Sickle Cell Disease - An Overview

Sickle Cell Explained - Its Symptoms & Types

Sickle cell disease (SCD) is a serious condition, but sickle cell trait (SCT) (carrying one HbS gene) can confer malaria resistance in endemic regions. This is an evolutionary advantage, though SCT can rarely cause complications., In SSD, the red blood cells (RBC) lose their shape, causing a blockage in blood flow. Due to this condition, you might experience extreme pain, organ damage, and other complications.

What is Sickle Cell Disease?

It is a genetic blood disorder that is one of the common inherited genetic disorders. It affects a protein, haemoglobin, that is a component of RBC. Haemoglobin is a protein that is responsible for carrying oxygen in the blood and other parts of the body.

The shape of the normal RBC is round and flexible, allowing it to easily move throughout the body. In SCD, the haemoglobin is of an abnormal shape known as haemoglobin S. This alters the normal shape of an RBC into a crescent shape. This causes the blood cell to stick together, become inflexible, and become rigid.  

What are the Types of Sickle Cell Disease?

There are multiple types of sickle cell disease. It varies based on the gene type that one inherits. Let's delve deeper into its types:

1. Haemoglobin SS (HbSS): HbSS is the most severe form of SCD that affects about 65% of people out of all the people suffering from SCD. People with this form inherit one gene encoding haemoglobin S from each parent. Therefore, in this case, most or all of your haemoglobin is abnormal, leading to chronic anaemia.
2. Haemoglobin SC (HbSC): It is the mildest form of SCD, with only 25% of overall SCD patients suffering from HbSC. While often less severe than HbSS, HbSC can still lead to organ damage. In this form, you inherit a haemoglobin S gene from one parent and inherit a haemoglobin C gene from the other parent.  Haemoglobin C is another form of abnormal haemoglobin type.
3. Haemoglobin (HbS) Beta Thalassaemia: In this type of SCD, you inherit the haemoglobin S gene from one parent. Moreover, you also inherit another abnormal cell from the other parent, beta-thalassaemia.

• HbSβ⁺ (some normal hemoglobin; milder).
• HbSβ⁰ (no normal hemoglobin; severe).
         

What is the Difference between Sickle Cell Anaemia and Sickle Cell Disease?

SCD is an umbrella term that includes all types of diseases related to sickle cell issues. Whereas the term "sickle cell anaemia" refers to a variety of SCD that leads to the most severe form of SCD.  

What are the Symptoms of Sickle Cell Disease?

The symptoms of SCD become prominent by the age of 5-6 months. These symptoms are often subjective and vary from person to person. Some people might exhibit milder symptoms. Whereas, in other cases, the symptoms can be more serious. Here is a list of symptoms to look out for if you suspect SCD:

• Anaemia causes feelings of fatigue and weakness.
• Frequent pain episodes.
• Swelling along with pain in the hands and feet.
• Jaundice
• Acute chest syndrome
• Stroke
   

What are the Problems of Sickle Cell Disease?

SCD disease disrupts the normal functioning of multiple organs. The effects of some are acute, and some can be chronic. Let us know about some complications:

• Pain: Sickle cell, while passing through blood vessels, causes pain due to sticking together and blocking regular blood flow.
• Anaemia: The longevity of RBCs is lowered due to SCD, which leads to anaemia. This condition occurs when you do not have enough healthy RBCs.
• Organ Damage and Failure: Due to a lack of oxygen supply, organs fail to perform optimally. 

Is Sickle Cell Disease Manageable? 

Yes, individuals can manage sickle cell disease and reduce complications by following the steps listed below:

1. Keep yourself hydrated by drinking at least 7-8 glasses of water every day.
2. Avoid extreme temperatures; don’t go to places that are too hot or too cold.
3. Stay in touch with your health professional. Attend all scheduled appointments with your provider. If anything is bothering you, talk to them.
4. Ask your healthcare professional about different effective ways to prevent the risk of infections. Ask them if you need to take any vaccines.
5. Regular monitoring of your overall health, including blood pressure if you have hypertension, is important.
6. Avoid places with exposure to low oxygen levels.
7. Do regular exercises. Consult a doctor to determine the appropriate amount of activity for you.
8. If you smoke and use any tobacco products, stop them.
9. Try to manage your stress through relaxation techniques.
10. Wash your hands frequently to avoid the risk of infections.

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