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Sickle cell anaemia is a serious inherited blood disorder where the red blood cells, which carry oxygen around the body, develop abnormally.
The disorder mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. Normal red blood cells are flexible and disc-shaped, but in sickle cell anaemia they can can become rigid and shaped like a crescent (or sickle).
The sickle-shaped cells contain defective haemoglobin, the iron-rich protein that enables red blood cells to carry oxygen from your lungs to the rest of the body.
The abnormal cells are also unable to move around as easily as normal shaped cells and can block blood vessels, resulting in tissue and organ damage and episodes of severe pain.
Such episodes are known as a sickle cell crisis or a vaso-occlusive crisis. They can last from a few minutes to several months, although on average most last five to seven days.
The abnormal blood cells also have a shorter lifespan and aren’t replaced as quickly as normal blood cells. This leads to a shortage of red blood cells, known as anaemia.
Anemia – It is where the amount of haemoglobin in the blood is lower than normal. Sickle cell are delicate and break easily, leaving one’s body without enough supply of red blood cells. Normal red blood cells live for up to 120 days but sickle cell live up to 20 days, vast difference. The result is a shortage of red blood cell and hence sicklers feeling tired.
Pain – I would call it excruciating pain; this is as a result of sickle-shaped red blood cells block blood flow through tiny blood vessels to parts of your body. Pain can also occur in your bones and joints. The pain may vary in intensity and can last for a few hours to a few weeks. This is called having a vaso occlusive crisis.
SOME COMPLICATIONS:
Infection – Sickle Cell damages the spleen and other organs that fight infection and hence your being prescribed the antibiotic Penicillin for life. Sicklers are more susceptible to infections.
Hand-Foot Syndrome – this is when you hands and foot are swollen with pain and tenderness. We have all had it as sicklers.
Acute Chest Syndrome – a lung related complication with sicklers; it lowers the level of oxygen in the blood and can be life-threatening. If you are coughing, you have chest pain, you are short of breath or feeling feverish, then do go and see your doctor.
Splenic Sequestration – Splenic sequestration happens when a lot of sickled red blood cells become trapped in the spleen. The spleen can enlarge, get damaged, and not work as it should. When the spleen doesn’t work well, a person is more likely to have serious, life-threatening infections with certain types of bacteria.
Vision Loss – Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images.
Leg Ulcers – It is a painful, open sore that develops on the lower leg, usually close to the ankle. Leg ulcers often develop after a skin infection or injury, such as a cut or puncture wound. People with Sickle Cell have a heightened risk of developing leg ulcers.
References: from the mayo clinic website, the nhs website and the webmd website.
