Health‐related quality of life in infants and children with interstitial lung disease

Clara Lauby (1) , Pierre-Yves Boëlle (2) , Rola Abou Taam (3) , Katia Bessaci (4) , Jacques Brouard (5, 6) , Marie‐laure Dalphin (7, 1) , Christophe Delacourt (8) , Céline Delestrain (1, 9) , Antoine Deschildre (10) , Christophe Dubus (11, 1) , Michael Fayon (12) , Lisa Giovannini‐chami (1, 13) , Véronique Houdouin (14) , Anne Houzel (15, 1) , Christophe Marguet (16) , Isabelle Pin (17, 18) , Philippe Reix (19) , Marie‐catherine Renoux (20, 1) , Cyril Schweitzer (21) , Aurélie Tatopoulos (13, 1) , Caroline Thumerelle (10) , Françoise Troussier (22) , Stéphanie Wanin (14) , Laurence Weiss (23) , Annick Clément (24, 25, 26) , Ralph Epaud (27) , Nadia Nathan (24, 25, 26)

1 CHU Trousseau [APHP]
2 Epidémiologie, Systèmes d'Information, Modélisation
3 MaMEA Necker - Centre Référence des Maladies Héréditaires du Métabolisme de l'Enfant et de l'Adulte [CHU Necker]
4 Hôpital universitaire Robert Debré [Reims]
5 Service de Pédiatrie Médicale [Caen]
6 GRAM 2.0 - Groupe de Recherche sur l'Adaptation Microbienne
7 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
8 LST - Laboratoire de Sciences de la Terre
9 CHIC - Centre Hospitalier Intercommunal de Créteil
10 CHRU Lille - Centre Hospitalier Régional Universitaire [Lille]
11 Service de pédiatrie spécialisée et médecine infantile (neurologie, pneumologie, maladies héréditaires du métabolisme) [Hôpital de la Timone - APHM]
12 CHU Bordeaux [Bordeaux]
13 CHU Nice - Centre Hospitalier Universitaire de Nice
14 AP-HP Hôpital universitaire Robert-Debré [Paris]
15 CHU Dijon - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
16 Service de pédiatrie médicale et médecine de l'adolescent [Rouen]
17 CHU Grenoble
18 Equipe Inserm U1163 - Inflammatory Responses and Transcriptomic Networks in diseases
19 HCL - Hospices Civils de Lyon
20 CHU Montpellier
21 Service de Médecine Infantile III et Génétique Clinique [CHRU Nancy]
22 CHU Angers - Centre Hospitalier Universitaire d'Angers
23 Service d'immunologie [HEGP, Paris]
24 Service de Pneumologie pédiatrique [CHU Trousseau]
25 Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau]
26 Inserm U933 - Maladies génétiques d'expression pédiatrique [CHU Trousseau]
27 BCR - Biomécanique cellulaire et respiratoire

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

Keywords: chILD; children; interstitial lung disease; quality of life; scale

Domains: Virology; Pulmonology and respiratory tract; Microbiology and Parasitology; Bacteriology

Clara Lauby, Pierre-Yves Boëlle, Rola Abou Taam, Katia Bessaci, Jacques Brouard, et al.. Health‐related quality of life in infants and children with interstitial lung disease. Pediatric Pulmonology, 2019, 54 (6), pp.828-836. ⟨10.1002/ppul.24308⟩. ⟨hal-02153909⟩