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What are sickle cells? -

Sickle cells are mutated red blood cells that are shaped and act differently from normal red blood cells. They have a far shorter lifespan than normal red blood cells, and inhibit hemoglobin's ability to carry oxygen throughout the body.

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What is hemoglobin? -

Hemoglobin is an iron-rich protein that carries oxygen throughout the body via red blood cells. The shape and nature of sickle cells makes the job of the body's hemoglobin protein much more difficult.

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Why are they called sickle cells? -

Sickle cells simply gain their name from their shape. As opposed to the round, globulous figure of healthy red blood cells, sickle cells take on a crescent shape, like that of a, you guessed it, sickle.

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How do sickle cells work? -

Because of their weakened figure, sickle cells have a lifespan of only around 20 days. In comparison, healthy blood cells live for around 120 days on average, giving them much more time to do their job. Additionally, the irregular shape of sickle cells causes them to group in clusters within the veins, causing painful and life-threatening disturbances in the body's blood circulation.

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What is the origin of sickle cells? -

Sickle cells are simply mutated blood cells, and have no exact cause or reason for existing. Genetics tells us the culprit of sickle cells is nothing more than a defective gene, a luck of the draw.

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What is sickle cell disease? -

Sickle cell disease, or SCD, is a broad term for the many ailments that can be caused by the presence of sickle cells in an individual's body.

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What is sickle cell disease? -

The presence of sickle cells isn't something that can be controlled, and sickle cell disease isn't contagious. It is at its core a genetic condition, passed down hereditarily.

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How does one get SCD? -

A person can only contract SCD if they inherit a sickle cell gene from each parent. If both parents of a child have a sickle cell trait gene, genetics tells us that there is a 25% chance that their child will be born with SCD.

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What's the difference between a sickle cell trait and SCD? -

People with a sickle cell "trait" gene have just that: one single gene copy of a sickle cell mutation. The vast majority of people with sickle cell traits feel no symptoms and face no danger, but it is still important for you and your partner to get tested if you are planning on having children. If both you and your partner have sickle cell traits, there is a chance your child will inherit both and be born with sickle cell disease.

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The many faces of SCD -

Sickle cell disease manifests itself in many ways and can vary in severity. There are five main types of SCD, and they're distinguished from one another based on the exact nature of the sickle cell genes, the number of sickle cells produced, and the way hemoglobins act within the blood cells. The most common and most dangerous strain of SCD is known as sickle cell anemia.

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What is sickle cell anemia? -

Sickle cell anemia occurs when sickle cells are misshapen, fragile, and large in numbers. All three of these factors inhibit the flow of oxygen throughout the body, causing pain, fatigue, and numerous other symptoms.

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How many people in the world have SCD? -

Annually, around 300,000 children worldwide are born with some form of sickle cell disease. A third of the worldwide cases are born within the United States.

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How can SCD affect your life? -

The flow of oxygen through one's body, the very thing that SCD inhibits, is such a fundamental function of the body that its effects on one's livelihood are wide-reaching and impossible to overstate.

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Common symptoms -

Vision problems are common in individuals with SCD, as the visual organs are deprived of oxygen and are unable to develop properly. Other growth issues can occur for the same reason, including a delay in puberty in children. Chronic pain and swelling of the hands and feet are other common symptoms.

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Higher risk of stroke -

The constant lack of oxygen flowing to the brain also makes sufferers of SCD much more likely to suffer strokes. By the age of 45, nearly a quarter of all individuals with SCD will have a stroke.

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Sickle cell crises -

Common and lengthy bursts of intense, localized pain known as "crises" are also common in those with SCD. Crises occur when a certain part of the body becomes severely deprived of oxygen for a time. The pain can be excruciating, and last for up to a full week with no letting up.

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Children are at the highest risk -

Children are at the highest risk when it comes to SCD. Babies born with any type of sickle cell condition must be closely monitored for most of their childhood, as life-threatening conditions can pop up frighteningly quickly.

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Serious complications of SCD in children -

In short, all of the symptoms of SCD are more severe and more dangerous in children. Their smaller blood vessels leave even less room for blood cells to move throughout the body, and crises, which can start at as young as five months old, can be too severe for their little bodies to handle.

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Can SCD be cured? -

So far, there is no completely safe cure for SCD. Stem cell research has proven to be promising, but stem cell transplants for blood cells are still too risky to be considered a viable cure. However, as science progresses, there is hope for the future.

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Can SCD be treated? -

Thankfully, there are many treatments available for sickle cell disease that can provide immense amounts of relief, and allow those afflicted to live relatively normal, happy lives.

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Treatments for SCD -

Numerous non-habit-forming pain medications have been developed specifically to lessen the frequency and severity of pain crises, and cardiovascular exercise is recommended to help promote the flow of blood and oxygen throughout the body.

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Treatments for SCD -

Treatment for children is trickier, as many of the medications prescribed to adults are too intense for children and can cause more harm than good. Some medications, however, like hydroxyurea, have been approved for pediatric use.

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Making great strides -

For centuries, sickle cell disease had no name and confounded medical professionals around the world. Thankfully, medical science in recent decades has made leaps and bounds when it comes to understanding this genetic disease, and how to treat it.

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Bone marrow transplants -

Bone marrow and stem cell transplants are the only procedures proven to cure SCD. Both procedures introduce new, healthy blood cells into your system that begin to multiply and outlive the body's sickle cells until there are no sickle cells left to reproduce. However, grave complications can arise through these types of transplants, no matter how good the doctor is. A common adverse reaction of such procedures is known as graft-versus-host disease, in which the new cells attack their new environment and destroy it from the inside.

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Gene therapy -

This FDA-approved therapy is still in its early stages, but data shows promising results, offering hope to those who suffer from sickle cell disease. The treatment works by correcting the mutation that causes the condition, modifying the blood-forming stem cells.

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Gene therapy -

In 2023, the FDA approved an innovative gene therapy treatment and 12-year-old Kendric Cromer became the first person to receive it. After 44 days of treatment, Cromer left the hospital on October 21, 2024. It was an emotional celebration for the doctors, nurses, family, and friends who supported him throughout his journey. Doctors believe the potentially life-changing treatment will make unprecedented improvements to his quality of life.

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Pediatric penicillin -

The advent of pediatric antibiotics like penicillin has also been a great help in the fight against SCD. Penicillin has been proven to protect children against numerous infections that are immensely common in kids with SCD.

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Blood transfusions -

Regular blood transfusions, while a hassle and can be considered invasive by some, are an indispensable aspect of treatment for those trying to live a normal life with SCD. The introduction of healthy blood into one's bloodstream lowers the percentage of sickle cells, thus minimizing the rick of clotting and pain crises, as well as promoting the flow of oxygen throughout the body.

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Newborn screenings in the United States -

It is also essential to catch SCD as soon as possible, in order to minimize the rate of infantry mortality due to sickle cells. Since doctors know that people with SCD are born with it, one of the first exams done to a newborn baby is a heel-stick test where their blood is tested for sickle cells so that treatment can begin immediately, if need be.

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The average lifespan of someone with SCD -

Despite the great advancements in the understanding and treatment of sickle cell disease, it is an affliction that is devastatingly hard on the body. The average lifespan for individuals afflicted by SCD is only around the mid-forties. That being said, the oldest known person to ever live with SCD, Ernestine Diamond, lived until she was 94.

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Living with SCD -

Although one must come to terms with a dishearteningly short lease on life, the treatments and therapies available today all but guarantee that anyone living with sickle cell disease is given the opportunity to live a happy and fulfilling life.

Sources: (Nationwide Children's Hospital) (Mayo Clinic)

See also: Signs you have iron deficiency

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