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A NEW HOPE: The techniques could improve the survival rate of sickle cell patients

COMPUTER MODELS developed by researchers in the United States have given new insights into understanding sickle cell anaemia. And health experts have hailed providing fresh hope that life-changing medication can be developed, which will provide a brighter future for thousands of people living with the condition.

In the UK, the condition mainly affects people of African and Caribbean heritage. Sickle cell disease produces unusually shaped red blood cells that can cause problems because they don’t live as long as healthy blood cells and they can become stuck in blood vessels.

For those living with the condition it can cause painful episodes called sickle cell crises, which can be very severe and can last up to a week. There is also an increased risk of serious infections, anaemia (where red blood cells can’t carry enough oxygen around the body), which in turn can cause tiredness and shortness of breath.

Mathematicians at Rhode Island’s Brown University – or Brown’s – created computer simulations of how sickle cell disease manifests inside red blood cells affected by the condition.

Sickle cell disease affects hemoglobin, molecules within red blood cells responsible for transporting oxygen. In normal red blood cells, hemoglobin is dispersed evenly throughout the cell. In sickle red blood cells, mutated hemoglobin can cause a chemical reaction when deprived of oxygen.

They assemble themselves into long polymer fibres that push against the membranes of the cells, forcing them out of shape. The stiff, ill-shaped cells can become lodged in small capillaries throughout the body, leading to painful episodes known as sickle cell crisis.

SIMULATE

The model uses detailed biomechanical data on how sickle hemoglobin molecules behave and bind with each other to simulate the assembly of a polymer fibre. Using the new computer simulations called MARS (mesoscopic adaptive resolution scheme), researchers
at Brown’s were able to show how different configurations of growing polymer fibres are able to produce cells with different shapes.

Though the disease gets its name because it causes many red blood cells to take on a sickle-like shape, the new technique shows a variety of abnormal cell shapes present. The study’s lead author Lu Lu, a research student at Brown’s said: “The goal of our work is to model both how these sickle hemoglobin fibres form as well as the mechanical properties of those fibres.

PICTURED: Sickle Cell Society members

“There had been separate models for each of these things individually developed by us, but this brings those together into one comprehensive model.” Karniadakis, who leads the Brown’s team, said: “The models give us a way to do preliminary testing on new approaches to stopping this disease.

“Now that we can simulate the entire polymerization process, we think the models will be much more useful.” The researchers said they believed that their research, published in the Biophysical Journal will help in pro- viding better targeted therapies to fight sickle cell disease.

The work has been enthusiastically welcomed by sickle cell campaigners. Kehinde Salami, founder and managing director of Sicklekan, a charity which advocates awareness of sickle cell believes the research is pioneering.

He said: “The research is great, because for the first time it is addressing the possibility of reversing the sickle caused by the disease. I’m hopeful that drug strategies developed by the new computers will not be restricted to sufferers in the West, but will be shared quickly in Africa and the Caribbean.”

While welcoming the new developments, Salami believes that sufferers need to be more vocal in discussing how the disease affects them. Through Sicklekan, he visits schools and colleges to heighten this awareness.

He said: “Sickle cell sufferers like myself need to do their part in educating others about their predicament, which can have the effect of mobilis- ing support from the community and medical profession.”

ENCOURAGED

John James, CEO of Sickle Cell Society, which represents and supports people living with the condition, is also encouraged by the Brown University research. He said: “Any research that sheds light on a possible cure for sickle cell is welcomed. The good news is there are a growing number of clinical trials focusing on new curative treatments, rather than just pain management.

“For a condition which the medical profession has been aware of for over a 100 years and which afflicts a minimum of 15,000 persons in the UK, the bulk of whom are BAME persons, there is room for improvement.”

However, he cautioned: “What sufferers need to be aware of, however, is that any new developments from the US will take time to receive approval in the UK.”

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