Sickle cell disease is a group of blood disorders in which the body produces abnormally shaped red blood cells. It is a genetic disease that is inherited from parents. If your parents each pass one sickle cell gene to you, then you will have the disease. If you are born with one sickle cell gene then you have the trait, and will usually not have any symptoms of the disease.
People with this disease have problems with the haemoglobin in their blood cells. Haemoglobin is a protein found in red blood cells which carries oxygen in the body. When a person has abnormal haemoglobin, the red cells assume an abnormal sickle shape instead of their regular disc-like shape. This abnormal sickle-shaped cells are not flexible and can stick to the walls of the blood vessels thus interrupting blood flow to organs in the body. This leads to many of the symptoms and complications seen in the disease.
Possible Complications Of Sickle Cell Disease
- Chronic anaemia: This means a shortage of red blood cells or haemoglobin. Normal red cells have a lifespan of about 90-120 days while sickle-shaped cells have a lifespan of 10-20 days. This means the body has to work extra hard to replace the lost red blood cells. This means the body has to work extra hard to replace the lost red blood cells and this leads to anaemia.
- Bone complications: Some portions of the bone could die due to blockage of blood supply by the sickle cells to those areas. This is known as avascular or aseptic necrosis and it usually occurs in long bones like the femur of the leg.
- Splenic manifestations: Sometimes, a large number of sickle cells accumulate in the spleen. This is called splenic sequestration. The spleen gets very large, but this doesn’t mean it functions better. Eventually, repeated accumulation of these cells cause the spleen to shrink in size until it eventually loses its function. This is called autosplenectomy The spleen plays a special role in protecting the body against microorganisms, therefore people with sickle cell disease are prone to coming down with many infections especially bacterial infections.
- Pain: Many patients with sickle cell have chronic low-level pain.
- Infections: Serious infections can occur in people with sickle cell disease. Common infections include infections of the lungs, kidneys, bladder, bone. Meningitis could also occur.
- Leg Ulcers: Chronic leg ulcers that do not heal can also occur in this people with this disease.
- Stroke This is one of the most feared complications. Here, the sickle cells block blood supply to the brain, thus leading to a stroke which can be fatal.
- Kidney problems: Including chronic kidney disease which can progress to end-stage kidney disease is a feared complication.
- Eye problems: Problems with vision and even loss of vision could occur.
- Priapism: This a prolonged painful erection of the penis caused by sickle cells blocking the blood vessels in the penis. This could lead to permanent erectile dysfunction
- Depression: Low mood and other psychological problems such as anxiety can also be present.
In conclusion, sickle cell disease is a lifelong disorder, and long-term follow up is necessary to prevent these complications from coming up.