Neurogenic bladder and bowel dysfunction - incontinence and life situation in adolescents and adults with spina bifida Magdalena Vu Minh Arnell Department of Pediatrics Institute of Clinical Sciences Sahlgrenska Academy, University of Gothenburg Gothenburg 2021 Neurogenic bladder and bowel dysfunction ̶ incontinence and life situation in adolescents and adults with spina bifida © Magdalena Vu Minh Arnell 2021 magdalena.vuminh-arnell@vgregion.se ISBN 978-91-8009-148-0 (PRINT) ISBN 978-91-8009-149-7 (PDF) http://hdl.handle.net/2077/66818 Printed by Stema specialtryck AB, Borås To all the fantastic children and families who I have had the privilege of meeting and getting to know and cooperate with in my role as a pediatric nurse and urotherapist. Trycksak 3041 0234 SV AN ENMÄRKET Trycksak 3041 0234 SV AN ENMÄRKET Neurogenic bladder and bowel dysfunction ̶ incontinence and life situation in adolescents and adults with spina bifida © Magdalena Vu Minh Arnell 2021 magdalena.vuminh-arnell@vgregion.se ISBN 978-91-8009-148-0 (PRINT) ISBN 978-91-8009-149-7 (PDF) http://hdl.handle.net/2077/66818 Printed by Stema specialtryck AB, Borås To all the fantastic children and families who I have had the privilege of meeting and getting to know and cooperate with in my role as a pediatric nurse and urotherapist. Neurogenic bladder and bowel dysfunction - incontinence and life situation in adolescents and adults with spina bifida Magdalena Vu Minh Arnell Department of Pediatrics, Institute of Clinical Sciences Sahlgrenska Academy, University of Gothenburg, Sweden ABS T RACT BACKGROUND: In individuals with myelomeningocele (MMC), survival rate has significantly increased over the past 40-50 years due to medical innovations. In the coming years, many adolescents will be transferred to adult care. AIMS: To investigate life situation, quality of life, follow-up in adult care and the incidence and experience of incontinence in adults with MMC. To compare the incidence of incontinence with a youth group and investigate whether continence is one of the prerequisites for an active life and close intimate relationships. METHODS AND RESULTS: Study I Sixty-nine adults (27– 50 years) with MMC participated in a structured telephone interview. Pads for urinary and fecal incontinence were used by 87% and 14% had contact with a urotherapist. About 60% were single, 90% had attended high school and 67% had a job. Study II The validated HRQoL instrument SF-36 was answered by 61 of 69 individuals. There were significantly lower scores for the overall physical quality of life while scores for the overall mental quality of life were higher than for the reference group. Neither physical nor mental quality of life was affected by whether the individual had fecal incontinence, lived with a partner or had children. Study IV In a descriptive qualitative semi-structured interview, 9 adults described their personal experience of living with incontinence. Study III All 16-18-year old’s with MMC (25) from west ern Sweden were included in a prospective cross-sectional study regarding urinary and fecal incontinence as well of life situation. All were followed according to the national care program and 68% (17/25) were urinary continent. Of these, 12 had an active social life and 8 had experience of having a partner. Of the 8 with incontinence, none had an active social life or a close physical intimate relationship. CONCLUSION: Incontinence is common in adults with MMC and few have uro- therapy support. Life situation and incontinence are not reflected in the generic HRQoL instrument SF-36. The adults' experience of how incontinence affects life is consistent with reports from individuals with acquired incontinence. In the adolescent study, the results indicate the importance of follow-up and active treatment strategies to achieve urinary continence. Continence seems to be a success factor for an active social life and close intimate relationships. KEYWORDS: spina bifida, myelomeningocele, adults, life situation, follow-up, HRQoL adolescents, continence, incontinence ISBN 978-91-8009-148-0 (PRINT) ISBN 978-91-8009-149-7 (PDF) http://hdl.handle.net/2077/66818 Neurogenic bladder and bowel dysfunction - incontinence and life situation in adolescents and adults with spina bifida Magdalena Vu Minh Arnell Department of Pediatrics, Institute of Clinical Sciences Sahlgrenska Academy, University of Gothenburg, Sweden ABS T RACT BACKGROUND: In individuals with myelomeningocele (MMC), survival rate has significantly increased over the past 40-50 years due to medical innovations. In the coming years, many adolescents will be transferred to adult care. AIMS: To investigate life situation, quality of life, follow-up in adult care and the incidence and experience of incontinence in adults with MMC. To compare the incidence of incontinence with a youth group and investigate whether continence is one of the prerequisites for an active life and close intimate relationships. METHODS AND RESULTS: Study I Sixty-nine adults (27– 50 years) with MMC participated in a structured telephone interview. Pads for urinary and fecal incontinence were used by 87% and 14% had contact with a urotherapist. About 60% were single, 90% had attended high school and 67% had a job. Study II The validated HRQoL instrument SF-36 was answered by 61 of 69 individuals. There were significantly lower scores for the overall physical quality of life while scores for the overall mental quality of life were higher than for the reference group. Neither physical nor mental quality of life was affected by whether the individual had fecal incontinence, lived with a partner or had children. Study IV In a descriptive qualitative semi-structured interview, 9 adults described their personal experience of living with incontinence. Study III All 16-18-year old’s with MMC (25) from west ern Sweden were included in a prospective cross-sectional study regarding urinary and fecal incontinence as well of life situation. All were followed according to the national care program and 68% (17/25) were urinary continent. Of these, 12 had an active social life and 8 had experience of having a partner. Of the 8 with incontinence, none had an active social life or a close physical intimate relationship. CONCLUSION: Incontinence is common in adults with MMC and few have uro- therapy support. Life situation and incontinence are not reflected in the generic HRQoL instrument SF-36. The adults' experience of how incontinence affects life is consistent with reports from individuals with acquired incontinence. In the adolescent study, the results indicate the importance of follow-up and active treatment strategies to achieve urinary continence. Continence seems to be a success factor for an active social life and close intimate relationships. KEYWORDS: spina bifida, myelomeningocele, adults, life situation, follow-up, HRQoL adolescents, continence, incontinence ISBN 978-91-8009-148-0 (PRINT) ISBN 978-91-8009-149-7 (PDF) http://hdl.handle.net/2077/66818 SAMMANFATTNING PÅ SVENSKA Hos vuxna med ryggmärgsbråck har överlevnaden ökat signifikant under de senaste 40–50 åren på grund av medicinska innovationer som t.ex. förbättrade operationsmetoder vid hydrocefalus och bevarande av njurfunktionen med hjälp av Ren Intermittent Kateterisering (RIK). Många ungdomar överförs därför nu till vuxenvården med ett stort behov av livslång vård och uppföljning av ett multiprofessionellt team. Urin- och avföringsinkontinens är mycket vanligt men ses ofta inte som orsak till behandling eller uppföljning. Syftet med avhandlingen var att hos vuxna individer med ryggmärgsbråck undersöka livssituationen, hälsorelaterad livskvalitet, förekomst av inkontinens, uro- logisk och uroterapeutisk uppföljning och upplevelsen av att leva med urin- inkontinens. Syftet var också att jämföra förekomst av inkontinens mellan vuxna och en ungdomsgrupp i åldern 16– 18 år och i den senare även undersöka om kontinens är en av förutsättningarna för ett aktivt liv och nära intima relat- ioner. Studie I Sextionio individer (27–50 år) med ryggmärgsbråck deltog i en struk- turerad telefonintervju. Av deltagarna använde 87% skydd för urin och/eller avföringsläckage och 14% hade kontakt med uroterapeut, drygt 60% var ensamstående, 90% hade gått på gymnasiet och 67% hade arbete. Av de individer i studien som inte genomgått någon urologisk operation följdes 69% sporadiskt eller inte alls inom vuxenvården. Studie II Det validerade HRQoL-instrumentet SF-36 besvarades av 61 av 69 individer. Resultaten visade att studiegruppen hade signifikant lägre poäng i fysisk funktion, allmän hälsa och övergripande fysisk livskvalitet jämfört med referensgruppen. Den övergripande livskvaliteten för psykisk hälsa var högre än för referensgruppen. Varken fysisk eller psykisk livskvalitet påverkades av om individen hade avföringsinkontinens, partner eller barn. Studie IV I en beskrivande kvalitativ semistrukturerad intervju berättar 9 individer från samma kohort som i studie I och II om sina personliga upp- levelser av att leva med inkontinens. Studie III Alla 16–18- åringar med ryggmärgsbråck (25 individer) från västra Sverige ingick i en prospektiv tvärsnittsstudie angående urin- och avförings- inkontinens samt deras livssituation. Alla följdes enligt ett nationellt vård- program. Av de 17 ungdomar (68%) som var urinkontinenta hade 12 ett aktivt socialt liv och 8 hade erfarenhet av att ha en partner. Av de 8 ungdomarna med urininkontinens hade ingen ett aktivt socialt liv, partner eller en nära intim relation. Sammanfattnin gsvi s tyder studierna på att inkontinens är vanligt före- kommande hos vuxna med ryggmärgsbråck och att få har stöd av uroterapeut. Livssituation och inkontinens återspeglas inte i det generiska HRQoL instru- mentet SF-36. De vuxnas upplevelse om hur inkontinens påverkar livet överensstämmer med rapporter från individer med förvärvad inkontinens. Individer med medfödd inkontinens verkar behöva samma tillgång till behand- ling som individer med förvärvad inkontinens. I tonårsstudien indikerar resul- taten på vikten av uppföljning och aktiva behandlingsstrategier för att uppnå urinkontinens. Kontinens verkar vara en framgångsfaktor för ett aktivt socialt liv och nära intima relationer. Nyckelord: ryggmärgsbråck, vuxna, ungdomar, livssituation, livskvalitet, uppföljning, kontinens, inkontinens ISBN978-91-8009-148-0(PRINT) ISBN978-91-8009-149-7(PDF) SAMMANFATTNING PÅ SVENSKA Hos vuxna med ryggmärgsbråck har överlevnaden ökat signifikant under de senaste 40– 50 åren på grund av medicinska innovationer som t.ex. förbättrade operationsmetoder vid hydrocefalus och bevarande av njurfunktionen med hjälp av Ren Intermittent Kateterisering (RIK). Många ungdomar överförs därför nu till vuxenvården med ett stort behov av livslång vård och uppföljning av ett multiprofessionellt team. Urin- och avföringsinkontinens är mycket vanligt men ses ofta inte som orsak till behandling eller uppföljning. Syftet med avhandlingen var att hos vuxna individer med ryggmärgsbråck undersöka livssituationen, hälsorelaterad livskvalitet, förekomst av inkontinens, uro- logisk och uroterapeutisk uppföljning och upplevelsen av att leva med urin- inkontinens. Syftet var också att jämföra förekomst av inkontinens mellan vuxna och en ungdomsgrupp i åldern 16– 18 år och i den senare även undersöka om kontinens är en av förutsättningarna för ett aktivt liv och nära intima relat- ioner. Studie I Sextionio individer (27– 50 år) med ryggmärgsbråck deltog i en struk- turerad telefonintervju. Av deltagarna använde 87% skydd för urin och/eller avföringsläckage och 14% hade kontakt med uroterapeut, drygt 60% var ensamstående, 90% hade gått på gymnasiet och 67% hade arbete. Av de individer i studien som inte genomgått någon urologisk operation följdes 69% sporadiskt eller inte alls inom vuxenvården. Studie II Det validerade HRQoL-instrumentet SF-36 besvarades av 61 av 69 individer. Resultaten visade att studiegruppen hade signifikant lägre poäng i fysisk funktion, allmän hälsa och övergripande fysisk livskvalitet jämfört med referensgruppen. Den övergripande livskvaliteten för psykisk hälsa var högre än för referensgruppen. Varken fysisk eller psykisk livskvalitet påverkades av om individen hade avföringsinkontinens, partner eller barn. Studie IV I en beskrivande kvalitativ semistrukturerad intervju berättar 9 individer från samma kohort som i studie I och II om sina personliga upp- levelser av att leva med inkontinens. Studie III Alla 16– 18-åringar med ryggmärgsbråck (25 individer) från västra Sverige ingick i en prospektiv tvärsnittsstudie angående urin- och avförings- inkontinens samt deras livssituation. Alla följdes enligt ett nationellt vård- program. Av de 17 ungdomar (68%) som var urinkontinenta hade 12 ett aktivt socialt liv och 8 hade erfarenhet av att ha en partner. Av de 8 ungdomarna med urininkontinens hade ingen ett aktivt socialt liv, partner eller en nära intim relation. Sammanfattnin gsvi s tyder studierna på att inkontinens är vanligt före- kommande hos vuxna med ryggmärgsbråck och att få har stöd av uroterapeut. Livssituation och inkontinens återspeglas inte i det generiska HRQoL instru- mentet SF-36. De vuxnas upplevelse om hur inkontinens påverkar livet överensstämmer med rapporter från individer med förvärvad inkontinens. Individer med medfödd inkontinens verkar behöva samma tillgång till behand- ling som individer med förvärvad inkontinens. I tonårsstudien indikerar resul- taten på vikten av uppföljning och aktiva behandlingsstrategier för att uppnå urinkontinens. Kontinens verkar vara en framgångsfaktor för ett aktivt socialt liv och nära intima relationer. Nyckelord: ryggmärgsbråck, vuxna, ungdomar, livssituation, livskvalitet, uppföljning, kontinens, inkontinens ISBN978-91-8009-148-0(PRINT) ISBN978-91-8009-149-7(PDF) i LIST OF PAPERS This thesis is based on the following studies, referred to in the text by their Roman numerals. I. Vu Minh Arnell M, Seljee Svedberg K, Lindehall B, Jodal U, Abrahamsson K. Adults with myelomeningocele: An interview study about life situation and bladder and bowel management. J Pediatr Urol. 2013 Jun;9(3):267-71. II. Vu Minh Arnell M, Seljee Svedberg K, Lindehall B, Möller A, Abrahamsson K. Health-related quality of life compared to life situation and incontinence in adults with myelomeningocele: Is SF-36 a reliable tool? J Pediatr Urol. 2013 Oct;9(5):559-66. III. Vu Minh Arnell M, Abrahamsson K. Urinary continence appears to enhance social participation and intimate relations in adolescents with myelomeningocele. J Pediatr Urol. 2019 Feb;15(1):33. e1-33. e6. IV. Vu Minh Arnell M, Korsgaard R, Abrahamsson K. The voice of adults with myelomeningocele - experience of urinary incontinence and how it affects life. In manuscript. i LIST OF PAPERS This thesis is based on the following studies, referred to in the text by their Roman numerals. I. Vu Minh Arnell M, Seljee Svedberg K, Lindehall B, Jodal U, Abrahamsson K. Adults with myelomeningocele: An interview study about life situation and bladder and bowel management. J Pediatr Urol. 2013 Jun;9(3):267-71. II. Vu Minh Arnell M, Seljee Svedberg K, Lindehall B, Möller A, Abrahamsson K. Health-related quality of life compared to life situation and incontinence in adults with myelomeningocele: Is SF-36 a reliable tool? J Pediatr Urol. 2013 Oct;9(5):559-66. III. Vu Minh Arnell M, Abrahamsson K. Urinary continence appears to enhance social participation and intimate relations in adolescents with myelomeningocele. J Pediatr Urol. 2019 Feb;15(1):33. e1-33. e6. IV. Vu Minh Arnell M, Korsgaard R, Abrahamsson K. The voice of adults with myelomeningocele - experience of urinary incontinence and how it affects life. In manuscript. ii iii CONTENT ABBREVIATIONS .............................................................................................. V DEFINITIONS IN SHORT .................................................................................. VII 1 INTRODUCTION ........................................................................................... 1 Myelomeningocele, MMC ......................................................................... 2 Normal bladder function ............................................................................ 3 Neurogenic bladder dysfunction ................................................................ 4 Normal bowel function ............................................................................... 5 Neurogenic bowel dysfunction ................................................................... 6 Urotherapy .................................................................................................. 7 Life situation .............................................................................................. 9 Cognitive function .................................................................................... 10 2 AIM ........................................................................................................... 11 3 PARTICIPANTS AND METHODS ................................................................. 13 4 RESULTS AND DISCUSSION ....................................................................... 19 5 GENERAL DISCUSSION AND FUTURE PERSPECTIVES ................................. 27 6 CONCLUSION ............................................................................................ 31 ACKNOWLEDGEMENTS .................................................................................. 33 REFERENCES .................................................................................................. 35 APPENDIX ...................................................................................................... 45 ii iii CONTENT ABBREVIATIONS .............................................................................................. V DEFINITIONS IN SHORT .................................................................................. VII 1 INTRODUCTION ........................................................................................... 1 Myelomeningocele, MMC ......................................................................... 2 Normal bladder function ............................................................................ 3 Neurogenic bladder dysfunction ................................................................ 4 Normal bowel function ............................................................................... 5 Neurogenic bowel dysfunction ................................................................... 6 Urotherapy .................................................................................................. 7 Life situation .............................................................................................. 9 Cognitive function .................................................................................... 10 2 AIM ........................................................................................................... 11 3 PARTICIPANTS AND METHODS ................................................................. 13 4 RESULTS AND DISCUSSION ....................................................................... 19 5 GENERAL DISCUSSION AND FUTURE PERSPECTIVES ................................. 27 6 CONCLUSION ............................................................................................ 31 ACKNOWLEDGEMENTS .................................................................................. 33 REFERENCES .................................................................................................. 35 APPENDIX ...................................................................................................... 45 iv v A BBREVIATIONS Ch Charrière catheter size, corresponds to millimeters in circumference CIC FCC Clean Intermittent Catheterization Family Centered Care HRQoL MACE Health Related Quality of Life Malone Antegrade Continence Enema MCS Mental component summary MMC NTD PCS SF-36 Myelo Meningo Cele Neural tube defects Physical component summary The Short Form (36) Health Survey iv v A BBREVIATIONS Ch Charrière catheter size, corresponds to millimeters in circumference CIC FCC Clean Intermittent Catheterization Family Centered Care HRQoL MACE Health Related Quality of Life Malone Antegrade Continence Enema MCS Mental component summary MMC NTD PCS SF-36 Myelo Meningo Cele Neural tube defects Physical component summary The Short Form (36) Health Survey vi vii D EFINITIONS IN SHORT Ability to walk Ambulatory everywhere or just walking indoor Ambulatory ability Walking without the need of a wheelchair Assistance Practical help Bodily pain Pain magnitude Clean Intermittent Catheterization The bladder is emptied regularly 4-6 times a day, with a clean catheter Dysphoria Interviewees is weeping during the interview or by the individual stating that he/she is depressed Emotional role Cut down time, accomplished less Fecal continence Dryness between regimens not including dripping of enema solution in a period up to 2 h after the enema was performed General health Sick easier, excellent health Independence in CIC and fecal elimination regimens Physically performing the total procedure without need of assistance Intimate relations Intimacy without sexual intercourse Mental health Nervous, happy, peaceful Micturition chart Volume of urine at each CIC and fluid were measured for 2 days Participation in social life At least twice a week spending free time with friends and being able to stay overnight when desired, without family or assistant attending. Partner Person with whom the individual can have intimate relations and/or sexual intercourse vi vii D EFINITIONS IN SHORT Ability to walk Ambulatory everywhere or just walking indoor Ambulatory ability Walking without the need of a wheelchair Assistance Practical help Bodily pain Pain magnitude Clean Intermittent Catheterization The bladder is emptied regularly 4-6 times a day, with a clean catheter Dysphoria Interviewees is weeping during the interview or by the individual stating that he/she is depressed Emotional role Cut down time, accomplished less Fecal continence Dryness between regimens not including dripping of enema solution in a period up to 2 h after the enema was performed General health Sick easier, excellent health Independence in CIC and fecal elimination regimens Physically performing the total procedure without need of assistance Intimate relations Intimacy without sexual intercourse Mental health Nervous, happy, peaceful Micturition chart Volume of urine at each CIC and fluid were measured for 2 days Participation in social life At least twice a week spending free time with friends and being able to stay overnight when desired, without family or assistant attending. Partner Person with whom the individual can have intimate relations and/or sexual intercourse viii Physical functioning Mobility Physical role Limitations caused by physical health problems Reminder Help to remember the time for CIC Sexual debut First occasion of sexual intercourse Social functioning Social excellent, social time Urinary continence Totally dry during day and night without need of incontinence pads Vitality Energy, tiered Young age 20-29 years of age ix THESIS AT A GLANCE AIM DESIGN/METHOD RESULTS CONCLUSION I To asses life situation, bladder and bowel management in adults with MMC after transferal to adult care. Cross-sectional cohort study with 69 adults participated in an individual structured interview. Of the individuals 90% had passed high school or had university education. Fifty-three (77%) had their own apartment. CIC was used by 71%. Of those with no urological operation, 31% had a consultation with urologist every 3 years. The corresponding number for those operated on were 53%. Few of the participants had urotherapy support. The majority used pads, and none had support with their fecal elimination regimen. If pads were used it was harder to get employed. About 60% were single. II To investigate if life situation and incontinence in adults with MMC are reflected in HRQoL. Cross-sectional, cohort study with 61of 69 from study I who answered the generic Health related Quality of life instrument SF-36. The individuals had lower scores in the overall physical quality of life but higher scores in the overall mental quality of life. Neither physical nor mental quality of life was affected by whether the individual had fecal incontinence, lived with a partner or had children. III To evaluate urinary continence in adolescents with MMC, involved in a urotherapy/urology program and if urinary continence is one condition to enable social participation and close physical intimacy. Cross-sectional, quantitative, cohort study. A structured individual interview with 25 adolescents, were conducted. Prospective investigation about incontinence was implemented. Seventeen of 25 (68%) were urinary continent. Of these, 12 had an active social life and 8 had experience of having a partner. Eight individuals were urinary incontinent. None of them had an active social life or a close physical intimate relationship. The importance of follow-up and active treatment strategies to achieve urinary continence seem to be one of the predictors for having an active social life and the possibility of close physical intimate relationship. IV To describe experience of urinary incontinence and how incontinence affects life. Cross-sectional, descriptive qualitative semi structured interview with 9 individuals from the cohort in study I and II. The participants described similar experiences as individuals with acquired urinary incontinence. Individuals with congenital incontinence seem to need the same access to treatment as individuals with acquired urinary incontinence. viii Physical functioning Mobility Physical role Limitations caused by physical health problems Reminder Help to remember the time for CIC Sexual debut First occasion of sexual intercourse Social functioning Social excellent, social time Urinary continence Totally dry during day and night without need of incontinence pads Vitality Energy, tiered Young age 20-29 years of age ix THESIS AT A GLANCE AIM DESIGN/METHOD RESULTS CONCLUSION I To asses life situation, bladder and bowel management in adults with MMC after transferal to adult care. Cross-sectional cohort study with 69 adults participated in an individual structured interview. Of the individuals 90% had passed high school or had university education. Fifty-three (77%) had their own apartment. CIC was used by 71%. Of those with no urological operation, 31% had a consultation with urologist every 3 years. The corresponding number for those operated on were 53%. Few of the participants had urotherapy support. The majority used pads, and none had support with their fecal elimination regimen. If pads were used it was harder to get employed. About 60% were single. II To investigate if life situation and incontinence in adults with MMC are reflected in HRQoL. Cross-sectional, cohort study with 61of 69 from study I who answered the generic Health related Quality of life instrument SF-36. The individuals had lower scores in the overall physical quality of life but higher scores in the overall mental quality of life. Neither physical nor mental quality of life was affected by whether the individual had fecal incontinence, lived with a partner or had children. III To evaluate urinary continence in adolescents with MMC, involved in a urotherapy/urology program and if urinary continence is one condition to enable social participation and close physical intimacy. Cross-sectional, quantitative, cohort study. A structured individual interview with 25 adolescents, were conducted. Prospective investigation about incontinence was implemented. Seventeen of 25 (68%) were urinary continent. Of these, 12 had an active social life and 8 had experience of having a partner. Eight individuals were urinary incontinent. None of them had an active social life or a close physical intimate relationship. The importance of follow-up and active treatment strategies to achieve urinary continence seem to be one of the predictors for having an active social life and the possibility of close physical intimate relationship. IV To describe experience of urinary incontinence and how incontinence affects life. Cross-sectional, descriptive qualitative semi structured interview with 9 individuals from the cohort in study I and II. The participants described similar experiences as individuals with acquired urinary incontinence. Individuals with congenital incontinence seem to need the same access to treatment as individuals with acquired urinary incontinence. Magdalena Vu Minh Arnell 1 1 INTRODUCTION Spina bifida is a diagnosis within neural tube defects, (NTD). Globally about 300,000 children are annually born with NTD, and the most common malformation is myelomeningocele (MMC) (1). The individuals in this thesis have either MMC or Lipo-MMC. Since the 1970s, there is a multi-professional team in Gothenburg that follows children and adolescents with MMC. The team at Queen Silvia Children´s hospital consists of urologist, pediatric urotherapist, neurologist, intestinal surgeon, occupational therapist and habilitation assistant. Our catchment area consists of western Sweden with a population of 2.3 million of the total 10,3 million inhabitants in Sweden 2019 (2). In the beginning of the 70s the incidence of MMC in Sweden was 5.5 per 10,000 births. In year 2016 the figure has decreased to 2,4 individuals in 10,000 births (3). The fact that MMC has become a rare diagnosis, the number of patients decreases in pediatric care while it still increases in adult care. During the last decades the pediatric MMC-team often discusses in terms of; - What happened to the individuals with MMC after leaving pediatric care and what does their life look like? These questions became the basis for my PhD-studies.  Magdalena Vu Minh Arnell 1 1 INTRODUCTION Spina bifida is a diagnosis within neural tube defects, (NTD). Globally about 300,000 children are annually born with NTD, and the most common malformation is myelomeningocele (MMC) (1). The individuals in this thesis have either MMC or Lipo-MMC. Since the 1970s, there is a multi-professional team in Gothenburg that follows children and adolescents with MMC. The team at Queen Silvia Children´s hospital consists of urologist, pediatric urotherapist, neurologist, intestinal surgeon, occupational therapist and habilitation assistant. Our catchment area consists of western Sweden with a population of 2.3 million of the total 10,3 million inhabitants in Sweden 2019 (2). In the beginning of the 70s the incidence of MMC in Sweden was 5.5 per 10,000 births. In year 2016 the figure has decreased to 2,4 individuals in 10,000 births (3). The fact that MMC has become a rare diagnosis, the number of patients decreases in pediatric care while it still increases in adult care. During the last decades the pediatric MMC-team often discusses in terms of; - What happened to the individuals with MMC after leaving pediatric care and what does their life look like? These questions became the basis for my PhD-studies.  Neurogenic bladder and bowel dysfunction 2 MYELOMENINGOCELE, MMC Dysmorphology on the neural tube can lead to many different malformations known as, neural tube defects, (NTD). The malformations vary in extent and symptomatology. Major defects include anencephaly, a large open defect meaning survival is impossible. Open spina bifida, myelomeningocele (MMC), could be extensive with severe neurological injuries. On the other hand, occult spina bifida could just be a small defect in the vertebral arches, with normal neurological function (4,5). MMC is a malformation due to a lack of closure of the spinal canal and it occurs during the third to fourth week of pregnancy. The spinal cord is often deformed, and the nerve fibers are stretched and damaged. The cause of myelomeningocele is not completely clarified (4). Nowadays, in the industrial world, there is a decline in the numbers of children born with MMC. There is a consensus that if the mother takes folic acid or eats food fortified with folic acid the risk of NTDs in the fetus declines (5,6). Another reason for the decline may be the increased number of abortions following prenatal screening (3). Certain heredity, and probably environmental factors can also affect the occurrence of NTD (5). During the last 60 years in Sweden, care for individuals with MMC has developed. Due to the improved hygiene conditions at Swedish hospitals during the 1950s, newborns with MMC could survive. Neurosurgeons began to operate on and cover the hernia in plastic surgery procedures, and in late 1950s, the first shunt procedure was carried out on at a little boy in the US. The first shunt operation was carried out on an individual with MMC in Sweden in the early 1960s. To be able to close the myelomeningocele and to do a shunt operation for hydrocephalus were two important conditions for survival (7). Today, most children with the condition survive, and we can expect most of them to reach adulthood. However, we do not really know how aging will affect individuals with MMC. There are articles that claim that adults with MMC have more hospital stays than the general population, they have more problems with pain, obesity, pressure ulcers, high blood pressure, and even premature death from uremia (8-11). Magdalena Vu Minh Arnell 3 NORMAL BLADDER FUNCTION At normal function the bladder stores the urine under low pressure and without leakage. Individuals should be able to decide voluntarily when to empty the bladder and should be able to empty it completely. Getting the micturition cycle to work depends on a functioning nervous system. When the bladder is filled during the storage phase, the sympathetic branch of the autonomic nervous system ensures that the detrusor is relaxed, and the internal sphincter is contracted. Voluntary micturition starts at brain level, the parasympathetic branch of the autonomic nervous system giving opposite signals, the detrusor contracting and the sphincter relaxing. The flow continues until the bladder is emptied. The detrusor muscle of the bladder consists of smooth muscle that ends in the bladder neck area where the inner sphincter is located. The urethra consists of both smooth and striated muscles. The striated muscles form the outer sphincter are will-controlled. Good pelvic floor muscles are important to counteract rapid rises of pressure in the abdominal cavity and to maintain urinary continence (12,13). Neurogenic bladder and bowel dysfunction 2 MYELOMENINGOCELE, MMC Dysmorphology on the neural tube can lead to many different malformations known as, neural tube defects, (NTD). The malformations vary in extent and symptomatology. Major defects include anencephaly, a large open defect meaning survival is impossible. Open spina bifida, myelomeningocele (MMC), could be extensive with severe neurological injuries. On the other hand, occult spina bifida could just be a small defect in the vertebral arches, with normal neurological function (4,5). MMC is a malformation due to a lack of closure of the spinal canal and it occurs during the third to fourth week of pregnancy. The spinal cord is often deformed, and the nerve fibers are stretched and damaged. The cause of myelomeningocele is not completely clarified (4). Nowadays, in the industrial world, there is a decline in the numbers of children born with MMC. There is a consensus that if the mother takes folic acid or eats food fortified with folic acid the risk of NTDs in the fetus declines (5,6). Another reason for the decline may be the increased number of abortions following prenatal screening (3). Certain heredity, and probably environmental factors can also affect the occurrence of NTD (5). During the last 60 years in Sweden, care for individuals with MMC has developed. Due to the improved hygiene conditions at Swedish hospitals during the 1950s, newborns with MMC could survive. Neurosurgeons began to operate on and cover the hernia in plastic surgery procedures, and in late 1950s, the first shunt procedure was carried out on at a little boy in the US. The first shunt operation was carried out on an individual with MMC in Sweden in the early 1960s. To be able to close the myelomeningocele and to do a shunt operation for hydrocephalus were two important conditions for survival (7). Today, most children with the condition survive, and we can expect most of them to reach adulthood. However, we do not really know how aging will affect individuals with MMC. There are articles that claim that adults with MMC have more hospital stays than the general population, they have more problems with pain, obesity, pressure ulcers, high blood pressure, and even premature death from uremia (8-11). Magdalena Vu Minh Arnell 3 NORMAL BLADDER FUNCTION At normal function the bladder stores the urine under low pressure and without leakage. Individuals should be able to decide voluntarily when to empty the bladder and should be able to empty it completely. Getting the micturition cycle to work depends on a functioning nervous system. When the bladder is filled during the storage phase, the sympathetic branch of the autonomic nervous system ensures that the detrusor is relaxed, and the internal sphincter is contracted. Voluntary micturition starts at brain level, the parasympathetic branch of the autonomic nervous system giving opposite signals, the detrusor contracting and the sphincter relaxing. The flow continues until the bladder is emptied. The detrusor muscle of the bladder consists of smooth muscle that ends in the bladder neck area where the inner sphincter is located. The urethra consists of both smooth and striated muscles. The striated muscles form the outer sphincter are will-controlled. Good pelvic floor muscles are important to counteract rapid rises of pressure in the abdominal cavity and to maintain urinary continence (12,13). Neurogenic bladder and bowel dysfunction 4 NEUROGENIC BLADDER DYSFUNCTION Neurogenic bladder dysfunction in MMC, almost always means a loss of function. Normal function depends on the nervous control from the brain via nerve roots in the spinal cord and peripheral nerves to the bladder and urethral muscles being intact (13). In neurogenic bladder dysfunction the nerves, which are important for emptying the bladder are damaged. The emptying of the bladder and the relaxation of the urethra sphincter which are usually coordinated, are dyssynergic. Neurogenic bladder dysfunction in individuals with MMC entails both a peripheral nerve and spinal cord damage. It is not usually possible to make a diagnosis according to the level of the location of the MMC, so the diagnosis has to be made according to a careful mapping of the function. About 10% of individuals have a normal bladder function while 90% have one of the four types of neurogenic bladder dysfunction below. (14). Sphincter + Sphincter - Detrusor + ~35% High-risk bladder ~10% Low-risk bladder Detrusor - ~10% Risk-bladder ~35% Low-risk bladder Magdalena Vu Minh Arnell 5 NORMAL BOWEL FUNCTION During bowel movements, an interaction between the colon, rectum and sphincter muscles begins. For most individuals, the urge to empty the bowel starts after eating. It is of importance to take advantage of the gastrocolic reflex. The feces in the colon are transported down into the rectum by a strong contraction. When the wall in the rectum expands, signals go through the pelvic nerve and a feeling of urgency arises. The bowel movement is started by influencing the pelvic floor, which then relaxes, and the anorectal angle is straightened. Usually a short straining is needed to start the emptying, which takes place automatically (15). Neurogenic bladder and bowel dysfunction 4 NEUROGENIC BLADDER DYSFUNCTION Neurogenic bladder dysfunction in MMC, almost always means a loss of function. Normal function depends on the nervous control from the brain via nerve roots in the spinal cord and peripheral nerves to the bladder and urethral muscles being intact (13). In neurogenic bladder dysfunction the nerves, which are important for emptying the bladder are damaged. The emptying of the bladder and the relaxation of the urethra sphincter which are usually coordinated, are dyssynergic. Neurogenic bladder dysfunction in individuals with MMC entails both a peripheral nerve and spinal cord damage. It is not usually possible to make a diagnosis according to the level of the location of the MMC, so the diagnosis has to be made according to a careful mapping of the function. About 10% of individuals have a normal bladder function while 90% have one of the four types of neurogenic bladder dysfunction below. (14). Sphincter + Sphincter - Detrusor + ~35% High-risk bladder ~10% Low-risk bladder Detrusor - ~10% Risk-bladder ~35% Low-risk bladder Magdalena Vu Minh Arnell 5 NORMAL BOWEL FUNCTION During bowel movements, an interaction between the colon, rectum and sphincter muscles begins. For most individuals, the urge to empty the bowel starts after eating. It is of importance to take advantage of the gastrocolic reflex. The feces in the colon are transported down into the rectum by a strong contraction. When the wall in the rectum expands, signals go through the pelvic nerve and a feeling of urgency arises. The bowel movement is started by influencing the pelvic floor, which then relaxes, and the anorectal angle is straightened. Usually a short straining is needed to start the emptying, which takes place automatically (15). Neurogenic bladder and bowel dysfunction 6 NEUROGENIC BOWEL DYSFUNCTION About 90% of the individuals with MMC have a neurogenic bowel dysfunction (16). Due to the congenital damage of the spinal cord, the intestine is affected with a lack of emptying reflex and there is also slower peristalsis to the colon and a weak rectal sphincter. These factors lead to both constipation and fecal incontinence in this patient group (17,18). Bowel regim ens Experience gained at our pediatric MMC-clinic since the 1970s, tells us that most of our patients need some kind of bowel emptying regimens. Every child gets an individual treatment plan according to the national and local care program (19). As soon as the child can sit up on a potty chair or on a special toilet seat, we initiate bowel regimen while sitting. The goals are to sit on a toilet when having an enema, that all feces should get into the toilet and that there will be no fecal leakage in between the times of the enemas. The definition “ to be without leakage in between the times of the enemas” is sometimes called pseudo-continence by Vande Velde et al (20,21). The method must be effective and sufficiently simple for patients to perform so they are able to handle the procedure independently as they get older. A review from Belgium establishes the importance of an individually stepwise adapted method for bowel emptying and concludes that conventional methods must be well-proven before any surgical procedure is considered. The importance of lifelong follow-up of the emptying routine is emphasized for all individuals with MMC (21). In a registry study from the United States, the bowel emptying methods were compared for three groups; children, adolescents and adults with MMC. Adults more often used digital stimulation, and colostomies were not uncommon. Only 17% of the adults used enemas compared with 27% in children (22). On the market, there is a variety of tools to administer an enema. For individuals with MMC the principle is to get the enema fluid to remain in the bowel for long enough to mimic the emptying reflex. In a retrospective study of individuals with MMC from 2 to 24 years of age, and who followed a bowel-emptying program, Schletker et al found that the most common challenge when administering an enema was leakage of the solution during infusion (23). Being able to follow bowel regimens independently is important for reasons of integrity and self-esteem. However, in children up to 16 years of age it is hard to administer an enema independently, either trans-anal or antegrade like in Malone Antegrade Continence Enema (MACE) (24). In a study from the UK, parents found it hard to hand over responsibility to their teenagers and teenagers found it difficult to become independent in the enema regimen (25). Magdalena Vu Minh Arnell 7 UROTHERAPY The first university education in urotherapy in the world started in 1987 at the University of Gothenburg. To become a urotherapist you must have a univer- sity degree and have qualifications including registered nurse, physiotherapist or physician. To be able to apply for the urotherapy course nurses also need a specialization degree (26). “ Urotherapy” is a combination of the word “uro” - from urology that means study of treatment and diseases of the urogenital tract and “therapy” - which means treatment of diseases or disorders by rehabili- tation or curative process” a quote from UTF-Nordic (Urotherapeutic association-Nordic) (on internet) (27). A urotherapist investigates, treats and makes follow-up interventions to deal with both bladder and bowel problems including leakage, urgency and emptying difficulties. The urotherapist conveys knowledge and understanding of cause and symptoms of the condition in order to achieve the best treatment results. The urotherapist have also education and knowledge about prescription of products for urinary and fecal incontinence and retention (26). Important conditions for survival in this patient group were surgical closure of the myelomeningocele in the 1950s and shunting of hydrocephalus in the 1960s. However, there was still one major problem that affected basically all individuals with MMC, namely the inability to empty the bladder which entailed a high risk of kidney deterioration, resulting in uremia with a fatal outcome. In the late 1970s, Clean Intermittent Catheterization (CIC) was introduced in Sweden and it became a revolution for individuals with MMC. From having a high risk of renal failure, the risk was now considerably minimized. The method was first described by Lapides et al in 1972 and introduced in Sweden in 1977 by Lindehall and Hellström. Regular complete emptying with a clean catheter was more important than a sterile technique (28,29). Before CIC was introduced, parents of children with MMC learned manual compression of the child's bladder and later children had to try abdominal straining in order to empty the bladder. These methods increased the risks of vesicoureteral reflux (29). In the medical world, a non-sterile method of catheterization was not a possibility. However, Lapides showed that two things were more important than a sterile catheter, a regular and an effective emptying with bladder volumes for an adult under 400 ml. The bacteria were inevitably introduced into the bladder but drained again after 3-4-hour intervals daytime. The CIC method became the second-best method for bladder emptying (28,30). Neurogenic bladder and bowel dysfunction 6 NEUROGENIC BOWEL DYSFUNCTION About 90% of the individuals with MMC have a neurogenic bowel dysfunction (16). Due to the congenital damage of the spinal cord, the intestine is affected with a lack of emptying reflex and there is also slower peristalsis to the colon and a weak rectal sphincter. These factors lead to both constipation and fecal incontinence in this patient group (17,18). Bowel regim ens Experience gained at our pediatric MMC-clinic since the 1970s, tells us that most of our patients need some kind of bowel emptying regimens. Every child gets an individual treatment plan according to the national and local care program (19). As soon as the child can sit up on a potty chair or on a special toilet seat, we initiate bowel regimen while sitting. The goals are to sit on a toilet when having an enema, that all feces should get into the toilet and that there will be no fecal leakage in between the times of the enemas. The definition “ to be without leakage in between the times of the enemas” is sometimes called pseudo-continence by Vande Velde et al (20,21). The method must be effective and sufficiently simple for patients to perform so they are able to handle the procedure independently as they get older. A review from Belgium establishes the importance of an individually stepwise adapted method for bowel emptying and concludes that conventional methods must be well-proven before any surgical procedure is considered. The importance of lifelong follow-up of the emptying routine is emphasized for all individuals with MMC (21). In a registry study from the United States, the bowel emptying methods were compared for three groups; children, adolescents and adults with MMC. Adults more often used digital stimulation, and colostomies were not uncommon. Only 17% of the adults used enemas compared with 27% in children (22). On the market, there is a variety of tools to administer an enema. For individuals with MMC the principle is to get the enema fluid to remain in the bowel for long enough to mimic the emptying reflex. In a retrospective study of individuals with MMC from 2 to 24 years of age, and who followed a bowel-emptying program, Schletker et al found that the most common challenge when administering an enema was leakage of the solution during infusion (23). Being able to follow bowel regimens independently is important for reasons of integrity and self-esteem. However, in children up to 16 years of age it is hard to administer an enema independently, either trans-anal or antegrade like in Malone Antegrade Continence Enema (MACE) (24). In a study from the UK, parents found it hard to hand over responsibility to their teenagers and teenagers found it difficult to become independent in the enema regimen (25). Magdalena Vu Minh Arnell 7 UROTHERAPY The first university education in urotherapy in the world started in 1987 at the University of Gothenburg. To become a urotherapist you must have a univer- sity degree and have qualifications including registered nurse, physiotherapist or physician. To be able to apply for the urotherapy course nurses also need a specialization degree (26). “ Urotherapy” is a combination of the word “uro” - from urology that means study of treatment and diseases of the urogenital tract and “therapy” - which means treatment of diseases or disorders by rehabili- tation or curative process” a quote from UTF-Nordic (Urotherapeutic association-Nordic) (on internet) (27). A urotherapist investigates, treats and makes follow-up interventions to deal with both bladder and bowel problems including leakage, urgency and emptying difficulties. The urotherapist conveys knowledge and understanding of cause and symptoms of the condition in order to achieve the best treatment results. The urotherapist have also education and knowledge about prescription of products for urinary and fecal incontinence and retention (26). Important conditions for survival in this patient group were surgical closure of the myelomeningocele in the 1950s and shunting of hydrocephalus in the 1960s. However, there was still one major problem that affected basically all individuals with MMC, namely the inability to empty the bladder which entailed a high risk of kidney deterioration, resulting in uremia with a fatal outcome. In the late 1970s, Clean Intermittent Catheterization (CIC) was introduced in Sweden and it became a revolution for individuals with MMC. From having a high risk of renal failure, the risk was now considerably minimized. The method was first described by Lapides et al in 1972 and introduced in Sweden in 1977 by Lindehall and Hellström. Regular complete emptying with a clean catheter was more important than a sterile technique (28,29). Before CIC was introduced, parents of children with MMC learned manual compression of the child's bladder and later children had to try abdominal straining in order to empty the bladder. These methods increased the risks of vesicoureteral reflux (29). In the medical world, a non-sterile method of catheterization was not a possibility. However, Lapides showed that two things were more important than a sterile catheter, a regular and an effective emptying with bladder volumes for an adult under 400 ml. The bacteria were inevitably introduced into the bladder but drained again after 3-4-hour intervals daytime. The CIC method became the second-best method for bladder emptying (28,30). Neurogenic bladder and bowel dysfunction 8 As the majority of children with MMC have neurogenic bladder dysfunction, CIC is vital and lifelong (31). From a medical point of view, the bladder emptying is the most serious problem. It means that when a child with MMC is newborn, CIC should be introduced as soon as possible. This is gold standard in many of the industrial countries (32). The urotherapist's most important role in the natal period is to teach and support the parents in how to perform CIC on their child. A child with MMC will be followed at the outpatient clinic in Queen Silvia Children`s hospital for 18 years. The team is inspired by “ family-centered care” FCC, a way to work with and for the family and their child with special needs. Parents and professionals should be seen as partners where continuity in care and accessibility are two key concepts. Respect, family strength and collaboration are central in FCC (33). In our unit, each child has a personal urotherapist who follows the child and knows the family. In an outpatient clinic for children and adolescents with MMC, most of the patients are expected to have the diagnosis neurogenic bladder and bowel dysfunction. The urotherapist has a prior knowledge of the diagnosis and a structured national and local care program to follow (20). Urodynamic examinations play a central role in the investigation and follow-up of neurogenic bladder dysfunction and are performed by the child´s personal urotherapist (34-37). An important complement to urodynamics is observation of the micturition list, leakage test and registration of bowel emptying. The urotherapist is responsible for the child receiving individually tested pads or diapers for leakage and catheters for CIC. Changes in the micturition or continence pattern should lead to follow-up cystometry. In connection with the visit an ultrasound is made after CIC or micturition to check that the child empties the bladder completely. In neonates and infants, a 4-hour micturition observation with provocation and ultrasound after micturition are conducted (38,39). From the integrity aspect, learning self CIC is of great importance. Most children learn to perform CIC by an age of 6-9 years old. However, the urothearapist and the parents may encourage the child to practice as soon as the child shows interest in any part of the treatment. Depending on the child's conditions such as hand function and cognitive ability, CIC is practiced in small steps and always adapted individually. If the child has a difficulty in time perception, the CIC may be related to another event such as meals (40- 42). The urotherapist starts early to motivate the child and the family to introduce self-CIC. We invite the child and parents to “CIC school” where they can meet others in the same age who also come to practice self-CIC. Magdalena Vu Minh Arnell 9 LIFE SITUATION During the last 40 years, individuals with MMC survive into adulthood. During these years it has been noticed that it is not only the physical problems that are in focus. Together with the cognitive problems, the entire life situation is affected. Almost 20 years ago, in 2001, an article was published by Bowman et al in which they pointed out that a major challenge was providing care for the growing adult population. Such care entails multidisciplinary follow-up for patients and an active network of healthcare professionals in adult care (43). Today, there is knowledge about what affects the life situation in individuals with MMC. Urine and fecal leakage are major problems and can be a contributing factor to loneliness (44). A life without social context like having a job, a partner or friends, affects health and leads to difficulties in life conditions. The physical problems become more and more accentuated the older you get. Inactivity leads to pain, obesity and incontinence which can contribute to pressure ulcers (45,46). In a study from the US they look at the fact that adults with MMC have physical and cognitive problems that affect their psychosocial situations which in turn can lead to mental illness. Since 2018, the Spina Bifida Association in the US has provided; Guidelines for the Care of People with Spina Bifida, with the intention of helping individuals achieve good mental health throughout life (47). Not being active in the society can contribute to loneliness and affect life situation. Barf et al found that young adults with MMC experienced difficulties in participating in the society due to physical or mental limitations (48). In a study on friendship, young people with MMC often described a relationship with a particular friend as close and called this friend their best friend. However, this did not correspond to the friend's opinion. The conclusion was that young people with MMC experience differences in the quality and reciprocity of friendship compared with their peers (49). Still 20 years after Bowman's article, we face the same challenge. Neurogenic bladder and bowel dysfunction 8 As the majority of children with MMC have neurogenic bladder dysfunction, CIC is vital and lifelong (31). From a medical point of view, the bladder emptying is the most serious problem. It means that when a child with MMC is newborn, CIC should be introduced as soon as possible. This is gold standard in many of the industrial countries (32). The urotherapist's most important role in the natal period is to teach and support the parents in how to perform CIC on their child. A child with MMC will be followed at the outpatient clinic in Queen Silvia Children`s hospital for 18 years. The team is inspired by “ family-centered care” FCC, a way to work with and for the family and their child with special needs. Parents and professionals should be seen as partners where continuity in care and accessibility are two key concepts. Respect, family strength and collaboration are central in FCC (33). In our unit, each child has a personal urotherapist who follows the child and knows the family. In an outpatient clinic for children and adolescents with MMC, most of the patients are expected to have the diagnosis neurogenic bladder and bowel dysfunction. The urotherapist has a prior knowledge of the diagnosis and a structured national and local care program to follow (20). Urodynamic examinations play a central role in the investigation and follow-up of neurogenic bladder dysfunction and are performed by the child´s personal urotherapist (34-37). An important complement to urodynamics is observation of the micturition list, leakage test and registration of bowel emptying. The urotherapist is responsible for the child receiving individually tested pads or diapers for leakage and catheters for CIC. Changes in the micturition or continence pattern should lead to follow-up cystometry. In connection with the visit an ultrasound is made after CIC or micturition to check that the child empties the bladder completely. In neonates and infants, a 4-hour micturition observation with provocation and ultrasound after micturition are conducted (38,39). From the integrity aspect, learning self CIC is of great importance. Most children learn to perform CIC by an age of 6-9 years old. However, the urothearapist and the parents may encourage the child to practice as soon as the child shows interest in any part of the treatment. Depending on the child's conditions such as hand function and cognitive ability, CIC is practiced in small steps and always adapted individually. If the child has a difficulty in time perception, the CIC may be related to another event such as meals (40- 42). The urotherapist starts early to motivate the child and the family to introduce self-CIC. We invite the child and parents to “CIC school” where they can meet others in the same age who also come to practice self-CIC. Magdalena Vu Minh Arnell 9 LIFE SITUATION During the last 40 years, individuals with MMC survive into adulthood. During these years it has been noticed that it is not only the physical problems that are in focus. Together with the cognitive problems, the entire life situation is affected. Almost 20 years ago, in 2001, an article was published by Bowman et al in which they pointed out that a major challenge was providing care for the growing adult population. Such care entails multidisciplinary follow-up for patients and an active network of healthcare professionals in adult care (43). Today, there is knowledge about what affects the life situation in individuals with MMC. Urine and fecal leakage are major problems and can be a contributing factor to loneliness (44). A life without social context like having a job, a partner or friends, affects health and leads to difficulties in life conditions. The physical problems become more and more accentuated the older you get. Inactivity leads to pain, obesity and incontinence which can contribute to pressure ulcers (45,46). In a study from the US they look at the fact that adults with MMC have physical and cognitive problems that affect their psychosocial situations which in turn can lead to mental illness. Since 2018, the Spina Bifida Association in the US has provided; Guidelines for the Care of People with Spina Bifida, with the intention of helping individuals achieve good mental health throughout life (47). Not being active in the society can contribute to loneliness and affect life situation. Barf et al found that young adults with MMC experienced difficulties in participating in the society due to physical or mental limitations (48). In a study on friendship, young people with MMC often described a relationship with a particular friend as close and called this friend their best friend. However, this did not correspond to the friend's opinion. The conclusion was that young people with MMC experience differences in the quality and reciprocity of friendship compared with their peers (49). Still 20 years after Bowman's article, we face the same challenge. Neurogenic bladder and bowel dysfunction 10 COGNITIVE FUNCTION Over the years, meeting many children and adolescents with MMC, we have noticed similar features among the individuals that we initially did not fully understand. However, many parents had the same experience. Their children had e.g. difficulties in mathematics, problems with memory, to interact with other children, and they had to be constantly pushed to get something done. In the 21 centuries, research has shown that most individuals with MMC have cognitive difficulties due to primary brain dysmorphology and the occurrence of hydrocephalus (50-52). The published articles have pointed out how this affects life situation for both children and adults (53-55). A Swedish study showed that the individuals have difficulties in estimating time, understanding time perspectives and plan time (54). Peny-Dahlstrand et al described in an article the executive difficulties in children with MMC. The hardest thing is not to learn how to do things but to get things done (53). This sentence says a lot about the challenge that is faced by the individual, the family and by the professionals. In a urotherapeutic unit where we meet individuals with MMC, we start CIC and bowel emptying regimens where the goal is to create independence in vital and lifelong treatments. If CIC is not performed, even if the person knows how, it could become a life-threatening situation resulting in renal failure (55). Aware of the cognitive and executive difficulties affecting individuals with MMC, it is of great importance to have an, of the diagnosis experienced occupational therapist, in the team. Magdalena Vu Minh Arnell 11 2 AIM Study I To assess life situation, bladder and bowel management and urological follow- up in individuals with MMC after transferal to adult medical care. Study II To evaluate HRQoL in adults with MMC measured by SF-36. Study III To evaluate urinary continence in adolescents with MMC, who are actively involved in a urotherapy/urology program. To evaluate if urinary continence is one of the conditions required to enable social participation and close physical intimacy. Study IV To describe the experience of urinary incontinence in individuals with MMC and how incontinence affects their lives. Neurogenic bladder and bowel dysfunction 10 COGNITIVE FUNCTION Over the years, meeting many children and adolescents with MMC, we have noticed similar features among the individuals that we initially did not fully understand. However, many parents had the same experience. Their children had e.g. difficulties in mathematics, problems with memory, to interact with other children, and they had to be constantly pushed to get something done. In the 21 centuries, research has shown that most individuals with MMC have cognitive difficulties due to primary brain dysmorphology and the occurrence of hydrocephalus (50-52). The published articles have pointed out how this affects life situation for both children and adults (53-55). A Swedish study showed that the individuals have difficulties in estimating time, understanding time perspectives and plan time (54). Peny-Dahlstrand et al described in an article the executive difficulties in children with MMC. The hardest thing is not to learn how to do things but to get things done (53). This sentence says a lot about the challenge that is faced by the individual, the family and by the professionals. In a urotherapeutic unit where we meet individuals with MMC, we start CIC and bowel emptying regimens where the goal is to create independence in vital and lifelong treatments. If CIC is not performed, even if the person knows how, it could become a life-threatening situation resulting in renal failure (55). Aware of the cognitive and executive difficulties affecting individuals with MMC, it is of great importance to have an, of the diagnosis experienced occupational therapist, in the team. Magdalena Vu Minh Arnell 11 2 AIM Study I To assess life situation, bladder and bowel management and urological follow- up in individuals with MMC after transferal to adult medical care. Study II To evaluate HRQoL in adults with MMC measured by SF-36. Study III To evaluate urinary continence in adolescents with MMC, who are actively involved in a urotherapy/urology program. To evaluate if urinary continence is one of the conditions required to enable social participation and close physical intimacy. Study IV To describe the experience of urinary incontinence in individuals with MMC and how incontinence affects their lives. Neurogenic bladder and bowel dysfunction 12 Magdalena Vu Minh Arnell 13 3 PARTICIPANTS AND METHOD S Table 1. Overview of methodological approaches. STUDY STUDY POPULATION STUDY DESIGN DATA COLLECTION DATA ANALYSIS I 69 participants 37 females Age Md 34 yrs (27-50) Observational, Cross-sectional, Quantitative, cohort study Individual structured interview Fisher´s exact test, p<0.05 II 61 participants 30 females Age Md 34 yrs (27-50) Observational, Cross-sectional, Quantitative, cohort study Questionnaire study Descriptive statistics, Fisher´s exact test, Mann- Whitney´s test, Kruskal- Wallis´ test, p<0.05 III 25 participants 10 females Age Md 17 yrs (16-18) Observational, Cross-sectional, Quantitative, cohort study Individual structured interview. Prospective investigations Descriptive statistics IV 9 participants 7 females Age Md 46 yrs (40-56) Observational, Cross-sectional, Descriptive Qualitative study Individual semi- structured interview. Selection of participants was purposeful Qualitative Content Analysis Neurogenic bladder and bowel dysfunction 12 Magdalena Vu Minh Arnell 13 3 PARTICIPANTS AND METHOD S Table 1. Overview of methodological approaches. STUDY STUDY POPULATION STUDY DESIGN DATA COLLECTION DATA ANALYSIS I 69 participants 37 females Age Md 34 yrs (27-50) Observational, Cross-sectional, Quantitative, cohort study Individual structured interview Fisher´s exact test, p<0.05 II 61 participants 30 females Age Md 34 yrs (27-50) Observational, Cross-sectional, Quantitative, cohort study Questionnaire study Descriptive statistics, Fisher´s exact test, Mann- Whitney´s test, Kruskal- Wallis´ test, p<0.05 III 25 participants 10 females Age Md 17 yrs (16-18) Observational, Cross-sectional, Quantitative, cohort study Individual structured interview. Prospective investigations Descriptive statistics IV 9 participants 7 females Age Md 46 yrs (40-56) Observational, Cross-sectional, Descriptive Qualitative study Individual semi- structured interview. Selection of participants was purposeful Qualitative Content Analysis Neurogenic bladder and bowel dysfunction 14 STUDY POPULATION (Fig) All children newborn to 18 years of age, with MMC and neurogenic bladder dysfunction, living in western Sweden, and attending Regional Rehabilitation Centre for Children and Adolescents. Inclusion criteria for the study population in study I, II and IV were patients with MMC, born before 1981. As children they had lived in western Sweden and had been assessed on at least two occasions by a pediatric urologist at Regional Rehabilitation Center for Children and Adolescents. They had been transferred to adult medical care before 2001. Figure. Individuals who met the inclusion criteria in study I, II and IV and drop-outs. Entry criteria in 134 individuals 69 individuals Study I 16, contact could not be established 22, unable to answer the questions 2, denied the diagnosis and 25, declined to participate 61 individuals Study II 8, not involved 9 individua ls Study IV 11, didn´t respond 1, denied to be tape-recorded 1, became acutely ill 47, didn´t meet the inclusion criteria. Magdalena Vu Minh Arnell 15 In study I 69 individuals (58%) participated. Twelve participants did not have a shunt due to hydrocephalus. Wheelchair was never used by 18, occasionally by 11, and always used by 40 participants. No evident differences in regard to age, gender, type of spina bifida, lesion level and having a shunt for hydrocephalus were noted between the group that declined to participate and the group that agreed. On the same occasion as interviews for study I took place, 61 of the 69 individuals answered the Heath Related Quality of Life questionnaire, SF-36, study II. Eight individuals participated in the pilot study for study I and did therefore not take part in study II. Four individuals only partly completed SF-36 because two became too emotional and two did not understand the questions. The study's selection of informants in study IV was purposeful for the aim and the method used. We intended to interview individuals from study I who had urinary incontinence and who, during the interview in study I, openly expressed opinions about their incontinence. The day before the planned interview one individual became acutely ill and one was anxious about being tape-recorded. All but one in study IV had a shunt. Study III. All 25 individuals with MMC from the ages of 16 to18 years, living in western Sweden, and born between 1996 and 1998, agreed to participate in a face-to-face interview at the same occasion as the yearly urological/uroterapeutic follow-up. The interviews were performed at the outpatient clinic. STUDY DESIGN All studies were observational and cross-sectional. Study I, II and III were also quantitative as well as cohort studies. The cohort was defined as all individuals who met the inclusion criteria for participating in the studies. In study IV the design was a descriptive qualitative semi-structured interview. The participants were selected from study I. With purposeful selection means that you want to get the best and as much information as possible about the topic. The individuals were selected for the research project based on their knowledge and experience (56). Neurogenic bladder and bowel dysfunction 14 STUDY POPULATION (Fig) All children newborn to 18 years of age, with MMC and neurogenic bladder dysfunction, living in western Sweden, and attending Regional Rehabilitation Centre for Children and Adolescents. Inclusion criteria for the study population in study I, II and IV were patients with MMC, born before 1981. As children they had lived in western Sweden and had been assessed on at least two occasions by a pediatric urologist at Regional Rehabilitation Center for Children and Adolescents. They had been transferred to adult medical care before 2001. Figure. Individuals who met the inclusion criteria in study I, II and IV and drop-outs. Entry criteria in 134 individuals 69 individuals Study I 16, contact could not be established 22, unable to answer the questions 2, denied the diagnosis and 25, declined to participate 61 individuals Study II 8, not involved 9 individua ls Study IV 11, didn´t respond 1, denied to be tape-recorded 1, became acutely ill 47, didn´t meet the inclusion criteria. Magdalena Vu Minh Arnell 15 In study I 69 individuals (58%) participated. Twelve participants did not have a shunt due to hydrocephalus. Wheelchair was never used by 18, occasionally by 11, and always used by 40 participants. No evident differences in regard to age, gender, type of spina bifida, lesion level and having a shunt for hydrocephalus were noted between the group that declined to participate and the group that agreed. On the same occasion as interviews for study I took place, 61 of the 69 individuals answered the Heath Related Quality of Life questionnaire, SF-36, study II. Eight individuals participated in the pilot study for study I and did therefore not take part in study II. Four individuals only partly completed SF-36 because two became too emotional and two did not understand the questions. The study's selection of informants in study IV was purposeful for the aim and the method used. We intended to interview individuals from study I who had urinary incontinence and who, during the interview in study I, openly expressed opinions about their incontinence. The day before the planned interview one individual became acutely ill and one was anxious about being tape-recorded. All but one in study IV had a shunt. Study III. All 25 individuals with MMC from the ages of 16 to18 years, living in western Sweden, and born between 1996 and 1998, agreed to participate in a face-to-face interview at the same occasion as the yearly urological/uroterapeutic follow-up. The interviews were performed at the outpatient clinic. STUDY DESIGN All studies were observational and cross-sectional. Study I, II and III were also quantitative as well as cohort studies. The cohort was defined as all individuals who met the inclusion criteria for participating in the studies. In study IV the design was a descriptive qualitative semi-structured interview. The participants were selected from study I. With purposeful selection means that you want to get the best and as much information as possible about the topic. The individuals were selected for the research project based on their knowledge and experience (56). Neurogenic bladder and bowel dysfunction 16 DATA COLLECTION Study I During 2007-2008, two pediatric urotherapists, who had never been involved in the care of the study individuals, conducted a structured telephone interview according to a protocol. The interview questions focused on the individuals´ life situation and bladder and bowel function. The personal opinion of the individuals characterized the answers. The mean time for the interviews was 58 min (range 30-120). As control material Official Statistics of Sweden data was used (57). Study II SF-36 was used as a generic HRQoL protocol evaluating eight domains: physical functioning (PF), role physical (limitation caused by physical health problems) (RP), bodily pain (BP), general health (GH), vitality (VT), social functioning (SF), role emotional (limitation caused by emotional problems) (RE), and mental health (MH) (58). The eight domains could be aggregated into two summary measures: physical component summary (PCS) and mental component summary (MCS). Scores were measured on a scale from 0 to 100, with 0 representing the lowest health status and with 100 representing the highest health status. Values for PCS < 20 or > 58 and MCS < 17 and >62 are calculated as artifacts (59). The questions asked during the telephone interview were done in accordance with the SF-36 Swedish manual for the interview user (58). Study IV The individuals were free to choose where the interview should take place but due to the nature of the issue, it had to be in a private room with no one else to overhear. The interviews were conducted in the participants´ homes or in a private room at the Regional Rehabilitation Center for Children and Adolescents. A pediatric nurse and urotheraphist, not involved in the care of the participants but with experience of the diagnosis, conducted the interviews. The interview was a tape-recorded face-to-face, semi-structured interview with two open-ended questions. All individuals were asked to share their experience of living with urinary incontinence and how the incontinence affected their lives. It was of great importance that the interviewer did not lead the interview by suggesting different predetermined areas, just be supportive by saying such as “ Tell me more about this. Can you explain further?” The interviews lasted between 30 and 120 minutes. Magdalena Vu Minh Arnell 17 Study III During 2014, a urotherapist with knowledge of the participants conducted a structured interview with a questionnaire. All 25 individuals agreed to participate (100%). The questions asked were about urinary and fecal continence, CIC, bowel regimens, the need of reminder or help from assistant, social participation, physical intimacy with a partner and sexual debut. To have an objective observation, prospectively urinary continence was evaluated with 24-hour leakage test or a two-day micturition chart for those who reported that they were continent and did not have pads for incontinence prescribed. A 24-hour leakage test was performed with: pre-weighed pads, each in its own airtight plastic bag. The pads were changed every third hour (CIC interval) during daytime. The night pad was changed in the morning. After use, they were put back into the plastic bag which was sealed and returned to the hospital ward. Leakage was evaluated by weighing the pads and CIC volume and fluid intake was also measured. DATA ANALYSIS Descriptive statistics (Study II, III) Continuous variables were expressed as mean, standard deviation (SD), median, min and max. Number and/or percentage described categorical variables. Statistical analysis For comparison between groups, a Mann-Whitney U test was used for continuous variables (study II) and Fischer´s exact test for dichotomous variables (study I, II). For tests between three or more groups with regard to continuous variables, a Kruskal-Wallis test was used (study II). All tests were two-tailed and conducted at a 5% statistical level. All statistical analyses were performed by using SAS_9.2 (Cary, NC, USA) or Statistical Package for Social Science (SPSS) for Windows 18.0. Neurogenic bladder and bowel dysfunction 16 DATA COLLECTION Study I During 2007-2008, two pediatric urotherapists, who had never been involved in the care of the study individuals, conducted a structured telephone interview according to a protocol. The interview questions focused on the individuals´ life situation and bladder and bowel function. The personal opinion of the individuals characterized the answers. The mean time for the interviews was 58 min (range 30-120). As control material Official Statistics of Sweden data was used (57). Study II SF-36 was used as a generic HRQoL protocol evaluating eight domains: physical functioning (PF), role physical (limitation caused by physical health problems) (RP), bodily pain (BP), general health (GH), vitality (VT), social functioning (SF), role emotional (limitation caused by emotional problems) (RE), and mental health (MH) (58). The eight domains could be aggregated into two summary measures: physical component summary (PCS) and mental component summary (MCS). Scores were measured on a scale from 0 to 100, with 0 representing the lowest health status and with 100 representing the highest health status. Values for PCS < 20 or > 58 and MCS < 17 and >62 are calculated as artifacts (59). The questions asked during the telephone interview were done in accordance with the SF-36 Swedish manual for the interview user (58). Study IV The individuals were free to choose where the interview should take place but due to the nature of the issue, it had to be in a private room with no one else to overhear. The interviews were conducted in the participants´ homes or in a private room at the Regional Rehabilitation Center for Children and Adolescents. A pediatric nurse and urotheraphist, not involved in the care of the participants but with experience of the diagnosis, conducted the interviews. The interview was a tape-recorded face-to-face, semi-structured interview with two open-ended questions. All individuals were asked to share their experience of living with urinary incontinence and how the incontinence affected their lives. It was of great importance that the interviewer did not lead the interview by suggesting different predetermined areas, just be supportive by saying such as “ Tell me more about this. Can you explain further?” The interviews lasted between 30 and 120 minutes. Magdalena Vu Minh Arnell 17 Study III During 2014, a urotherapist with knowledge of the participants conducted a structured interview with a questionnaire. All 25 individuals agreed to participate (100%). The questions asked were about urinary and fecal continence, CIC, bowel regimens, the need of reminder or help from assistant, social participation, physical intimacy with a partner and sexual debut. To have an objective observation, prospectively urinary continence was evaluated with 24-hour leakage test or a two-day micturition chart for those who reported that they were continent and did not have pads for incontinence prescribed. A 24-hour leakage test was performed with: pre-weighed pads, each in its own airtight plastic bag. The pads were changed every third hour (CIC interval) during daytime. The night pad was changed in the morning. After use, they were put back into the plastic bag which was sealed and returned to the hospital ward. Leakage was evaluated by weighing the pads and CIC volume and fluid intake was also measured. DATA ANALYSIS Descriptive statistics (Study II, III) Continuous variables were expressed as mean, standard deviation (SD), median, min and max. Number and/or percentage described categorical variables. Statistical analysis For comparison between groups, a Mann-Whitney U test was used for continuous variables (study II) and Fischer´s exact test for dichotomous variables (study I, II). For tests between three or more groups with regard to continuous variables, a Kruskal-Wallis test was used (study II). All tests were two-tailed and conducted at a 5% statistical level. All statistical analyses were performed by using SAS_9.2 (Cary, NC, USA) or Statistical Package for Social Science (SPSS) for Windows 18.0. Neurogenic bladder and bowel dysfunction 18 Qualitative Content Analysis, QCA As method used in study IV, qualitative content analysis, was conducted according to the technique described by Krippendorff (60). Data were analyzed with an inductive approach based on the content of the interview texts. To get a sense of the whole and identify what was relevant to the issue, the text was read several times. The text was then condensed to find the significant core or pattern. The analysis was described step by step to give the reader an idea of the thoughts of the individuals described in the content. Quotes from the interviews also exemplify the content. The first step was to find meaning units that answered to the research question. The meaning unit were condensed to shorten the text but retain the content, then the condensed meaning units were coded. To reflect the core of the interviews, categories were created, and categories were then divided into subcategories (61). Quotes from the participants from the interview material, to exemplify each subcategory, were illustrated in the text. The text was analyzed with a manifest qualitative content analysis, which means that no interpretation of the text was made. ETHICAL CONSIDERATION Study designs for study I, II and III were accepted by the Regional Ethical Review Board in Gothenburg and the study design for Study IV was approved by the Ethics Review Authority in Uppsala, Sweden. Participation was voluntary and informed consents were obtained. The ethical principles of the Declaration of Helsinki - Ethical Principles for Medical Research Involving Human Subjects - were followed. They included the principle stating the care of the individual must take precedence over the interests of both science and society (62). These principles were followed in the dissertation. In Study I, III and IV, where there were sensitive topics and questions of a private nature, we were careful to respect decisions of individuals not to answer. If participants in interviews raised issues related to healthcare contacts, we tried to help solve them. In connection with requests to participate in the studies, no more than two reminders were sent. Information was given to the participants that results would be treated confidentially. Magdalena Vu Minh Arnell 19 4 RESULTS AND DISCUSSION 1 . Life situation in adults In a telephone interview study with 69 adult individuals 27-50 years of age, many expressed frustration and despair over their life situation. Many of the participants lived isolated lives far beyond what they had expected when young. As many as 90% of the individuals had gone to high school and over 50% had a driving license, but despite this several participants told that it was difficult to get a job, and difficult to keep it. Of participants, 67% had some form of employment, but 26% had no daily activity at all, corresponding to 7% in the control group from Official Statistics of Sweden (57). Many of the males pointed out that they lived alone (75%) and longed for someone to live with. Among females, about 50% lived with a partner. Internationally, Sweden stands out with 53 of 69 adult individuals with MMC having their own hous- ing, but at the same time only 27 individuals of 69 told they were independent. This means that many of those living in their own homes need some sort of support. Several adults described how their parents, in their 80s, helped with cleaning and enemas (63). International studies have shown that many adults with MMC continue to live at home with their parent’s (64-66). Asking ques- tions about sexual experience, when the individual said he or she never had had a close intimate relation, felt unethical. What emerged was that out of those who now lived in a relationship, were also those who answered yes to the question on physically intimate relationships. Of the females 19% had children compared to 6% of the males. Corresponding figures from the Swedish population show that 86% of females and 82% of males had children (57). Neurogenic bladder and bowel dysfunction 18 Qualitative Content Analysis, QCA As method used in study IV, qualitative content analysis, was conducted according to the technique described by Krippendorff (60). Data were analyzed with an inductive approach based on the content of the interview texts. To get a sense of the whole and identify what was relevant to the issue, the text was read several times. The text was then condensed to find the significant core or pattern. The analysis was described step by step to give the reader an idea of the thoughts of the individuals described in the content. Quotes from the interviews also exemplify the content. The first step was to find meaning units that answered to the research question. The meaning unit were condensed to shorten the text but retain the content, then the condensed meaning units were coded. To reflect the core of the interviews, categories were created, and categories were then divided into subcategories (61). Quotes from the participants from the interview material, to exemplify each subcategory, were illustrated in the text. The text was analyzed with a manifest qualitative content analysis, which means that no interpretation of the text was made. ETHICAL CONSIDERATION Study designs for study I, II and III were accepted by the Regional Ethical Review Board in Gothenburg and the study design for Study IV was approved by the Ethics Review Authority in Uppsala, Sweden. Participation was voluntary and informed consents were obtained. The ethical principles of the Declaration of Helsinki - Ethical Principles for Medical Research Involving Human Subjects - were followed. They included the principle stating the care of the individual must take precedence over the interests of both science and society (62). These principles were followed in the dissertation. In Study I, III and IV, where there were sensitive topics and questions of a private nature, we were careful to respect decisions of individuals not to answer. If participants in interviews raised issues related to healthcare contacts, we tried to help solve them. In connection with requests to participate in the studies, no more than two reminders were sent. Information was given to the participants that results would be treated confidentially. Magdalena Vu Minh Arnell 19 4 RESULTS AND DISCUSSION 1. Life situation in adults In a telephone interview study with 69 adult individuals 27-50 years of age, many expressed frustration and despair over their life situation. Many of the participants lived isolated lives far beyond what they had expected when young. As many as 90% of the individuals had gone to high school and over 50% had a driving license, but despite this several participants told that it was difficult to get a job, and difficult to keep it. Of participants, 67% had some form of employment, but 26% had no daily activity at all, corresponding to 7% in the control group from Official Statistics of Sweden (57). Many of the males pointed out that they lived alone (75%) and longed for someone to live with. Among females, about 50% lived with a partner. Internationally, Sweden stands out with 53 of 69 adult individuals with MMC having their own hous- ing, but at the same time only 27 individuals of 69 told they were independent. This means that many of those living in their own homes need some sort of support. Several adults described how their parents, in their 80s, helped with cleaning and enemas (63). International studies have shown that many adults with MMC continue to live at home with their parent’s (64-66). Asking ques- tions about sexual experience, when the individual said he or she never had had a close intimate relation, felt unethical. What emerged was that out of those who now lived in a relationship, were also those who answered yes to the question on physically intimate relationships. Of the females 19% had children compared to 6% of the males. Corresponding figures from the Swedish population show that 86% of females and 82% of males had children (57). Neurogenic bladder and bowel dysfunction 20 Table 2. Life situation and civil status in 69 individuals (32 males and 37 females) with MMC. Control-group from Statistics Sweden 2011 No. (%) Control - Group % Ambulatory 29 (42) Shunt 57 (83) Educational level School for intellectual disability 5 (7) Elementary school 2 (3) High school 51 (74) 76 University 11 (16) 23 Driving license Yes 37 (54) 82 Employment No daily activity 18 (26) 7 Day center 3 (4) Education 2 (3) Employment 46 (67) Accommodation Own living 53 (77) Living in institution 10 (14) Living with parents 6 (9) Independent Yes 27 (39) No 42 (61) Single Total 42 (61) Males 24 (75) Females 18 (49) Sexual experience Total 27 (39) Males 8 (25) Females 19 (51) Individuals with offspring Total 9 (13) Males 2 (6) 82 Females 7 (19) 86 Magdalena Vu Minh Arnell 21 2. Urinary continenc e and bladder/bow el manageme nt Table 3. Urine and fecal continence, independence and support; comparison between adults and teenagers with MMC. When the results from study I were compared with study III, it turned out that adult individuals with MMC had not been given the opportunity to participate in the development of urotherapeutic care and bowel regimens. Many of the adults still had exactly the same sizes of pads, diapers or catheters that they had when they left pediatric care 10 to 30 years ago. Some pointed out that they had stopped CIC as it was too difficult to get catheters prescribed. One man told us, “If you get catheters prescribed th ere are just enough for weekends only, when I meet my friends, and the rest of the week I have to wear big diapers.” Today there are studies that show that a thicker catheter is a better choice than a thin one. There is a reduced risk for complication and the bladder empties faster, more efficiently and it is easier to empty completely (28,29). In study I, 22% of the adults used catheter ch10, a size suitable for preschool children with MMC. None of the teenagers used catheters ch10. CIC was conducted in 100% of teenagers and 71% of adults. Anticholinergics were used by 12% of adults, and 44% of adolescents. When comparing the continence levels of the two groups, there were large differences. In the youth group, 68% were continent for urine and 56% did not use pads at all. Comparative figures in the adult study showed a different situation where 29% were continent for urine and 13% did not use incontinence pads. Study I Study III Adults 27-50 yrs (no 69) Teenagers 16-18 yrs (no 25) 29% Urinary continence 68% 45% Fecal continence 76% 13% No pads 56 71% CIC 100% 22% Catheter size, 10 Ch 0 12% Anticholinergic drugs 44% 94% Independent in CIC 100% 14% Urotherapy support 100% Neurogenic bladder and bowel dysfunction 20 Table 2. Life situation and civil status in 69 individuals (32 males and 37 females) with MMC. Control-group from Statistics Sweden 2011 No. (%) Control - Group % Ambulatory 29 (42) Shunt 57 (83) Educational level School for intellectual disability 5 (7) Elementary school 2 (3) High school 51 (74) 76 University 11 (16) 23 Driving license Yes 37 (54) 82 Employment No daily activity 18 (26) 7 Day center 3 (4) Education 2 (3) Employment 46 (67) Accommodation Own living 53 (77) Living in institution 10 (14) Living with parents 6 (9) Independent Yes 27 (39) No 42 (61) Single Total 42 (61) Males 24 (75) Females 18 (49) Sexual experience Total 27 (39) Males 8 (25) Females 19 (51) Individuals with offspring Total 9 (13) Males 2 (6) 82 Females 7 (19) 86 Magdalena Vu Minh Arnell 21 2. Urinary continenc e and bladder/bow el manageme nt Table 3. Urine and fecal continence, independence and support; comparison between adults and teenagers with MMC. When the results from study I were compared with study III, it turned out that adult individuals with MMC had not been given the opportunity to participate in the development of urotherapeutic care and bowel regimens. Many of the adults still had exactly the same sizes of pads, diapers or catheters that they had when they left pediatric care 10 to 30 years ago. Some pointed out that they had stopped CIC as it was too difficult to get catheters prescribed. One man told us, “If you get catheters prescribed th ere are just enough for weekends only, when I meet my friends, and the rest of the week I have to wear big diapers.” Today there are studies that show that a thicker catheter is a better choice than a thin one. There is a reduced risk for complication and the bladder empties faster, more efficiently and it is easier to empty completely (28,29). In study I, 22% of the adults used catheter ch10, a size suitable for preschool children with MMC. None of the teenagers used catheters ch10. CIC was conducted in 100% of teenagers and 71% of adults. Anticholinergics were used by 12% of adults, and 44% of adolescents. When comparing the continence levels of the two groups, there were large differences. In the youth group, 68% were continent for urine and 56% did not use pads at all. Comparative figures in the adult study showed a different situation where 29% were continent for urine and 13% did not use incontinence pads. Study I Study III Adults 27-50 yrs (no 69) Teenagers 16-18 yrs (no 25) 29% Urinary continence 68% 45% Fecal continence 76% 13% No pads 56 71% CIC 100% 22% Catheter size, 10 Ch 0 12% Anticholinergic drugs 44% 94% Independent in CIC 100% 14% Urotherapy support 100% Neurogenic bladder and bowel dysfunction 22 In adult care, it is expressed that healthy individuals cannot be followed up for preventive purposes. CIC or bowel regimens are not regarding as requiring follow-ups. Of the adults in the study, 14% had contact with a urotherapist but no one had contact regarding the bowel emptying regimens. In the youth group, 100% followed a national care program and all had support for bladder and bowel regimens. Since 2018 there is also a follow-up program for adults with MMC (19). 3. Social partici pation and intimacy in rel ation to continence Table 4. Social participation and intimacy in 25 individuals with MMC between 16-18 years of age. In study III, 25 teenagers from 16 to 18 years of age were interviewed about current lives. The results of the study showed that 17 out of 25 individuals were urinary continent. To reach continence, 9 had undergone continence surgery, 6 had CIC in combination with anticholinergic treatment and 2 were continent using CIC only. Eight teenagers were incontinent, 5 of those had tried anticholinergic treatment to enhance continence but had finished the medication as it did not give desired result. Another 3 used anticholinergics but were still incontinent due to sphincter insufficiency. All 8, had been offered continence surgery but had declined. 2 5 participa nts Urinary continent no.17 Urinary incontinent no.8 12 Social participation 0 8 Physical intimacy 0 5 Sexual intercourse 0 11 (65%) Males 4 (50%) 8 (47%) Ambulatory (11) 3 (38%) 12 (71%) Shunt (20) 8 (100%) Magdalena Vu Minh Arnell 23 Of the individuals who were continent, 12 of 17 had a shunt and of those who were incontinent all had a shunt. We discovered that 12 of the 17 teenagers who were continent expressed that they were active in social life and had sports activities, met friends and were not afraid to sleep over at a friend's home. Eight of the 12 who were active in social life had experience of a close physical intimate relation. In those with urinary incontinent, no one said that they had a social life with activities outside the home, no one said they met friends in their free time, and no one had experience of close physical intimacy. In a qualitative study from Canada about incontinence and social participation, children and adolescents who were continent had more possibilities for social participation (67). However, we are aware that incontinence may not be the only reason why individuals with MMC do not socialize with friends. In a Palestinian study with children and youth with MMC it was shown more like a total stigmatization about their disability and not only their incontinence situation that made them lonely, vulnerable and without an active social life (68). Individuals cognitive difficulties can also contribute to exclusion (69). In a qualitative interview study (IV) where 9 adults with MMC described their experience of living with incontinence, the results showed that incontinence had a great impact on their lives. In our study we recognized that the individuals with MMC described similar experiences to those suffering from acquired incontinence. The participants described their thoughts about urinary leakage. The results are not generalizable as this was a qualitative study. However, the experience of congenital urinary incontinence and ways of adapting life is consistent with reports from participants with acquired incontinence (70-72). Neurogenic bladder and bowel dysfunction 22 In adult care, it is expressed that healthy individuals cannot be followed up for preventive purposes. CIC or bowel regimens are not regarding as requiring follow-ups. Of the adults in the study, 14% had contact with a urotherapist but no one had contact regarding the bowel emptying regimens. In the youth group, 100% followed a national care program and all had support for bladder and bowel regimens. Since 2018 there is also a follow-up program for adults with MMC (19). 3. Social partici pation and intimacy in rel ation to continence Table 4. Social participation and intimacy in 25 individuals with MMC between 16-18 years of age. In study III, 25 teenagers from 16 to 18 years of age were interviewed about current lives. The results of the study showed that 17 out of 25 individuals were urinary continent. To reach continence, 9 had undergone continence surgery, 6 had CIC in combination with anticholinergic treatment and 2 were continent using CIC only. Eight teenagers were incontinent, 5 of those had tried anticholinergic treatment to enhance continence but had finished the medication as it did not give desired result. Another 3 used anticholinergics but were still incontinent due to sphincter insufficiency. All 8, had been offered continence surgery but had declined. 2 5 participa nts Urinary continent no.17 Urinary incontinent no.8 12 Social participation 0 8 Physical intimacy 0 5 Sexual intercourse 0 11 (65%) Males 4 (50%) 8 (47%) Ambulatory (11) 3 (38%) 12 (71%) Shunt (20) 8 (100%) Magdalena Vu Minh Arnell 23 Of the individuals who were continent, 12 of 17 had a shunt and of those who were incontinent all had a shunt. We discovered that 12 of the 17 teenagers who were continent expressed that they were active in social life and had sports activities, met friends and were not afraid to sleep over at a friend's home. Eight of the 12 who were active in social life had experience of a close physical intimate relation. In those with urinary incontinent, no one said that they had a social life with activities outside the home, no one said they met friends in their free time, and no one had experience of close physical intimacy. In a qualitative study from Canada about incontinence and social participation, children and adolescents who were continent had more possibilities for social participation (67). However, we are aware that incontinence may not be the only reason why individuals with MMC do not socialize with friends. In a Palestinian study with children and youth with MMC it was shown more like a total stigmatization about their disability and not only their incontinence situation that made them lonely, vulnerable and without an active social life (68). Individuals cognitive difficulties can also contribute to exclusion (69). In a qualitative interview study (IV) where 9 adults with MMC described their experience of living with incontinence, the results showed that incontinence had a great impact on their lives. In our study we recognized that the individuals with MMC described similar experiences to those suffering from acquired incontinence. The participants described their thoughts about urinary leakage. The results are not generalizable as this was a qualitative study. However, the experience of congenital urinary incontinence and ways of adapting life is consistent with reports from participants with acquired incontinence (70-72). Neurogenic bladder and bowel dysfunction 24 4. Questionnai r e and Intervi ew Table 5. HRQoL SF-36 and statistics. Participants versus Swedish population. Domains Pa rticipa nts, mea n Referents, mea n p - v a lue Physical function 42,2 93,0 <0.0001 Role physical 64,8 88,9 <0.0001 Body pain 75,2 76,9 ns General health 67,7 79,1 <0.0001 Vitality 63,1 68,4 ns Social function 82,2 88,5 ns Emotional role 81,4 87,4 ns Mental health 74,0 80,9 ns Physical component summary 34,5 52,1 <0.0001 Mental component summary 51,0 49,4 <0.0001 Magdalena Vu Minh Arnell 25 Table 6. Life situation compared to HRQoL: PCS and MCS Variable PCS PCS, p - v a lue MCS MCS, p - v a lue Sex ns ns Age, 20-29 increased p<0.05 ns Age, 30-50 ns ns Shunt ns ns Walking, yes ns decreased p<0.05 Children ns ns Partnership ns ns Employment, yes increased p<0.05 ns University ns ns Pos. experience ns ns Neg. experience ns decreased p<0.05 Dysphoria, yes decreased p<0.05 ns Incontinence urine ns ns Incontinence feces ns ns Neurogenic bladder and bowel dysfunction 24 4. Questionnai r e and Intervi ew Table 5. HRQoL SF-36 and statistics. Participants versus Swedish population. Domains Pa rticipa nts, mea n Referents, mea n p - v a lue Physical function 42,2 93,0 <0.0001 Role physical 64,8 88,9 <0.0001 Body pain 75,2 76,9 ns General health 67,7 79,1 <0.0001 Vitality 63,1 68,4 ns Social function 82,2 88,5 ns Emotional role 81,4 87,4 ns Mental health 74,0 80,9 ns Physical component summary 34,5 52,1 <0.0001 Mental component summary 51,0 49,4 <0.0001 Magdalena Vu Minh Arnell 25 Table 6. Life situation compared to HRQoL: PCS and MCS Variable PCS PCS, p - v a lue MCS MCS, p - v a lue Sex ns ns Age, 20-29 increased p<0.05 ns Age, 30-50 ns ns Shunt ns ns Walking, yes ns decreased p<0.05 Children ns ns Partnership ns ns Employment, yes increased p<0.05 ns University ns ns Pos. experience ns ns Neg. experience ns decreased p<0.05 Dysphoria, yes decreased p<0.05 ns Incontinence urine ns ns Incontinence feces ns ns Neurogenic bladder and bowel dysfunction 26 In study I, several of the participants cried during the telephone interview and some expressed that they were dysphoric. They said that they felt forgotten and lonely. We also asked participants to answer SF-36, which is one of the world's most widely used generic instrument for measuring physical and mental health, study II. The questions were difficult for the individuals to answer, such as asking them how they felt now compared to four weeks ago, this due to the individuals' memory problems (51). We could also see that the scale with six different answer options were too difficult for participants, which is in accordance with the findings of Lindqvist and Dennis (73,74). Sometimes the individual did not answer what was intended by the question. The results showed, despite the individuals saying that they were dysphoric, that the overall mental health (MCS) was better than in the reference group. The total group had significantly lower scores, compared to the reference group in physical component summary (PCS). HRQoL, both physical and mental, was not affected by whether the individual had fecal incontinence, had a partner or had children. The PCS was lower but MCS was unaffected in the 12 individuals who expressed dysphoria during the interview. We considered that for individuals with MMC, SF-36 was not a reliable tool for measuring HRQoL. A better instrument to assess QoL in individuals with MMC is required. Magdalena Vu Minh Arnell 27 5 GENERAL DISCUSSION A ND FUTURE PERSPECTIVES In this dissertation, the overall questions were life situation and the prevalence of incontinence in adults and adolescents with MMC. The quantitative interview studies focused on the life situation of adults, such as education, work, housing and personal partnership. Urine elimination, incontinence and urological and urotherapeutic follow-up in adult care were also evaluated. To investigate whether the adult individuals' HRQoL was affected by the life situation and the incidence of incontinence, they answered SF-36, a generic HRQoL form with a focus on physical and mental health. In the fourth article, a qualitative interview, with focus on the individual's personal experience of living with incontinence and the way it affected their lives. In the adolescent group, who are followed through a urological/urotherapeutic care program, we prospectively evaluated urinary incontinence. Two issues were central, whether urinary continence affects the ability to live an active life in society and whether urinary continence is one of the pre-conditions for close physical intimacy. Life situation We chose to interview adult individuals by telephone so they would have the opportunity to answer the questions themselves. Furthermore, the study is not an objective observation but based on the personal opinion, thus, the reality for the individual. It is not uncommon when postal questionnaires are to be answered, individuals with MMC often receive help from relatives or assistants (64). A telephone interview was also a conscious choice as dropouts are common when individuals have to remember the place and time for an interview as their prospective memory both for time and events are impaired (51,54,73,75). Some studies have analyzed success factors for having a job, such as male gender and the ability to walk (75,76). In our study, it turned out that the significant factors to get a job were university education and continence, not to male gender, level of independence or ambulatory status. Taking this into account, Sweden is very well adapted to the disabled (78). Törnbom et al followed a group of individuals with spina bifida and cerebral palsy regarding their work situation. When they compared the group of individuals in 1997 with 2009, the number of individuals who had a job decreased significantly, but the level of education was a success factor for having a job (79) which also emerged in our study. Neurogenic bladder and bowel dysfunction 26 In study I, several of the participants cried during the telephone interview and some expressed that they were dysphoric. They said that they felt forgotten and lonely. We also asked participants to answer SF-36, which is one of the world's most widely used generic instrument for measuring physical and mental health, study II. The questions were difficult for the individuals to answer, such as asking them how they felt now compared to four weeks ago, this due to the individuals' memory problems (51). We could also see that the scale with six different answer options were too difficult for participants, which is in accordance with the findings of Lindqvist and Dennis (73,74). Sometimes the individual did not answer what was intended by the question. The results showed, despite the individuals saying that they were dysphoric, that the overall mental health (MCS) was better than in the reference group. The total group had significantly lower scores, compared to the reference group in physical component summary (PCS). HRQoL, both physical and mental, was not affected by whether the individual had fecal incontinence, had a partner or had children. The PCS was lower but MCS was unaffected in the 12 individuals who expressed dysphoria during the interview. We considered that for individuals with MMC, SF-36 was not a reliable tool for measuring HRQoL. A better instrument to assess QoL in individuals with MMC is required. Magdalena Vu Minh Arnell 27 5 GENERAL DISCUSSION A ND FUTURE PERSPECTIVES In this dissertation, the overall questions were life situation and the prevalence of incontinence in adults and adolescents with MMC. The quantitative interview studies focused on the life situation of adults, such as education, work, housing and personal partnership. Urine elimination, incontinence and urological and urotherapeutic follow-up in adult care were also evaluated. To investigate whether the adult individuals' HRQoL was affected by the life situation and the incidence of incontinence, they answered SF-36, a generic HRQoL form with a focus on physical and mental health. In the fourth article, a qualitative interview, with focus on the individual's personal experience of living with incontinence and the way it affected their lives. In the adolescent group, who are followed through a urological/urotherapeutic care program, we prospectively evaluated urinary incontinence. Two issues were central, whether urinary continence affects the ability to live an active life in society and whether urinary continence is one of the pre-conditions for close physical intimacy. Life situation We chose to interview adult individuals by telephone so they would have the opportunity to answer the questions themselves. Furthermore, the study is not an objective observation but based on the personal opinion, thus, the reality for the individual. It is not uncommon when postal questionnaires are to be answered, individuals with MMC often receive help from relatives or assistants (64). A telephone interview was also a conscious choice as dropouts are common when individuals have to remember the place and time for an interview as their prospective memory both for time and events are impaired (51,54,73,75). Some studies have analyzed success factors for having a job, such as male gender and the ability to walk (75,76). In our study, it turned out that the significant factors to get a job were university education and continence, not to male gender, level of independence or ambulatory status. Taking this into account, Sweden is very well adapted to the disabled (78). Törnbom et al followed a group of individuals with spina bifida and cerebral palsy regarding their work situation. When they compared the group of individuals in 1997 with 2009, the number of individuals who had a job decreased significantly, but the level of education was a success factor for having a job (79) which also emerged in our study. Neurogenic bladder and bowel dysfunction 28 Lifelong foll ow - up and support Today, we have extensive knowledge in several aspects of MMC. The condition is often associated with a great need for care due to complex physical and cognitive profile (47,51,53,55). In pediatric care, we work preventively in the care of individuals with MMC. At the age of 18 and followed up in Sweden since birth, there are no individuals with severe kidney damage (19). In our adult study participants who had not undergone urological surgery 69% were in adult care, followed very sporadically or not at all. We are convinced that these adults were undertreated in comparison to the adolescents with the same conditions. By urologists in adult care, it is a common opinion that they cannot follow individuals without obvious medical needs. The ICCS (International Children's Continence Society) recommendation of follow-up in adult care is every third year with ultrasound over the urinary tract but at the same time be checked closer with regard to CIC routines and urinary tract infections (80). With improved pediatric care, it is now the adults who become ill. There should be no reason why adult individuals not could have healthy kidneys during their lifetime (81). A regular multi-professional and urological follow- up that involves preservation of kidney function, CIC checkup and medication to maintain continence must become real for all adults with MMC (81,82). Close cooperation between the urologist and the urotherapist is necessary for follow-up when it comes to CIC, bowel regimens, medical treatment and continence surgery. It is medically difficult for several reasons to achieve urinary continence in this patient group (81,90). To reach the best results medical professionals must build up a trusting and respectful relationship with patients, to lead and support them so they can cope with vital, lifelong treatments. The adults need a lot of help, which is time-consuming for healthcare professionals and it takes years of experience of the condition to understand the needs of individuals with MMC. In a study from Miller et al where parents of children with spina bifida expressed what was important to them in the care of their child. The desire of continuity in care, thorough knowledge of the child and its diagnosis, communication and telephone follow-up and available care coordinator were predominated (83). The same desire the parents had for their child and for themselves could be a good suggestion for how the care would be structured even for adult individuals with MMC. Magdalena Vu Minh Arnell 29 CIC, incontinence and HRQoL The individuals in study I told that they experienced low levels of competence in the primary healthcare professionals with respect to products for urine retention and incontinence. It was obvious that the persons neither were guided in updating their aids, nor in following the development of products or new research in the field. This shows that education and up-dated information are required for those who prescribe aids for CIC and incontinence. There have been discussions among urologists that urinary incontinence in individuals with MMC does not appear to affect HRQoL negatively (84-86). Ehrén et al reported that only 20% of the adult individuals with MMC in their study used anticholinergic drugs, and one explanation could be that urinary leakage was not a major problem for the individual (87). When we evaluated the results of SF-36 in this patient group we found that life situation and incontinence were not truly reflected by this instrument. With a diagnosis specific HRQoL instrument, Szymanski et al have been able to show that children with MMC and urinary incontinence had impaired HRQoL already at the age of 10 (88). In addition, Verheof et al reported that 69% of young adults with MMC perceive their urinary incontinence as a problem and in a study from US the authors reported that HRQoL is impaired in adults with MMC who have urinary incontinence (88,89). In the adolescents’ study we define continence as, “always dry ” without prescription of incontinence pads, which correspond to the definition in a French study (90). The definition of urinary incontinence is not consistent in the literature with regard to individuals with MMC. The most common definition was “always dry” but only 37% of the authors described which method they had used to evaluate the continence status of participants (91). In another registry study from the National Spina Bifida Patient Registry in US “Less than once per month” were considered as continent (22). Furthermore, the result from a study by Szymanski et al indicate it was quantity of urinary leakage that was the most bothersome (92). Perrouin-Verbe discussed in their article the medical risk of operating on individuals with MMC because of their cognitive disturbances. Until adulthood, there is support from the parents in coping with CIC, regular bladder washout, but when the individuals have to manage independently major problems may occur. Before decision about continence surgery, the question is how to select those individuals with MMC who can cope independently after the operation. However, continence surgery is the only thing that provides complete urinary continence, which is important to a good life in the company of others. (90). Neurogenic bladder and bowel dysfunction 28 Lifelong foll ow - up and support Today, we have extensive knowledge in several aspects of MMC. The condition is often associated with a great need for care due to complex physical and cognitive profile (47,51,53,55). In pediatric care, we work preventively in the care of individuals with MMC. At the age of 18 and followed up in Sweden since birth, there are no individuals with severe kidney damage (19). In our adult study participants who had not undergone urological surgery 69% were in adult care, followed very sporadically or not at all. We are convinced that these adults were undertreated in comparison to the adolescents with the same conditions. By urologists in adult care, it is a common opinion that they cannot follow individuals without obvious medical needs. The ICCS (International Children's Continence Society) recommendation of follow-up in adult care is every third year with ultrasound over the urinary tract but at the same time be checked closer with regard to CIC routines and urinary tract infections (80). With improved pediatric care, it is now the adults who become ill. There should be no reason why adult individuals not could have healthy kidneys during their lifetime (81). A regular multi-professional and urological follow- up that involves preservation of kidney function, CIC checkup and medication to maintain continence must become real for all adults with MMC (81,82). Close cooperation between the urologist and the urotherapist is necessary for follow-up when it comes to CIC, bowel regimens, medical treatment and continence surgery. It is medically difficult for several reasons to achieve urinary continence in this patient group (81,90). To reach the best results medical professionals must build up a trusting and respectful relationship with patients, to lead and support them so they can cope with vital, lifelong treatments. The adults need a lot of help, which is time-consuming for healthcare professionals and it takes years of experience of the condition to understand the needs of individuals with MMC. In a study from Miller et al where parents of children with spina bifida expressed what was important to them in the care of their child. The desire of continuity in care, thorough knowledge of the child and its diagnosis, communication and telephone follow-up and available care coordinator were predominated (83). The same desire the parents had for their child and for themselves could be a good suggestion for how the care would be structured even for adult individuals with MMC. Magdalena Vu Minh Arnell 29 CIC, incontinence and HRQoL The individuals in study I told that they experienced low levels of competence in the primary healthcare professionals with respect to products for urine retention and incontinence. It was obvious that the persons neither were guided in updating their aids, nor in following the development of products or new research in the field. This shows that education and up-dated information are required for those who prescribe aids for CIC and incontinence. There have been discussions among urologists that urinary incontinence in individuals with MMC does not appear to affect HRQoL negatively (84-86). Ehrén et al reported that only 20% of the adult individuals with MMC in their study used anticholinergic drugs, and one explanation could be that urinary leakage was not a major problem for the individual (87). When we evaluated the results of SF-36 in this patient group we found that life situation and incontinence were not truly reflected by this instrument. With a diagnosis specific HRQoL instrument, Szymanski et al have been able to show that children with MMC and urinary incontinence had impaired HRQoL already at the age of 10 (88). In addition, Verheof et al reported that 69% of young adults with MMC perceive their urinary incontinence as a problem and in a study from US the authors reported that HRQoL is impaired in adults with MMC who have urinary incontinence (88,89). In the adolescents’ study we define continence as, “always dry ” without prescription of incontinence pads, which correspond to the definition in a French study (90). The definition of urinary incontinence is not consistent in the literature with regard to individuals with MMC. The most common definition was “always dry” but only 37% of the authors described which method they had used to evaluate the continence status of participants (91). In another registry study from the National Spina Bifida Patient Registry in US “Less than once per month” were considered as continent (22). Furthermore, the result from a study by Szymanski et al indicate it was quantity of urinary leakage that was the most bothersome (92). Perrouin-Verbe discussed in their article the medical risk of operating on individuals with MMC because of their cognitive disturbances. Until adulthood, there is support from the parents in coping with CIC, regular bladder washout, but when the individuals have to manage independently major problems may occur. Before decision about continence surgery, the question is how to select those individuals with MMC who can cope independently after the operation. However, continence surgery is the only thing that provides complete urinary continence, which is important to a good life in the company of others. (90). Neurogenic bladder and bowel dysfunction 30 FUTURE PERSPECTIVES Transition from pediatric to adult care For adults with MMC, there is a great need for continuous follow-up (81,82,90,93). When transition from pediatric to adult care occurs, the process should be individually designed, and account must be taken of the individuals´ maturity and disability. NOBAB-standard (Nordic standard for children and young people in health care proclaims that, at the time of the transition to adult care, the adolescent has the right to continuity, preparation, information, FRRSHUDWLRQ UHVSHFW LQWHJULW\ DQG TXDOL¿HG personnel (94). Adult health care is not organized to meet these multi-medical needs (95-97). TRAQ-SB (Transition Readiness Assessment Questionnaire Spina Bifida) is used in many countries to evaluate whether the individual is mature enough to be transferred to adult care (98). Roth et al found out that only older age was associated with transition readiness (99). A successful organization according to transition readiness for adolescents with MMC could be a closer cooperation between pediatric and adult care. However, there is still questions raised about how to overcome the difficulties for this patient group described in examples given by the researchers; The adult ward was just around the corner from pediatric ward but only 40% attended the adult care (100). Roth et al asked; do we demand too much of the young people with MMC? (101). Hettel et al found out that even with new urological problems the adults did not seek care in the adult ward (102). In the dissertation, we have not focused on fecal incontinence, but we are fully aware that it is a major problem for individuals with MMC. In tables in Studies I and III, we have shown the frequency of fecal incontinence. In Study IV, questions about experience of fecal incontinence were also asked, but the results will be presented in a further study. Szymanski et al evaluated the impact of fecal incontinence on HRQoL in children from the age of 8, as well as in adolescents and adults. Results showed that the quality of life was impaired (103). Magdalena Vu Minh Arnell 31 6 CONCLUSION In adults with MMC from western Sweden, born between 1957 and 1980, about 60% were single and just a few had children. It seemed to be easier for females to establish close relations. Few had urotherapy support, none had support with their fecal elimination regimen and the majority used pads. To use pads had a negative influence on becoming employed. To evaluate HRQoL. SF-36 was used. As an overall group they had significant impairment of PCS and improvement of MCS compared to the Swedish population. Surprisingly, HRQoL was unaffected by fecal incontinence, partnership and offspring. Even in the individuals who were dysphoric during the interview, MCS was unaffected, while PCS was decreased. As we believe SF-36 to be an unreliable instrument, a personal interview with a qualitative approach was performed in 9 of the individuals who had urinary incontinence and who could openly recount how incontinence restricts and affects their lives. The results are consistent with reports from individuals with incontinence due to acquired conditions. With standardized follow-up, active treatment strategy, and urinary/bowel therapy, almost 70% of adolescents with MMC can reach urinary continence. Urinary continence seems to be a success factor in creating an age-appropriate active social life and seems to enhance the possibility of finding a partner and becoming physically intimate. Neurogenic bladder and bowel dysfunction 30 FUTURE PERSPECTIVES Transition from pediatric to adult care For adults with MMC, there is a great need for continuous follow-up (81,82,90,93). When transition from pediatric to adult care occurs, the process should be individually designed, and account must be taken of the individuals´ maturity and disability. NOBAB-standard (Nordic standard for children and young people in health care proclaims that, at the time of the transition to adult care, the adolescent has the right to continuity, preparation, information, FRRSHUDWLRQ UHVSHFW LQWHJULW\ DQG TXDOL¿HG personnel (94). Adult health care is not organized to meet these multi-medical needs (95-97). TRAQ-SB (Transition Readiness Assessment Questionnaire Spina Bifida) is used in many countries to evaluate whether the individual is mature enough to be transferred to adult care (98). Roth et al found out that only older age was associated with transition readiness (99). A successful organization according to transition readiness for adolescents with MMC could be a closer cooperation between pediatric and adult care. However, there is still questions raised about how to overcome the difficulties for this patient group described in examples given by the researchers; The adult ward was just around the corner from pediatric ward but only 40% attended the adult care (100). Roth et al asked; do we demand too much of the young people with MMC? (101). Hettel et al found out that even with new urological problems the adults did not seek care in the adult ward (102). In the dissertation, we have not focused on fecal incontinence, but we are fully aware that it is a major problem for individuals with MMC. In tables in Studies I and III, we have shown the frequency of fecal incontinence. In Study IV, questions about experience of fecal incontinence were also asked, but the results will be presented in a further study. Szymanski et al evaluated the impact of fecal incontinence on HRQoL in children from the age of 8, as well as in adolescents and adults. Results showed that the quality of life was impaired (103). Magdalena Vu Minh Arnell 31 6 CONCLUSION In adults with MMC from western Sweden, born between 1957 and 1980, about 60% were single and just a few had children. It seemed to be easier for females to establish close relations. Few had urotherapy support, none had support with their fecal elimination regimen and the majority used pads. To use pads had a negative influence on becoming employed. To evaluate HRQoL. SF-36 was used. As an overall group they had significant impairment of PCS and improvement of MCS compared to the Swedish population. Surprisingly, HRQoL was unaffected by fecal incontinence, partnership and offspring. Even in the individuals who were dysphoric during the interview, MCS was unaffected, while PCS was decreased. As we believe SF-36 to be an unreliable instrument, a personal interview with a qualitative approach was performed in 9 of the individuals who had urinary incontinence and who could openly recount how incontinence restricts and affects their lives. The results are consistent with reports from individuals with incontinence due to acquired conditions. With standardized follow-up, active treatment strategy, and urinary/bowel therapy, almost 70% of adolescents with MMC can reach urinary continence. Urinary continence seems to be a success factor in creating an age-appropriate active social life and seems to enhance the possibility of finding a partner and becoming physically intimate. Neurogenic bladder and bowel dysfunction 32 Magdalena Vu Minh Arnell 33 ACKN OWLEDGEMENT S First, to the adults and teenagers who participated in the studies, thank you so much for your invaluable contribution. I am grateful for the support, help and commitment from those who have been involved in my work with the thesis. I would like to express my gratitude especially to: Kate Abrahamss on my main supervisor, colleague for almost 25 years, and friend. We have a common great interest in children with MMC and work together in the team to develop care and improve the individual's quality of life. You have always advocated and pointed out the importance of a close collaboration between the urologist and the urotherapist to achieve the best treatment results for the patient. I'm so honored to be urotherapist by your side! It has been a pleasure to work with you in both the clinic and with the dissertation. Always with mutual respect and with never-failing enthusiasm you guide me when my self-confidence has waned and help me move forward with the research work. I am deeply grateful to you. Anders Möller my co-supervisor in 2011-2012. Always kind and supporting. When we had thoughts about ethical considerations you were always available for rewarding discussions. Birgitta Lindehall my co-author and dear friend, role model as a pediatric nurse and urotherapist. As a young nurse 1983 I was lucky to meet a competent, curious, dedicated and proud nurse colleague. I am so glad that you 30 years ago asked me to work together with you and the team in the urotherapeutic ward. You also pushed and inspired me and persuaded me to start the research education. I have the best work a pediatric nurse and urotherapist ever can have and that is thanks to you, Birgitta. Katarina Seljee Svedberg my co-author and another outstanding pediatric nurse and urotherapist and my soul mate. We started to study caring science together and we made the interviews to the two first articles. You took a different path in working life and I continued with what we started . Ingrid Bobo Olsson former neurologist in our team. I have had the privilege to work with you and learn so much about the diagnosis MMC. I admire your great knowledge, interest in, and strong commitment to the children with MMC and their families. Neurogenic bladder and bowel dysfunction 32 Magdalena Vu Minh Arnell 33 ACKN OWLEDGEMENT S First, to the adults and teenagers who participated in the studies, thank you so much for your invaluable contribution. I am grateful for the support, help and commitment from those who have been involved in my work with the thesis. I would like to express my gratitude especially to: Kate Abrahamss on my main supervisor, colleague for almost 25 years, and friend. We have a common great interest in children with MMC and work together in the team to develop care and improve the individual's quality of life. You have always advocated and pointed out the importance of a close collaboration between the urologist and the urotherapist to achieve the best treatment results for the patient. I'm so honored to be urotherapist by your side! It has been a pleasure to work with you in both the clinic and with the dissertation. Always with mutual respect and with never-failing enthusiasm you guide me when my self-confidence has waned and help me move forward with the research work. I am deeply grateful to you. Anders Möller my co-supervisor in 2011-2012. Always kind and supporting. When we had thoughts about ethical considerations you were always available for rewarding discussions. Birgitta Lindehall my co-author and dear friend, role model as a pediatric nurse and urotherapist. As a young nurse 1983 I was lucky to meet a competent, curious, dedicated and proud nurse colleague. I am so glad that you 30 years ago asked me to work together with you and the team in the urotherapeutic ward. You also pushed and inspired me and persuaded me to start the research education. I have the best work a pediatric nurse and urotherapist ever can have and that is thanks to you, Birgitta. Katarina Seljee Svedberg my co-author and another outstanding pediatric nurse and urotherapist and my soul mate. We started to study caring science together and we made the interviews to the two first articles. You took a different path in working life and I continued with what we started . Ingrid Bobo Olsson former neurologist in our team. I have had the privilege to work with you and learn so much about the diagnosis MMC. I admire your great knowledge, interest in, and strong commitment to the children with MMC and their families. Neurogenic bladder and bowel dysfunction 34 Gloria Davidsson, Anna - Lena Kronander , Rikke Korsgaard , Rigmo r Linder , Claery Andersson and Karin Gildebrand my co-workers and my former co-workers Inger Odeholm , Carina Perdsjö Runemark , Yvonne Gröning , Kirsti Ahlberg and Marika Perss on , committed, wise, knowledgeable and wonderful persons. We share the same values and children and youths with disabilities have a special place in our hearts. Lisa Bondjers, Ana Dos Santos Rufino and Matilda Bräutigam you are all an important part of our team. With different expertise we provide the best conditions for optimal care for the children we follow. Thank you for always good cooperation. Kristin Edbom for transcribing the interviews and for excellent help and support with the layout of the thesis. I cannot thank you enough! All coll eagues at Regional Rehabilitation Centre for children and adolescents at Queen Silvia Children´s Hospital, Sahlgrenska University Hospital. Thank you for good cooperation throughout the years. To my fami ly Mattias my husband with an outstanding patience. Never once during all these years have you complained that I have invested so many hours, evenings and weekends in my work. In recent months, I have received all the support imaginable to be able to finish my dissertation. From the bottom of my heart, thank you darling! Emma, Julia and Lisa my strong, beautiful, intelligent, caring girls, who make your mother extremely proud and grateful to have you all in my life. Not to forget your nice, loving husbands Jimmy, Olle and Erik. The great change of recent years in our family make life even more wonderful to live, the joy of our adorable grandchildren; Lo, Nora, Edda, Inez and Frans. Ingrid my positive, life-loving mother who always supporting me and believing in me. This thesis was financially supported by grants from Petter Silfverskiölds Foundation, Willhelm and Martina Lundgren Foundation, Norrbacka-Eugenia Foundation, The Research Foundation of Queen Silvia Children’s Hospital, RBU Swedish National Association for Disabled Children and Young People, Regional University grants from Sahlgrenska University Hospital and Bertha and Felix Neuberghs Foundation. Magdalena Vu Minh Arnell 35 REFERENCES 1. Zaganjor A, Sekkarie, BLTsang et al. Describing the prevalence of neural tube defects worldwide: a systematic Literature Review PLoS 2016 Apr 11;11(4):e0151586. doi: 10.1371/journal.pone.0151586. 2. Statistikmyndigheten, statistiska central byrån, SCB (internet) https://www.scb.se/hitta-statistik/sverige-i-siffror/manniskorna-i-sverige/ sveriges-befolkning/ Accessed October 10, 2020. 3. Socialstyrelsen (The Swedish national board of health and welfare). Official statistics of Sweden; statistics for health and medical care; Birth Defects (internet). Available from: www.socialstyrelsen.se; Accessed January 20, 2020. 4. The Swedish Peadiatric Society (internet) https://snpf.barnlakarforeningen.se/vardprogram-2/nationella-riktlinjer-for- medicinsk-uppfoljning-vid-ryggmargsbrack-mmc/ Accessed July 31, 2020. 5. Collins J, Atkinson K, Dean J, et al. Longterm maint(1)enance of neural tube defects prevention in high prevalence state. J Pediatr 2011Jul;159(1):143-9. doi: 10.1016/j.jpeds.2010.12.037. 6. Atta CA, Fiest KM, Frolkis AD et.al Global Birth Prevalence of Spina Bifida by Folic Acid Fortification Status: A Systematic Review and Meta- Analysis. Am J Public Health. 2016 Jan;106(1):e24-34. doi: 10.2105/AJPH.2015.302902. 7. BilleB,OlowI. Barnhabilitering vid rörelsehinder.1ed. Uppsala: Almqvist& Wiksell Förlag AB; 1992. Chapter 7, Missbildningar, skador och sjukdomar i centrala nervsystemet och perifera nerver; p104-108. 8. Peyronnet B, GaoF, Brochard E. Urologic Disorders are Still the Leading Cause of In-hospital Death in Patients with Spina Bifida. Urology 2020 Mar; 137:200-204.doi: 10.1016/j.urology.2019.11.006. 9. Lidal IB, Lundberg Larsen K, Hoff M. 50 Years and older - born with spina bifida: participation, health issues and physical function. Disabil Rehabil. 2019 Jun;10:1-10. doi: 10.1080/09638288.2019.1621953. 10. Liptak GS, Robinson LM, Davidson PW, Dziorny A, Lavalley R, Flaherty M et al. Life course health and healthcare utilization among adults with spina bifida. Dev Med Child Neurol. 2016 Jul;58(7):714-20. doi: 10.1111/dmcn.12952. Neurogenic bladder and bowel dysfunction 34 Gloria Davidsson , Anna - Lena Kronander , Rikke Korsgaard , Rigmo r Linder , Claery Andersson and Karin Gildebrand my co-workers and my former co-workers Inger Odeholm , Carina Perdsjö Runemark , Yvonne Gröning , Kirsti Ahlberg and Marika Perss on , committed, wise, knowledgeable and wonderful persons. We share the same values and children and youths with disabilities have a special place in our hearts. Lisa Bondjers, Ana Dos Santos Rufino and Matilda Bräutigam you are all an important part of our team. With different expertise we provide the best conditions for optimal care for the children we follow. Thank you for always good cooperation. Kristin Edbom for transcribing the interviews and for excellent help and support with the layout of the thesis. I cannot thank you enough! All coll eagues at Regional Rehabilitation Centre for children and adolescents at Queen Silvia Children´s Hospital, Sahlgrenska University Hospital. Thank you for good cooperation throughout the years. To my fami ly Mattias my husband with an outstanding patience. Never once during all these years have you complained that I have invested so many hours, evenings and weekends in my work. In recent months, I have received all the support imaginable to be able to finish my dissertation. From the bottom of my heart, thank you darling! Emma, Julia and Lisa my strong, beautiful, intelligent, caring girls, who make your mother extremely proud and grateful to have you all in my life. Not to forget your nice, loving husbands Jimmy, Olle and Erik. The great change of recent years in our family make life even more wonderful to live, the joy of our adorable grandchildren; Lo, Nora, Edda, Inez and Frans. Ingrid my positive, life-loving mother who always supporting me and believing in me. This thesis was financially supported by grants from Petter Silfverskiölds Foundation, Willhelm and Martina Lundgren Foundation, Norrbacka-Eugenia Foundation, The Research Foundation of Queen Silvia Children’s Hospital, RBU Swedish National Association for Disabled Children and Young People, Regional University grants from Sahlgrenska University Hospital and Bertha and Felix Neuberghs Foundation. Magdalena Vu Minh Arnell 35 REFERENCES 1. Zaganjor A, Sekkarie, BLTsang et al. Describing the prevalence of neural tube defects worldwide: a systematic Literature Review PLoS 2016 Apr 11;11(4):e0151586. doi: 10.1371/journal.pone.0151586. 2. Statistikmyndigheten, statistiska central byrån, SCB (internet) https://www.scb.se/hitta-statistik/sverige-i-siffror/manniskorna-i-sverige/ sveriges-befolkning/ Accessed October 10, 2020. 3. Socialstyrelsen (The Swedish national board of health and welfare). Official statistics of Sweden; statistics for health and medical care; Birth Defects (internet). 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Neurourology and Urodynamics 2012;31:1170– 1174. 18. Martinez L, Neshatian L, Khavario R. Neurgenic Bowel Dysfunction in Patients with Neurogenic Bladder. Curr Bladder Dysfunct Rep.2016 Dec;11(4):334-340. https;//doi: 10.1007/s11884-016-0390-3. 19. MMCUP. The National MMC Follow-Up Program and Quality of Care Registry(internet) www.MMCUP.se Accessed October 10, 2020. 20. Vande Velde S, Van Biervliet S, Van Laecke E. Colon enemas for fecal incontinence in patients with spina bifida. J Urol 2013;189:300e4. https://doi.org/10.1016/j.juro.2012.09.007. 21. Vande Velde S, Biervliet S, Bruyne RD, Winckel MV. A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients. Spinal Cord 2013;51:873-881.doi: 10.1038/sc.2013.123. Magdalena Vu Minh Arnell 37 22. Freeman K, Castillo H, Castillo J, Liu T, Schechter M, Wiener J et al. Bowel and bladder continence across US spina bifida programs: A descriptive study.J Pediatr Rehabil Med.2017 Dec11;10(3-4):231-241. doi: 10.3233/PRM-170450. 23. Schletker J, Edmonds T, Jacobson R, Ketzer J, Hall J, Trecartin et al. Bowel management program in patients with spina bifida Pediatr Surg Int. 2019 Feb;35(2):243-245. doi: 10.1007/s00383-018-4403-5 24. Wide P, Glad Mattsson G, Drott P, Mattsson S. Independence does not come with the method – treatment of neurogenic bowel dysfunction in children with myelomeningocele. ActaPed.2014;103:11591164.doi:10.1111/apa.12756. 25. Sanders C, Bray L, Driver C, Harris V. Parents of children with neurogenic bowel dysfunction: their experiences of using transanal irrigation with their child. Child Care Health Dev.2014Nov;40(6):863-9. doi: 10.1111/cch.12117. 26. HellströmA-L,LindehallB. URO-TARMTERAPI.2ed Lund: Studentlitteratur AB; 2019. Chapter 01, Inledning; p 19– 20. 27. Nordisk uroterapeutisk förening(internet).https://utfnordic.org/Accessed July 31, 2020. 28. Lapides J, Diokno AC, Silber SM, Lowe BS. Clean intermittent self- catheterization in the treatment of urinary tract disease. 1972. J Urol. 2002 Apr;167(4):1584-6 doi:10.1016/s0022-5347(17)61055-3. 29. Lindehall B, Claesson I, Hjälmås K, Jodal U. Effect of clean intermittent catheterization on radiological appearance of the upper urinary tract in children with myelomeningocele. Br J Urol. 1991 Apr;67(4):415-9. doi: 10.1111/j.1464-410x.1991.tb15169.x. 30. Proesmans W. The neurogenic bladder: introducing four contributions. Pediatr Nephrol 2008 Apr;23(4):537-40. doi: 10.1007/s00467-008-0768-3. 31. Clayton D, Brock J. The Urologist's Role in the Management of Spina Bifida: A Continuum of Care. Urology 2010Jul;76(1):32-8. doi: 10.1016/j.urology.2009.12.063. 32. Elzeneini W, Waly R, Marshall D, Bailie A. Early start of clean intermittent catheterization versus expectant management in children with spina bifida. J Pediatr Surg. 2019 Feb;54(2):322-325. doi:10.1016/j.jpedsurg.2018.10.096. Neurogenic bladder and bowel dysfunction 36 11. Alriksson-Schmidt A, Lundkvist Josenby A, Lindquist B, Westbom L. Pain and health status in adults with myelomeningocele living in Sweden. J Pediatr Rehabil Med. 2018;11(4):255–264. doi: 10.3233/PRM -170517. 12. Griffiths D, Tadic SD. Bladder control, urgency, and urge incontinence: evidence from functional brain imaging. Neurourol Urodyn.2008;27(6): 466–74. doi: 10.1002/nau.20549. 13. SUF Svensk Urologisk Förening. State of Art 2015, Störningar av de nedre urinvägarnas funktioner(internet)2015.Normal utveckling och funktion och anatomi. Miktionens fysiologi;p.16–25. https://urologi.org/wp- content/uploads/2016/12/Sota_NU_funktion_150629.pdf; Accessed July 19,2020. 14. Bille B, Olow I. Barnhabilitering vid rörelsehinder.1ed. Uppsala: Almqvist& Wiksell Förlag AB;1992. Chapter 23, Urologi; p.235–241 . 15. Leif Hultén, Svante Nordgren, Tom Öresland. Fekal inkontinens ett gömt problem.1 ed. Växjö: Teknikinfo AB; 1995. Rektoanal anatomi-fysiologi; p.9–16. 16. Mattsson S, Gladh G. Tap water enema for children with myelomeningocele and neurogenic bowel dysfunction. Acta Paediatr 2006; 95: 369–37417. 17. De Kort LM, Bower WF, Swithinbank LV, Marschall-Kehrel D, De Jong T, Bauer S B. The Management of Adolescents with Neurogenic Urinary Tract and Bowel Dysfunction. Neurourology and Urodynamics 2012;31:1170– 1174. 18. Martinez L, Neshatian L, Khavario R. Neurgenic Bowel Dysfunction in Patients with Neurogenic Bladder. Curr Bladder Dysfunct Rep.2016 Dec;11(4):334-340. https;//doi: 10.1007/s11884-016-0390-3. 19. MMCUP. The National MMC Follow-Up Program and Quality of Care Registry(internet) www.MMCUP.se Accessed October 10, 2020. 20. Vande Velde S, Van Biervliet S, Van Laecke E. Colon enemas for fecal incontinence in patients with spina bifida. J Urol 2013;189:300e4. https://doi.org/10.1016/j.juro.2012.09.007. 21. Vande Velde S, Biervliet S, Bruyne RD, Winckel MV. A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients. Spinal Cord 2013;51:873-881.doi: 10.1038/sc.2013.123. Magdalena Vu Minh Arnell 37 22. Freeman K, Castillo H, Castillo J, Liu T, Schechter M, Wiener J et al. Bowel and bladder continence across US spina bifida programs: A descriptive study.J Pediatr Rehabil Med.2017 Dec11;10(3-4):231-241. doi: 10.3233/PRM-170450. 23. Schletker J, Edmonds T, Jacobson R, Ketzer J, Hall J, Trecartin et al. Bowel management program in patients with spina bifida Pediatr Surg Int. 2019 Feb;35(2):243-245. doi: 10.1007/s00383-018-4403-5 24. Wide P, Glad Mattsson G, Drott P, Mattsson S. Independence does not come with the method – treatment of neurogenic bowel dysfunction in children with myelomeningocele. ActaPed.2014;103:11591164.doi:10.1111/apa.12756. 25. Sanders C, Bray L, Driver C, Harris V. Parents of children with neurogenic bowel dysfunction: their experiences of using transanal irrigation with their child. Child Care Health Dev.2014Nov;40(6):863-9. doi: 10.1111/cch.12117. 26. HellströmA-L,LindehallB. URO-TARMTERAPI.2ed Lund: Studentlitteratur AB; 2019. Chapter 01, Inledning; p 19–20. 27. Nordisk uroterapeutisk förening(internet).https://utfnordic.org/Accessed July 31, 2020. 28. Lapides J, Diokno AC, Silber SM, Lowe BS. Clean intermittent self- catheterization in the treatment of urinary tract disease. 1972. J Urol. 2002 Apr;167(4):1584-6 doi:10.1016/s0022-5347(17)61055-3. 29. Lindehall B, Claesson I, Hjälmås K, Jodal U. Effect of clean intermittent catheterization on radiological appearance of the upper urinary tract in children with myelomeningocele. Br J Urol. 1991 Apr;67(4):415-9. doi: 10.1111/j.1464-410x.1991.tb15169.x. 30. Proesmans W. The neurogenic bladder: introducing four contributions. Pediatr Nephrol 2008 Apr;23(4):537-40. doi: 10.1007/s00467-008-0768-3. 31. Clayton D, Brock J. The Urologist's Role in the Management of Spina Bifida: A Continuum of Care. Urology 2010Jul;76(1):32-8. doi: 10.1016/j.urology.2009.12.063. 32. Elzeneini W, Waly R, Marshall D, Bailie A. Early start of clean intermittent catheterization versus expectant management in children with spina bifida. J Pediatr Surg. 2019 Feb;54(2):322-325. doi:10.1016/j.jpedsurg.2018.10.096. Neurogenic bladder and bowel dysfunction 38 33. Shelton TL, Stepanek, JS. Excerpts from Family-Centered Care for Children Needing Specialized Health and Developmental Services. Pediatric Nursing 1995; 21(4): 362-64. 34. Sinha S. Follow-up urodynamics in patients with neurogenic bladder. Indian J Urol. 2017 Oct-Dec;33(4):267–275. doi: 10.4103/iju.IJU_358_16 . 35. Musco S, Padilla-Fernández B, Del Popolo G, Bonifazi M, Blok BFM, Groen J. Value of urodynamic findings in predicting upper urinary tract damage in neuro-urological patients: A systematic review. Neurourol Urodyn. 2018;37:1522-1540. doi: 10.1002/nau.23501. 36. Abrahamsson K, Olsson I, Sillen U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol. 2007; 177:331-334. doi: 10.1016/j.juro.2006.08.146. 37. Averbeck MA, Madersbacher H. Follow-up of the neuro-urological patient: a systematic review. BJUInt.2015;115 Suppl 6:39-46. doi: 10.1111/bju.13084. 38. Wide P, Duchén K, Mattsson S, Glad Mattsson G. Four-hour voiding observation with provocation test reveals significant abnormalities of bladder function in newborns with spinal dysraphism. J Pediatr Urol 2020 Aug;16(4): 491.e1-491.e7. doi: 10.1016/j.jpurol.2020.06.022. 39. Gladh G, Persson D, Mattsson S, Lindström S. Voiding pattern in healthy newborns. Neurourol Urodyn 2000;19:177-184.doi:10.1002/(sici)1520- 6777(2000)19:2<177::aid-nau8>3.0.co;2-0. 40. Lindehall B, Abrahamsson K, Jodal U, Olsson I, Sillén U. Complications at clean intermittent catheterization in boys and young males with neurogenic bladder dysfunction. J Urol 2004;172:1986-8. doi:10.1097/01.ju.0000138847.14680.7d. 41. Lindehall B, Möller A, Hjälmås K, Jodal U, Abrahamsson K. Psychosocial factors in teenagers and young adults with myelomeningocele and clean intermittent catheterization. Scand J Urol Nephrol. 2008;42(6):539-44. doi: 10.1080/00365590802273218. 42. Lindquist B, Carlsson G, Persson EK, Uvebrant P. Learning disabilities in a population-based group of children with hydrocephalus. Acta Paediatr 2005; 94:878-83.doi: 10.1111/j.1651-2227.2005.tb02005.x. 43. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 2001 Mar;34:114e20. doi: 10.1159/000056005. Magdalena Vu Minh Arnell 39 44. Bendt M, Gabrielsson H, Riedel D, Hagman G, Hultling C, Franzén E et al. Adults with spina bifida: A cross-sectional study of health issues and living conditions. Brain Behav.2020;00:e01736. doi: 10.1002/brb3.1736. 45. Olsson I, Dahl M, Mattsson S, Wendelius M, Astrom E, Westbom L. Medical problems in adolescents with myelomeningocele (MMC):an inventory of the Swedish MMC population born during1986-1989. ActaPaediatr.2007Mar;96(3):446-9. doi:10.1111/j.1651-2227.2006.00153.x 46. Patel S, Staarmann B, Heilman A, Mains A , Woodward J, Bierbrauer K. Growing up with spina bifida: bridging the gaps in the transition of care from childhood to adulthood. Neurosurg Focus.2019 Oct1;47(4):E16.doi: 10.3171/2019.7.FOCUS19441. 47. Kritikos TK, Smith K, Holmbeck GN. Mental health guidelines for the care of people with spina bifida. J Pediatr Rehabil Med. 2020 Sep 18. doi: 10.3233/PRM-200719. Online ahead of print. 48. Barf A, Post HM, Verhoef M, Jennekens-Schinkel A, Gooskens M, Prevo H.Restrictions in social participation of young adults with spina bifida. Disabil Rehabil 2009;31:921e7. doi: 10.1080/09638280802358282. 49. Devine K, Holmbeck G, Gayes L, Purnell J. Friendships of children and adolescents with spina bifida: social adjustment, social performance, and social skills. J Pediatr Psychol 2011;37:220e31. doi:10.1093/jpepsy/jsr075. 50. Juranek J, Sahlman M. Anomalous development of brain structure and function in spina bifida myelomeningocele. Developmental Disabilities Res Rev 2010;16:23e30.doi: 10.1002/ddrr.88. 51. Dennis M, Nelson R, Jewell D, Fletcher JM. Prospective memory in adults with spina bifida. Childs Nerv Syst. 2010Dec;26(12):1749-55. doi: 10.1007/s00381-010-1140-z. 52. Schneider J, Mohr N, Aliatakis N, Seidel U, John R, Promnitz G et al. Brain malformations and cognitive performance in spina bifida. Dev Med Child Neurol. 2020 Nov 2doi: 10.1111/dmcn.14717. 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Value of urodynamic findings in predicting upper urinary tract damage in neuro-urological patients: A systematic review. Neurourol Urodyn. 2018;37:1522-1540. doi: 10.1002/nau.23501. 36. Abrahamsson K, Olsson I, Sillen U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol. 2007; 177:331-334. doi: 10.1016/j.juro.2006.08.146. 37. Averbeck MA, Madersbacher H. Follow-up of the neuro-urological patient: a systematic review. BJUInt.2015;115 Suppl 6:39-46. doi: 10.1111/bju.13084. 38. Wide P, Duchén K, Mattsson S, Glad Mattsson G. Four-hour voiding observation with provocation test reveals significant abnormalities of bladder function in newborns with spinal dysraphism. J Pediatr Urol 2020 Aug;16(4): 491.e1-491.e7. doi: 10.1016/j.jpurol.2020.06.022. 39. Gladh G, Persson D, Mattsson S, Lindström S. Voiding pattern in healthy newborns. Neurourol Urodyn 2000;19:177-184.doi:10.1002/(sici)1520- 6777(2000)19:2<177::aid-nau8>3.0.co;2-0. 40. Lindehall B, Abrahamsson K, Jodal U, Olsson I, Sillén U. Complications at clean intermittent catheterization in boys and young males with neurogenic bladder dysfunction. J Urol 2004;172:1986-8. doi:10.1097/01.ju.0000138847.14680.7d. 41. Lindehall B, Möller A, Hjälmås K, Jodal U, Abrahamsson K. Psychosocial factors in teenagers and young adults with myelomeningocele and clean intermittent catheterization. Scand J Urol Nephrol. 2008;42(6):539-44. doi: 10.1080/00365590802273218. 42. Lindquist B, Carlsson G, Persson EK, Uvebrant P. Learning disabilities in a population-based group of children with hydrocephalus. Acta Paediatr 2005; 94:878-83.doi: 10.1111/j.1651-2227.2005.tb02005.x. 43. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 2001 Mar;34:114e20. doi: 10.1159/000056005. Magdalena Vu Minh Arnell 39 44. Bendt M, Gabrielsson H, Riedel D, Hagman G, Hultling C, Franzén E et al. 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Quality of performance of everyday activities in children with spina bifida: a population-based study. Acta Paediatr 2009 Oct;98:1674e9. doi: 10.1111/j.1651-2227.2009.01410.x. 54. Persson M, Janeslätt G, Peny-Dahlstrand M. Daily time management in children with spina bifida. J Pediatr Rehabil Med. 2017 Dec 11;10(3-4):295- 302. doi: 10.3233/PRM-170459. Neurogenic bladder and bowel dysfunction 40 55. Ehrén I, Hagman G, Lindbo L, Gabrielsson H, Bendt M, Seiger Å. Cognitive challenges in persons with spina bifida: Bearing on urological dysfunctions? Neurourol Urodyn. 2020 Nov;39(8):2139-2145. doi: 10.1002/nau.24515. 56. Palinkas L, Horwitz S, Green C, Wisdom J, Duan N, Hoagwood K. Purposeful Sampling for Qualitative Data Collection and Analysis in Mixed Method Implementation Research. Adm Policy Ment Health 2015 Sep;42(5):533-44. doi: 10.1007/s10488-013-0528-y. 57. Official Statistics of Sweden (internet) https://www.scb.se/ Accessed sept 13, 2020. 58. Sullivan M, Karlsson J, Taft C. SF-36 Hälsoenkät: Svensk manual och Tolkningsguide. 2ed. Göteborg: Sahlgrenska Universitetssjukhuset; 2002. 59. Taft C, Karlsson J, Sullivan M. Do SF-36 summary component scores accurately summarize subscale scores? Qual Life Res 2001;10:395-404. doi: 10.1023/a:1012552211996. 60. Krippendorff K. Content Analysis: An introduction to its Methodology. Thousand Oaks.CA: Sage; 2004 p 87-90. 61. Elo S, Kyngäs H: The qualitative content analysis process. Journal of Advanced Nursing 2008;62:107-15 doi: 10.1111/j.1365-2648.2007.04569.x 62. WMA Declaration of Helsinki - Ethical Principles for Medical Research InvolvingHumanSubjects(internet)https://www.wma.net/policies- post/wma-declaration-of-helsinki-ethical-principles-for-medical-research- involving-human-subjects/ Accessed Aug 10, 2020. 63. Betz C, Smith K, Kysh L, Roland M, Van Speybroeck A, Castillo P et al. Psychosocial outcomes for adults with spina bifida: a scoping review protocol. JBIEvidSynth2020May;18(5):1135-1143.doi: 10.11124/JBISRIR- D-19-00072. 64. Oakeshott P, Poulton A, Hunt GM, Reid F. Walking and living independently with spina bifida: a 50-year prospective cohort study. Dev Med Child Neurol. 2019 Oct;61(10):1202-1207. doi: 10.1111/dmcn.14168. 65. Roach J, Short B, Saltzman H. Adult consequences of spina bifida: a cohort study. Clin Orthop Relat Res 2011 May;469(5):1246-52. doi: 10.1007/s11999-010-1594-z. 66. Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J 2015 Oct;8(4):492-8. doi: 10.1016/j.dhjo.2015.03.012 Magdalena Vu Minh Arnell 41 67. Fischer N, Church P, Lyons J, McPherson AC. A qualitative exploration of the experiences of children with spina bifida and their parents around incontinence and social participation. Child Care Health Dev. 2015 Nov;41(6):954-62. doi: 10.1111/cch.12257. 68. Nahal MS, Axelsson ÅB, Imam A, Wigert H..Palestinian children's narratives about living with spina bifida: Stigma, vulnerability, and social exclusion. Child Care Health Dev. 2019 Jan;45(1):54-62. doi: 10.1111/cch.12625. 69. Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R, Christenson M. et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015 Oct;8(4):492-8. doi: 10.1016/j.dhjo.2015.03.012. 70. Hägglund D, Ahlström G.The meaning of women's experience of living with long-term urinary incontinence is powerlessness J Clin Nurs. 2007 Oct;16(10):1946-54. doi: 10.1111/j.1365-2702.2007.01787.x. 71. Ortuño Esparza A, Ángel Cánovas T, Pina-Roche F. Experiences of women and men living with urinary incontinence: A phenomenological study. Appl Nurs Res. 2018;April 40: 68-75. doi: 10.1016/j.apnr.2017.12.007. 72. Lindgren A, Dunberger G, Enblom A. Experiences of incontinence and pelvic floor muscle training after gynaecologic cancer treatment. Support Care Cancer. 2017Jan; (1) 25: 157-166. doi: 10.1007/s00520-016-3394-9. 73. Lindquist B, Persson EK, Uvebrant P, Carlsson G. Learning, memory and executive functions in children with hydrocephalus. Acta Paediatr 2008 May; 97:596-601 doi: 10.1111/j.1651-2227.2008.00747.x. 74. Dennis M, Barnes MA. The cognitive phenotype of spina bifida meningo- myelocele. Dev Disabil Res Rev 2010;16(1):31-9. doi: 10.1002/ddrr.89. 75. Treble-Barna A, Juranek J, Stuebing KK, Cirino PT, Dennis M, Fletcher JM. Prospective and episodic memory in relation to hippocampal volume in adults with spina bifida myelomeningocele. Neuropsychology. 2015 Jan;29(1):92-101.doi: 10.1037/neu0000111. 76. Van Mechelen MC, Verhoef M, van Asbeck FW, Post MW. Work participation among young adults with spina bifida in the Netherlands. Dev Med Child Neurol2008Oct;(50)10:772-7. doi:10.1111/j.1469- 8749.2008.03020.x. 77. Valtonen K, Karlsson AK, Alaranta H, Viikari-Juntura E. Work participation among persons with traumatic spinal cord injury and meningomyelocele1 J Rehabil Med. 2006 May;38(3):192-200. Neurogenic bladder and bowel dysfunction 40 55. Ehrén I, Hagman G, Lindbo L, Gabrielsson H, Bendt M, Seiger Å. Cognitive challenges in persons with spina bifida: Bearing on urological dysfunctions? Neurourol Urodyn. 2020 Nov;39(8):2139-2145. doi: 10.1002/nau.24515. 56. Palinkas L, Horwitz S, Green C, Wisdom J, Duan N, Hoagwood K. Purposeful Sampling for Qualitative Data Collection and Analysis in Mixed Method Implementation Research. Adm Policy Ment Health 2015 Sep;42(5):533-44. doi: 10.1007/s10488-013-0528-y. 57. Official Statistics of Sweden (internet) https://www.scb.se/ Accessed sept 13, 2020. 58. Sullivan M, Karlsson J, Taft C. SF-36 Hälsoenkät: Svensk manual och Tolkningsguide. 2ed. Göteborg: Sahlgrenska Universitetssjukhuset; 2002. 59. Taft C, Karlsson J, Sullivan M. Do SF-36 summary component scores accurately summarize subscale scores? Qual Life Res 2001;10:395-404. doi: 10.1023/a:1012552211996. 60. Krippendorff K. Content Analysis: An introduction to its Methodology. Thousand Oaks.CA: Sage; 2004 p 87-90. 61. Elo S, Kyngäs H: The qualitative content analysis process. Journal of Advanced Nursing 2008;62:107-15 doi: 10.1111/j.1365-2648.2007.04569.x 62. WMA Declaration of Helsinki - Ethical Principles for Medical Research InvolvingHumanSubjects(internet)https://www.wma.net/policies- post/wma-declaration-of-helsinki-ethical-principles-for-medical-research- involving-human-subjects/ Accessed Aug 10, 2020. 63. Betz C, Smith K, Kysh L, Roland M, Van Speybroeck A, Castillo P et al. Psychosocial outcomes for adults with spina bifida: a scoping review protocol. JBIEvidSynth2020May;18(5):1135-1143.doi: 10.11124/JBISRIR- D-19-00072. 64. Oakeshott P, Poulton A, Hunt GM, Reid F. Walking and living independently with spina bifida: a 50-year prospective cohort study. Dev Med Child Neurol. 2019 Oct;61(10):1202-1207. doi: 10.1111/dmcn.14168. 65. Roach J, Short B, Saltzman H. Adult consequences of spina bifida: a cohort study. Clin Orthop Relat Res 2011 May;469(5):1246-52. doi: 10.1007/s11999-010-1594-z. 66. Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J 2015 Oct;8(4):492-8. doi: 10.1016/j.dhjo.2015.03.012 Magdalena Vu Minh Arnell 41 67. Fischer N, Church P, Lyons J, McPherson AC. A qualitative exploration of the experiences of children with spina bifida and their parents around incontinence and social participation. Child Care Health Dev. 2015 Nov;41(6):954-62. doi: 10.1111/cch.12257. 68. Nahal MS, Axelsson ÅB, Imam A, Wigert H..Palestinian children's narratives about living with spina bifida: Stigma, vulnerability, and social exclusion. Child Care Health Dev. 2019 Jan;45(1):54-62. doi: 10.1111/cch.12625. 69. Wagner R, Linroth R, Gangl C, Mitchell N, Hall M, Cady R, Christenson M. et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015 Oct;8(4):492-8. doi: 10.1016/j.dhjo.2015.03.012. 70. Hägglund D, Ahlström G.The meaning of women's experience of living with long-term urinary incontinence is powerlessness J Clin Nurs. 2007 Oct;16(10):1946-54. doi: 10.1111/j.1365-2702.2007.01787.x. 71. Ortuño Esparza A, Ángel Cánovas T, Pina-Roche F. Experiences of women and men living with urinary incontinence: A phenomenological study. Appl Nurs Res. 2018;April 40: 68-75. doi: 10.1016/j.apnr.2017.12.007. 72. Lindgren A, Dunberger G, Enblom A. Experiences of incontinence and pelvic floor muscle training after gynaecologic cancer treatment. Support Care Cancer. 2017Jan; (1) 25: 157-166. doi: 10.1007/s00520-016-3394-9. 73. Lindquist B, Persson EK, Uvebrant P, Carlsson G. Learning, memory and executive functions in children with hydrocephalus. Acta Paediatr 2008 May; 97:596-601 doi: 10.1111/j.1651-2227.2008.00747.x. 74. Dennis M, Barnes MA. The cognitive phenotype of spina bifida meningo- myelocele. Dev Disabil Res Rev 2010;16(1):31-9. doi: 10.1002/ddrr.89. 75. Treble-Barna A, Juranek J, Stuebing KK, Cirino PT, Dennis M, Fletcher JM. Prospective and episodic memory in relation to hippocampal volume in adults with spina bifida myelomeningocele. Neuropsychology. 2015 Jan;29(1):92-101.doi: 10.1037/neu0000111. 76. Van Mechelen MC, Verhoef M, van Asbeck FW, Post MW. Work participation among young adults with spina bifida in the Netherlands. Dev Med Child Neurol2008Oct;(50)10:772-7. doi:10.1111/j.1469- 8749.2008.03020.x. 77. Valtonen K, Karlsson AK, Alaranta H, Viikari-Juntura E. Work participation among persons with traumatic spinal cord injury and meningomyelocele1 J Rehabil Med. 2006 May;38(3):192-200. Neurogenic bladder and bowel dysfunction 42 78. MDF, Myndigheten för delaktighet arbetar för att främja ett samhälle som ska vara användbart och tillgängligt för alla(internet).www.mfd.se Accessed November 3 2020. 79. Törnbom M, Jonsson U, Sunnerhagen KS. Work participation among middle-aged persons with cerebral palsy or spina bifida--a longitudinal study Disabil Health J. 2014 Apr;7(2):251-5doi: 10.1016/j.dhjo.2013.06.005. 80. Bauer SB, Austin PF, Rawashdeh YF, de Jong TP, Franco I, Siggard C, Jorgensen TM; International Children's Continence Society International Children's Continence Society's recommendations for initial diagnostic evaluation and follow-up in congenital neuropathic bladder and bowel dysfunction in children. Neurourol Urodyn. 2012 Jun;31(5):610-4. doi: 10.1002/nau.22247. 81. Ahmad I, Granitsiotis P. Urological follow-up of adult spina bifida patients. Neurourol Urodyn. 2007;26(7):978-80. doi: 10.1002/nau.20447. 82. Dik P, Klijn AJ, von Gool JD, et al. Early start to therapy preserves kidney function in spina bifida patients. Eur Urol 2006; 49:908–13. doi: 10.1016/j.eururo.2005.12.056. 83. Miller AR, Condin C, McKellin W, Shaw N, Klassen A, Sheps S. Continuity of care for children with complex chronic health conditions: parents' perspectives. BMC Health Serv Res 2009 Dec 21; 9:242. doi: 10.1186/1472- 6963-9-242. 84. Lemelle JL, Guillemin F, Aubert D et al: Quality of life and continence in patients with spina bifida. Qual Life Res 2006 Nov;15(9):1481-92 doi: 10.1007/s11136-006-0032-x. 85. MacNeily AE, Jafari S, Scott H, Dalgetty A, Afshar K. Health related quality of life in patients with spina bifida: a prospective assessment before and after lower urinary tract reconstruction. J Urol 2009 Oct;182(4suppl):1984-91. doi: 10.1016/j.juro.2009.02.060. 86. Liu J, Dong C, Casey J, Greiman A, Mukherjee S, Kielb S. Quality of life related to urinary continence in adult spina bifida patients. Cent European J Urol 2015;68(1):61-7 doi: 10.5173/ceju.2015.01.494. 87. Ehrén I, Lindbo L, Gabrielsson H, Bendt M, Seiger Å. Voiding conditions, renal and bowel function in a cohort of adults with spina bifida. Neurourol Urodyn. 2020 Aug;39(6):1796-1802. doi: 10.1002/nau.24422. Magdalena Vu Minh Arnell 43 88. Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. Incontinence affects health-related quality of life in children and adolescents with spina bifida. J Pediatr Urol.2018 Jun;14(3):279.e1-279.e8. doi: 10.1016/j.jpurol.2018.02.021. 89. Verhoef M, Lurvink M, Barf H et al. High prevalence of incontinence among young adults with spina bifida: description, prediction and problem perception. Spinal Cord 2005jun;43(6): 331e40.doi: 10.1038/sj.sc.3101705. 90. Perrouin-Verbe MA, Léon V, Denys P, Mongiat-Artus P, Chartier-Kastler E, Phé V. Long-term functional outcomes of augmentation cystoplasty in adult spina bifida patients:A single-center experience in a multidisciplinary team. Neurourol Urodyn. 2019 Jan;38(1):330-337. doi: 10.1002/nau.23857. 91. Lloyd JC, Nseyo U, Madden-Fuentes RJ, Ross SS, Wiener JS, Routh JC. Reviewing definitions of urinary continence in the contemporary spina bifida literature: a call for clarity. J Pediatr Urol. 2013 Oct;9(5):567-74. doi: 10.1016/j.jpurol.2013.02.006. 92. Szymanski KM, Misseri R, Whittam B, Kaefer M, Rink R, Cain M. Quantity, Not Frequency, Predicts Bother with Urinary Incontinence and its Impact on Quality of Life in Adults with Spina Bifida. Urol 2016 Apr;195(4 Pt 2):1263-9. doi: 10.1016/j.juro.2015.07.108. 93. Duplisea J, Rodrigo L, Romao P et al: Urological Follow-up in Adult Spina Bifida Patients: Is There an Ideal Interval? J. Urology 2016 Nov;97: 269-72 doi: 10.1016/j.urology.2016.06.025. 94. Nordic network for children's rights and needs in health care (internet). http://www.nobab.se/ Accessed October 28 2020. 95. Mattsson S, Gladh G. Children with meningomyelocele become adults. Lakartidningen. 2005 Sep 12-18;102(37):2566-7. 96. Sawyer SM, Macnee S. Transition to adult health care for adolescents with spina bifida: research issues. Dev Disabil Res Rev2010;16(1):60-5.doi: 10.1002/ddrr.98. 97. Liptak GS, Kennedy JA, Dosa NP. Youth with spina bifida and transitions: health and social participation in a nationally represented sample. J Pediatr 2010 Oct;157(4):584-8, 588e1.doi: 10.1016/j.jpeds.2010.04.004. 98. Wood D, Rocque B, Hopson B, Barnes K, Johnson KR. Transition Readiness Assessment Questionnaire Spina Bifida (TRAQ-SB) specific module and its association with clinical outcomes among youth and young adults with spina bifida. J Pediatr Rehabil Med. 2019;12(4):405-413. doi: 10.3233/PRM-180595 Neurogenic bladder and bowel dysfunction 42 78. MDF, Myndigheten för delaktighet arbetar för att främja ett samhälle som ska vara användbart och tillgängligt för alla(internet).www.mfd.se Accessed November 3 2020. 79. Törnbom M, Jonsson U, Sunnerhagen KS. Work participation among middle-aged persons with cerebral palsy or spina bifida--a longitudinal study Disabil Health J. 2014 Apr;7(2):251-5doi: 10.1016/j.dhjo.2013.06.005. 80. Bauer SB, Austin PF, Rawashdeh YF, de Jong TP, Franco I, Siggard C, Jorgensen TM; International Children's Continence Society International Children's Continence Society's recommendations for initial diagnostic evaluation and follow-up in congenital neuropathic bladder and bowel dysfunction in children. Neurourol Urodyn. 2012 Jun;31(5):610-4. doi: 10.1002/nau.22247. 81. Ahmad I, Granitsiotis P. Urological follow-up of adult spina bifida patients. Neurourol Urodyn. 2007;26(7):978-80. doi: 10.1002/nau.20447. 82. Dik P, Klijn AJ, von Gool JD, et al. Early start to therapy preserves kidney function in spina bifida patients. Eur Urol 2006; 49:908– 13.doi: 10.1016/j.eururo.2005.12.056. 83. Miller AR, Condin C, McKellin W, Shaw N, Klassen A, Sheps S. Continuity of care for children with complex chronic health conditions: parents' perspectives. BMC Health Serv Res 2009 Dec 21; 9:242. doi: 10.1186/1472- 6963-9-242. 84. Lemelle JL, Guillemin F, Aubert D et al: Quality of life and continence in patients with spina bifida. Qual Life Res 2006 Nov;15(9):1481-92 doi: 10.1007/s11136-006-0032-x. 85. MacNeily AE, Jafari S, Scott H, Dalgetty A, Afshar K. Health related quality of life in patients with spina bifida: a prospective assessment before and after lower urinary tract reconstruction. J Urol 2009 Oct;182(4suppl):1984-91. doi: 10.1016/j.juro.2009.02.060. 86. Liu J, Dong C, Casey J, Greiman A, Mukherjee S, Kielb S. Quality of life related to urinary continence in adult spina bifida patients. Cent European J Urol 2015;68(1):61-7 doi: 10.5173/ceju.2015.01.494. 87. Ehrén I, Lindbo L, Gabrielsson H, Bendt M, Seiger Å. Voiding conditions, renal and bowel function in a cohort of adults with spina bifida. Neurourol Urodyn. 2020 Aug;39(6):1796-1802. doi: 10.1002/nau.24422. Magdalena Vu Minh Arnell 43 88. Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. Incontinence affects health-related quality of life in children and adolescents with spina bifida. J Pediatr Urol.2018 Jun;14(3):279.e1-279.e8. doi: 10.1016/j.jpurol.2018.02.021. 89. Verhoef M, Lurvink M, Barf H et al. High prevalence of incontinence among young adults with spina bifida: description, prediction and problem perception. Spinal Cord 2005jun;43(6): 331e40.doi: 10.1038/sj.sc.3101705. 90. Perrouin-Verbe MA, Léon V, Denys P, Mongiat-Artus P, Chartier-Kastler E, Phé V. Long-term functional outcomes of augmentation cystoplasty in adult spina bifida patients:A single-center experience in a multidisciplinary team. Neurourol Urodyn. 2019 Jan;38(1):330-337. doi: 10.1002/nau.23857. 91. Lloyd JC, Nseyo U, Madden-Fuentes RJ, Ross SS, Wiener JS, Routh JC. Reviewing definitions of urinary continence in the contemporary spina bifida literature: a call for clarity. J Pediatr Urol. 2013 Oct;9(5):567-74. doi: 10.1016/j.jpurol.2013.02.006. 92. Szymanski KM, Misseri R, Whittam B, Kaefer M, Rink R, Cain M. Quantity, Not Frequency, Predicts Bother with Urinary Incontinence and its Impact on Quality of Life in Adults with Spina Bifida. Urol 2016 Apr;195(4 Pt 2):1263-9. doi: 10.1016/j.juro.2015.07.108. 93. Duplisea J, Rodrigo L, Romao P et al: Urological Follow-up in Adult Spina Bifida Patients: Is There an Ideal Interval? J. Urology 2016 Nov;97: 269-72 doi: 10.1016/j.urology.2016.06.025. 94. Nordic network for children's rights and needs in health care (internet). http://www.nobab.se/ Accessed October 28 2020. 95. Mattsson S, Gladh G. Children with meningomyelocele become adults. Lakartidningen. 2005 Sep 12-18;102(37):2566-7. 96. Sawyer SM, Macnee S. Transition to adult health care for adolescents with spina bifida: research issues. Dev Disabil Res Rev2010;16(1):60-5.doi: 10.1002/ddrr.98. 97. Liptak GS, Kennedy JA, Dosa NP. Youth with spina bifida and transitions: health and social participation in a nationally represented sample. J Pediatr 2010 Oct;157(4):584-8, 588e1.doi: 10.1016/j.jpeds.2010.04.004. 98. Wood D, Rocque B, Hopson B, Barnes K, Johnson KR. Transition Readiness Assessment Questionnaire Spina Bifida (TRAQ-SB) specific module and its association with clinical outcomes among youth and young adults with spina bifida. J Pediatr Rehabil Med. 2019;12(4):405-413. doi: 10.3233/PRM-180595 Neurogenic bladder and bowel dysfunction 44 99. Roth JD, Szymanski KM, Cain MP, Misseri R. Factors impacting transition readiness in young adults with neuropathic bladder. J Pediatr Urol. 2020 Feb;16(1):45.e1-45.e7 doi: 10.1016/j.jpurol.2019.10.017. 100. Szymanski KM, Cain MP, Hardacker TJ, Misseri R. How successful is the transition to adult urology care in spina bifida? A single center 7-year experience. J Pediatr Urol. 2017 Feb;13(1):40.e1-40e6.doi: 10.1016/j.jpurol.2016.09.020. 101. Roth JD, Szymanski KM, Ferguson EJ, Cain MP, Misseri R. Transitioning young adults with neurogenic bladder-Are providers asking too much? J Pediatr Urol. 2019 Aug;15(4):384.e1-384.e6doi: 10.1016/j.jpurol.2019.04.013. 102. Hettel D, Tran C, Szymanski K, Misseri R, Wood H. Lost in transition: Patient-identified barriers to adult urological spina bifida care. J Pediatr Urol. 2018 Dec;14(6):535.e1-535.e.4.doi: 10.1016/j.jpurol.2018.06.009. 103. Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. Incontinence affects health-related quality of life in children and adolescents with spina bifida. J Pediatr Urol. 2018 Jun;14(3):279.e1-279.e8. doi: 10.1016/j.jpurol.2018.02.021. 45 APPENDI X 1.The questionnaire for study I in Swedish. 2.The questionnaire for study III in Swedish. Neurogenic bladder and bowel dysfunction 44 99. Roth JD, Szymanski KM, Cain MP, Misseri R. Factors impacting transition readiness in young adults with neuropathic bladder. J Pediatr Urol. 2020 Feb;16(1):45.e1-45.e7 doi: 10.1016/j.jpurol.2019.10.017. 100. Szymanski KM, Cain MP, Hardacker TJ, Misseri R. How successful is the transition to adult urology care in spina bifida? A single center 7-year experience. J Pediatr Urol. 2017 Feb;13(1):40.e1-40e6.doi: 10.1016/j.jpurol.2016.09.020. 101. Roth JD, Szymanski KM, Ferguson EJ, Cain MP, Misseri R. Transitioning young adults with neurogenic bladder-Are providers asking too much? J Pediatr Urol. 2019 Aug;15(4):384.e1-384.e6doi: 10.1016/j.jpurol.2019.04.013. 102. Hettel D, Tran C, Szymanski K, Misseri R, Wood H. Lost in transition: Patient-identified barriers to adult urological spina bifida care. J Pediatr Urol. 2018 Dec;14(6):535.e1-535.e.4.doi: 10.1016/j.jpurol.2018.06.009. 103. Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. Incontinence affects health-related quality of life in children and adolescents with spina bifida. J Pediatr Urol. 2018 Jun;14(3):279.e1-279.e8. doi: 10.1016/j.jpurol.2018.02.021. 45 APPENDI X 1.The questionnaire for study I in Swedish. 2.The questionnaire for study III in Swedish. 1.The questionnai re for study I in Swedish. Grundlägg ande data Namn Personnummer Tidigare aktuell barnhabilitering Skadenivå enligt barnjournalen 1 = cervikalt 2 = thorakalt 3 = thorakolumbalt 4 = lumbosakralt 6 = s akralt Hydrocefalus enligt barnjournalen 0 = ingen hydrocefalus 1 = opererad hydrocefalus 2 = hydrocefalus ej opererad (om data finns) Interv jufrå go r Förflyttningssätt 0 = utan hjälpmedel men kan ha ortopediska inlägg/skor 1 = skenor/kryckor (vanligen) 2 = delvis rullstol 3 = alltid rullstol Skolgång/utbildning Högsta utbildning 1 = särskola inklusive särgymnasium 2 = normalklass grundskola 3 = gymnasium 4 = folkhögskola 5 = universitet Nuvarande sysselsättning 0 = sysslolös 1 = dagcenter/annan liknade lösning 2 = studier 3 = lönearbete Genomgått körkortsutbildning 0 = nej 1 = ja Boendeform 1 = eget boende 2 = eget boende i servicehus 3 = gruppboende 4 = föräldrahem Assistent 0 = nej 1 = ja om ja, vad gör assistenten? Civilstånd 1 = single 2 = sambo/gift Sexuella erfarenheter 0 = inga 1 = ja om ja, ”vill du berätta om detta?” Graviditeter 0 = inga 1 = ja; antal Barn 0 = inga 1 = ja; antal Fertilitetsförsök 0 = nej 1 = ja om ja, vilken typ av fertilitetsförsök Urolo g i ska fråg o r Blåstömningsmetod 1 = spontan/normal miktion 2 = krystning/komprimering 3 = RIK 4 = annan metod 0 = ingen (bara rinner) Självständighet i samband med blåstömning 0 = nej ”vad behöver du hjälp med?” 1 = ja (klarar allt) Antal tömningar/dygn 0 = ingen (bara rinner) 1 = 1 -3 2 = 4 -6 3 = >6 Urinläckage, dag 0 = inget läckage 1 = enstaka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid våt F ö rb ruknin g sa rtikla r Katetertyp 1 = obehandlad 2 = behandlad 3 = förgelad 4 = annan 1.The questionnai re for study I in Swedish. Grundlägg ande data Namn Personnummer Tidigare aktuell barnhabilitering Skadenivå enligt barnjournalen 1 = cervikalt 2 = thorakalt 3 = thorakolumbalt 4 = lumbosakralt 6 = s akralt Hydrocefalus enligt barnjournalen 0 = ingen hydrocefalus 1 = opererad hydrocefalus 2 = hydrocefalus ej opererad (om data finns) Interv jufrå go r Förflyttningssätt 0 = utan hjälpmedel men kan ha ortopediska inlägg/skor 1 = skenor/kryckor (vanligen) 2 = delvis rullstol 3 = alltid rullstol Skolgång/utbildning Högsta utbildning 1 = särskola inklusive särgymnasium 2 = normalklass grundskola 3 = gymnasium 4 = folkhögskola 5 = universitet Nuvarande sysselsättning 0 = sysslolös 1 = dagcenter/annan liknade lösning 2 = studier 3 = lönearbete Genomgått körkortsutbildning 0 = nej 1 = ja Boendeform 1 = eget boende 2 = eget boende i servicehus 3 = gruppboende 4 = föräldrahem Assistent 0 = nej 1 = ja om ja, vad gör assistenten? Civilstånd 1 = single 2 = sambo/gift Sexuella erfarenheter 0 = inga 1 = ja om ja, ”vill du berätta om detta?” Graviditeter 0 = inga 1 = ja; antal Barn 0 = inga 1 = ja; antal Fertilitetsförsök 0 = nej 1 = ja om ja, vilken typ av fertilitetsförsök Urolo g i ska fråg o r Blåstömningsmetod 1 = spontan/normal miktion 2 = krystning/komprimering 3 = RIK 4 = annan metod 0 = ingen (bara rinner) Självständighet i samband med blåstömning 0 = nej ”vad behöver du hjälp med?” 1 = ja (klarar allt) Antal tömningar/dygn 0 = ingen (bara rinner) 1 = 1 -3 2 = 4 -6 3 = >6 Urinläckage, dag 0 = inget läckage 1 = enstaka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid våt F ö rb ruknin g sa rtikla r Katetertyp 1 = obehandlad 2 = behandlad 3 = förgelad 4 = annan Kateterstorlek, Ch 1 = Ch8 2 = Ch10 3 = Ch12 4 = Ch14 5 = Ch16 6 = annan, vilken Kontinensskydd 0 = nej 1 = ja om ja, vilken typ Toaletthjälpmedel 0 = inget 1 = ja om ja, vilken typ Tarmtömningsmetod 1 = spontan/normal tarmtömning 2 = krystning 3 = tarmsköljning om ja Resulax Klysma Vatten Blandning vilken 4 = annan metod vilken Avföringsläckage, dag 0 = inget läckage 1 = enstaka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid avföringsläckage Avföringsläckage, natt 0 = inget läckage 1 = enst aka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid avföringsläckage Självständighet i samband med tarmtömning 0 = nej om nej, ”vad behöver du hjälp med?” 1 = ja Frekvens 0 = ingen 1 = 1 ggr/vecka 2 = 2 ggr/vecka 3 = varannan d ag 4 = varje dag 5 = annat Förbrukningsartiklar av avföringsinkontinens 0 = inga 1 = lavemangspip/påse 2 = tarmtratt/påse eller behållare 3 = ballongkateter/påse 4 = annat Särskilt inkontinensskydd 0 = nej 1 = ja om ja, vilken typ Aktuella läkemedel 0 = inga 1 = urologiska läkemedel vilka 2 = övriga läkemedel vilka Genomgången kirurgi 0 = ingen 1 = ja blåsa vilken tarm vilken Behandlande UVI:er 0 = nej 1 = ja ofta >1 ggr/år färre < 1 ggr/år Senaste urologiska kontroll 0 = ej sedan överföringen 1 = årtal Uroterapeutkontakt 0 = nej 1 = ja var Kateterstorlek, Ch 1 = Ch8 2 = Ch10 3 = Ch12 4 = Ch14 5 = Ch16 6 = annan, vilken Kontinensskydd 0 = nej 1 = ja om ja, vilken typ Toaletthjälpmedel 0 = inget 1 = ja om ja, vilken typ Tarmtömningsmetod 1 = spontan/normal tarmtömning 2 = krystning 3 = tarmsköljning om ja Resulax Klysma Vatten Blandning vilken 4 = annan metod vilken Avföringsläckage, dag 0 = inget läckage 1 = enstaka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid avföringsläckage Avföringsläckage, natt 0 = inget läckage 1 = enst aka läckage/månad 2 = enstaka läckage/vecka 3 = enstaka läckage/dag 4 = alltid avföringsläckage Självständighet i samband med tarmtömning 0 = nej om nej, ”vad behöver du hjälp med?” 1 = ja Frekvens 0 = ingen 1 = 1 ggr/vecka 2 = 2 ggr/vecka 3 = varannan d ag 4 = varje dag 5 = annat Förbrukningsartiklar av avföringsinkontinens 0 = inga 1 = lavemangspip/påse 2 = tarmtratt/påse eller behållare 3 = ballongkateter/påse 4 = annat Särskilt inkontinensskydd 0 = nej 1 = ja om ja, vilken typ Aktuella läkemedel 0 = inga 1 = urologiska läkemedel vilka 2 = övriga läkemedel vilka Genomgången kirurgi 0 = ingen 1 = ja blåsa vilken tarm vilken Behandlande UVI:er 0 = nej 1 = ja ofta >1 ggr/år färre < 1 ggr/år Senaste urologiska kontroll 0 = ej sedan överföringen 1 = årtal Uroterapeutkontakt 0 = nej 1 = ja var 2.The questionnai re for study III in Swedish. Grundlägg ande data Namn Personnummer Barnhabilitering Skadenivå enligt journal Cervikalt Thorakalt Thorakolumbalt Lumbalt Lumbosakralt Sakralt Hydrocefalus Ingen Op hydrocefalus Ej op hydrocefalus Förflyttning Utan hjälpmedel Kryckor Delvis rullstol Alltid rullstol Utbildning Särskola Grundskola Särgymnasium Gymnasium Körkort Ja Nej Boende Eget Eget i servicehus Gruppboende Föräldrahem Assistans Ja Nej om ja, vad gör assistenten? Urolo g i ska fråg o r Blåstömningsmetod Spontan Krystning RIK Annan metod Ingen Antal tömningar/dygn 0 1-3 4-6 >6 Självständig Ja Nej om nej, vad behöver du ha hjälp med? Vid RIK – katetertyp Obehandlad Hydrofil Förgelad Annan Kateterstorlek/Ch Ch8 CH10 Ch12 Ch14 Ch16 Annan typ eller storlek Ch18 Annan sort, vilken? Urinläckage, dag Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid våt Urinläckage, natt Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid våt Kontinensskydd Ja Nej om ja, vilken sort? Toaletthjälpmedel Ja Nej Vad? 2.The questionnai re for study III in Swedish. Grundlägg ande data Namn Personnummer Barnhabilitering Skadenivå enligt journal Cervikalt Thorakalt Thorakolumbalt Lumbalt Lumbosakralt Sakralt Hydrocefalus Ingen Op hydrocefalus Ej op hydrocefalus Förflyttning Utan hjälpmedel Kryckor Delvis rullstol Alltid rullstol Utbildning Särskola Grundskola Särgymnasium Gymnasium Körkort Ja Nej Boende Eget Eget i servicehus Gruppboende Föräldrahem Assistans Ja Nej om ja, vad gör assistenten? Urolo g i ska fråg o r Blåstömningsmetod Spontan Krystning RIK Annan metod Ingen Antal tömningar/dygn 0 1-3 4-6 >6 Självständig Ja Nej om nej, vad behöver du ha hjälp med? Vid RIK – katetertyp Obehandlad Hydrofil Förgelad Annan Kateterstorlek/Ch Ch8 CH10 Ch12 Ch14 Ch16 Annan typ eller storlek Ch18 Annan sort, vilken? Urinläckage, dag Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid våt Urinläckage, natt Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid våt Kontinensskydd Ja Nej om ja, vilken sort? Toaletthjälpmedel Ja Nej Vad? Tarmtömn i ng Metod Spontant Krystning Tarmsköljning med vad? Annan metod Hur ofta? Hjälpmedel vid tarmtömning Vilket? Avföringsläckage/dag Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid Avföringsläckage/natt Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid Självständighet Ja Nej om nej, vad behöver du hjälp med? Kontinensskydd enbart för avföringsläckage Ja Nej om ja, vad för sort? Öv ri g a fråg o r Aktuella läkemedel/sort Inga Urologiska Övriga Genomgången kirurgi Ja Nej Blåsa Tarm Behandlade UVI:er Ja Nej Hur ofta? Senaste urologiska kontroll Årtal Kontakt med uroterapeut Ja Nej Genomgått psykologtest Ja Nej Kognitiv nivå enligt utlåtandet Nära intima relationer/erfarenheter? Sexuella erfarenheter? Ja Nej om ja, vill du berätta? Fritidsaktiviteter/kamratkontakt? Beskriv kamratkontakten. Hur många? Vilken typ av relation? Ja Nej Lätt att ta/få kontakt med andra? Vem umgås du helst med? Sover du över hos någon? Idrottar du? Har du något annat fritidsintresse? Ja Nej Om kontinensskirurgi, hur var det före/efter op angående idrott/andra fritidsintressen/umgås med andra/övernatta? Ja Tarmtömn i ng Metod Spontant Krystning Tarmsköljning med vad? Annan metod Hur ofta? Hjälpmedel vid tarmtömning Vilket? Avföringsläckage/dag Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid Avföringsläckage/natt Inget Enstaka/månad Enstaka/vecka Enstaka/dag Alltid Självständighet Ja Nej om nej, vad behöver du hjälp med? Kontinensskydd enbart för avföringsläckage Ja Nej om ja, vad för sort? Öv ri g a fråg o r Aktuella läkemedel/sort Inga Urologiska Övriga Genomgången kirurgi Ja Nej Blåsa Tarm Behandlade UVI:er Ja Nej Hur ofta? Senaste urologiska kontroll Årtal Kontakt med uroterapeut Ja Nej Genomgått psykologtest Ja Nej Kognitiv nivå enligt utlåtandet Nära intima relationer/erfarenheter? Sexuella erfarenheter? Ja Nej om ja, vill du berätta? Fritidsaktiviteter/kamratkontakt? Beskriv kamratkontakten. Hur många? Vilken typ av relation? Ja Nej Lätt att ta/få kontakt med andra? Vem umgås du helst med? Sover du över hos någon? Idrottar du? Har du något annat fritidsintresse? Ja Nej Om kontinensskirurgi, hur var det före/efter op angående idrott/andra fritidsintressen/umgås med andra/övernatta? Ja