Sickle Cell Crises (Overview)

Sickle cell disease is an inherited hemoglobin disorder in the red blood cells. It is passed down from both parents.

Acute painful sickle cell events, also known as painful crises or vaso-occlusive episodes (VOEs), are painful episodes that occur when sickled red blood cells block small blood vessels.

In sickle cell disease, red blood cells are abnormally shaped and rigid, resembling a crescent or sickle. Normal red blood cells are disc-shaped and flexible, but in sickle cell disease, they are distorted and rigid.

Their red blood cells become deformed, turning into sickle-shaped. Sickle cells die sooner than healthy cells. Normal red blood cells live about 90 to 120 days, but sickled cells only last 10 to 20 days. The bone marrow tries to make more red blood cells to makeup with the loss but can’t keep up, causing anemia. A shortage of red blood cells (RBCs) is called anemia, and people with anemia often feel breathless. Their new shapes keep them from moving properly through the body. and the shapes themselves can jam up and stick to the walls of the blood vessels. These clogs cut off the oxygen supply to these tissues, delaying a child’s normal growth and causing fatigue and extreme pain.

Sickle cell disease is a health condition caused by a gene mutation that affects the hemoglobin molecule in red blood cells, causing it to form into stiff rods.

Sickle cell disease can cause a range of serious health complications, including:

• Painful episodes, called pain crises, that can last several hours or days
• Chronic pain
• Stroke
• Lung problems
• Eye problems
• Infections
• Kidney disease 
• Stroke

A simple blood test will tell whether you have sickle cell trait or the disorder.  Sickle Cell Society estimates that 17,500 people in the UK have sickle cell disorder.

Sickle cell disease is a lifelong illness, but with the right care, many people with the disease can live fulfilling lives. Treatments include fluids, painkillers, antibiotics, and transfusions. 

Children with SCD are at increased risk for stroke, the risk is highest between the ages of 2 and 16.

Treatments include fluids, painkillers, antibiotics, and transfusions. In rare cases, bone marrow transplants may be advised.