Updates in Pediatric Sickle Cell Lung Disease.

Sickle cell lung disease presents a challenging care paradigm involving acute and chronic lower airway disease, sleep-disordered breathing, pulmonary vascular disease, and modification by environmental factors. Understanding the presentation, pathophysiology, and diagnostic approaches is essential for accurate identification and management. While significant progress has been made, there remains a need for research to develop effective treatments and interventions to decrease disease burden in these children.

Time to be blunt: Substance use in cystic fibrosis.

Background: As the population of people with cystic fibrosis (pwCF) continues to age, attention is shifting towards addressing the unique challenges teenagers and adults face, including substance use. Changing attitudes and legality regarding marijuana and cannabidiol (CBD) may influence their use among pwCF, but data on the rate of use, reasons for use, and administration methods are lacking.

Objective: Investigate marijuana, CBD, e-cigarette, and cigarette usage among pwCF and explore differences in demographics, disease severity, and cystic fibrosis transmembrane receptor (CFTR) modulator use between recent users and nonusers.

Pregnancy outcomes in women with sickle cell disease in California.

Adverse pregnancy outcomes occur frequently in women with sickle cell disease (SCD) across the globe. In the United States, Black women experience disproportionately worse maternal health outcomes than all other racial groups. To better understand how social determinants of health impact SCD maternal morbidity, we used California's Department of Health Care Access and Information data (1991-2019) to estimate the cumulative incidence of pregnancy outcomes in Black women with and without SCD-adjusted for age, insurance status, and Distressed Community Index (DCI) scores.

Food insecurity and mental health during the COVID-19 pandemic in cystic fibrosis households.

Background: The COVID-19 pandemic impacted many households due to shelter-in-place orders and economic hardship. People with cystic fibrosis (CF) experienced increased food insecurity compared to the general population before the pandemic, even though adequate food access is needed to maintain nutrition goals associated with improved health-related outcomes. Little is known about the impact the pandemic had on the food insecurity of people with CF and their families.

Asthma in children with sickle cell disease.

Purpose Of Review: Asthma is common in children with sickle cell disease (SCD) and appears to be associated with increased morbidity. Providers caring for children with SCD have struggled with the question of whether asthma exists as a true comorbidity or whether certain aspects of the chronic inflammatory disease gives children with SCD an asthma-like phenotype.

Recent Findings: Clinical signs and symptoms seen in children with asthma in the general population, such as wheezing, airway hyperresponsiveness, atopy, elevated leukotrienes, and abnormal lung function are seen in children with SCD both with and without a diagnosis of asthma.

The Epidemiology and Management of Lung Diseases in Sickle Cell Disease: Lessons Learned from Acute and Chronic Lung Disease in Cystic Fibrosis.

Although sickle cell disease and cystic fibrosis are two of the most common monogenic diseases presenting in childhood worldwide, cystic fibrosis and sickle cell disease enjoy vastly different funding and collaborative research efforts. Pulmonary complications in cystic fibrosis have well established guidelines and multidisciplinary involvement focusing on comorbidities, routine monitoring, infectious complications, nutrition, and treatment recommendations. These guidelines can provide a framework on which to build knowledge of lung disease in sickle cell disease.

Aeroallergen sensitization predicts acute chest syndrome in children with sickle cell anaemia.

Asthma is associated with higher rates of acute chest syndrome (ACS) and vaso-occlusive pain episodes among children with sickle cell anaemia (SCA). Aeroallergen sensitization is a risk factor for asthma. We hypothesized that aeroallergen sensitization is associated with an increased incidence of hospitalizations for ACS and pain.

Sleep disordered breathing does not predict acute severe pain episodes in children with sickle cell anemia.

Conflicting evidence has suggested that low mean nocturnal hemoglobin oxygen saturation (SpO ) predicts future hospital days for acute severe pain in children with sickle cell anemia (SCA). In an unselected multicenter prospective cohort study, we tested the hypothesis that either low mean nocturnal SpO or high obstructive apnea-hypopnea index (OAHI; the number of obstructive apneas and hypopneas with ≥ 3% desaturation or arousal per hour of sleep) or high oxygen desaturation index (ODI; number of ≥ 3% desaturation from baseline saturation per hour of sleep) is associated with increased incidence rates of pain. A total of 140 children with SCA with a median age of 10.

Age is a predictor of a small decrease in lung function in children with sickle cell anemia.

The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: (1) FEV % predicted declines over time; and (2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study.

What is the role of screening for pulmonary hypertension in adults and children with sickle cell disease?

Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema.

Travelers with sickle cell disease.

Background: Sickle cell disease (SCD) is the most common genetic disease among persons with African ancestry. This article provides a background to SCD and reviews many important aspects of travel preparation in this population.

Methods: The medical literature was searched for studies on travel-associated preparedness and complications in individuals with SCD.