Lightning Learning: Sickle Cell Disease

Lightning Learning: Sickle Cell Disease

80% of admissions relating to sickle cell disease are also related to vaso-occlusive crises. Not only shortening life expectancy but potentially life-threatening.
— advice is based on local guidelines & procedures

What?

Sickle Cell disease is a genetic disease that causes red blood cells to become sickle-shaped. The sickle-shaped cells are more rigid and stick together causing obstruction to blood flow.

The obstruction stops oxygen reaching the tissues which is called a vaso-occlusive crisis. A crisis causes sudden severe pain often requiring strong analgesia.

Causes: infection or sepsis, hypoxia, cold weather and dehydration

Treatment: high flow oxygen, strong analgesia and fluids

Why?

80% of admissions related to sickle cell disease are related to vaso-occlusive crises. Can be life-threatening and shortens life expectancy. Contact haematology and get advice regarding transfusion.

Other crisis presentations: chest syndrome, splenic sequestration and aplastic crisis.

Complications: stroke, priapism, renal failure and can be more prone to infections due to splenic damage.

Further Reading

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