Center for Advanced Intestinal Rehabilitation (CAIR), Boston Children's Hospital.
Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA.
J Pediatr Gastroenterol Nutr. 2021 Sep 1;73(3):338-344. doi: 10.1097/MPG.0000000000003102.
The aim of the study was to assess overall and disease-specific health-related quality of life (hrQOL) in patients with pediatric intestinal failure (PIF) and caregivers and elucidate differences from healthy and chronic gastrointestinal (GI) illness cohorts.
Cross-sectional study of patients with PIF and their caregivers managed at a multidisciplinary intestinal rehabilitation program using the PedsQL Generic Core and the Gastrointestinal Symptoms Module to assess generic and disease-specific hrQOL, respectively. These data were compared to established healthy and chronic GI disease controls.
A total of 53 patients (mean age 6.2 ± 3.9 years) and their caregivers were studied. Patients reported lower generic hrQOL than healthy children (73.0 vs 83.84, P < 0.001), but no difference from patients with chronic GI disease (73.0 vs 77.79). In contrast, PIF caregivers perceived similar generic hrQOL compared to a healthy cohort (78.9 vs 82.70), but higher when compared to the GI disease cohort (78.9 vs 72.74, P < 0.01). Patients with PIF and caregivers reported lower psychosocial health scores than healthy controls. Patients and caregivers reported similar disease-specific hrQOL to a cohort with chronic GI disease but significantly lower disease-specific hrQOL than a healthy cohort (P < 0.001 both groups).
Patients with PIF and their caregivers have disparate perceptions of generic hrQOL when compared to healthy and chronic GI disease controls. Both patients and caregivers, however, had significantly lower scores in psychosocial health than healthy controls. In addition, disease-specific hrQOL was substantially lower than healthy controls for PIF patients and caregivers. Further investigation to expand on these findings and identify modifiable variables to improve the psychosocial health score and disease-specific factors would be of high value.
本研究旨在评估儿科肠衰竭（PIF）患者及其照护者的总体和疾病特异性健康相关生活质量（HRQOL），并阐明其与健康人群和慢性胃肠道（GI）疾病人群的差异。
采用 PedsQL 通用核心量表和胃肠道症状模块对接受多学科肠康复计划管理的 PIF 患者及其照护者进行横断面研究，分别评估通用和疾病特异性 HRQOL。将这些数据与既定的健康和慢性 GI 疾病对照组进行比较。
共纳入 53 例患者（平均年龄 6.2±3.9 岁）及其照护者。与健康儿童相比，患者报告的通用 HRQOL 较低（73.0 比 83.84，P<0.001），但与慢性 GI 疾病患者无差异（73.0 比 77.79）。相比之下，PIF 照护者的通用 HRQOL 与健康人群相似（78.9 比 82.70），但与 GI 疾病人群相比更高（78.9 比 72.74，P<0.01）。PIF 患者及其照护者报告的心理社会健康评分低于健康对照组。PIF 患者和照护者报告的疾病特异性 HRQOL 与慢性 GI 疾病患者相似，但明显低于健康对照组（两组均 P<0.001）。
与健康人群和慢性 GI 疾病人群相比，PIF 患者及其照护者对通用 HRQOL 的看法存在差异。然而，与健康对照组相比，患者和照护者的心理社会健康评分均显著降低。此外，PIF 患者及其照护者的疾病特异性 HRQOL 明显低于健康对照组。进一步的研究来扩展这些发现，并确定可改变的变量，以提高心理社会健康评分和疾病特异性因素，将具有很高的价值。
