Sickle cell disease (SCD) is a group of genetic diseases that affects hemoglobin
The red blood cells in sickle cell disease begin to form the shape of a crescent moon instead of the normal round shape of red blood cells.
While round red blood cells can move easily through the blood vessels, sickle-shaped cells interconnect, resulting in blood clots.
These blood clots usually cause extreme pain in the back, chest, hands, and feet, while the disrupted blood flow causes damage to the bones, muscles, and organs, thus causing sufferers to feel weak, tired, and look pale.
The eyes, as well as the skin, also usually look yellowish.
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Other names for Sickle Cell Disease are: HbS disease, Hemoglobin S disease, Sickle cell disorders, and Sickle cell disorder due to hemoglobin S.
According to WHO, SCD, or sickle cell anaemia, affects most countries in the African region, such as Cameroon, Republic of Congo, Gabon, Ghana, and Nigeria.
Research conducted by some research experts, as of 2022, discovered that, of the 50 million people living with sickle cell disease globally, Nigeria accounts for about 4-6 million people, i.e., 1 in every 4 Nigerians has a sickle cell trait, making her the epicentre zone for the disease.
In addition, out of the 300,000 children born with SCD globally, Nigeria accounts for 33% of the global burden of newborns born with SCD.
Hb-SS is the predominant hemoglobin variant in Nigeria, while Hb-SC occurs sporadically, especially in the south-western part of the country.
News Round The Clock in this article talks about the symptoms, causes, triggers, and treatment of SCD.
Symptoms of SCD
SCD symptoms vary according to carrier, but some of the most common symptoms are categorized into early and severe symptoms.
Early Symptoms:
- Yellow-coloured eyes or skin (jaundice): This occurs when many red cells undergo hemolysis.
The liver filters red blood cells, among its many functions, but, in SCD, fast-dying sickled cells release bilirubin that builds up in the system, causing jaundice.
- Extreme tiredness or fussiness from anemia, which occurs when the body does not get enough oxygen because of a shortage of healthy red blood cells.
- Painful swelling of the hands and feet, known as dactylitis.
This symptom is more common among babies who suffer from sickle cell. The sickled cells get stuck in their blood vessels, preventing blood flow to their hands and feet, thereby causing unbearable pain.
Severe Symptoms:
- Severe pain: SCD causes tissue damage when the tissues don’t get enough oxygen, thereby creating pain, sometimes excruciating, in the arms, legs, chest, and back.
It may start as an ache, before becoming an excruciating pain.
This serious pain event is sometimes called a “sickle cell crisis” or “vaso-occlusive crisis.”
Most adults with sickle cell disease suffer from this “sickle cell crisis,” however, it may occur in 1 year-old babies.
This level of pain often requires treatment in a clinic or hospital.
- Fatigue, shortness of breath, dizziness, and irregular heartbeat are also severe symptoms of severe anaemia.
Severe anemia can be caused by other conditions, such as a splenic sequestration crisis or chronic kidney disease.
Meanwhile, babies with sickle cell anemia may seem unusually fussy and irritable.
- Frequent infections: The disease affects the spleen, which weakens the sufferer’s immune system so they are more likely to develop infections.
However, because of the widespread use of preventive antibiotics and immunizations, the risk of developing infections is now significantly reduced.
- Fever. If you have a fever of more than 38.5 degrees Celsius, seek medical attention from your doctor.
- Chest pain, coughing, fever, and shortness of breath.
These symptoms usually suggest acute chest syndrome, requiring treatment in a hospital, where you may receive antibiotics, oxygen therapy, and/or a blood transfusion.
- Sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking.
This symptom is indicative of a stroke. Therefore, consult your doctor or a hematologist— a doctor who specializes in blood conditions and diseases.
- Priapism: A symptom where the sufferer experiences long hours of erection for up to four hours or more.
This is an emergency!
Therefore, go to the hospital and see your hematologist or urologist— a doctor who specializes in treating conditions of the reproductive and urinary systems.
Priapism can lead to erectile dysfunction and impotence, it is therefore important to get treatment as soon as possible.
- Vision problems
- Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells, thus damaging the retina and causing blindness.
Note: SCD symptoms change over time as the body makes more sickled cells.
SCD Triggers
Environmental factors often play a role in the occurrence of painful attacks.
Common triggers include: cold temperatures, dehydration, excessive amounts of exercise, and tobacco smoke.
Other triggers, such as plane flights and high altitudes, can also trigger an attack.
What causes SCD
Sickle cell anemia stems from a genetic mutation that alters the production of hemoglobin (the essential, iron-rich protein in red blood cells responsible for transporting oxygen throughout the body).
This altered form of hemoglobin causes red blood cells to become stiff, sticky, and deformed, taking on a crescent or “sickle” shape.
For a child to inherit sickle cell anemia, both parents must carry and pass on the sickle cell gene.
If only one parent passes the gene, the child will have what is known as the sickle cell trait.
In this case, their blood contains a mix of normal and sickled hemoglobin, but they typically don’t experience symptoms.
Instead, they serve as carriers, meaning they can still pass the gene to their offspring.
Complications caused by SCD
Sickle cell anaemia carriers suffer many complications like chronic kidney disease, blindness, stroke, as well as avascular necrosis— the death of bone tissue due to a lack of blood supply.
Other complications include leg ulcers, priapism, acute chest syndrome, and pregnancy complications, among many others.
Sickle Cell Disease Treatment
Treatment for sickle cell anaemia depends on your symptoms and your overall health.
For example, sufferers who experience severe complications like acute chest syndrome, frequent acute pain crises, or stroke, may be advised by their doctor to undergo allogeneic stem cell transplant.
Note: This procedure is the only cure for sickle cell anemia.
Meanwhile, blood transfusions and antibiotics are other anaemia treatments, often adopted to treat infections and medications that ease specific symptoms.
However, consult your doctor about possible treatments, preventive measures and reproductive options before undergoing any anaemia treatment.
Warning: Do not self-medicate on antibiotics!
Recap
Sickle cell disease remains a significant health challenge, particularly in regions like Nigeria, where the burden is among the highest globally.
While advancements in medical research have improved symptom management and life expectancy, a cure remains elusive for many.
The key to combating SCD lies in increased awareness, genetic counseling, and access to better healthcare.
With ongoing research, emerging treatments, and improved support systems, the future holds promise for those affected.
Until then, education, early intervention, and proper medical care remain the best weapons in this battle against sickle cell disease.
