Quality of life in children and adolescents 1-year after cure of Cushing syndrome: a prospective study

Abstract

Objective: Cushing syndrome (CS) in children is associated with symptoms that may impair health related quality of life (HRQL). There are no prospective reports of HRQL in children with CS.

Methods: Prospective study of 40 children (mean age 13 +/- 3.2 years) with CS evaluated prior to and 1-year post-treatment. The Child Health Questionnaire (CHQ) was used to assess HRQL; Wechsler Intelligence Scale for Children (WASI) was used to assess cognitive function, and patient-reported symptoms were assessed with a CS symptom checklist.

Results: Active CS was associated with low physical and psychosocial summary scores compared to US population data (P < 0.001). Despite improvement from pre- to 1-year postcure, residual impairment remained in physical summary and function, and role-physical, global health and emotional impact (parent) scores. Incomplete recovery of adrenal function at 1-year post-treatment was associated with impaired scores. WASI IQ scores declined and a correlation was noted between age at first evaluation and IQ score changes. Most self-reported CS symptoms showed improvement, but forgetfulness, unclear thinking and decreased attention span did not improve after cure of CS.

Conclusion: CS in children and adolescents is associated with impaired HRQL, with residual impairment 1 year after cure. Our results also suggest that younger children are more likely to experience negative changes in cognitive function. HRQL is an important outcome measure in children and adolescents with CS and identification of factors that contribute to HRQL may help to diminish the physical and psychological burden of disease in this population of patients.

Trial registration: ClinicalTrials.gov NCT00001595.

Figures

Figure 1

Individual CHQ domain z scores baseline (pre) and 1-yr after (post) treatment in 40 children with CS treated with either TSS or adrenalectomy. Results are expressed as z scores compared to age-matched normative data from the US population. The CHQ domains represented are those that showed significant improvement from pre- to post-treatment and include: physical summary score (PhS), physical function (PF), role-physical (RP), global health (GHP) (note: GHP was the only score that declined from pre- to post- treatment), emotional impact on parent (PE), mental health (MH), bodily pain (BP), self esteem (SE), time impact- parent (PT), and psychosocial summary score (PsS). p