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QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A): development and international validation of a novel health-related quality of life instrument

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Abstract

Purpose

Health-related quality of life (HRQOL) is important in spina bifida (SB) management. No clinically useful, comprehensive instrument incorporating bladder/bowel domains exists. We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A).

Methods

We drafted the 53-question pilot instrument using a comprehensive item generation/refinement process. It was administered to an international convenience sample of adults with SB and controls recruited online via social media and in person at outpatient SB clinics (January 2013–September 2014). Final questions were determined by: clinical relevance, high factor loadings and domain psychometrics in an Internal Validation Sample randomly selected from United States participants (n = 250). External validity was evaluated in United States and International External Validation Samples (n = 165 and n = 117, respectively). Adults with SB completed the validated general WHOQOL-BREF and International Consultation on Incontinence Questionnaire (ICIQ).

Results

Mean age of 532 participants was 32 years (32.7 % males, 85.0 % Caucasian), similar to 116 controls (p ≥ 0.08). There were 474 online and 58 clinical participants (61.1 % eligible). Face validity and content validity of the 3-domain, 15-question QUALAS-A were established by patients, families and experts. Internal consistency and test–retest reliability were high for all domains (Cronbach’s alpha ≥ 0.70, ICC ≥ 0.77). Correlations between QUALAS-A and WHOQOL-BREF were low (r ≤ 0.60), except for high correlations with Health and Relationships domain (0.63 ≤ r ≤ 0.71). Bladder and Bowel domain had a high correlation with ICIQ (r = −0.70). QUALAS-A scores were lower among adults with SB than without (p < 0.0001). QUALAS-A had good statistical properties in both External Validation Samples (Cronbach’s alpha 0.68–0.77).

Conclusions

QUALAS-A is a short, valid HRQOL tool for adults with SB.

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Abbreviations

HRQOL:

Health-related quality of life

QOL:

Quality of life

SB:

Spina bifida

QUALAS-A:

QUAlity of Life Assessment in Spina bifida for Adults

WHOQOL-BREF:

World Health Organization Quality of Life instrument

SD:

Standard deviation

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Acknowledgments

We would like to thank the hundreds of individuals with spina bifida and their parents and caregivers participating in this study. We also want to thank the following individuals and groups for their help: Concept and Content Consultant: Kathleen J. Sawin, PhD CPNP-PC FAAN (College of Nursing, University of Wisconsin-Milwaukee/Children’s Hospital of Wisconsin); Content Consultants: Pediatric Urology: John Paul Capolicchio, MD (Montreal Children’s Hospital, McGill University), Mohamed Elsherbini, MD (Montreal Children’s Hospital, McGill University), Walid Farhat, MD (Hospital for Sick Children, University of Toronto), Richard Grady, MD (Seattle Children’s Hospital, University of Washington), Roman Jednak, MD (Montreal Children’s Hospital, McGill University), David B. Joseph, MD (University of Alabama at Birmingham), Alison C. Keenan, MD (Riley Hospital for Children, Indiana University Health), Martin A. Koyle, MD (Hospital for Sick Children, University of Toronto), Andrew L. MacNeilly, MD (British Columbia Children’s Hospital, University of British Columbia), JL Pippi Salle, MD (Hospital for Sick Children, University of Toronto), Melissa A. Young, CPNP (Riley Hospital for Children, Indiana University Health). Pediatrics: Timothy J. Brei, MD (Seattle Children’s Hospital, University of Washington), Joseph O’Neil, MD (Riley Hospital for Children, Indiana University Health). Clinical Pediatric Psychology: Khush Amaria, PhD (Hospital for Sick Children, University of Toronto). Health Literacy: Cynthia Latty, RN (Riley Hospital for Children, Indiana University Health); Statistics Consultants: Robert Haley, MD (University of Texas Southwestern), Patrick Monahan, PhD (Richard M. Fairbanks School of Public Health, Indiana University); Participant Recruitment: Robin Bowman, MD (Lurie Children’s Hospital of Chicago, Northwestern University), Timothy J. Brei, MD (Seattle Children’s Hospital, Seattle Children’s Hospital), Jerry Clayton, MD (Children’s Hospital Colorado, University of Colorado), Dominic C. Frimberger, MD (The Children’s Hospital of Oklahoma, University of Oklahoma), Betsy Hopson, MSHA (Children’s Hospital of Alabama, University of Alabama at Birmingham), Jill Mazurek, MD (Indiana University Health), Brandon G. Rocque, MD (Children’s Hospital of Alabama, University of Alabama at Birmingham), Dorota A. Szczepaniak, MD (Riley Hospital for Children, Indiana University Health); Record Entry and Review: Sable Amstutz, MD (Indiana University Health), Kyle Hardacker (Indiana University Health), Zoe King (Indiana University Health), Sophie Mazurek (Indiana University Health), Meredith Metcalf, MD (University of Tennessee Health Sciences Centre), David Yang, BSc (Indiana University Health); Organizations assisting in participant recruitment: International: International Federation for Spina Bifida and Hydrocephalus. United States: Spina Bifida Association, Spina Bifida Associations of Alabama, the Carolinas, Community of Memphis, Connecticut, Georgia, Greater New England, Illinois, Indiana, Kentucky, Nassau County, New Orleans, North Texas, Texas, Washington, Western Pennsylvania, Wisconsin, SB Resource Network, Spina Bifida Connection, National Birth Defects Prevention Network, Disabled Children of Vietnam Veterans. Canada: Spina Bifida & Hydrocephalus Association of Canada, Spina Bifida & Hydrocephalus Association of Ontario, Association de Spina-Bifida et d’Hydrocéphalie du Québec. Europe: Spina Bifida Hydrocephalus Ireland (Ireland), Mid-West Spina Bifida and Hydrocephalus Association (Ireland), Cork Spina Bifida and Hydrocephalus Association (Ireland), Spina bifida Hydrocephalus Information Networking Equality (SHINE, UK), Arbeitsgemeinschaft Spina Bifida und Hydrocephalus e. V. (ASBH, Germany), Swedish National Association for Disabled Children and Young People (FUB) (Sweden), Asociación Bizkaia Elkartea Espina Bífida e Hidrocefalia (Spain), Fundacja Spina (Poland), Spina Bifida ir Hidrocefalija asociacija (SBHA) (Lithuania). Australia and Oceania: Spina Bifida Foundation Victoria (Australia), Spina Bifida Hydrocephalus Queensland (Australia), Spina Bifida Awareness (New Zealand). Middle East: Lebanese Association for Neuromuscular Diseases for Hope (Lebanon). South America: Associação de Espinha Bífida e Hidrocefalia (AEBH, Brazil). Africa: Festus Fajemilo Foundation (Nigeria), National Council for Persons with Physical Disabilities in South Africa (South Africa).

Conflict of interest

Dr. Misseri is a site Investigator and Shelly King is a site Coordinator of the Allergan study 191622-120. None of the other authors declare potential competing personal interests. No authors declare any potential financial conflict of interest.

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Authors and Affiliations

  1. Division of Pediatric Urology, Riley Hospital for Children at IU Health, Indiana University School of Medicine, 705 Riley Hospital Dr. #4230, Indianapolis, IN, 46205, USA

    Konrad M. Szymanski, Rosalia Misseri, Benjamin Whittam, Sonia-Maria Raposo, Shelly J. King, Martin Kaefer, Richard C. Rink & Mark P. Cain

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  1. Konrad M. Szymanski
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  2. Rosalia Misseri
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  3. Benjamin Whittam
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  4. Sonia-Maria Raposo
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  8. Mark P. Cain
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Correspondence to Konrad M. Szymanski.

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Szymanski, K.M., Misseri, R., Whittam, B. et al. QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A): development and international validation of a novel health-related quality of life instrument. Qual Life Res 24, 2355–2364 (2015). https://doi.org/10.1007/s11136-015-0988-5

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