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Disease severity impacts perceived quality of life in congenital diaphragmatic hernia: a prospective observational study
  1. Alexandra Dimmer1,
  2. Madison Meehan2,
  3. Sabrina Beauseigle1,
  4. Louise Koclas2,
  5. Katryn Paquette2,
  6. Carolina Michel Macias2,
  7. Shiran S Moore3,
  8. Ana Sant'Anna2,
  9. Adam Shapiro4,
  10. Jessica Simoneau2,
  11. Daniela Villegas Martinez2,
  12. Gabriel Altit2,
  13. Pramod Puligandla1
  1. 1 Harvey E Beardmore Division of Pediatric Surgery, Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, Québec, Canada
  2. 2 Division of Neonatology, Department of Pediatrics, Montreal Children's Hospital, Montreal, Québec, Canada
  3. 3 Division of Neonatology, Dana-Dwek Children's Hospital, Tel Aviv, Israel
  4. 4 Division of Respiratory Medicine, Department of Pediatrics, Montreal Children's Hospital, Montreal, Québec, Canada
  1. Correspondence to Dr Pramod Puligandla, Division of Pediatric Surgery, Department of Pediatric Surgery, Montreal Children's Hospital, Montreal, H4A 3J1, Canada; pramod.puligandla{at}mcgill.ca

Abstract

Background While research indicates comparable quality of life (QOL) in congenital diaphragmatic hernia (CDH) and healthy populations, the effect of CDH severity on patients’ health perceptions remains unexplored. We aimed to assess QOL perception in CDH, hypothesising a decline correlated with increased disease severity.

Methods In this prospective observational study, we analysed patients with CDH aged 5 years and above participating in a longitudinal outpatient programme. We excluded bilateral CDH, genetic/syndromic conditions, prematurity and late diagnosis. Participants self-administered the age-adapted Pediatric Quality of Life Inventory (PedsQL) survey, covering four domains (physical, emotional, social, school). After enrolment, data were collected blind to severity status (larger defects denoting significant/‘severe’ disease). Repeated measurements were managed using a random mixed-effects model.

Results Of 34 participants (50% males) who completed the PedsQL, 10 provided measurements at two visits. Eight required a patch (type C), while 26 had primary repairs (type A=8; type B=18). Age at first evaluation was comparable across groups (no patch: median 11 (7–16), patch: 13 (8–15) years, p=0.78). Severe CDH correlated significantly with lower PedsQL scores (adjusted β: −18%, 95% CI −28%; −7%, adjusted for age at visit and sex). Lower scores specifically occurred in walking, exercising, social and academic functioning.

Conclusion Severe CDH significantly lowers QOL. This finding is crucial for resource allocation in long-term CDH health surveillance and advocates for regular inclusion of patient experiences in quality improvement efforts.

  • Child Health
  • Paediatrics
  • Child Development
  • Follow-Up Studies
  • Infant Development

Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information. Not applicable.

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Data availability statement

All data relevant to the study are included in the article or uploaded as supplemental information. Not applicable.

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Footnotes

  • X @CMichelK, @DrPuligandla

  • Contributors AD contributed to conceptualising and designing the study, collected the data, analysed the data, drafted the initial and revised manuscript, and is the acting guarantor of the work. MM collected the data and critically reviewed the manuscript. LK was involved in the design of the study, collected the data, contributed to the interpretation of the findings and critically reviewed the manuscript. SB, CMM, SSM, KP, AS'A, AS, JS and DVM collected the data, contributed to the interpretation of the findings and critically reviewed the manuscript. GA and PP conceptualised and designed the study, supervised data analysis, contributed to the interpretation of the findings and critically reviewed the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

  • Funding This work was funded by the Grand Défi Pierre Lavoie, the Mirella and Lino Saputo Foundation Chair in Pediatric Surgical Education and Patient and Family-Centered Care, Department of Pediatric Surgery, McGill University Faculty of Medicine and Health Sciences, the John McCrae Fellowship, McGill University Faculty of Medicine and Health Sciences, as well as the Montreal Children’s Hospital Foundation.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.