Relationships Among Health-Related Quality of Life, Pulmonary Health, and Newborn Screening for Cystic Fibrosis

Abstract

Medical advances have signifi cantly improved the longevity of patients with cystic fi brosis (CF). The median survival age is approximately 37 years, and many patients live well into middle age. 1 Newborn screening (NBS) is an innovation that provides early intervention for affected infants. Although neo natal diagnosis offers nutritional advantages, over time, patients experience complications that require increasingly complex treatments 2-4 for which they and their families typically shoulder most of the burden. The progressive nature of CF and treatment side effects can adversely affect every aspect of life. Thus, there has been a growing interest in understanding how this chronic illness and related life-long care affect health-related quality of life (HRQOL) for persons with CF.This study examined relationships between pulmonary health and HRQOL in patients with CF who participated in the Wisconsin CF Neonatal Screening randomized clinical trial (RCT). 2 Although HRQOL Background: The objective of this study was to examine relationships between pulmonary health and health-related quality of life (HRQOL) in patients with cystic fi brosis (CF) evaluated longitudinally in the Wisconsin Newborn Screening Project. Methods: Patients aged 8 to 18 years (mean Ϯ SD, 13.5 Ϯ 2.8) in early diagnosis (n 5 45) and control (n 5 50) groups completed Cystic Fibrosis Questionnaires (CFQs) to measure HRQOL at three data points over a 2-year period. Pulmonary health was evaluated concurrently by the Wisconsin chest x-ray scoring system (WCXR) and pulmonary function tests (PFTs). Results: WCXR showed signifi cant group differences ( P Յ .023), with the early diagnosis group showing more-severe lung disease. When adjusted for group differences in mucoid Pseudomonas aeruginosa status and pancreatic status, however, WCXR differences and PFT data were not signifi cant. Most patients (74%) had FEV 1 values Ն 80% predicted (within normal range). For patients aged , 14 years, as WCXR scores worsened CFQ respiratory and physical domain scores decreased (both P Յ .007). FEV 1 /FVC showed a positive relationship with the respiratory and physical domains (both P Յ .006). WCXR scores for patients aged Ն 14 years were associated with CFQ weight, respiratory, and health domains (all P Յ .011). FEV 1 was associated with CFQ weight, respiratory, health, and physical domains (all P Յ .003). Changes in pulmonary health were not associated with changes in CFQ over time. Signifi cant group differences on the CFQ-Child social functioning domain favored the control group. Conclusions: To our knowledge, this study is the fi rst to compare pulmonary outcomes with HRQOL indicators assessed by serial, standardized, patient-reported outcome measures for patients with CF identifi ed either through newborn screening or diagnosed by use of traditional methods. This study found no benefi ts of newborn screening for pulmonary health or HRQOL after controlling for risk factors. Using WCXR and PFT data collectively helped to identify associations between pulmo...

Health-Related Quality of Life and Psychological Distress in Italian Patients With Cystic Fibrosis

“…Koscik and colleagues (29) reported a link between NBS diagnosis and improved cognitive outcomes, but failed to demonstrate any benefit for HRQoL (7). On the other hand, delayed diagnosis in the face of clinical symptoms is in itself a major source of anxiety for parents, leading to lack of trust in the medical profession and to "doctor shopping" (30). Most parents appear to be supportive of NBS, in the belief that early detection and intervention will improve the health of their children and reduce the stress of a delayed diagnosis (30,31).…”

“…On the other hand, delayed diagnosis in the face of clinical symptoms is in itself a major source of anxiety for parents, leading to lack of trust in the medical profession and to "doctor shopping" (30). Most parents appear to be supportive of NBS, in the belief that early detection and intervention will improve the health of their children and reduce the stress of a delayed diagnosis (30,31). This positive parental attitude might affect patients' beliefs and the likelihood of developing psychological distress, as suggested by Szyndler et al (22) who attributed the low levels of psychopathology found in adolescents to the large screening uptake in Australia.…”

Obesity and its Therapy: A Short Review

“…Broad information have been distributed uncovering falling apart personal satisfaction and psychosocial working as an aftereffect of a wide range of complexities [33][34][35][36][37][38]. In a meta-examination, Body Mass Index (BMI) classifications were connected with higher all-reason and reason particular mortality; peril proportions (HR) 0.94 for overweight and 1.18 for heftiness all evaluations joined [9].…”

“…33). Despite what might be expected, it was watched that there was an expanded number of postagent inconveniences in the populace that had an Operation performed (Chances Proportion 6.0571).…”

Genetic Screening

“…Newborn screening is also estimated to provide a small mortality benefit (41,42). However, a follow-up study of patients in the randomized trial of newborn screening, now between 8 and 18 years of age, showed no benefit from newborn screening for pulmonary disease or health-related quality of life (43). The evolution toward screening that offered less dramatic benefit was described by one expert as moving from ''public health emergency'' to ''public health service'' (44).…”