What is sickle cell disease?
Sickle cell disease is a disorder that affects the red blood cells, which use a protein called hemoglobin to transport oxygen from the lungs to the rest of the body. The most common type of sickle cell disease is sickle cell anemia.
In most people, red blood cells are round and smooth. This round and smooth shape allows the cells to move easily through the blood vessels. People with sickle cell disease have abnormal hemoglobin, which is a protein found in the red blood cells.
The sickle cell hemoglobin can cause the red blood cells to change shapes, from the normal round shape into a crescent (or sickle) shape. The sickle shaped red blood cells are hard and sticky and do not pass well through blood vessels. As a result, blood vessels can sometimes become clogged, preventing blood from flowing through them. A clogged blood vessel is called an “occlusion.” Occlusions in blood vessels can lead to a painful sickle cell crisis.
Both children and adults who have sickle cell anemia have a hard time fighting infections. This is because sickle cell anemia can damage the spleen, an organ that helps fight infections. Infants and young children who have damaged spleens are more likely to get infections that can kill them within hours or days.
Pneumonia is the most common cause of death in young children who have sickle cell anemia. Meningitis, influenza and hepatitis are other infections that are common in people who have sickle cell anemia. Therefore, we must administer IV antibiotics and other drugs for hepatitis and/or meningitis when ordered.
Two forms of stroke can occur in people who have sickle cell anemia. One form occurs when a blood vessel in the brain is blocked. The other form occurs when a blood vessel in the brain bursts. A stroke can cause learning disabilities and/or lasting brain damage, long-term disability, paralysis or death. As with any stroke, nurses must carefully monitor all vital signs on a regular basis and a head-to-toe assessment must be completed daily.
While there is no cure for sickle cell disease, there are precautions for preventing painful sickle cell crises and treatments to provide relief during pain crises.
Please note that Someone who inherits only one sickle cell gene and a normal gene from the other parent will have the sickle cell trait, but not the disease