Let’s Start with Your Blood

You may know that blood flows through your body every second of every day. But have you ever wondered what blood actually does?

Blood is a remarkable body fluid that travels through a network of blood vessels, delivering oxygen and nutrients to the tissues and organs of your body while carrying away waste products.

Blood has four main components:

• Red blood cells
• White blood cells
• Platelets
• Plasma

Each has an important job to do.

Red Blood Cells: Your Oxygen Carriers

Red blood cells are the most abundant cells in your blood.

Inside them is a special protein called haemoglobin. Haemoglobin picks up oxygen from your lungs and carries it to your brain, heart, muscles and other organs. After delivering oxygen, red blood cells help carry carbon dioxide back to the lungs, where it is breathed out.

Healthy red blood cells are normally round, soft and flexible. Their shape and flexibility allow them to move easily through even the smallest blood vessels. And that flexibility is very important.

White Blood Cells: Your Body’s Defense Team

White blood cells help protect your body against infections.

Different types of white blood cells have different roles, but together they form an important part of your immune system.

Think of them as part of your body’s security team, helping to identify and fight harmful organisms and other threats.

Platelets: Helping to Stop Bleeding

Platelets are tiny cell fragments that circulate in your blood.

When a blood vessel is injured, platelets help form a plug at the site of the injury. This is an important part of the body’s process for stopping bleeding.

Plasma: The Transport System

Plasma is the pale-yellow liquid part of blood.

It carries blood cells throughout the body and transports nutrients, hormones, proteins, antibodies, medicines and waste products.

So, although blood may look like one red liquid, it is actually a carefully organized system in which different components work together to keep you alive and healthy.

Where Are Blood Cells Made?

Most blood cells are produced in the bone marrow.

Bone marrow is the soft, spongy material found inside some of our bones. It is the body’s major blood-cell factory, continually producing new blood cells to replace older cells.

Your body is constantly working to maintain a healthy supply of blood cells.

This is why healthy blood is so important.

Without enough healthy blood cells and adequate oxygen delivery:

• your brain may not function properly;
• your muscles may become weak and tired;
• your organs may not function normally; and
• your body may have difficulty maintaining normal body functions.

Now that we understand a little about blood, let’s talk about sickle cell disease.

So, What Is Sickle Cell Disease?

Sickle cell disease (SCD) is an inherited blood disorder.

This means that the condition is passed from parents to their children through genes.

Sickle cell disease affects haemoglobin, the protein inside red blood cells that carries oxygen around the body.

Types of sickle cell disease include:

• Sickle cell anaemia (HbSS)
• Sickle cell disease with haemoglobin C (HbSC)
• Sickle beta-thalassaemia (HbS/β-thalassaemia)

There are also other, less common forms of sickle cell disease.

So, What Happens Inside the Body?

This is where understanding your red blood cells becomes especially important.

People living with sickle cell disease have an abnormal type of haemoglobin called haemoglobin S (HbS).

When HbS loses oxygen, it can form long, rigid structures inside the red blood cell.

As a result, the normally round and flexible red blood cell can change into a crescent or sickle shape.

And this change in shape matters.

Sickled red blood cells can become:

• harder and less flexible;
• sticky;
• more likely to block small blood vessels; and
• more fragile and easily destroyed.

Why Is the Shape So Important?

Imagine trying to push a soft, flexible ball through a very narrow tube.

It can squeeze through easily.

Now imagine trying to push a hard, rigid object through the same narrow tube.

It may get stuck.

Something similar can happen in sickle cell disease.

When sickled cells block small blood vessels, blood flow to tissues can be reduced. This can cause pain and, over time, contribute to damage to important organs.

Because sickled red blood cells also break down more quickly than normal red blood cells, people living with SCD can develop anaemia (shortage of blood). This helps explain why some of the symptoms and complications associated with sickle cell disease occur.

The more you understand about sickle cell disease, the better prepared you can be to recognize changes in your body, take appropriate steps to protect your health and follow your treatment plan.

At PHI, we are here to provide information, encouragement and a safe space where you can ask questions and learn.

Until our next post, stay informed. Stay Sickle Smart. And keep believing in yourself.