Blood travels all over the body, so Sickle Cell can affect many parts of the body:
- Brain: A stroke occurs when blood flow to the brain is blocked.
As a child you should have had a test called a Transcranial Doppler (TCD) that measures the flow of blood to the brain. This test helps predict a person’s risk of having a stroke.
- Eyes:Jaundice is a word used to describe the yellow color most easily seen in the normally white part of the eyes (sclera)
People with sickle cell have jaundice because of the breakdown of red blood cells that releases bilirubin, a yellow substance. Many people with sickle cell always have some mild jaundice because their red bloods cells are broken down much more often than people who do not have sickle cell. This jaundice is not concerning. Also, People with sickle cell should see an ophthalmologist to look for retinopathy every year starting at around age 10.
- Lungs: Acute chest syndrome (ACS) is a term used to describe a type of sickle cell crisis involving the lungs. Some doctors may use the word “pneumonia” – In general, pneumonia in a person with sickle cell disease is the same thing as acute chest syndrome.
- Heart: Pulmonary hypertension is increased pressure in the blood vessels that go to the lung for oxygen transport. This increased pressure is caused by narrowing of these blood vessels.
Pulmonary hypertension is a problem because it can cause a strain on the heart that has to pump blood.
- Spleen: Splenic sequestration is the sudden enlargement of the spleen due to trapping of red blood cells. This pooling of blood in the spleen causes problems because fewer red blood cells are able to circulate throughout the rest of the body to transport oxygen. People with splenic sequestration need to be hospitalized and may need a blood transfusion.
Functional asplenia – The spleen in individuals with sickle cell disease usually becomes damaged over time by the many abnormal red blood cells traveling through it. Usually by adolescence, the spleen in individuals with sickle cell disease is non-functional because of this damage. Functional asplenia simply means not having a working spleen.
- Gallbladder: Gallstones. People with sickle cell disease typically can develop types of gallstones called bilirubin gallstones. Bilirubin is a yellow substance made when hemoglobin in red blood cells is broken down. People with sickle cell disease have elevated bilirubin levels because of the constant break down of damaged red blood cells. This bilirubin can then collect to form gallstones. A few gallstones may not cause any problems.
If a person is having significant problems from gallstones, the treatment is surgery to remove the gallbladder (cholecystectomy).
Sickle Cell can also affect the kidney, bone, bone marrow, and the BLOOD itself due to having iron overload from frequent blood transfusions.