Sickle cell disease (SCD) is a term that describes a group of inherited defective red blood cells. People with SCD have abnormal hemoglobin, otherwise known as hemoglobin S in their red blood cells.

Hemoglobin is the protein present in the blood that facilitates the transportation of oxygen to tissues and nearby organs. The red blood cells, in this case, are defective and have a rod-like form that looks like a sickle instead of the normal form which is roundish and doughnut-like.

SCD gives rise to sickle cell anemia, so most times both terms are interchanged and believed to be one and the same.

To create a better understanding of the subject, we will take a look at the unique factors that influence sickle cell disease and examine the effects that it has on the body.

Causes of Sickle Cell Disease (SCD)

At the start of this overview, we described SCD as “inherited”. This means that SCD is genetic and can be passed through the bloodline of people who have it.

Sickle cell disease is not contagious; it cannot be transmitted through the exchange of body fluids, touch, and sexual intercourse. It is purely genetic and can only be inherited.

However, it is possible for a person to have the sickle cell gene and not have the disease/order. In all the many forms of sickle cell, at least one of them and abnormal hemoglobin make the body to produce hemoglobin S. When a person has two hemoglobin S genes (hemoglobin SS), this is called sickle cell anemia. This is the most common and severe form of the sickle cell disease.

Persons with the sickle gene do not have the disease themselves but are capable of passing it on to their children if they have a partner that has the gene also.

SCD affects races and can be found in almost every nation of the world.

What Does the Sickle Cell disease do To the Body?

SCD attacks the hemoglobin present in the blood. Hemoglobin helps in the transportation of oxygen to every part of the body. Hence, they are vital to the sustenance of tissues and organs.

 Sickle-shaped hemoglobin cells are abnormal and not as elastic as the normal doughnut-shaped (without a hole) cells. While blood flows through blood vessels, the sickle-shaped cells tend to cluster together and block passageways, making it difficult for blood and oxygen to get to nearby tissues.

This accumulation of sickle red blood cells around blood vessels causes sudden pain and aches which is normally referred to as “crisis”. Persons with sickle cell disease go through this pain often and it comes without prior notice.

Also, sickle-shaped cells do not last as long as the normal red blood cells do. In most cases, they last for just 10 to 20 days before dying while the normal cells can last for as long as 100 to 120 days before dying.

Although the blood is always making red blood cells, a person with SCD cannot cope with how fast the sickle-shaped cells are dying. This leads to a decrease in the number of healthy cells in the body which results in complications in nearly all parts of the body.

Symptoms

Asides few direct symptoms of sickle cell disease, the major indicator of SCD is commonly known as the “sickle cell crisis.” A sickle cell crisis is a sudden development of pain in a specific region of the body. This usually requires urgent medical attention to prevent further complications.

Acute chest syndrome is another disturbing symptom of the sickle cell disease that may also require medical attention in the intensive care unit of a hospital. Acute chest syndrome results when blood vessels located in the get blocked, or it could be a sign of an emerging chest disease.

Other common symptoms of SCD include:

-           Jaundice (yellowing of the eyes and skin)

-           Anemia

-           Prolonged and painful erections in men

-           Kidney problems

-           Spleen disorder

-           Swollen hands and feet

-           Bones and joints complications

-           Infections

-           Leg Sores

-           Eye disease

-           High blood pressure in the heart

-           Eye disease

-           Stroke

-           Sores

The list can go on and on because SCD affects the blood and can generate complications at any location in the body. This is why people with SCD must see a doctor for regular screening.

Diagnosis

Unlike when the condition was not so popular, it has been relatively easy to detect the disease. It that is required is a simple blood test to ascertain whether a person has the condition or not. It is also possible to detect the disease at birth through a test for newborns.

However, anyone with a genetic history of the sickle disease should ensure that they consult their doctor if they intend to have children of their own. Women, on the other hand, should be well taken care of so as to detect problems before they arise.

A prenatal test can as well help you detect whether the unborn child has SCD or not.

How to live with Sickle Cell Disease

SCD is a lifelong medical condition. As a result, it is important for a person with SCD to take lifelong decisions, cut excessive so as to lead a long, healthy and happy life. To do this successfully, a combination of lifestyle choices are usually recommended. This includes:

- Cutting down on smoking and alcohol intake

- Going for a regular checkup with the doctor

- Maintaining a healthy diet at all time

Doing all these and religiously following your doctor’s directives should ensure that you minimize the harmful effects of sickle cell disease.

Source: National Heart, Lung, and Blood Institute publication, and other online sources