Evaluating the Empowerment Potential of an International Sexual Support Website for Patients with Anorectal Malformations and Hirschsprung Disease, their Parents and Healthcare Providers

Abstract

Introduction

Research indicates that the sexual support needs of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) are often not addressed by patients, parents, and healthcare professionals (HPs) in their interactions. An international support website was developed to empower stakeholders, by addressing identified barriers. This study aimed to explore the empowerment potential of this disease-specific tool.

Materials and Methods

Two online surveys were disseminated between May 1 and October 1, 2023; one for HPs and another for patients/parents. The surveys sought to assess and understand the website's expected empowerment effect. Empowerment was conceptualized using patient/professional empowerment models. Data were descriptively analyzed.

Results

A total of 12 patients (ARM, n = 11; HD, n = 1), 17 parents (ARM, n = 9; HD, n = 8), and 20 HPs responded to the survey. HPs largely expected the website to have a positive empowerment effect, by providing a sense of meaning, information, support, and opportunities to learn and grow. Less of an effect was expected for “freeing up resources.” For patients and parents, an empowerment effect was also expected, by generating the knowledge, skills, attitudes, and self-awareness necessary to influence their own behavior and by providing a sense of meaning and coherence. Respondents experienced the website positively, yet one patient and one parent considered the website “fully complete.” Inclusivity, cultural sensitivity, and accessibility were highlighted as focus points.

Conclusions

To increase the website's empowerment potential, attention should be paid to inclusivity, cultural sensitivity, and accessibility, as well as its implementation within the (institutional) contexts where patients, parents, and HPs interact.

Keywords

ERNICA - ERN eUROGEN - European Reference Network - rare disease - digital health

Publikationsverlauf

Eingereicht: 25. September 2024

Angenommen: 07. Juni 2025

Artikel online veröffentlicht:
09. Juli 2025

• References

• 1 Trinidad S, Garrison A, Encisco EM. et al. Long-term male sexual function and fecal incontinence outcomes for adult patients with Hirschsprung disease or anorectal malformation. J Pediatr Surg 2023; 58 (08) 1573-1577
• 2 Rigueros Springford L, Connor MJ, Jones K, Kapetanakis VV, Giuliani S. Prevalence of active long-term problems in patients with anorectal malformations: a systematic review. Dis Colon Rectum 2016; 59 (06) 570-580
• 3 Danielson J, Karlbom U, Graf W, Wester T. Outcome in adults with anorectal malformations in relation to modern classification—which patients do we need to follow beyond childhood?. J Pediatr Surg 2017; 52 (03) 463-468
• 4 Neuvonen MI, Kyrklund K, Rintala RJ, Pakarinen MP. Bowel function and quality of life after transanal endorectal pull-through for Hirschsprung disease: controlled outcomes up to adulthood. Ann Surg 2017; 265 (03) 622-629
• 5 Kyrklund K, Pakarinen MP, Rintala RJ. Long-term bowel function, quality of life and sexual function in patients with anorectal malformations treated during the PSARP era. Semin Pediatr Surg 2017; 26 (05) 336-342
• 6 Zimmer J, Tomuschat C, Puri P. Long-term results of transanal pull-through for Hirschsprung's disease: a meta-analysis. Pediatr Surg Int 2016; 32 (08) 743-749
• 7 Bjoersum-Meyer T, Kaalby L, Lund L. et al. Long-term functional urinary and sexual outcomes in patients with anorectal malformations—a systematic review. Eur Urol Open Sci 2021; 25: 29-38
• 8 Reppucci ML, Harris KT, Wilcox DT, Peycelon M, Bischoff A. Adult urological outcomes for patients with anorectal malformation. J Pediatr Urol 2024; 20 (02) 193-199
• 9 Stenström P, Kockum CC, Benér DK, Ivarsson C, Arnbjörnsson E. Adolescents with anorectal malformation: physical outcome, sexual health and quality of life. Int J Adolesc Med Health 2014; 26 (01) 49-59
• 10 Reppucci ML, Wehrli LA, Wilcox D. et al. Sexual function and fertility of adult males with anorectal malformations or Hirschsprung disease. Pediatr Surg Int 2022; 38 (12) 1693-1699
• 11 Witvliet MJ, van Gasteren S, van den Hondel D, Hartman E, van Heurn L, van der Steeg A. Predicting sexual problems in young adults with an anorectal malformation or Hirschsprung disease. J Pediatr Surg 2018; 53 (08) 1555-1559
• 12 van den Hondel D, Sloots CE, Bolt JM, Wijnen RM, de Blaauw I, IJsselstijn H. Psychosexual well-being after childhood surgery for anorectal malformation or Hirschsprung's disease. J Sex Med 2015; 12 (07) 1616-1625
• 13 Bicelli N, Trovalusci E, Zannol M. et al. Gynecological and psycho-sexual aspects of women with history of anorectal malformations. Pediatr Surg Int 2021; 37 (08) 991-997
• 14 Davidson JR, Kyrklund K, Eaton S. et al. Sexual function, quality of life, and fertility in women who had surgery for neonatal Hirschsprung's disease. Br J Surg 2021; 108 (02) e79-e80
• 15 Mitchell KR, Lewis R, O'Sullivan LF, Fortenberry JD. What is sexual wellbeing and why does it matter for public health?. Lancet Public Health 2021; 6 (08) e608-e613
• 16 Yu LX, McGowan M, Bendle K, Mullins C, Streich-Tilles T, Breech LL. Sexual and reproductive health concerns in adults with cloacal anomalies: a qualitative study. J Pediatr Surg 2023; 58 (12) 2300-2307
• 17 Svetanoff WJ, Kapalu CL, Lopez JJ, Fraser JA, Briggs KB, Rentea RM. Psychosocial factors affecting quality of life in patients with anorectal malformation and Hirschsprung disease—a qualitative systematic review. J Pediatr Surg 2022; 57 (03) 387-393
• 18 Feng X, Lacher M, Quitmann J, Witt S, Witvliet MJ, Mayer S. Health-related quality of life and psychosocial morbidity in anorectal malformation and Hirschsprung's disease. Eur J Pediatr Surg 2020; 30 (03) 279-286
• 19 Eleuteri S, Aminoff D, Lucidi F, Violani C, Grano C. Sexual well-being in adolescent and young adults born with arm: the perspective of the patients. Pediatr Surg Int 2019; 35 (09) 945-951
• 20 Grano C, Bucci S, Aminoff D, Lucidi F, Violani C. Feelings of depression in people with ARM: the role of critical incidents and perceived difficulties in close and sexual relationships. Pediatr Surg Int 2014; 30 (08) 823-828
• 21 Eleuteri S, Aminoff D, Midrio P. et al. Talking about sexuality with your own child. The perspective of the parents of children born with arm. Pediatr Surg Int 2022; 38 (12) 1665-1670
• 22 Petit-Steeghs V, Mogami-Asselin GIK, Nijenkamp MD, Spoel M, Broerse JEW, Pittens CACM. Empowering patients and health professionals to address sexual health in the context of anorectal malformations and Hirschsprung's disease. Qual Health Res 2020; 30 (13) 2033-2048
• 23 Hoel AT, Tofft L, Bjørnland K. et al. Reaching adulthood with Hirschsprung's disease: patient experiences and recommendations for transitional care. J Pediatr Surg 2021; 56 (02) 257-262
• 24 Violani C, Grano C, Fernandes M. et al. The transition of care for patients with anorectal malformations and Hirschsprung disease: a European survey. Eur J Pediatr Surg 2023; 33 (03) 191-197
• 25 Amerstorfer EE, Grano C, Verhaak C. et al; ARM-Net consortium. What do pediatric surgeons think about sexual issues in dealing with patients with anorectal malformations? The ARM-Net consortium members' opinion. Pediatr Surg Int 2019; 35 (09) 935-943
• 26 Macdowall W, Parker R, Nanchahal K. et al. “Talking of Sex”: developing and piloting a sexual health communication tool for use in primary care. Patient Educ Couns 2010; 81 (03) 332-337
• 27 Taylor B, Davis S. The extended PLISSIT model for addressing the sexual wellbeing of individuals with an acquired disability or chronic illness. Sex Disabil 2007; 25 (03) 135-139
• 28 van der Stege HA, van Staa A, Hilberink SR, Visser AP. Using the new board game SeCZ TaLK to stimulate the communication on sexual health for adolescents with chronic conditions. Patient Educ Couns 2010; 81 (03) 324-331
• 29 Annon JS. The PLISSIT model: a proposed conceptual scheme for the behavioral treatment of sexual problems. J Sex Educ Ther 1976; 2 (01) 1-15
• 30 The European Reference Network for rare Inherited Congenital Anomalies (ERNICA). Accessed September 16, 2024 at: https://www.ern-ernica.eu/
• 31 The European Reference Network for Rare Urogenital Diseases and Complex Conditions (ERN eUROGEN). Accessed September 16, 2024 at: https://eurogen-ern.eu/
• 32 European Reference Networks. Accessed September 16, 2024 at: https://health.ec.europa.eu/rare-diseases-and-european-reference-networks/european-reference-networks_en
• 33 Kanter RM. Men and women of the corporation revisited: interview with Rosabeth Moss Kanter. Human Resource Management. 1987; 26 (02) 257-263
• 34 Thomas KW, Velthouse BA. Cognitive elements of empowerment: an “interpretive” model of intrinsic task motivation. Acad Manage Rev 1990; 15 (04) 666-681
• 35 Bravo P, Edwards A, Barr PJ, Scholl I, Elwyn G, McAllister M. Cochrane Healthcare Quality Research Group, Cardiff University. Conceptualising patient empowerment: a mixed methods study. BMC Health Serv Res 2015; 15: 252
• 36 Spence Laschinger HK, Gilbert S, Smith LM, Leslie K. Towards a comprehensive theory of nurse/patient empowerment: applying Kanter's empowerment theory to patient care. J Nurs Manag 2010; 18 (01) 4-13
• 37 Teixeira AC, Nogueira A, Barbieri-Figueiredo MDC. Professional empowerment and evidence-based nursing: a mixed-method systematic review. J Clin Nurs 2023; 32 (13-14): 3046-3057
• 38 Nilsen P. Making sense of implementation theories, models and frameworks. Implement Sci 2015; 10: 53