Top

Open Access 07-05-2024 | Spina Bifida | REVIEW

Long-Term Quality of Life for Patients with Spina Bifida

Authors: Madeleine A. Z. Ball, Douglass B. Clayton

Published in: Current Bladder Dysfunction Reports

Abstract

Purpose of Review

This review explores the literature that currently exists regarding quality of life for patients with spina bifida and assesses screening tools available for practitioners to assess these outcomes. Domains addressed include health related quality of life, relationships, bowel and bladder dysfunction, sexuality, pain, and mood.

Recent Findings

Several recent papers have assessed available questionnaires and reviewed findings of key quality of life domains, with varying results.

Summary

Historically, the literature has focused on surgical outcomes for patients with spina bifida. Though some authors have begun to explore quality of life for this patient population, particularly in the short-term, a paucity of quality, long-term studies exist that describe quality of life domains or offer potential targets for improvements in outcomes.

• Gilbertson K, Liu T, Wiener JS, et al. Age-Specific Probability of 4 Major Health Outcomes in Children with Spina Bifida. J Dev Behav Pediatr. Published online 2023. https://doi.org/10.1097/DBP.0000000000001218. Study of outcomes including bowel/bladder using large national database.

Atta CAM, 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;106(1). https://doi.org/10.2105/AJPH.2015.302902.

Davis BE, Daley CM, Shurtleff DB, et al. Long-term survival of individuals with myelomeningocele. Pediatr Neurosurg. 2005;41(4). https://doi.org/10.1159/000086559.

Patel N, Rizk E, Simon S. Spina Bifida. American Association of Neurological Surgeons.Published online 2022.

Shin M, Kucik JE, Siffel C, et al. Improved survival among children with spina bifida in the United States. J Pediatr. 2012;161(6). https://doi.org/10.1016/j.jpeds.2012.05.040.

Spina Bifida Association. What is Spina Bifida? https://www.spinabifidaassociation.org/what-is-spina-bifida-2/.

Li Y, Stern N, Wang P (Zhantao), Braga L, Dave S. Systematic review and meta-analysis to study the outcomes of proactive versus delayed management in children with a congenital neurogenic bladder. J Pediatr Urol. Published online 2023. https://doi.org/10.1016/j.jpurol.2023.08.033.

•• Raveendran L, Koyle M, Bagli D, et al. Integrative review and evaluation of quality of life related instruments in pediatric urology. J Pediatr Urol. 2021;17(4). https://doi.org/10.1016/j.jpurol.2021.03.011. Systematic review evaluating all QOL surveys available in pediatric urology.

Varni JW, Burwinkle TM, Lane MM. Health-related quality of life measurement in pediatric clinical practice: An appraisal and precept for future research and application. Health Qual Life Outcomes. 2005;3. https://doi.org/10.1186/1477-7525-3-34.

10.

Panicker JN. Neurogenic Bladder: Epidemiology, Diagnosis, and Management. Semin Neurol. 2020;40(5). https://doi.org/10.1055/s-0040-1713876.

11.

The World Health Organization quality of life assessment (WHOQOL): Position paper from the World Health Organization. Soc Sci Med. 1995;41(10). https://doi.org/10.1016/0277-9536(95)00112-K.

12.

Sawin KJ, Brei TJ, Buran CF, Fastenau PS. Factors Associated with Quality of Life in Adolescents with Spina Bifida. J Holist Nurs. 2002;20(3). https://doi.org/10.1177/089801010202000307.

13.

Tasian GE, Ellison JS. Measuring patient-centered outcomes: The need to move beyond quality of life. J Pediatr Urol. 2021;17(4). https://doi.org/10.1016/j.jpurol.2021.03.033.

14.

Ridosh MM, Sawin KJ, Roux G, Brei TJ. Quality of life in adolescents and young adults with and without Spina Bifida: An exploratory analysis. J Pediatr Nurs. 2019;49. https://doi.org/10.1016/j.pedn.2019.08.004.

15.

•• Szymanski KM, Kaefer M, Fossum M, et al. What are validated questionnaires and which ones measure quality of life? J Pediatr Urol. 2023;19(4). https://doi.org/10.1016/j.jpurol.2023.03.034. Summary educational article defining validated questionnaires and QOL.

16.

Irwin DE, Stucky BD, Thissen D, et al. Sampling plan and patient characteristics of the PROMIS pediatrics large-scale survey. Qual Life Res. 2010;19(4):585–94. https://doi.org/10.1007/s11136-010-9618-4.CrossRefPubMedPubMedCentral

17.

McCormick MC, Charney EB, Stemmler MM. Assessing the Impact of a Child with Spina Bifida on the Family. Dev Med Child Neurol. 1986;28(1). https://doi.org/10.1111/j.1469-8749.1986.tb03831.x.

18.

Radojicic Z, Milivojevic S, Lazovic JM, Becanovic S, Korićanac I, Milic N. The impact of bowel management on the quality of life in children with spina bifida with overactive bladder and detrusor sphincter dyssynergia. J Pediatr Urol. 2019;15(5). https://doi.org/10.1016/j.jpurol.2019.05.005.

19.

Freeman KA, Smith K, Adams E, Mizokawa S, Neville-Jan A. Is continence status associated with quality of life in young children with spina bifida?. J Pediatr Rehabil Med. 2013;6(4). https://doi.org/10.3233/PRM-140263.

20.

Sawin KJ, Brei TJ, Houtrow AJ. Quality of life: Guidelines for the care of people with spina bifida. J Pediatr Rehabil Med. 2020;13(4). https://doi.org/10.3233/PRM-200732.

21.

Starowicz J, Cassidy C, Brunton L. Health Concerns of Adolescents and Adults With Spina Bifida. Front Neurol. 2021;12. https://doi.org/10.3389/fneur.2021.745814.

22.

U.S. Department of Health and Human Services FDA Center for Drug Evaluation and Research. Guidance for industry: patient-reported outcome measures: use in medical product development to support labeling claims: draft guidance. Health Qual Life Outcomes. 2006;4(1):79. https://doi.org/10.1186/1477-7525-4-79.

23.

Luijten MAJ, Haverman L, van Litsenburg RRL, Roorda LD, Grootenhuis MA, Terwee CB. Advances in measuring pediatric overall health: the PROMIS® Pediatric Global Health scale (PGH-7). Eur J Pediatr. 2022;181(5). https://doi.org/10.1007/s00431-022-04408-9.

24.

• Rague JT, Kim S, Hirsch J, et al. The Association of Health Literacy with Health-Related Quality of Life in Youth and Young Adults with Spina Bifida: A Cross-Sectional Study. Journal of Pediatrics. 2022;251. https://doi.org/10.1016/j.jpeds.2022.08.005. Studying using SB-validated PROMIS to study HRQOL.

25.

Rocque BG, Bishop ER, Scogin MA, et al. Assessing health-related quality of life in children with spina bifida. J Neurosurg Pediatr. 2015;15(2). https://doi.org/10.3171/2014.10.PEDS1441.

26.

•• Fremion E, Madey R, Staggers KA, et al. Factors associated with self-management independence and quality of life for adolescents and young adults with spina bifida engaged in a guideline-based transition clinic. J Pediatr Rehabil Med. 2021;14(4). https://doi.org/10.3233/PRM-200758. Prospective survey study using SB-validated QUALAS studying multiple domains.

27.

Szymanski KM, Misseri R, Whittam B, et al. Validation of QUALAS-T, a health-related quality of life instrument for teenagers with spina bifida. Cent European J Urol. 2017;70(3). https://doi.org/10.5173/ceju.2017.1195.

28.

Wagner R, Linroth R, Gangl C, et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015;8(4). https://doi.org/10.1016/j.dhjo.2015.03.012.

29.

Liu T, Ouyang L, Walker WO, et al. Education and employment as young adults living with spina bifida transition to adulthood in the USA: A study of the National Spina Bifida Patient Registry. Dev Med Child Neurol. 2023;65(6). https://doi.org/10.1111/dmcn.15456.

30.

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;14(3). https://doi.org/10.1016/j.jpurol.2018.02.021.

31.

Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. All Incontinence is Not Created Equal: Impact of Urinary and Fecal Incontinence on Quality of Life in Adults with Spina Bifida. J Urol. 2017;197(3). https://doi.org/10.1016/j.juro.2016.08.117.

32.

Romero-Maroto J, Martinez-Cayuelas L, Gómez-Pérez L, Sarrió-Sanz P, Olarte Barragán E, López-López AI. Long-term effectiveness and safety of bladder augmentation in spina bifida patients. Neurourol Urodyn. 2021;40(6). https://doi.org/10.1002/nau.24713.

33.

Coco CT, Meenakshi-Sundaram B, Eldefrawy A, et al. A cross sectional single institution study of quality of life in adult patients with spina bifida. Neurourol Urodyn. 2018;37(5). https://doi.org/10.1002/nau.23511.

34.

Brownrigg N, Braga LH, Rickard M, et al. The impact of a bladder training video versus standard urotherapy on quality of life of children with bladder and bowel dysfunction: A randomized controlled trial. J Pediatr Urol. 2017;13(4). https://doi.org/10.1016/j.jpurol.2017.06.005.

35.

Valeska Halstead N, Hirsch J, Rosoklija I, et al. Association Between Quality of Life and Neurogenic Bowel Symptoms by Bowel Management Program in Spina Bifida. Urology. Published online December 2023. https://doi.org/10.1016/j.urology.2023.12.012.

36.

Cardenas DD, Topolski TD, White CJ, McLaughlin JF, Walker WO. Sexual Functioning in Adolescents and Young Adults With Spina Bifida. Arch Phys Med Rehabil. 2008;89(1). https://doi.org/10.1016/j.apmr.2007.08.124.

37.

Sawyer SM, Roberts K V. Sexual and reproductive health in young people with spina bifida. Dev Med Child Neurol. 1999;41(10). https://doi.org/10.1017/S0012162299001383.

38.

Choi EK, Ji Y, Han SW. Sexual Function and Quality of Life in Young Men With Spina Bifida: Could It Be Neglected Aspects in Clinical Practice?. Urology. 2017;108. https://doi.org/10.1016/j.urology.2016.11.052.

39.

Rague JT, Hirsch J, Rosoklija I, et al. Male perspectives on clinical communication about sexual health in spina bifida. Dev Med Child Neurol Published online. 2023. https://doi.org/10.1111/dmcn.15709.CrossRef

40.

De Win G, Dautricourt S, Deans R, et al. Fertility and sexuality issues in congenital lifelong urology patients: female aspects. World J Urol. 2021;39(4). https://doi.org/10.1007/s00345-020-03461-z.

41.

Akre C, Light A, Sherman L, Polvinen J, Rich M. What young people with spina bifida want to know about sex and are not being told. Child Care Health Dev. 2015;41(6). https://doi.org/10.1111/cch.12282.

42.

• Streur CS, Corona L, Smith JE, Lin M, Wiener JS, Wittmann DA. Sexual Function of Males and Females with Spina Bifida: A Scoping Literature Review. Sex Med Rev. 2021;9(2):244–266. https://doi.org/10.1016/j.sxmr.2020.09.001. Literature review of sexual function and sexuality in patients with SB.

43.

• Hughes TL, Simmons KL, Tejwani R, et al. Sexual Function and Dysfunction in Individuals with Spina Bifida: A Systematic Review. Urology. 2021;156. https://doi.org/10.1016/j.urology.2021.03.042. Systematic review of sexual function and sexuality in patients with SB.

44.

Rague JT, Hirsch J, Meyer T, et al. “i Just Haven’t Done Any of That”: Applicability of the International Index of Erectile Function in Young Men with Spina Bifida. J Urol. 2023;210(3). https://doi.org/10.1097/JU.0000000000003556.

45.

Peterson MD, Haapala H, Kamdar N, Lin P, Hurvitz EA. Pain phenotypes among adults living with cerebral palsy and spina bifida. Pain. 2021;162(10). https://doi.org/10.1097/j.pain.0000000000002240.

46.

• Lidal IB, Lundberg Larsen K, Hoff M. 50 Years and older–born with spina bifida: participation, health issues and physical function. Disabil Rehabil. 2021;43(2). https://doi.org/10.1080/09638288.2019.1621953. Specific focus on pain and quality of life in older adults with spina bifida.

47.

Werhagen L, Hultling C, Borg K. Pain, especially neuropathic pain, in adults with spina bifida, and its relation to age, neurological level, completeness, gender and hydrocephalus. J Rehabil Med. 2010;42(4). https://doi.org/10.2340/16501977-0529.

48.

Smith A V., Richardson EJ, Cowan R. Pain interference, pain type, and quality of life among adults with spina bifida. PM&R. Published online December 19, 2023. https://doi.org/10.1002/pmrj.13084.

49.

Dicianno BE, Kinback N, Bellin MH, et al. Depressive symptoms in adults with spina bifida. Rehabil Psychol. 2015;60(3). https://doi.org/10.1037/rep0000044.

50.

Wiener JS, Chaudhry R. Neurogenic Lower Urinary Tract Dysfunction. Urol Clin N Am. 2023;50(3). https://doi.org/10.1016/j.ucl.2023.04.002.

Title: Long-Term Quality of Life for Patients with Spina Bifida
Authors: Madeleine A. Z. Ball
Douglass B. Clayton
Publication date: 07-05-2024
Publisher: Springer US
Keywords: Spina Bifida
Spina Bifida
Neurogenic Bladder
Published in: Current Bladder Dysfunction Reports
Print ISSN: 1931-7212
Electronic ISSN: 1931-7220
DOI: https://doi.org/10.1007/s11884-024-00755-5

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Long-Term Quality of Life for Patients with Spina Bifida

Abstract

Purpose of Review

Recent Findings

Summary

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose of Review

Recent Findings

Summary

Please log in to get access to this content