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Sickle cell anaemia (lack of blood) is a genetically inherited red blood cell disorder. Sickle cell disease can cause organ damage, stroke and even death

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Sickle cell anemia is one of a group of disorders known as sickle cell disease. Sickle cell anemia is an inherited red blood cell disorder in which there aren’t enough healthy red blood cells to carry oxygen throughout your body.

Normally, the flexible, round red blood cells move easily through blood vessels. In sickle cell anemia, the red blood cells are shaped like sickles or crescent moons. These rigid, sticky cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.

People also ask, these are the answers:

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What is the main cause of sickle cell disease?

What causes sickle cell disease? Sickle cell is an inherited disease caused by a defect in a gene. A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father. A person who inherits just one gene is healthy and said to be a “carrier” of the disease.

How do you get sickle cell disease?

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You inherit 1 set from your mother and 1 set from your father. To be born with sickle cell disease, a child has to inherit a copy of the sickle cell gene from both their parents. This usually happens when both parents are “carriers” of the sickle cell gene, also known as having the sickle cell trait.

How does sickle cell disease affect a person?

Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen. In sickle cell anemia, blood is also chronically low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including your kidneys, liver and spleen, and can be fatal. Blindness.

How long can a person live with sickle cell disease?

With a national median life expectancy of 42–47 years, people with sickle cell disease (SCD) face many challenges, including severe pain episodes, stroke, and organ damage

What is sickle cell pain like?

The pain may feel sharp, stabbing, intense, or throbbing. Some people with sickle cell disease say it’s worse than childbirth or the pain after surgery. You may have pain anywhere in your body and in more than one place.

What should sickle cell patients avoid?

Avoid very strenuous exercise – people with sickle cell disease should be active, but intense activities that cause you to become seriously out of breath are best avoided. avoid alcohol and smoking – alcohol can cause you to become dehydrated and smoking can trigger a serious lung condition called acute chest syndrome.

What is the difference between sickle cell disease and sickle cell anemia?

Sickle cell disease (SCD) is a serious group of conditions which are inherited (genetic). It affects the red blood cells in the blood. Sickle cell anaemia is the name of a specific form of SCD in which there are two sickle cell genes (see below)

Can you survive sickle cell?

People who have sickle cell disease have a reduced life expectancy. Some people with the disease can remain without symptoms for years, while others do not survive beyond infancy or early childhood. New treatments for SCD are improving life expectancy and quality of life

Can a man with sickle cell get a woman pregnant?

For males with sickle cell disease, there are higher chances for sperm issues, including lower sperm counts and testicular dysfunction. For females with the condition, their ability to conceive may be reduced as well. For people with Sickle Cell disease, fertility treatment can make it possible to achieve pregnancy.

Does sickle cell get worse with age?

SCD is a disease that worsens over time. Treatments are available that can prevent complications and lengthen the lives of those who have this condition.

Can you get malaria with sickle cell?

Patients with sickle cell disease (SCD), an inherited haemoglobinopathy, have increased risk of malaria, at least in part due to impaired splenic function.

Why is sickle cell more common in Africa?

The disease is most common in sub-Saharan Africa, where as many as 45% of people are carriers. It has become so widespread there because being a carrier offers a survival advantage against malaria.

Is sickle cell more common in males or females?

No sex predilection exists, since sickle cell anemia is not an X-linked disease. Although no particular gender predilection has been shown in most series, analysis of the data from the US Renal Data System demonstrated marked male predominance of sickle cell nephropathy in affected patients.

How does sickle cell affect sperm?

Sperm abnormalities are frequent in males with SCD, with rates as high as 91%. Low sperm density, low sperm counts, poor motility, and increased abnormal morphology occur more frequently in males with SCD than in controls.

Symptoms

Signs and symptoms of sickle cell anemia usually appear around 5 months of age. They vary from person to person and change over time. Signs and symptoms can include:

o          Anemia. Sickle cells break apart easily and die, leaving you with too few red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia).

o          Without enough red blood cells, your body can’t get enough oxygen, causing fatigue.

o          Episodes of pain. Periodic episodes of pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones.

o          The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain crises a year. Others have a dozen or more pain crises a year. A severe pain crisis requires a hospital stay.

o          Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes.

o          Swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.

o          Frequent infections. Sickle cells can damage your spleen, leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.

o          Delayed growth or puberty. Red blood cells provide your body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

o          Vision problems. Tiny blood vessels that supply your eyes can become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images — and lead to vision problems.

When to see a doctor

Sickle cell anemia is usually diagnosed in infancy through newborn screening programs. If you or your child develops any of the following problems, see your doctor right away or seek emergency medical care:

o          Fever. People with sickle cell anemia have an increased risk of serious infection, and fever can be the first sign of an infection.

o          Unexplained episodes of severe pain, such as pain in the abdomen, chest, bones or joints., Swelling in the hands or feet., Abdominal swelling, especially if the area is tender to the touch.

o          Pale skin or nail beds., Yellow tint to the skin or whites of the eyes.

o          Signs or symptoms of stroke. If you notice one-sided paralysis or weakness in the face, arms or legs; confusion; trouble walking or talking; sudden vision changes or unexplained numbness; or a severe headache, call 911 or your local emergency number right away.

Causes

Sickle cell anemia is caused by a mutation in the gene that tells your body to make the iron-rich compound that makes blood red and enables red blood cells to carry oxygen from your lungs throughout your body (hemoglobin). In sickle cell anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen.

Both mother and father must pass the defective form of the gene for a child to be affected.

If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one normal hemoglobin gene and one defective form of the gene, people with the sickle cell trait make both normal hemoglobin and sickle cell hemoglobin.

Their blood might contain some sickle cells, but they generally don’t have symptoms. They’re carriers of the disease, however, which means they can pass the gene to their children.

Risk factors

For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects black people.

Prevention

If you carry the sickle cell trait, seeing a genetic counselor before trying to conceive can help you understand your risk of having a child with sickle cell anemia. They can also explain possible treatments, preventive measures and reproductive options.

o          Take folic acid supplements daily, and choose a healthy diet. Bone marrow needs folic acid and other vitamins to make new red blood cells. Ask your doctor about a folic acid supplement and other vitamins. Eat a variety of colorful fruits and vegetables, as well as whole grains.

o          Drink plenty of water. Dehydration can increase your risk of a sickle cell crisis. Drink water throughout your day, aiming for about eight glasses a day. Increase the amount of water you drink if you exercise or spend time in a hot, dry climate.

o          Avoid temperature extremes. Exposure to extreme heat or cold can increase your risk of a sickle cell crisis.

o          Exercise regularly, but don’t overdo it. Talk with your doctor about how much exercise is right for you.

o          Don’t smoke. Smoking increases your risk of pain cries

For further information, sickle cell association Banjul, email to [email protected], send text messages to Dr Azadeh WhatsApp 002207774469.

Dr Hassan Azadeh MD, senior lecturer at the University of The Gambia, Clinical Director at Medicare Health Services.

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