Development and Validation of a Disease-Specific Quality-of-Life Measure for Children with Achalasia

Abstract

Background: Achalasia is an uncommon oesophageal motility disorder occasionally affecting children. Children with achalasia can experience significant morbidity, and quality-of- life (QoL) has never been studied in this population.

Aim: The aim was to develop a disease-specific quality-of-life (DS-QoL) measure for children with achalasia.

Methods: Item response theory methods were used to develop the DS-QoL measure. The construct validity of this measure was assessed by comparing items and domains with the generic PedsQL™ questionnaire. Reliability was assessed using Cronbach's alpha coefficient for internal consistency.

Results: 17 children completed the final DS-QoL measure, which consisted of 20 items in three domains. The completion rate for items was 99%. “Floor and ceiling effects” ranged from 0–19%. Construct validity was good with significant correlation between 2 domains and 2 items on the PedsQL™. Reliability was excellent, with Cronbach's alpha coefficient ranging from 0.78–0.93.

Conclusions: This DS-QoL measure is appropriate for use in children with achalasia and has shown good results in this validation study. Further work in higher numbers is necessary to determine discriminant validity and test-retest reliability.

oesophageal achalasia - quality-of-life - paediatrics

References

• 1 Ben-Meir A, Urbach DR, Khajanchee YS. et al . Quality-of-life before and after laparoscopic Heller myotomy for achalasia.  Am J Surg. 2001;  181 ((5)) 471-474
• 2 Cronbach LJ. Coefficient alpha and the internal structure of tests.  Psychometrika. 1951;  16 ((3)) 297-334
• 3 Decker G, Borie F, Bouamrirene D. et al . Gastrointestinal quality-of-life before and after laparoscopic heller myotomy with partial posterior fundoplication.  Ann Surg. 2002;  236 ((6)) 750-758
• 4 Eiser C, Morse R. Quality-of-life measures in chronic diseases of childhood.  Health Technol Assess. 2001;  5 ((4)) 1-157
• 5 Erling A. Methodological considerations in the assessment of health-related quality-of-life in children.  Acta Paediatr Suppl. 1999;  88 ((428)) 106-107
• 6 Frankhuisen R, Heijkoop R, van Herwaarden MA. et al . Validation of a disease-specific quality-of-life questionnaire in a large sample of Dutch achalasia patients.  Dis Esophagus. 2008;  21 ((6)) 544-550
• 7 Frankhuisen R, van Herwaarden MA, Heijkoop R. et al . Persisting symptoms and decreased health-related quality-of-life in a cross-sectional study of treated achalasia patients.  Aliment Pharmacol Ther. 2007;  26 ((6)) 899-904
• 8 Lawson ML, Cash TF, Akers R. et al . A pilot study of the impact of surgical repair on disease-specific quality-of-life among patients with pectus excavatum.  J Pediatr Surg. 2003;  38 ((6)) 916-918
• 9 Loonen HJ, Derkx BH, Otley AR. Measuring health-related quality-of-life of pediatric patients.  J Pediatr Gastroenterol Nutr. 2001;  32 ((5)) 523-526
• 10 Maity S, Thomas AG. Quality-of-life in paediatric gastrointestinal and liver disease: a systematic review.  J Pediatr Gastroenterol Nutr. 2007;  44 ((5)) 540-554
• 11 Meshkinpour H, Haghighat P, Meshkinpour A. Quality-of-life among patients treated for achalasia.  Dig Dis Sci. 1996;  41 ((2)) 352-356
• 12 Modi AC, Quittner AL. Validation of a disease-specific measure of health-related quality-of-life for children with cystic fibrosis.  J Pediatr Psychol. 2003;  28 ((8)) 535-545
• 13 Morris-Stiff G, Khan R, Foster ME. et al . Long-term results of surgery for childhood achalasia.  Ann R Coll Surg Engl. 1997;  79 ((6)) 432-434
• 14 Nurko S. Other Motor Disorders. In: Walker WA, Durie PR, Hamilton RJ, Walker-Smith JA, Watkins JB, ed. Paediatric Gastrointestinal Disease. 2nd ed. St. Louis, Missouri: Mosby-Year Book Inc 1996 pp 474-489
• 15 Otley A, Smith C, Nicholas D. et al . The IMPACT questionnaire: A valid measure of health-related quality-of-life in pediatric inflammatory bowel disease.  J Pediatr Gastroenterol Nutr. 2002;  35 ((4)) 557-563
• 16 Pensabene L, Nurko S. Approach to the child who has persistent dysphagia after surgical treatment for esophageal achalasia.  J Pediatr Gastroenterol Nutr. 2008;  47 ((1)) 92-97
• 17 Ponce M, Ortiz V, Juan M. et al . Gastroesophageal reflux, quality-of-life, and satisfaction in patients with achalasia treated with open cardiomyotomy and partial fundoplication.  Am J Surg. 2003;  185 ((6)) 560-564
• 18 Raftopoulos Y, Landreneau RJ, Hayetian F. et al . Factors affecting quality-of-life after minimally invasive Heller myotomy for achalasia.  J Gastrointest Surg. 2004;  8 ((3)) 233-239
• 19 Upton P, Eiser C, Cheung I. et al . Measurement properties of the UK-English version of the Pediatric Quality-of-life Inventory 4.0 (PedsQL) generic core scales.  Health Qual Life Outcomes. 2005;  3 22
• 20 Urbach DR, Tomlinson GA, Harnish JL. et al . A measure of disease-specific health-related quality-of-life for achalasia.  Am J Gastroenterol. 2005;  100 ((8)) 1668-1676
• 21 Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality-of-life Inventory version 4.0 generic core scales in healthy and patient populations.  Med Care. 2001;  39 ((8)) 800-812
• 22 Voskuijl WP, van der Zaag-Loonen HJ, Ketel IJG. et al . Health-related quality-of-life in disorders of defection: the Defecation Disorders List.  Arch Dis Child. 2004;  89 1124-1127
• 23 Youssef Y, Richards WO, Sharp K. et al . Relief of dysphagia after laparoscopic Heller myotomy improves long-term quality-of-life.  J Gastrointest Surg. 2007;  11 ((3)) 309-313

Correspondence