Physical health conditions and quality of life in adults with primary immune deficiency diagnosed during childhood: a CEREDIH study

Publication date: Available online 30 September 2016
Source:Journal of Allergy and Clinical Immunology
Author(s): Vincent Barlogis, Nizar Mahlaoui, Pascal Auquier, Isabelle Pellier, Fanny Fouyssac, Camille Vercasson, Maya Allouche, Carolina Brito De Azevedo, Felipe Suarez, Despina Moshous, Bénédicte Neven, Marlène Pasquet, Eric Jeziorski, Nathalie Aladjidi, Nicolas Schleinitz, Caroline Thomas, Virginie Gandemer, Françoise Mazingue, Patrick Lutz, Olivier Hermine, Capucine Picard, Stéphane Blanche, Gérard Michel, Alain Fischer
BackgroundMost children with primary immune deficiency (PID) now reach adulthood. However, few studies have evaluated their health status and health related quality of life (HRQoL).ObjectiveTo investigate long-term morbidity, the French Reference Center for PIDs initiated a prospective multicenter cohort: the F-CILC (French Childhood Immune deficiency Long-term Cohort). The data collected was used to assess the physical health condition of patients who reached adulthood and the impact on their quality of life.MethodsPatients were asked to complete health status questionnaires. A severity score (grade1 ["mild"] to grade 4 ["life-threatening"]) was assigned to each health condition. The HRQoL of patients was compared to age- and sex-matched French normal values using the SF36 HRQoL questionnaire.ResultsAmong 329 participants, the mean age at evaluation was 27.6 years with a 21-year mean follow-up after diagnosis, 43% reported at least one grade 4 health condition and 86% reported at least one grade 3 (severe) or 4 health condition. Twenty-five patients (7.6%) had been treated for cancer. Compared with the French norms, adults with PID scored significantly lower for all HRQoL domains. HRQoL was strongly associated with the burden of health conditions. The association with grade 4 or grade 3-4 health conditions was highly significant for all physical and mental domains.ConclusionAdults with PID diagnosed during childhood experienced a heavy burden of health conditions, which affected their HRQoL. Our results emphasize the need to closely monitor this vulnerable population.

Teaser

This is the first study of adult survivors of childhood PID describing how the burden of health conditions affect their quality of life. Our results emphasize the need to closely monitor this vulnerable population.


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