Health outcomes and services in children with sickle cell trait, sickle cell anemia, and normal hemoglobin

Abstract

The health effects of sickle cell trait among children are unknown. We compared select health outcomes and health services utilization among children with sickle cell trait, sickle cell anemia (SCA), and normal hemoglobin. Newborn screening records were used to identify children with sickle cell trait and SCA born in Michigan (1997-2014) who were enrolled in Michigan Medicaid for ≥1 year from 2012 to 2014. Each select health outcome (acute otitis media, acute respiratory infections, fever, invasive pneumococcal disease, pneumonia and influenza, renal complications, spleen problems, stroke) was defined as ≥1 claim with a diagnosis code for the respective outcome within a study year. Health services utilization was summarized as counts of emergency department, inpatient, and outpatient encounters. The relationship between hemoglobin status and each health outcome or utilization was assessed by logistic or negative binomial regression with generalized estimating equations. The study population consisted of 18 257 children with sickle cell trait, 368 with SCA, and 74 523 with normal hemoglobin (227 188 total person-years). Compared with those with normal hemoglobin, children with sickle cell trait had lower odds of acute otitis media (odds ratio [OR], 0.88; 95% confidence interval [CI], 0.84-0.91), acute respiratory infections (OR, 0.94; 95% CI, 0.92-0.97), pneumonia and influenza (OR, 0.93; 95% CI, 0.87-0.99), and outpatient visits (incidence rate ratio, 0.95; 95% CI, 0.93-0.97). Children with SCA had higher or nonsignificant odds of all outcomes and types of health services utilization. These results indicate that children with sickle cell trait may not be at additional health risk for these outcomes. However, additional case-control studies may be necessary to identify rare events.

COVID-19 in individuals with sickle cell disease/trait compared with other Black individuals

“…This is consistent with sickle cell trait being largely an asymptomatic disease. [16][17][18] After matching on age, sex, and other known comorbidities, there were no significant differences between the 2 groups. It is noteworthy that in our sickle cell trait cohort, the majority were female, most likely because they are tested for trait at the time of their obstetrics/gynecology care.…”

Host genetic effects in pneumonia

“…Previous studies have also reported associations between carrier status for CFTR and CF-associated symptoms. 20 In contrast, SCA carriers had slightly lower risk of pneumonia and influenza (OR = 0.93) compared to subjects with normal hemoglobin, 22 although pneumonia severity was not studied. We applied Fisher’s exact test to investigate the effect of the risk allele in heterozygous carriers of the risk allele compared to those homozygous for the reference allele.…”

“… 21 Children with SCA are more likely to have pneumonia and influenza (OR = 7.38) and acute respiratory infections (OR = 1.29). 22 Acute chest syndrome (ACS) is a common complication of SCD and can be triggered by pneumonia and vaso-occlusive crises and is the leading cause of death in individuals with SCA. 23 Clinically differentiating between ACS and pneumonia can be difficult, and they often overlap in the EHR.…”

COVID-19 Vaccine Perception and Hesitancy Among Patients With Sickle Cell Disease in the Western Region of Saudi Arabia

“…Throughout the COVID-19 pandemic, patients with SCD were thought of as "high risk" due to impaired immunity, resulting from hyposplenism and systemic vasculopathy, which gives them the tendency for endorgan dysfunction and high risk for thrombosis [5,7]. In addition, previous studies have reported their vulnerability to acute viral illnesses [8] and subsequent exacerbation of vaso-occlusive crises and acute chest syndromes [9].…”