What to know about sickle cell anemia and hemoglobin

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Sickle cell disease (SCD) is a genetic condition that causes red blood cells to take on the shape of a sickle or the letter “C”. Levels of hemoglobin, a protein that helps carry oxygen to red blood cells, are also generally low.

red blood cells are typically round. When a person has SCD, abnormal types of hemoglobin cause their red blood cells to change in shape. This change makes it harder for red blood cells to move around the body and carry oxygen throughout the body. Reduced oxygen and blood flow to vital organs, such as the kidneys, can cause pain and even life-threatening illness.

Low hemoglobin is common in people with sickle cell disease and can cause anemia. Sickle cell disease is a form of SCD.

Read on to learn more about how sickle cell anemia can affect hemoglobin structure and levels, and how doctors can diagnose and treat this disease.

Hemoglobin levels in people with SCD are usually between 6 and 11 grams per deciliter (g/dL), while they are around 12 mg/dL in people without SCD.

Lower hemoglobin levels can make it harder for red blood cells to transport oxygen throughout the body. It can cause a wide range of symptoms, including pain and exhaustion. Over time, SCD May affect most organs, including eyes, kidneys and liver.

In addition to causing low hemoglobin levels, SCD alters hemoglobin, causing cells to sickle. 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 they carry one gene for SCD, not two. Sickle cell trait is usually a mild 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 keep them from developing symptoms.

Can physical activity affect hemoglobin levels?

When a person is under intense physical stress, such as when they are at high altitudes or when an untrained person trains with great intensity, some of their red blood cells may become deformed.

As a result, a person can develop a life-threatening complication called rhabdomyolysis, which damages muscles, causing them to break down and release harmful substances into the blood.

In sickle cell anemia, the hemoglobin protein is not normal. A genetic mutation within the HBB fact that hemoglobin contains a chemical called valine instead of glutamic acid in the protein chains that form hemoglobin. This damages the red blood cells, causing them to sickle and die sooner than usual.

The HBB The gene is responsible for the formation of beta-globin chains, which are a component of hemoglobin.

Premature death of red blood cells can mean that hemoglobin and red blood cell counts are chronically low. So, in addition to having blood cells that stick together and transport oxygen less efficiently, a person will not have enough red blood cells to transport oxygen effectively.

Changes in hemoglobin structure

Hemoglobin is made up of four protein subunits: two alpha-globin and two beta-globin subunits.

In people with SCD, certain mutations within the HBB gene can change the structure of hemoglobin. Specifically, the mutations code for an abnormal version of beta-globin, or HbS. This can occur in only one of the two beta-globin subunits.

However, in sickle cell anemia, the two beta-globin subunits of hemoglobin are replaced by abnormal HbS. This leads to the distortion of the red blood cells and causes them to adopt a sickle shape.

In other types of SCD, hemoglobin subunits may be replaced with other abnormal hemoglobin types, such as HbC. These can also change the shape of red blood cells.

The type of SCD a person has will influence the symptoms they experience. The genes a person inherits determine the type of SCD they develop.

The most common types of SCD are:

  • SS homozygous hemoglobin: The most common form of SCD, this variant means that a person inherits two copies of the sickle “S” gene – one from each parent. This is the most serious manifestation of SCD. Doctors call this sickle cell anemia.
  • Hemoglobin sickle cell anemia: People with this milder type of SCD inherit a sickle “S” gene and a sickle “C” gene, which is another type of abnormal hemoglobin gene.
  • Beta-thalassemia sickle hemoglobin: This version of SCD means that a person receives a sickle “S” protein and a gene for beta-thalassemia, which is another type of abnormal hemoglobin. There are two subtypes: beta0-thalassemia, which is a more severe form, and beta+-thalassemia, which tends to be milder.

People with access to medical care usually get a diagnosis as newborns. Routine newborn screening in the United States looks for signs of hemoglobin abnormalities using a drop of blood. This screening can help healthcare professionals diagnose SCD.

If a person has not had a newborn screening test or does not live in a country that offers this type of screening, they can have blood tests as an adult to detect sickle cell disease.

Quantification of hemoglobin

Some lab tests can separate hemoglobin to look for different variants and quantify them from blood samples. These tests include:

  • Hemoglobin electrophoresis: This technique makes it possible to identify the different types of hemoglobin.
  • High performance liquid chromatography: This technique also detects most types of hemoglobin.
  • Isoelectric focusing: It is a very sensitive detection method.

Although hematopoietic stem cell transplants have potential as cure for SCD, they are not widely available. No cure is available for most people, which means treatment mainly focuses on managing symptoms.

A doctor can recommend lifestyle management strategies to prevent certain complications associated with the disease. These strategies may include:

  • to drink a lot of water
  • wash your hands often to reduce risk of infection
  • avoid high altitudes

Pain relievers can help people with sickle cell disease manage episodes of pain or pain attacks. During a sickle cell crisis, a person may need to stay in the hospital. If their red blood cell and hemoglobin count becomes very low, a person may need a blood transfusion.

It is important for people with sickle cell disease to keep up to date with their vaccinations and practice safe food handling, as they are at higher risk of serious complications if they contract an infection.

Sickle cell disease can lead to many complications and shorten a person’s life, especially if they do not have access to adequate medical care. Possible complications include:

In sickle cell anemia, a person’s hemoglobin level is chronically low. This can lead to several complications. However, the severity of complications varies from person to person and is highly dependent on a person’s type of SCD.

It is important that everyone with sickle cell disease gets quality medical care, maintains a healthy lifestyle, and knows the signs of an emergency so they can be seen quickly if needed.

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