Influence of Diet on Bowel Function and Abdominal Symptoms in Children and Adolescents with Hirschsprung Disease—A Multinational Patient-Reported Outcome Survey
Judith Lindert,
Hannah Day,
Marta de Andres Crespo,
Eva Amerstorfer,
Sabine Alexander,
Manouk Backes,
Carlotta de Filippo,
Andrzej Golebiewski,
Paola Midrio,
Mazeena Mohideen,
Anna Modrzyk,
Anette Lemli,
Roxana Rassouli-Kirchmeier,
Marijke Pfaff-Jongman,
Karolina Staszkiewicz,
Lovisa Telborn,
Pernilla Stenström,
Karolin Holström,
Martina Kohl,
Joe Curry,
Stavros Loukogeorgakis,
Joseph R Davidson
Affiliations
Judith Lindert
Department of Paediatric Surgery, University Rostock, Ernst-Heydemann-Str. 8, 18057 Rostock, Germany
Hannah Day
Department of Specialist Neonatal and Paediatric Surgery Great, Ormond Street Hospital NHS Trust, London WC1N 3JH, UK
Marta de Andres Crespo
Department of Paediatric Surgery, University Rostock, Ernst-Heydemann-Str. 8, 18057 Rostock, Germany
Eva Amerstorfer
Department of Paediatric Surgery, University Hospital Graz, Auenbruggerplatz 34, 8036 Graz, Austria
Sabine Alexander
SoMA e.V.—Selbsthilfeorganisation für Betroffene von Morbus Hirschsprung und Anorektale Fehlbildungen Patient Organization, Munich, Blombergstr. 9, 81825 München, Germany
Manouk Backes
Department of Paediatric Surgery, Radboudumc Njjmegen, Geert Grooteplein Zuid 32, 6525 Nijmege, The Netherlands
Carlotta de Filippo
Italian Association of Hirschsprung’s Disease A.Mor.Hi, Via dei Castani 116, 00172 Rome, Italy
Andrzej Golebiewski
Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, 80-210 Gdansk, Poland
Paola Midrio
Pediatric Surgery Unit, Ca’Foncello Hospital, Piazzale dell´Ospedale 1, 31100 Treviso, Italy
Mazeena Mohideen
SoMA Austria—Selbsthilfeorganisation für Betroffene von Morbus Hirschsprung und Anorektale Fehlbildungen, Am-Ostrom-Park 11/7, 1220 Wien, Austria
Anna Modrzyk
Department of Children’s Developmental Defects Surgery and Traumatology, Medical University of Silesia, 41-800 Zabrze, Poland
Anette Lemli
SoMA e.V.—Selbsthilfeorganisation für Betroffene von Morbus Hirschsprung und Anorektale Fehlbildungen Patient Organization, Munich, Blombergstr. 9, 81825 München, Germany
Roxana Rassouli-Kirchmeier
Department of Paediatric Surgery, Radboudumc Njjmegen, Geert Grooteplein Zuid 32, 6525 Nijmege, The Netherlands
Marijke Pfaff-Jongman
Dutch Patient Association, Vereniging Ziekte van Hirschsprung, Hambakenwetering 15, 5231 ‘S-Hertogenbosch, The Netherlands
Karolina Staszkiewicz
Polish Hirschsprung Facebook Group, Poland
Lovisa Telborn
Department of Pediatric Surgery, Lund University, Skåne University Hospital, 22100 Lund, Sweden
Pernilla Stenström
Department of Pediatric Surgery, Lund University, Skåne University Hospital, 22100 Lund, Sweden
Karolin Holström
Swedish Patient Association-Hirschsprungs Sjukdom Patientförening, Sockervägen 25, 23253 Akarp, Sweden
Martina Kohl
Paediatric Gastroenterology, Paediatric Department, University Lübeck, Ratzeburger Alle 160, 23538 Lübeck, Germany
Joe Curry
Department of Specialist Neonatal and Paediatric Surgery Great, Ormond Street Hospital NHS Trust, London WC1N 3JH, UK
Stavros Loukogeorgakis
Department of Specialist Neonatal and Paediatric Surgery Great, Ormond Street Hospital NHS Trust, London WC1N 3JH, UK
Joseph R Davidson
Department of Specialist Neonatal and Paediatric Surgery Great, Ormond Street Hospital NHS Trust, London WC1N 3JH, UK
Introduction: This study aimed to understand the influence of diet and nutrition items on gastrointestinal symptoms in patients with Hirschsprung Disease (HD). Method: An online questionnaire was created to obtain patient-reported outcomes using the multinational Holistic Care in Hirschsprung Disease Network. This was distributed in Dutch, English, German, Italian, Polish, and Swedish via patient associations. Information on demographics, the extension of disease, current diet, and the influence of food ingredients on bowel function were obtained. Results: In total, 563 questionnaires were answered by parents or patients themselves. The length of the aganglionic segment was short in 33%, long in 45%, total colonic aganglionosis (TCA) in 11%, and involved the small intestine in 10%. Overall, 90% reported following a mixed diet, and 31% reported taking probiotics, with twice as many patients taking probiotics in the TCA group compared to standard HD. Mealtimes and behaviours around eating were affected by 61%, while 77% had established food items that worsened symptoms, and of these, 80% stated that they had worked these items out themselves. A high-fibre diet was followed by 24% and 18% a low-fibre diet. Symptoms were reported, particularly from dairy in 30%, fruits in 39%, pulses in 54%, and sugar in 48%. Conclusions: This first multinational survey on diet and bowel function in HD reports an association between certain dietary items with gastrointestinal symptoms. This study can support an improved understanding of the interaction between food items and bowel function in children with HD. We suggest a multidisciplinary approach to balance dietary exclusions and support adequate growth, preventing nutrition deficiencies and enhancing quality of life.
