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What do we know in The Gambia about the genetic and inherited disease called sickle anaemia?

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How does sickle cell anaemia affect childhood and in pregnancy?

What is sickle cell disease?

Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues.

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Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell haemoglobin are stiff and sticky, when they lose their oxygen, they form into the shape of a sickle or crescent, like the letter C. These cells stick together and can’t easily move through the blood vessels. This can block small blood vessels and the movement of healthy, normal oxygen-carrying blood. The blockage can cause pain.

Normal red blood cells can live up to 120 days. But sickle cells only live for about 10 to 20 days. Also, sickle cells may be destroyed by the spleen because of their shape and stiffness. The spleen helps filter the blood of infections. Sickled cells get stuck in this filter and die. With less healthy red blood cells circulating in the body, you can become chronically anaemic. The sickled cells also damage the spleen. This puts you at greater risk for infections.

What causes sickle cell disease?

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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. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier., For parents who are each carrier of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.

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 anemia is the name of a specific form of SCD in which there are two sickle cell genes.

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.

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: anaemia. 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).

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

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.

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.

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

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

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.

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.

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.

How does sickle cell disease affect pregnancy?

How sickle cell disease affects pregnancy depends on whether you have sickle cell disease or sickle cell trait. Some women with sickle cell disease have no change in their disease during pregnancy. In others, the disease may get worse. Painful events called sickle cell crises may still occur in pregnancy. These events may be treated with medicines that are safe to use during pregnancy.

If you have kidney disease or heart failure before you get pregnant, it may get worse during pregnancy.

Generally, women with sickle cell trait don’t have problems from the disorder. But they may have a lot of urinary tract infections during pregnancy. Pregnant women with sickle cell trait can also have a kind of anemia caused by not having enough iron in their blood. If you have this type of anemia, you may need to take iron supplements., In pregnancy, it’s important for blood cells to be able to carry oxygen. With sickle cell anemia, the abnormal red blood cells and anemia may result in lower amounts of oxygen going to your developing baby. This can slow down the baby’s growth.

What are possible complications of sickle cell disease in pregnancy?

Because sickling affects so many organs and body systems, you are more likely to have complications in pregnancy if you have sickle cell disease. Complications and increased risks may include:

Infections, including infection in the urinary tract, kidneys, and lungs, Gallbladder problems, including gallstones, Heart enlargement and heart failure from anemia, Miscarriage, Death, Complications and increased risks for your developing baby may include: Severe anemia, poor fetal growth, preterm birth (before 37 weeks of pregnancy), low birth weight (less than 5.5 pounds), stillbirth and new-born death.

How is sickle cell disease in pregnancy treated?

Pregnant women with sickle cell trait may not have any complications. But the baby may be affected if the father also carries the trait. If you have sickle cell trait, experts advise that your partner should be tested before you become pregnant. Or he should be tested at the first prenatal visit.

If the baby’s father has sickle cell trait, you may need other tests to see if your developing baby has the traitor disease.

Early and regular prenatal care is important if you are pregnant and have sickle cell disease. Having prenatal visits more often allows your healthcare provider to keep a close watch on the disease and on the health of your developing baby., Some women may need blood transfusions to replace the sickle cells with fresh blood. These may be done several times during the pregnancy. Blood transfusions can help the blood carry oxygen and lower the number of sickle cells.

If you get blood transfusions, you’ll be screened for antibodies that may have been transferred in the blood and that may affect your baby. The most common antibodies are to the blood factor Rh., Healthcare providers? don’t advise using the medicine hydroxyurea during pregnancy. This medicine is often used in sickle cell disease. You may be able to take lower doses of this medicine.

Sickle cell disease may affect your developing baby. So, your provider may start testing in the second trimester to check on the health and well-being of your baby., During labor, your healthcare provider will give you IV (intravenous) fluids to help prevent fluid loss (dehydration). You may also get extra oxygen through a mask during labor. A baby’s heart rate monitor is often used to watch for changes in your baby’s heart rate. It also watches for signs of baby’s distress. Most women can deliver vaginally, unless there are other complications.

Prevention

Give your child with sickle cell disease regular doses of a prescribed antibiotic until age 5. Get your child immunized on schedule. For more information, see the topic Immunizations. Avoid contact with anyone suspected of having fifth disease, which is caused by parvovirus. A serious condition called aplastic anemia can develop.

Prevent adult infection

Check your immunization schedule and keep your immunizations up to date. Prevent vision problems. Get routine eye examinations as a child and as an adult. Early detection can help prevent vision problems.

Prevent dehydration

Drink water and other fluids. Drink enough so that your urine is light yellow or clear like water. Drink extra fluids before, during, and after exertion and when in the heat. Drink plenty of fluid if you have a fever or infection. Children should keep a water bottle with them during school, play, and outings.

Key points about sickle cell disease in children

Sickle cell disease (SCD) is an inherited blood disorder that is present at birth. This means it is passed down through a parent’s genes.

With SCD, the red blood cells have an abnormal C shape. They get stuck in small blood vessels and block blood flow.

This blockage can cause pain and lead to infection. It can also damage a child’s major organs, and cause a stroke.

New born are checked for sickle cell disease as part of a group of screening tests.

Most children will start to have symptoms during the first year of life, often around 5 months.

When should you call the doctor?

Get emergency medical care right away if your child has any of these problems: fever of 101°F (38°C) or higher pain that isn’t getting better with medicine, chest pain, severe headaches or dizziness severe stomach pain or swelling, shortness of breath or trouble breathing, extreme tiredness skin that’s yellow or very pale, a penile erection that is not going away or is painful sudden change in vision, seizures, weakness or trouble moving part of the body, slurred speech loss of consciousness (passing out), numbness or tingling.

For further information visit WHO website, Gambian Sickle Cell Association, email: [email protected], address: Serekunda, Suleyman Nimaga Street, Tel: 3671344, 9988814, 3705482.

Dr H Azadeh MD, senior lecturer at the University of The Gambia, clinical director at the Medicare Health Services.

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