Clara Lauby1, Pierre-Yves Boelle2, Rola Abou Taam1,3, Katia Bessaci1,4, Jacques Brouard1,5, Marie-Laure Dalphin1,6, Christophe Delacourt1,3, Céline Delestrain1,7, Antoine Deschildre1,8, Jean-Christophe Dubus1,9, Michaël Fayon1,10, Lisa Giovannini-Chami1,11, Véronique Houdouin1,12, Anne Houzel1,13, Christophe Marguet1,14, Isabelle Pin1,15, Philippe Reix1,16, Marie-Catherine Renoux1,17, Cyril Schweitzer1,18, Aurélie Tatopoulos1,18, Caroline Thumerelle1,8, Françoise Troussier1,19, Stéphanie Wanin1,12, Laurence Weiss1,20, Annick Clement1,21, Ralph Epaud1,7, Nadia Nathan1,21. 1. Reference Centre for Rare Lung Diseases, RespiRare, France. 2. Inserm UMR S 1136, Pierre Louis Institute of Epidemiology and Public Health, Paris, France. 3. Pediatric Pulmonology Department, APHP, Necker Enfants Malades Hospital, Paris, France. 4. Pediatric Pulmonology Department, University Hospital, Reims, France. 5. Pediatric Pulmonology Department, University Hospital, Caen, France. 6. Pediatric Pulmonology Department, University Hospital, Besançon, France. 7. Pediatric Pulmonology Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France. 8. Pediatric Pulmonology Department, University Hospital, Lille, France. 9. Pediatric Pulmonology Department, La Timone University Hospital, Marseille, France. 10. Pediatric Pulmonology Department, Pellegrin University Hospital, Bordeaux, France. 11. Pediatric Pulmonology Department, Lenval University Hospital, Nice, France. 12. Pediatric Pulmonology Department, APHP, Robert Debré Hospital, Paris, France. 13. Pediatric Pulmonology Department, University Hospital, Dijon, France. 14. Pediatric Pulmonology Department, University Hospital, Rouen, France. 15. Pediatric Pulmonology Department, University Hospital, Grenoble, France. 16. Pediatric Pulmonology Department, Femme Mere Enfants University Hospital, Hospices Civils de Lyon, Lyon, France. 17. Pediatric Pulmonology Department, University Hospital, Montpellier, France. 18. Pediatric Pulmonology Department, University Hospital, Nancy, France. 19. Pediatric Pulmonology Department, University Hospital, Angers, France. 20. Pediatric Pulmonology Department, University Hospital, Strasbourg, France. 21. Pediatric Pulmonology Department and Inserm UMR S933, APHP and Sorbonne Université, Armand Trousseau Hospital, Paris, France.
Abstract
INTRODUCTION: Interstitial lung disease in children (chILD) is a highly heterogeneous group of rare and severe respiratory disorders. The disease by itself, the burden of the treatments (oxygen therapy, corticosteroid pulses, nutritional support) and recurrent hospitalizations may impair the quality of life (QoL) of these children. The aim of the study was to compare the health-related QoL (HR-QoL) in chILD compared to a healthy population and to find out the predictive factors of an altered QoL. METHODS: Patients aged 1 month to 18 years with ILD of known or unknown etiology were prospectively included. Parents and children over 8 years old were asked to fill the PedsQL 4.0 Generic Core Scale ranging from 0 to 100 points. RESULTS: A total of 78 children were recruited in 13 French pediatric centers. Total scores were 11.94 points (P = 0.0003) less for child self-report and 14.08 points ( P < 0.0001) less for parent proxy-report with respect to the healthy population. The clinical factors associated with a lower total score were: extrapulmonary expression of the disease, higher Fan severity score, long-term oxygen therapy, nutritional support, and a number of oral treatments. CONCLUSION: Using a validated quality of life (QoL) scale, we showed that health-related-QoL is significantly impaired in chILD compared with a healthy population. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL.
INTRODUCTION:Interstitial lung disease in children (chILD) is a highly heterogeneous group of rare and severe respiratory disorders. The disease by itself, the burden of the treatments (oxygen therapy, corticosteroid pulses, nutritional support) and recurrent hospitalizations may impair the quality of life (QoL) of these children. The aim of the study was to compare the health-related QoL (HR-QoL) in chILD compared to a healthy population and to find out the predictive factors of an altered QoL. METHODS:Patients aged 1 month to 18 years with ILD of known or unknown etiology were prospectively included. Parents and children over 8 years old were asked to fill the PedsQL 4.0 Generic Core Scale ranging from 0 to 100 points. RESULTS: A total of 78 children were recruited in 13 French pediatric centers. Total scores were 11.94 points (P = 0.0003) less for child self-report and 14.08 points ( P < 0.0001) less for parent proxy-report with respect to the healthy population. The clinical factors associated with a lower total score were: extrapulmonary expression of the disease, higher Fan severity score, long-term oxygen therapy, nutritional support, and a number of oral treatments. CONCLUSION: Using a validated quality of life (QoL) scale, we showed that health-related-QoL is significantly impaired in chILD compared with a healthy population. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL.
Authors: Robin R Deterding; Emily M DeBoer; Michal J Cidon; Terry E Robinson; David Warburton; Gail H Deutsch; Lisa R Young Journal: Am J Respir Crit Care Med Date: 2019-11-15 Impact factor: 21.405
Authors: Lauren Kelada; Claire Wakefield; Nada Vidic; David S Armstrong; Bruce Bennetts; Kirsten Boggs; John Christodoulou; Joanne Harrison; Gladys Ho; Nitin Kapur; Suzanna Lindsey-Temple; Tim McDonald; David Mowat; André Schultz; Hiran Selvadurai; Andrew Tai; Adam Jaffe Journal: BMJ Open Respir Res Date: 2022-02
Authors: Suzanna E L Temple; Gladys Ho; Bruce Bennetts; Kirsten Boggs; Nada Vidic; David Mowat; John Christodoulou; André Schultz; Thet Gayagay; Tony Roscioli; Ying Zhu; Sebastian Lunke; David Armstrong; Joanne Harrison; Nitin Kapur; Tim McDonald; Hiran Selvadurai; Andrew Tai; Zornitza Stark; Adam Jaffe Journal: Orphanet J Rare Dis Date: 2022-09-09 Impact factor: 4.303