory status only significantly affected the Physical domain but no other aspects of the HRQOL. (9) In contrast, a study by Abresch et al. concluded that patients with SBthat have mobility problems and rely on wheelchairs for mobility have lower HRQOL sub-scores in all domains of the PedsQL compared to those with no mobility issues. (8)Nevertheless, the PedsQL as a questionnaire has limitations in assessing physical problems for individuals who use wheelchairs as mentioned before.

Studies in general, sadly have shown that after operative interventions,there is no significant improvement in the activity of daily living, while other studies show that there are no significant

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relationship between surgery and physical activity. One main surgical operation still used without knowing the clear benefits is spinal fusion. One particular study tried to show ''the long-term outcome of patients treated operatively and non-operatively for scoilosis deformity secondary to SB''. In this study 34 fusions were performed using pedicle screws and rods, sublaminar wiring and rods, or a combination of both methods. While 11 patients were on the non-operatively treatment arm. The results did not differ using the ''SBSQ or SF-36 PCS and MCS scores''. 11 patients who were able to walk before the operation, became only 6 people that could walk during the follow-up. By contrast, 4 patients who were treated non-operatively were able to walk during both periods. This study not only showed that surgery is ineffective but complication can easily develop post-operatively. (17)

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Research Methodology and Methods:

Subject; For our study, patients with SB born between 1999and 2012 were chosen from different cities

throughout Lithuania. They were identified from neurology outpatient and ambulatory clinics and from various hospital birth registries. This study included patients with all forms of SB. Seventy subjects that met the criteria were identified; however only thirty one participated in the study.

The eligible subjects were invited via invitation letters and follow-up telephone calls with detailed information regarding the study.

Possible reasons for lack of patient participation could be llack of interest by the parents or lack of time or travel expenses.To reduce the percentage of dropouts due to financial reasons such as travel expenses,an option of home visitations if preferred was offered to parents in the invitation letter sent, especially to those who lived far in rural areas.

Questionnaires were given to the child to be answered either at home or during the follow-up consultation. Two different questionnaires were given which were specific to theage group 5-12 years of age and age group 13 -20 age groups. In some cases however, due to the age of the child or cognitive impairment, answers were received by the help of the parents.

Before questionnaires were handed out, the study was approved by the “LSMU Bioetikos Centras” (Reference Nr.: BEC-MF-453) and an Informed consent was obtained from each of the participants involved in the study. Before the subjects received the questionnaire, it needed to be translated from English into Lithuanian.

Questionnaire:

At first, a literature search was performed to compare different questionnairs used in the assessment of HRQOL, in order to select the most suitable one for our study population. In the end, a questionnaire developed by Parkins et al (1997) was used. It was used to assess HRQOL in the Social, Emotional, Intellectual, Financial, Medical, Environmental, Physical, Recreational and Vocational Domains. Subjects were given a questionnaire with forty-four items which were needed to be completed by the children aged 5-12 years of age and fourty-seven items by the chidren aged 13-20 years of age which included all of the domains. The instructions were given for the items to be filled out fully and to “indicate how important each of these statements is to you when considering your quality of life...”.

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Each item had to be evaluated on a 5 point Likert scale according to importance. The scale ranged from 1 point for “Does not apply” till 5 points “Extremely Important”.

Sensibility: The questions used in this scale were short, simple and closed ended. The child answered

the questions in a direct manner. Afew children that were of the youngest age or had intellectual problems required some help from their parents and these parents were told to answer the questions 'from the child‟s point of view'. When the questionnaire was being translated into Lithuanian, some words and phrases were reconstructed to cause less confusion when the questions are answered and evaluated.

Questionnaire Scoring:All the questions were added up according to what score was chosen from the

5 point Likert Scale. Differentiation was not necessary and each question was given equal weighting. However, as a consequence of the inconsistency in the number of questions, the results were subscaled to values between 0 to 100, where 100 corresponds to perfect QOL, in order to allow for easy and accurate comparability of the statistical analysis.

Statistical Analysis: The results of the survey were examined and various statistical tests were

performed using SPSS version 23 software package. T-test was used to compare the mean in 2 groups and ANOVA to compare the mean scores in 3 or more groups along with a Bonferroni post Hoc tests. A P value less than 0.05 was used a statistically significant result.

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Results:

Population Characteristics:

A total of thirty one children with SB were interviewed during the period between July 2016 – February 2017. Twenty of these were aged between 5-12 years and eleven children were aged between 13-20 years.

As can be seen in Table 1, there were eighteen males and thirteen females included, the majority of which lived with their parents.The level of lesions were divided into four groups: upper (thoracic lesions), lumbar L1-2 and L3-5 and sacral lesions with a good spread between all levels. Using the “Hoffer Ambulation scale” sixteen children were non-ambulatory and fifteen were ambulatory. From the ambulatory patients, eight were able to walk normally, and the remaining seven ha limited mobility; either walking with aids or only part of physiotherapy guided exercises.

Table 1: Patients Characteristics.

Gender 1. Male 2. Females (n) 18 13 Lives With 1. Father/one parent 2. Guardians 3. Care Facility 27 1 3 Level of Lesion 1. Thoracic 2. L1-L2 3. L3-L5 4. Sacral 8 7 10 6

Walking (Hoffer Scale)

1. Do not walk

2. Walk only during physiotheraphy 3. Walk at home with aid

4. Walk in all environment with aid 5. Walk normally 16 2 1 4 8

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Surgically eighteen out of thirty one children have been treated for hyrdorcephalus while only four children for Chiari-II-Malformation. Five children suffered from Epilepsy. Eighteen children (58%) had spinal deformities four of which four were treated surgically. Eighteen children had hip deformities, three of which were treated surgically. The most common deformity was foot deformities with twenty one children (68%) affected, eleven of which had surgical treatment, as shown in Table 2.

Regarding urinary incontinence, 68% (twenty one children) were affected and52% (sixteen children) used urinary catheters. Onechild had a surgical bladder reconstruction. Fifteen children had fecal incontinence, while sixteen children suffered fromconstipation, of which six were conservatively treated with medication and four with a routine washout.

Table 2: Patients co-morbidities.

Hyrdorcephalus Tx Surgically 1. None 2. 1 or more times 13 18 Chiari-II-Malformation Tx Surgically 1. None 2. Yes 27 4 Bladder Catherization Tx 1. None

2. Yes (3 or more times)

15 16 Urinary Incontinence 1. None 2. Sometimes 3. Always 5 5 21 Urology Operation 1. None 2. Yes 30 1 Syringomegaly Tx Surgically 1. None 2. Yes 30 1

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2. Yes 5 Inserting Catheter 1. Alone 2. With help 11 23 Constipation 1. None 2. Tx with medication 3. Washouts 15 6 10 Spinal Deformities 1. None 2. Tx conservatively 3. Tx surgically 13 14 4

Hip Joint Deformities

1. None 2. Tx conservatively 3. Tx Surgically 13 14 4 Foot Deformities 1. None 2. Tx conservatively 3. Tx surgically 10 10 11

Based on the questionnare, mental retardation features were divided into 5 groups: none, mild, moderate, severe and very severe. Sixteen children had no intellectual disability, one child had mild retardation, one moderate retardation, and five had severe levels; Whilst in eight children, it was not defined.

None of the children in the study had any of the following comorbidities: chronic pyelonephritis, renal failure or tethered spinal cord injuries.

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HRQOL Questionnaire

When examining the results of the questionnaire and the various domains assessing quality of life, it was found that the overall highest scores were in the emotional, medical, intellectual, financial and social domains. Whereas the lowest sub-scores were in the recreational, vocational, environmental and then physical domains, as shown in Graph 1.

Graph 1:QOL in the different domains.

The emotional domain in both age groups scored relatively high. Children in this domain showed that they were able to express their emotions and feel comfortable about themselves. This was seen with patients scoring the highest in being able ”express his/her emotions?” and “feel positive about themselves”.

The medical domain scored highly in both age groups and there was an overall high satisfaction regarding the care and respect they receive in hospital, aswell as the level of understanding shown regarding their condition. The patients involved in the study scored high in the intellectual domain with an average of 79.58in the 5-12 age group, as shown in table 3. They were satisfied with their school programme, with all patients scoring full marks with ''receives praises for things that he/she is able to do? ''. 57,26 58,33 59,35 60,81 69,35 72,18 72,63 73,2 77,42 0 20 40 60 80 100 Recreational Vocational Environment Physical Financial Social Intelectual Emotional Medical Mean

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In the social domain the overall average score was 78; The children scored the highest in“support from the family” andscored lowest in the relation to having a 'special friend' or 'someone to confide to outside of their immediate family'.

The Recreational domain scored relatively high in the 5-12 age group, whereas it scored very low in the 13-20 age group (mean of 73 vs 29 respectively), as shown in table 3. This could perhaps be explained by the fact that only 25 % of children aged 5-12 were wheelchair bound, compared to 67% in age group 13-20. In the older age group they did not participate or feel challenged or encouraged through outdoor or indoor sport acitivities.

In addition the Environmental domain scored realtively low in both age groups. Children of all age groups did not feel that the outdoor resources were sufficient for their disabilities, scoring the lowest ''in the ablity to use and access public washrooms''.

In addition Vocational domain also scored relatively low in both age groups. However, children aged 5-12 felt they '' had the chance to study in things he/she is interested in'', scoring the highest in that question whereas children aged 13-20 had less hope, scoring relatively low in ''being able to get an education for a job that interests you ?''

In relation to the different age groups (5-12years vs 13-20years), the younger age group scored statistically significantly higher in the overal HRQOL (p=0.022); with the following domains showing statistical difference: Intellectual (p=0.034), Medical (p=0.037), Recreational (p=0.00), Vocational (p=0.032).

There was no difference in the HRQOL between different genders, with only the emotional domain showing statitical difference (P=0.030), with boys scoring lower in this domain.

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Table 3 : Comparison of HRQOL in the different age groups. (rescaled 0-100)

Level of Lesion & Co-Morbidities HRQOL.

Using the ANOVA one-way test for statistical analysis, we found some statistical difference between the level of lesion and the HRQOL in the following domains: vocational, recreational, environmental, physical and emotional. However, following the application of the Bonferroni correction, there was no statistically significant difference between the level of the lesion and the HRQOL.

In those with Chiari-II-Malformation there was no statistical difference in the HRQOL. However, there was signficant difference found in those with Hydrocephalus (p=0.002) , with only the social domain showing no difference between the groups.

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In relation to mobility and QOL, those unable to walk scored lower (p= 0.002). They scored significantly lower in the physical, recreational, environmental, medical and vocational domains. Regarding deformity, spinal and hip deformities had a statistically significant difference in QOL (p= 0.000 and p= 0.006 respectively) but there was no statistical difference in the overall QOL in those with knee or foot deformity. Spinal deformity affected all domains except for financial in the children's perspective, while the hip deformity mainly affected the physical, environmental, recreational and vocational domains. Foot deformity showed a statistical difference in the emotional, medical and social domains.

Patients who suffered with epilespy had a statistically significant lower QOL (p= 0.004), with all domains affected except for intellectual, recreational and environment.

The children which were affected by urinary incontinence (68%), had statistically significant lower QOL (p=0.000), with lower sub-scores in all domains except for financial. Nonetheless, requiring urinary catheterization did not appear to influence the QOL. Fecal incontinence also had negative effects on QOL but to a lower extent than urinary incontinence (p= 0.009) with only five out of the nine domains affected. With incontinence, the highest significant difference was in the emotional domain.

Furthermore, those who had no intellectual disability scored significantly higher in all domains (p=0.00)

Children with upper lesion “thoracic” had a lower QOL in the physical and recreational domain compared to lower level of lesions. The higher the lesion also resulted in the lower the scores for the vocational domain. In the recreational domain over 80% of the children with sacral and L3-5, aged between 5-12 years scored 5 and had no difficulties in participating in outdoor activities, going out on dates. This was seen in the vocatioanl domain where they scored highly regarding 'having the chance to continue to study the things he/she is interested in''. In the 13-20 years age group, similar findings were seen regarding the physical, recreational and vocational domains. However, interestingly it was found that in the children aged 13-20, those with lower lesions scored lower than those with higher lesions in the emotional domain.

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Discussion:

Our study is one of the first to look at HRQOL in children with SB in Lithuania. It is community based, focused on specific age groups which can have both pros and cons. The main drawback from this study is it's relatively small sample size which negatively affects the power of the study and therefore some potentially significant differences may not have been identified.

Although the questionnaire we use (the Parkins et al.1997) is a disease-specific questionnaire that was developed for patients with SB and is more relevant than other questionnaires, it also however, has some limitations especially when used to assess the physical domain in children that are not mobile. Several questions are not appropriate to measure the physical function of individuals who use wheelchair, which may have led to biased lower scores who are reliant upon wheelchairs for mobility. Furthermore, the environment also had some influnece on certain questions. For example, problems with showering or bathing may be a combination of financial and physical capability rather than just a physical issue alone. Unfortunately,, it was not possible to differentiate influences from the environment on the results.

Regarding the level of lesions,there was a good but not equal spread of patients with the various levels of lesions. We found no statistical difference between the level of the lesions which is not consistent with some of the other studies, which may be due to the power of the study. One study showed that the QOL scores decreased in the L2 and above lesions compared with L3-L5.(18) One main limitation in this study is the fact that only the parents were interviewed and therefore the QOL scores are from the parents„ own perceptions and this could have signficantly affected the results. Another study evaluating '' Health Outcomes among youths and adults with spina bifida'' also showed significant difference, with the lower levelof lesions having a better QOL. This study used both HUI3 and AQoL questionnaires to assess the QOL, allowing it to have a better mean score compared to our study. (19)

Our study also suggested that co-morbidities including both urinary and fecal incontinence are indeed associated with a decreased QOL. This was the same case in other studies, eventhough different questionnaires were used. (9)(20) However one study “Quality of Life and continence in patients with spina bifida“ did not show any significance between urinary incontinence and QOL.(21)

This might be due to the population of the study with 65% being 18 years of ageand older. Although significant

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improvement to QOL was shown in their study for those with surgical interventions for continent diversion. Our study was not able to show this difference due to the small sample size as we only had one child with surgical diversions compared to the large sample size of the other study (460 patients).

Also, other co-morbidities such as epilepsy decreased the QOL, which reiterates how being affected with multiple conditions can be very challenging to the indivdual and negatively attenuates the QOL. Unlike other studies, we found that having Chiari-II-Malformation did not make any statistical difference to the QOL, while Hydrocephalus did. A potential bias could have resulted from the sample size as only four patients (13%) had Chiari-II-Malformation, compared to eighteen children (58%) that had hydrocephalus and surgery.

Regarding ambulation, the ambulatory group scored significantly higher in the overall QOL, which was expected. There was a statistically significant difference in the physical domain, despite the fact that the questionannaire as mentioned was not sensitive to assess physical domain in patients that are wheelchair bound, and therefore the difference might actually still be underestimated. Furthermore, because of the small numbers in our study we did not differentiate between those that are mobile with aid in our analysis. However one study “Self-Reported health-related quality of life in children and adolescents with myelomeningocele“ showed no significant difference between the QOL and the ambulatory group.(22) Their population sample was relatively small for wheelchair bound users, with only seven participants compared to thirty eight subjects being able to walk, which could have had a significant impact on the results.(22)

In relation to the physical and vocational domains, they received some of the lowest scores compared to the other domains and this is consistintly seen with other studies.(23) Although some other studies showed an influence between resource availability and QOL, our sub-scores for 'financial' domain were good but we had poor scores for the 'environment' domain, where it ranked the second lowest. This can be seen to relate indirectly to resource availability and funding for more making public areas and services more suitable for patients with disabilities and suggests potential need for improvement.

Our patient characteristics that were identified were not comprehensive. For example, we did not have any data on obesity for our study, but some other studies have shown a relationship between obesity,

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SB and HRQOL, with a negative impact on various domains. Abresch RT documented in his study that obesity had a significant impact in the QOL with lower scores found in the physical, social and emotional domains. (8)Furthermore, there are no studies or evidence as to whether or not interventions to improve weight or fitness in children with SB would lead to a better HRQOL in the long-run. In addition, this study did not have a healthy control group of children to compare the QOL. Therefore, recommendations for future studies would include these as part of their assessment.

Conclusion:

1. Positive results were obtained regarding the medical aspects of patients with SB in Lithuania as they scored high in this domain. However, the environment and vocational domains scored low, suggesting further examination needs to be carried in these domains. 2. We concluded, that having various co-morbidities including; hydrocephalus and

incontinence all have negative impacts on the QOL. Patients whom suffered from epilespy had a statistically significant lower QOL.

3. No significant difference was found in the association between the level of lesion and QOL in our study.

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Literature List:

1.

Elena Slobodyanik. Gerosios patirties partnerystė tarp Lietuvos ir Norvegijos Spina Bifida asociacijų. Spina Bifida and Hydrocephalus. Pirmadienis, 2016 gruodžio 05 10:10. Available from:http://www.sbh.lt/index.php/44-gerosios-patirties-partneryste-tarp-lietuvos-ir-norvegijos-spina-bifida-asociaciju [Accessed 2nd of December 2016].

2.

Lisa J. Pruitt.Living with spina bifida: a historical perspective. Pediatrics. 2012 Aug;130(2):181-3. Available from: doi: 10.1542/peds.2011-2935. [Accessed 2nd of December 2015].

3.

Samantha E. Parker,Cara T. Mai,Mark A. Canfield,Russel Rickard,Ying Wang,Robert E.

Meyer,Patrick Anderson,Craig A. Mason,Julianne S. Collins,Russell S. Kirby,and Adolfo Correafor the National Birth Defects Prevention Network. Updated National Birth Prevalence Estimates for selected birth defects in the United States. Birth Defects Research Part A Clinical and Molecular Teratolgy. 2010 Dec;88(12):1008-16. Available from: doi: 10.1002/bdra.20735. [Accessed 4th of December 2015].

4.

Pippa Oakeshott, Gillian M Hunt, Alison Poulton, Fiona Reid. Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study.

Developmental Medicine and Child Neurolgy. 2010 Aug;52(8):749-53. Available from: doi: 10.1111/j.1469-8749.2009.03543.x. [Accessed 5th of December 2015].

5.

Liptak GS, El Samra A. Optimizing health care for children with spina bifida. Developmental Disabilities Research Reviews 2010;16(1):66-75. Available from: doi: 10.1002/ddrr.91. [Accessed 10th of December 2015].

6.

Esther Cornegé-Blokland, H. E. Jansen, C. C. E. de Jong-de Vos van Steenwijk, Dan Poenaru. Quality of life of children with spina bifida in Kenya is not related to the degree of the spinal defects.Tropical Medicine & International Health. 2011 Jan;16(1):30-6. Available from: doi: 10.1111/j.1365-3156.2010.02680.x. [Accessed 15th of December 2015].

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7.

Jack M. Fletcher, Timothy J. Brei. Introduction: Spina Bfida a multidisciplinary perspective. Developmental Disabilities Research Reviews.2010; 16(1): 1–5. Available from: doi:

10.1002/ddrr.101. [Accessed 16th of December 2015].

8.

Abresch RT, McDonald DA, Widman LM, McGinnis K, Kathryn J. Hickey. Impact of spinal cord dysfunction and obesity on the Health-Related Quality of Life of Children and Adolescents. The Journal Spinal Cord Medicine. 2007;30 Suppl 1:S112-8. [Accessed 7th of January 2016].

9.

Åsa Bartonek, Helena Saraste, Aina Danielsson. Health-related quality of life and ambulation in children with myelomeningocele in a Swedish population. Acta Paediatrica. 2012 Sep;101(9):953-6. Available from: doi: 10.1111/j.1651-2227.2012.02742.x. [Accessed 10th of January 2016].

10.

Ineke M Pit-ten Cate MSc, Colin Kennedy MD,Jim Stevenson PhD. Disability and quality of life in spina bifida and hydrocephalus. Developmental Medicine Child Neurolgy. 2002 May;44(5):317-22. [Accessed 12th of January 2016].

11.

Jamie D Olesen, Darcie A Kiddoo, Peter D Metcalfe. The association between urinary continence and quality of life in paediatric patients with spina bifida and tethered cord.Paediatrics Child Health. 2013 Aug-Sep; 18(7): e32–e38. [Accessed 14th of January 2016].

12.

Brandon G. Rocque, MD, E. Ralee‟ Bishop, BS, Mallory A. Scogin, MD, Betsy D. Hopson, MSHA, Anastasia A. Arynchyna,Christina J. Boddiford, Chevis N. Shannon and Jeffrey P. Blount. Assessing health-related quality of life in children with spina bifida.Journal of Neurosurgery: PediatricsFeb 2015 / Vol. 15 / No. 2 / Pages 144-149. [Accessed 15th of January 2016].

13.

Pedro L Ferreira, Carla F Baltazar, Luís Cavalheiro, Jan Cabri and Rui S Gonçalves. Reliability and validity of PedsQL for Portuguese children aged 5–7 and 8–12 years. Health and Quality of Life Outcomes. 2014 Sep 11;12:122. Available from: doi: 10.1186/s12955-014-0122-3.[Accessed 22nd of January2016].

(29)

14.

Saskia Vande Velde & Jolien Laridaen & Eline Van Hoecke & Stephanie Van Biervliet & Ruth De Bruyne & Myriam Van Winckel & Liesbet Goubert. Development and validation of a spina bifida-specific pediatric quality of life questionnaire: the Spina Bifida Pediatric Questionnaire, SBPQ. Childs Nervous System. 2016 Jan;32(1):105-10. Available from: doi: 10.1007/s00381-015-2903-3. [Accessed 26th of January 2016].

15.

P. C. Parkin,H. M. Kirpalani, P. L. Rosenbaum, D. L. Fehlings, A. Van Nie, A. R. Willan and D. King. Development of a health-related quality of life instrument for use in children with spina bifida. Qual Life Res. 1997 Mar;6(2):123-32. [Accessed 3rd of July 2016].

16.

Rocque BG, Bishop ER, Scogin MA, Hopson BD, Arynchyna AA, Boddiford CJ, Shannon CN, Blount JP. Assessing health-related quality of life in children with spina bifida. Journal of

Neurosurgery Pediatrics. 2015 Feb;15(2):144-9. Available from: doi: 10.3171/2014.10.PEDS1441. [Accessed 4th of July 2016].

17.

A. Khoshbin, L. Vivas, P. W. Law, D. Stephens, A. M. Davis, A. Howard, J. G. Jarvis, J. G. Wright. The long-term outcome of patients treated operatively and non-operatively for scoliosis deformity secondary to spina bifida. The Bone & Joint Journal. 2014 Sep;96-B(9):1244-51. Available from: doi: 10.1302/0301-620X.96B9.33857. [Accessed 5th of July 2016].

18.

Flanagan A, Gorzkowski M, Altiok H, Hassani S, Ahn KW. Activity Level, Functional Health, and Quality of Life of Children with Myelomeningocele as Perceived by Parents. Clinical Orthopaedics and Related Research. 2011 May;469(5):1230-5. Available from: doi: 10.1007/s11999-010-1651-7. [Accessed 6th of July 2016].

19.

Yong NL, Sheridan K, Burke TA, Mukherjee S, McCormick A. Health Outcomes among youths and adults with spina bifida. Journal of Pediatrics. 2013 May;162(5):993-8. Available from: doi: 10.1016/j.jpeds.2012.10.042. [Accessed 7th of July 2016].

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20.

Bahriye Türkücüoğlu and Tülay Tarsuslu Şimşek. Relationship Between Functional Level and Quality of Life in Children with Spina Bfida. Clinical Orthopaedics Related Research. 2011 May; 469(5): 1230–1235. Published online 2010 Nov 2. Available from:doi: 10.1007/s11999-010-1651-7. [Accessed 8th of July 2016].

21.

J.L. Lemelle F. Guillemin, D. Aubert, J.M. Guys, H. Lottmann, S. Lortat-Jacob, P. Mouriquand, A. Ruffion, J. Moscovici & M. Schmitt. Quality of life and continence in patients with spina

bifida.Quality of Life Research. 2006 Nov;15(9):1481-92.[Accessed 10th of July 2016].

22.

Müller-Godeffroy E, Michael T, Poster M, Seidel U, Schwarke D, Thyen U. Self-Reported health-related quality of life in children and adolescents with myelomeningocele.Developmental Medicine & Child Neurolgy. 2008 Jun;50(6):456-61.Available from: doi: 10.1111/j.1469-8749.2008.02054.x. [Accessed 12th of July 2016].

23.

Jia-Chi Wang & Chih-Jou Lai & Tai-Tong Wong & Muh-Lii Liang & Hsin-Hung Chen & Rai-Chi Chan & Tsui-Fen Yang. Health-related quality of life in children and adolescents with spinal dysraphism: results from a Taiwanese sample. Child's Nervous System2013 Sep;29(9):1671-9. Available from: doi: 10.1007/s00381-013-2117-5. [Accessed 13th of July 2016].

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Annex:

Health Variables and level of comorbodities Questionnaire

1. Date of Birth: ___________ 2. Date of Consultation:________ 3. Gender: 1.Male 2. Female

4. Lives With: 1.Father/ One of the Parents 2.Guardian 3. Care Facility

5. Spinal cord Injury level: 1. Chest 2. Waist L1-L2 3. Lumbar L3-L5 4. Sacral 6. Hydrocephalus (treated surgically): 0. No 1. How many times (if yes)

7. Chiari II Malformations (treated surgically):0. No 1.Yes 8. Syringomegaly (treated surgically): 0. No 1.Yes

9. Tethered SpinalCord Injury ( treated surgically): 0. No 1.Yes 10. Epilepsy (treated with drugs): 0. No 1.Yes

11. Bone Fractures: 0. No 1. How many time (if yes)

12. Bladder Catheterization:0. None 1. For nr. Years (if yes) 13. Inserting Bladder Catherization: 1. Alone 2. With help

14. Urinary Incontinence: 0. None 1. Uses security measures sometimes 2. Always use protective equipment

15. Chronic Pyelonephritis: 0.None 1. For nr. of Years (if yes) 16. Renal Failure: 0.None 1. For nr. Years(if yes)

17. Urology Operation: 0. None 1. Bladder Augmentation 2. Suprapubic Cystoscopy 3. Bladder Augmentation + Suprapubic Cystoscopy

18. Constipation: 0.None 1. Treated Medically 2. Enema not regularly 3. Enema regularly (anterograde) 4. Enema regularly (retrograde) 5. Manual Eradication

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19. Walking (Hoffer Scale): 0. Do not walks 1. Walks only during physiotheraphy 2. Walks at home with Aid 3. Walks in all environment with Aid 4. Walks normally

20. Spinal Deformities: 0. None 1.Treated conservatively 2. Treated Surgically 21. Hip Deformities: 0. None 1.Treated conservatively 2. Treated Surgically 22. Foot Deformites: 0. None 1.Treatet conservatively 2. Treated Surgically

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Results

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