A holistic perspective on sickle cell disease

Sickle cell disease is caused by a mutation in the beta globin gene that results in misshapen red blood cells. These cells can impact the flow of blood and result in problems with circulation, which are well known and treatable with varying levels of efficacy. However, the resulting stress caused by circulation issues can cause additional problems in nearly every organ in the body.

Palomarez et al. examined the cardiovascular complications of sickle cell disease and how they develop from the stress applied on blood vessels. They also described methods of detecting and treating these complications.

Sickle red blood cells differ from healthy cells not just in shape, but also in mechanical properties. Changes to adhesiveness, density, and viscosity can affect the cardiovascular system in different ways. Cells can clump and trigger vaso-occlusion crises, and the additional stress can lead to heart disease and heart failure.

While sickle cell disease therapies show promise, many of these secondary conditions can cause a lifetime of issues for patients. The authors highlighted potential advances in microfluidics and personalized medicine that could target and effectively alleviate symptoms.

The team hopes to motivate researchers to think more broadly about sickle cell symptoms and treatments to benefit patients suffering from the disease.

“We want to look at sickle cell disease a little bit differently,” said author Renita Horton. “Not just from the red blood cell standpoint, but also from a systemic disease standpoint. If we can put all of these pieces together, we could improve the overall patient outcome for individuals living with sickle cell disease.”

Source: “The cardiovascular consequences of sickle cell disease,” by Alexis Palomarez, Manisha Jha, Ximena Medina Romero, and Renita Horton, Biophysics Reviews (2022). The article can be accessed at https://doi.org/10.1063/5.0094650 .