Background

Cystic fibrosis (CF) is one of the most common recessively inherited disorders diagnosed in early childhood in the United States. Determining the phenotype of CF patients likely to experience a significant drop in FEV1% predicted will help target efforts for mitigating this deleterious disorder.

Methods

This retrospective cohort study evaluated potential risk variables that account for the decline in FEV1% predicted in 81 CF patients treated at Miller Children's and Women's Hospital, CA. Cystic fibrosis risk of disease progression (CF RD-Pro) score was evaluated as a tool to identify high-risk patients for accelerated disease progression (event = drop in FEV1% predicted ≥10 percentage points) based on risk variables identified as significant.

Results

ROC analysis determined classification of high versus low-moderate risk of FEV1% decline during year two based on RD-Pro score. Scores ≥2 applied as threshold for high-risk revealed relatively good validity estimates: sensitivity =82.8%, specificity =66.7%, PVP =77.4%, PVN =73.7%, and correct classification =76%. Patients with CF RD-Pro scores suggestive of high (≥2 points) vs. low-moderate (<2 points) risk were nearly 10 times more likely to experience significant disease progression (OR 9.6, 95% CI, 2.6-36.1, P=0.001).

Conclusions

Identification of patients at high risk for significant decline in lung function will enable address of potential therapeutic modalities, environmental exposures, and behavioral variants that may improve outcomes in these patients. The power of the CF RD-Pro Score lies in its simplicity. Our study provides a novel readily available score, which incorporates body mass index (BMI) and Staphylococcus aureus infection, both being alterable targets for slowing CF progression.

Purpose

The pulmonary complications of sickle cell disease (SCD) are a leading cause of morbidity and mortality (MacLean et al. Am J Respir Crit Care Med 178:1055-1059, 2008; Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; National Heart, Lung, and Blood Institute, 2009). Despite this recognition, predictive markers of lung dysfunction progression remain elusive (Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; Platt et al. N Engl J Med 330:1639-1644, 1994; Caboot et al. Curr Opin Pediatr 20:279-287, 2008; Field et al. Am J Hematol 83:574-576, 2008; Shirlo et al. Peadiatr Respir Review 12:78-82, 2011). This study was designed describe the longitudinal progression and identify specific markers that influence bronchial disease in SCD.

Methods

A retrospective, chart review of 89 patients with SCD was conducted. All patients underwent spirometry in conjunction with body plethysmography as part of routine care. Eleven lung function variables were assessed, five of which were selected to establish patterns of normal, obstructive, restrictive, or mixed obstructive-restrictive physiology (Klings et al. Am J Respir Crit Care Med 173:1264-1269, 2006; Field et al. Am J Hematol 83:574-576, 2008).

Results

In the unadjusted model, forced expiratory volume in one second (FEV1)% of predicted trended downward with age, while total lung capacity (TLC)% of predicted showed a bimodal distribution and carbon monoxide diffusion capacity corrected for hemoglobin (DLCOcor)% of predicted remained stable. Adjusting for acute chest syndrome (ACS) episodes, medication status, and growth velocity (GV), the final model demonstrated that the downward trend between FEV1% of predicted with age was further influenced by the latter two factors.

Conclusions

Initial decline in FEV1% of predicted is associated with worsening pulmonary dysfunction over time. Independent of ACS episodes, the factors most influential on the progression of FEV1% predicted include the introduction of medications as well as the promotion of adequate prepubertal growth. Efforts to ensure normal prepubertal GV and treatment with bronchodilators, such as short-acting beta(2) agonists and inhaled corticosteroids (ICS), should be considered at an early age to delay progression of pulmonary dysfunction.

Background

COVID-19 pandemic introduced significant challenges that may have exacerbated healthcare worker (HCW) burnout. To date, assessments of burnout during COVID-19 pandemic have been cross-sectional, limiting our understanding of changes in burnout. This longitudinal study assessed change across time in pediatric HCW burnout during the COVID-19 pandemic and whether demographic and psychological factors were associated with changes in burnout.

Methods

This longitudinal study included 162 physicians, physician assistants, nurses, and medical technicians within the emergency department (ED), intensive care, perioperative, and inter-hospital transport services in a children's hospital. HCW demographics, anxiety and personality traits were reported via validated measures. HCWs completed the Maslach Burnout Inventory in April 2020 and March 2021. Data were analyzed using generalized estimating equations.

Results

The percentage of HCWs reporting high emotional exhaustion and/or depersonalization burnout increased significantly across time (18.5% to 28.4%, P = 0.010). Factors associated with increased emotional exhaustion included working in the ED (P = 0.011) or perioperative department (P < 0.001), being a nurse or medical technician (P's < 0.001), not having children (P < 0.001), and low conscientiousness (P < 0.001).

Conclusions

Pediatric HCW burnout significantly increased over 11-months of the COVID-19 pandemic. Results suggest that certain demographic and psychological factors may represent potential area to target for intervention for future pandemics.

Impact

This longitudinal study revealed that the COVID-19 pandemic has had a significant impact on pediatric healthcare worker burnout. The percentage of healthcare workers reporting high levels of emotional exhaustion and depersonalization burnout increased significantly over 11-months of the COVID-19 pandemic. Results suggest that certain demographic and psychological factors may represent potential targets for future interventions.

This study examined the frequency and type of complementary and alternative medicine (CAM) therapy, as well as characteristics associated with CAM usage. A survey about parental preferences and beliefs regarding CAM usage was distributed to 5 schools with predominantly low socioeconomic Vietnamese children. For the 360 Vietnamese children previously diagnosed with asthma whose families responded, most preferred conventional therapy as prescribed by the physician. The prevalence of CAM usage was 38.1%. Common CAM therapies were steam inhalation, creams/topical oils, foods, prayer, oil inhalation, massage, herbal medication, coining, and cupping. Significant predictors of CAM usage were older age (11-12 years) (P = .038), English language of survey response (P = .001), environmental tobacco smoke exposure (P = .001), fear of long-term medication usage, and perception of asthma as a condition related to genetics (P = .023). These findings suggest that assessing CAM therapy will provide a more holistic approach to asthma therapy.