Sickle cell disease SCD is a serious, inherited condition affecting the blood and various organs in the body. It affects the red blood cells, causing episodes of 'sickling', which produce episodes of pain and other symptoms. In between episodes of sickling, people with SCD are normally well.
Long-term complications can occur. Good treatment, started early in life, can prevent complications. So, early diagnosis and specialist treatment are advised for SCD. Sickle cell trait is not the same as sickle cell disease. Sickle cell disease SCD is a serious group of conditions which are inherited genetic. It affects the red blood cells in the blood.
Sickle cell anaemia is the name of a specific form of SCD in which there are two sickle cell genes see below. In SCD, the red blood cells have a tendency to go out of shape and become sickle-shaped like a crescent moon - instead of their normal disc shape.
This can cause various problems - as described later. In between the episodes of illness, people with SCD feel well. Sickle cell trait is not the same as SCD or sickle cell anaemia. Sickle Hookup someone with sickle cell anemia trait means you carry a single sickle cell gene, but it does not normally cause illness. Read more about sickle cell trait. The rest of this leaflet will discuss SCD, which includes sickle cell anaemia and the other less common disorders.
It is rare in people of North European origin.
On average, 1 in 2, babies born in England have SCD, but rates are much higher in some urban areas - about 1 in in some places. The cause is inherited genetic. It is a change in the genes which tell the body how to make an important protein called haemoglobin. To get SCD, you need to have two altered haemoglobin genes, Hookup someone with sickle cell anemia from each parent.
If you only have one of these genes, you will have sickle cell trait, which is very much milder. The most common type of SCD occurs where you have two sickle cell genes sickle cell anaemia. Other types of SCD involve one sickle cell gene plus another abnormal haemoglobin gene of a different type.
Sickle cell genes affect the production of an important chemical called haemoglobin. Haemoglobin is located in red blood cells, which are part of the blood. Haemoglobin carries oxygen and gives blood its red colour. The sickle cell genes make the body produce abnormal haemoglobin called HbS. Normal haemoglobin is called HbA. HbS behaves differently from HbA. Under certain conditions, HbS makes the red blood cells change shape - instead of the normal doughnut shape, they become sickle-shaped, like a crescent moon.
This is called sickling. Conditions which trigger sickling are cold, infection, lack of fluid in the body dehydrationlow oxygen, and acid acid is produced in hard physical exercise. The sickle cells containing mostly HbS are harder and less flexible than normal red blood cells. So, they can get stuck in small blood vessels and block them. This can happen quite suddenly, causing various symptoms which are known as a sickle cell crisis explained below.
Repeated blockages can also lead to complications occurring. The sickle cells are destroyed more easily than normal red blood cells. This means that people with SCD tend to be short of red blood cells and have a moderate and persistent anaemia. moderate anaemia is not usually a problem because the HbS the different haemoglobin carries oxygen well, and the body can compensate.
However, you may get bouts of severe anaemia for various reasons. For example, if too much blood goes to the spleen, if too many red blood cells break down at the same time, or due to certain infections which stop blood cells being made. A severe anaemia can make you very ill. The diagnosis is made by a blood test. The blood sample is analysed to see what type of haemoglobin is present in the blood using a test called haemoglobin electrophoresis or other methods.