Protecting the brain in sickle cell anemia: the value of beginning early in life

A recently published article in Experimental Biology and Medicine (Volume 246, Issue 23, December, 2021) highlights the importance of early-in-life screening for brain damage in children with sickle cell.  The study, led by Dr. Jane Hankins in the Department of Hematology at St. Jude Children's Research Hospital (SJCRH) in Memphis, along with colleagues at SJCRH, the University of Tennessee Health Science Center and Washington University (USA), reports that children with sickle cell anemia which present with alterations in MRI/MRA imaging at a young age are more likely to have new or worsened brain lesions as young adults.

Brain lesions among children living with sickle cell anemia is a prevalent problem with serious consequences later in life. In addition to the risk of overt stroke, which can be devastating for a child with sickle cell anemia, micro (silent) infarcts and vessel stenoses (vessel narrowing) can occur and accumulate over time. Lesions to the brain impair the brain's function, namely, the person's cognitive (or thinking) abilities, limiting their academic success and future earning potentials. Treatments to prevent or reduce the impact of brain damage in individuals with sickle cell anemia have the potential to mitigate the effects of brain damage, but have not been adequately studied, nor have they been described in real-world settings, outside of the very controlled (and artificial) environment of clinical trials.

Dr. Hankins and colleagues performed one of the longest observational studies of children with sickle cell anemia. Children were followed for up to 25 years, and their brains monitored for brain and vessel damage in young adulthood. From childhood to young adulthood, approximately one-fifth of these children had new or worsened brain lesions. Among those patients whose brain lesions were present since childhood (approximately 30%), brain damage progressed with more micro infarcts and vessel narrowing more frequently (up to 11 times more likely) compared with those whose lesions appeared after childhood. However, the development of brain lesions seemed to attenuate after adolescent years, a finding that suggests a possible protective role of the treatments these children had received since early in life, including hydroxyurea and monthly blood transfusions. Dr. Hankins said, "Brain damage among individuals with sickle cell anemia causes grave consequences, therefore more efforts are needed to fully understand, mitigate and prevent them from occurring in first place. Although the treatments these children received (hydroxyurea and monthly blood transfusions) might have attenuated the further development of brain damage, they did not fully prevent it, therefore alternative treatments must be investigated and considered, including curative ones, such as hematopoietic cell transplant and gene therapies, to fully protect these children's brains."

Dr. Steven R. Goodman, Editor-in-Chief of Experimental Biology and Medicine, said, "Hankins and colleagues have performed a long longitudinal follow-up made possible through a strong collaboration between those taking care of children and young adults with sickle cell disease in Memphis, Tennessee. Their important findings argue for early and frequent imaging of potential brain lesions, along with therapies to slow or eliminate progression in those most effected with central nervous system damage."

Experimental Biology and Medicine is a global journal dedicated to the publication of multidisciplinary and interdisciplinary research in the biomedical sciences. The journal was first established in 1903. Experimental Biology and Medicine is the journal of the Society of Experimental Biology and Medicine. To learn about the benefits of society membership, visit www.sebm.org. If you are interested in publishing in the journal, please visit http://ebm.sagepub.com.

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Source: Experimental Biology and Medicine

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Categories: Medical Research

Tags: brain, EBM, sickle cell


About Experimental Biology and Medicine

Experimental Biology and Medicine is a journal dedicated to the publication of multidisciplinary and interdisciplinary research in the biomedical sciences. The journal was first established in 1903.

Danna B. Zimmer
Assistant to the Editor-in-Cheif, Experimental Biology and Medicine