Sickle cell disease (SCD) is an inherited disorder that causes red blood cells to take the shape of a sickle or the letter “C.” Levels of hemoglobin, a protein that helps carry oxygen to red blood cells, are usually low as well.
Red blood cells
low hemoglobin subscriber In people with sickle cell anemia it can cause anemia. Sickle cell anemia is a form of sickle cell anemia.
Read on to learn more about how sickle cell anemia affects the structure and levels of hemoglobin, as well as how doctors diagnose and treat the disease.
Hemoglobin levels in people with sickle cell anemia are: usually It is in the range of 6-11 grams per deciliter (g/dL), while it is about 12 mg/dL in people without sickle cell disease.
Low levels of hemoglobin can make it more difficult for red blood cells to carry oxygen throughout the body. This can cause a wide range of symptoms, including pain and fatigue. Over time, SCD
In addition to causing low hemoglobin levels, sickle cell disease alters hemoglobin, causing cells to rupture. These cells die early.
Sickle cells are also more likely to stick together, which can increase the risk of dangerous blood clots and pulmonary embolism.
Does sickle cell trait affect hemoglobin levels?
When a person has the sickle cell trait, it means that they carry one sickle cell gene, not two genes. Sickle cell trait is usually a benign condition that does not affect health or hemoglobin levels.
Because people with sickle cell trait have only one gene that affects hemoglobin, they usually have enough normal hemoglobin to prevent them from developing symptoms.
Can physical activity affect hemoglobin levels?
When a person experiences extreme physical stress — such as when they are at a height or when an untrained person works very intensely — some of the red blood cells may sickle.
As a result, a person may develop a life-threatening complication called rhabdomyolysis, which destroys muscle, causing it to break down and release harmful substances into the blood.
In sickle cell anemia, the hemoglobin protein is abnormal. a
The HBB The gene responsible for the formation of beta-globin chains, which are a component of hemoglobin.
premature death of red blood cells
Changes in the composition of hemoglobin
Hemoglobin is made up of four protein subunits: two alpha globin subunits and two beta globin subunits.
In people with sickle cell anemia, certain mutations are present within HBB The gene can change the structure of hemoglobin. Specifically, the mutations encode for an abnormal version of beta globin, or HbS. This may occur in only one of the two beta globin subunits.
However, in sickle cell anemia, both beta-globin subunits of hemoglobin are replaced by abnormal HbS. This causes the red blood cells to deform and take the sickle shape.
In other types of sickle cell disease, the hemoglobin subunits may be replaced by other types of abnormal hemoglobin, such as HbC. These can also change the shape of red blood cells.
The type of sickle cell disease a person has will affect their symptoms. The genes a person inherits determine the type of sickle cell disease they develop.
The most common type of sickle cell disease
- Hemoglobin SS Homozygous: The most common form of SCD, this variant means a person inherits two copies of the sickle ‘S’ gene – one from each parent. This is the most severe manifestation of sickle cell disease. Doctors call it sickle cell anemia.
- Hemoglobin sickle cell disease: People with this milder form of sickle cell disease inherit the sickle “S” gene and the sickle “C” gene, another type of abnormal hemoglobin gene.
- Sickle cell beta thalassemia: This version of sickle cell disease means the person gets sickle protein “S” and one gene for beta thalassemia, another type of abnormal hemoglobin. There are two subtypes: beta 0 thalassemia, which is a more severe condition, and beta + thalassemia, which tends to be milder.
People with access to medical care
If a person does not have a newborn screening test or does not live in a country that provides this type of screening, they can undergo blood tests as an adult to detect SCD.
Some lab tests can separate hemoglobin to look for and identify different variants from blood samples. These tests include:
- Hemoglobin electrophoresis: This technique can identify different types of hemoglobin.
- High quality color separator liquid: This technology also detects most types of hemoglobin.
- Photoelectric focus: This is a very sensitive method of detection.
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Your doctor may recommend lifestyle management strategies to prevent some of the complications associated with the condition. These strategies may include:
- Drink a lot of water
- Wash your hands often to reduce infection risk
- Avoid high altitudes
Pain medications can help people with sickle cell anemia manage episodes of pain or crises. During a sickle cell crisis, a person may need to stay in the hospital. If the red blood cell count and hemoglobin are severely reduced, a person may need a blood transfusion.
Sickle heart disease can cause many complications and may shorten a person’s life, especially if they do not have access to proper medical care. Possible complications include:
In sickle cell anemia, a person’s hemoglobin levels are chronically low. This can cause several complications. However, the severity of complications varies from person to person and is highly dependent on the type of sickle cell disease a person has.
It is important for all people with sickle cell anemia to get good medical care, maintain a healthy lifestyle, and know the signs of an emergency so that they can seek immediate care when necessary.