Article
Pediatrics
Marianne E. Yee, Kristina W. Lai, Nitya Bakshi, Joanna K. Grossman, Preeti Jaggi, Alexander Mallis, Yun F. Wang, Robert C. Jerris, Peter A. Lane, Inci Yildirim
Summary: This retrospective cohort study examined children with SCD who had blood cultures collected from 2010 to 2019, and found that BSI remains a risk for these children with an incidence rate of 0.89 per 100 person-years. Sickle cell anemia genotypes and chronic transfusions were associated with higher odds of BSI, while hydroxyurea was associated with lower odds of BSI.
Article
Medicine, General & Internal
Ivie C. Egiebor, Karl J. McCleary, Jim E. Banta, Ronald Mataya, Wendy Shih
Summary: The study aims to identify multi-level barriers to medication adherence among adults with Sickle Cell Disease (SCD) and examine the relationship between these barriers and medication adherence levels. The study found that barriers at the community, institutional, and individual levels can impact medication adherence. Depression severity and patient concerns about medication were negatively correlated with adherence. Patients with fewer barriers tend to have higher medication adherence.
Article
Oncology
Susan E. Creary, Chase Beeman, Joseph Stanek, Kathryn King, Patrick T. McGann, Sarah H. O'Brien, Robert Liem, Jane Holl, Sherif M. Badawy
Summary: The study evaluates the relationship between hydroxyurea exposure, defined by average prescribed dose and adherence, and hematologic parameters, finding that higher exposure is associated with better hematologic parameters.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Genetics & Heredity
Marsha J. Treadwell, Lisa Du, Neha Bhasin, Anne M. Marsh, Theodore Wun, M. A. Bender, Trisha E. Wong, Nicole Crook, Jong H. Chung, Shannon Norman, Nicolas Camilo, Judith Cavazos, Diane Nugent
Summary: Sickle cell disease affects a significant number of people, primarily Blacks/African-Americans, and an FDA-approved medication called hydroxyurea is underutilized for its treatment. This study gathered perspectives from providers, individuals with SCD, and families, and found that over half of eligible patients reported using hydroxyurea. However, older adults and those facing barriers were less likely to be on the medication. Unintentional barriers such as forgetting and intentional barriers like worrying about side effects were identified. Additionally, providers were less likely to prescribe hydroxyurea for certain diagnoses. These findings highlight the importance of addressing barriers and promoting shared decision making in SCD treatment.
FRONTIERS IN GENETICS
(2022)
Article
Hematology
V. Munaretto, P. Corti, E. Bertoni, S. I. Tripodi, M. E. Guerzoni, S. Cesaro, F. Arcioni, C. Piccolo, T. Mina, M. Zecca, D. Cuzzubbo, M. Casale, G. Palazzi, L. D. Notarangelo, N. Masera, P. Samperi, S. Perrotta, G. Russo, L. Sainati, R. Colombatti
Summary: Acute chest syndrome (ACS) is a common reason for hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings lack knowledge about ACS best practices. A retrospective study in Italy showed that standardized management improved diagnostic and therapeutic pathways of ACS in children, but discrepancies were found between reference centers and general hospitals.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Health Care Sciences & Services
Ravindra Kumar, Aparup Das
Summary: Sickle cell disease is a chronic genetic disease that requires lifelong therapy and monitoring. Mobile health apps are being tested for their potential in increasing drug adherence in patients with SCD. In the Indian context, these mHealth apps may have applicability and feasibility for the management of SCD.
JMIR MHEALTH AND UHEALTH
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Olurotimi O. Komolafe, Victor A. Adetiloye, Oluwagbemiga O. Ayoola, Olufemi Adefehinti, Chidiogo Onwuka
Summary: This study found abnormal peripheral hemodynamic characteristics in the lower limbs of children with sickle cell anemia, but no evidence of occlusive peripheral artery disease.
Article
Immunology
Hannah K. Peng, Kevin J. Dombkowski, Gary L. Freed, Susan E. Creary, Dominic Smith, Sarah L. Reeves
Summary: Children with sickle cell disease (SCD) had higher annual flu immunization rates than those without SCD, but over 50% remained unimmunized. Among children with SCD, there were no significant differences in immunization rates by age; however, adolescents aged 13-17 without SCD were less likely to receive annual flu immunization compared to children aged 6-35 months.
Article
Hematology
Chibuzo J. J. Aguwa, Alicia D. D. Cannon, James F. F. Casella, Bruce K. K. Shapiro, Eboni I. I. Lance
Summary: Despite the availability of developmental screening guidelines, children with sickle cell disease (SCD) are not receiving regular and appropriate screening for neurodevelopmental disorders (NDDs). Many children are screened outside the recommended ages, and there is a lack of autism-specific screening.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Genetics & Heredity
Beatrice E. Gee, Andrea Pearson, Iris Buchanan-Perry, Roger P. Simon, David R. Archer, Robert Meller
Summary: Whole transcriptome RNA-sequencing revealed differential expression of 223 genes and differential transcript expression of 441 genes in whole blood samples from sickle cell anemia (SCA) patients compared to controls. The differentially expressed RNA were enriched for genes associated with hemoglobin and ubiquitin-proteasome pathway. Further analysis showed higher expression of gamma globin genes in SCA patients, and identified non-coding RNAs associated with HBG1 and HBG2 mRNA levels. These findings suggest novel regulatory mechanisms for fetal hemoglobin regulation in SCA.
FRONTIERS IN GENETICS
(2022)
Article
Pediatrics
Khalid I. Elsayh, Khaled Saad, Helal F. Hetta, Mervat A. M. Youssef, Mostafa M. Embaby, Ismail L. Mohamed, Safwat M. Abdel-Aziz, Zeinab Albadry M. Zahran, Amira Elhoufey, Aliaa M. A. Ghandour, Asmaa M. Zahran
Summary: Hydroxyurea treatment can restore the immune parameters in children with sickle cell anemia, including increased CD3+, CD4+, Th1, and CD8+ T cells and decreased NK, Th2, and Tc2 cells, resulting in an increase in the Th1/Th2 and Tc1/Tc2 ratios.
PEDIATRIC RESEARCH
(2023)
Article
Hematology
Wally R. Smith, Donna K. McClish, Richard Lottenberg, India Y. Sisler, Daniel Sop, Shirley Johnson, Anthony Villella, Darla Liles, Elizabeth Yang, Ian Chen
Summary: Patients eligible for hydroxyurea treatment who were exposed to patient navigators showed improvements in starting hydroxyurea treatment and adherence, but there were no significant differences in hematological parameters between the experimental and control groups at 12 months.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Pediatrics
Julianne E. Burns, Cary Thurm, James W. Antoon, Carlos G. Grijalva, Matt Hall, Adam L. Hersh, Gabrielle Z. Hester, Emilie Korn, Mario A. Reyes, Samir S. Shah, Balagangadhar R. Totapally, Ronald J. Teufel
Summary: During the COVID-19 pandemic, treatment guidelines for children with coronavirus disease 2019 (COVID-19) have evolved rapidly. The December 2020 guideline from the Infectious Diseases Society of America (IDSA) recommended the use of steroids for critical disease and suggested steroids and remdesivir for severe disease. This study evaluated the changes in medication use for children hospitalized with COVID-19 after the publication of the guideline. The findings showed a decline in the uptake and incomplete adherence to guideline-directed therapies for children with severe and critical disease.
Editorial Material
Multidisciplinary Sciences
Anna Nowogrodzki
Summary: The United States has made progress in reducing child mortality, but adults have been neglected in this regard.
Article
Nutrition & Dietetics
Chinenye R. R. Dike, Jeffrey Lebensburger, Ciara Mitchell, Betty Darnell, Casey D. D. Morrow, Wendy Demark-Wahnefried
Summary: This study tested the acceptability of flaxseed in children with sickle cell disease. The results showed that flaxseed baked in brownies and cookies, as well as ground flaxseed added to yogurt, were the most popular. More than 80% of participants expressed willingness to participate in a follow-up study to evaluate the effectiveness of a flaxseed-supplemented diet in mitigating sickle cell disease-associated pain.
