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How To Avoid Sickle Cell Anemia.

In recent times, the fear of sickle cell disease is the beginning of wisdom to young couples who see their future together, but when there is a symptom of sickle cell in one, that relationship comes to a halt.

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle Hemoglobin-C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

Sickle Cell Anemia (SS): When a child inherits one substitution beta globin genes (the sickle cell gene) from each parents, the child has Sickle Cell Anemia (SS). Populations that have a high frequency of sickle cell anemia are those of African and Indian descents.

Sickle Hemoglobin- C Disease (SC): Individuals with Sickle Hemoglobin-C Disease (SC) have a slightly different substitution in their beta globin genes that produces both hemoglobin C and hemoglobin S. Sickle Hemoglobin-C disease may cause similar symptoms as sickle cell anemia but less anemia due to a higher blood count level. Populations that have a high frequency of Sickle Hemoglobin-C disease are those of West African, Mediterranean and Middle Eastern descents. Sickle Beta-Plus Thalassemia: Individuals with Sickle Beta Thalassemia (SB) disease also contain substitutions in both beta globin genes. The severity of the disease varies according to the amount of normal beta globin produced. When no beta globin is produced, the symptoms are almost identical to sickle cell anemia, with severe cases needing chronic blood transfusions. Populations that have a high frequency of Sickle Beta Thalassemia are those of Mediterranean and Caribbean descents.

Sickle Hemoglobin-D Disease: Through research, hemoglobin D, which is a different substitution of the beta globin gene, has been found to interact with the sickle hemoglobin gene. Individuals with Sickle Hemoglobin-D disease (SD) have moderately severe anemia and occasional pain episodes. Populations that have a high frequency of Sickle Hemoglobin-D disease are those of Asian and Latin American descents. And finally, Sickle Hemoglobin-O Disease: Hemoglobin O, another type of substitution in the beta globin gene, also interacts with sickle hemoglobin. Individuals with Sickle Hemoglobin- O disease (SO) can have symptoms of sickle cell anemia. Populations that have a high frequency of Sickle Hemoglobin-O disease are those of Arabian, North African and Eastern Mediterranean descents.

The most common of them all sickle cell anemia is an inherited condition that causes a type of faulty hemoglobin in red blood cells. It is the most common blood disorder passed down from parents to children. People with this disorder have a typical hemoglobin molecules called hemoglobin S, which can distort red blood cells into sickles, crescent moon or make them shapeless.

The Red Blood vessel carries oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C shaped farm tool unlike the healthy red blood cell which is round shaped.

The sickle cells die early, which causes a constant shortage of red blood cells and when they travel through small blood vessels, they get stuck and clog the blood flow, this causes pain and other problems like infection, acute chest syndrome and stroke to its victim, which can be known as sickle cell crisis.

Most SCD patients do not manifest clinically till about age of 6 months when the level of hemoglobin F (HbF) begins to fall. They may present with pallor, Jaundice, hepatosplenomegaly and swelling of dorsal of hands/ feet ( hand foot syndrome ) failure to thrive, infections, Sickle cell habits etc

According to WHO, the African region are mostly affected by this disease. Also, the majority of children with the most severe form of the disease die before the age of 5, usually from an infection or severe blood loss. However, I'm countries such as Cameroon, Republic of Congo, Gabon, Ghana and Nigeria, the prevalence is between 20% to 30% while in some parts of Uganda, it is as high as 45%.

A recent study by Nwogoh etal in Benin city, Nigeria, revealed an SCD prevalence of 2.39% and a carrier rate of about 23% in Nigeria.

Most times a person suffering from SCD has some physical attributes that ranges from a normal build to a tall lanky physique depending on the clinical severity. In childhood, sickle cell patients are shorter than normal, puberty is often delayed and considerable growth takes place in late adolescence such that adults with SCD are at least tall as normal. Many of these physical changes are due to the chronic hypothermia associated with severe anemia.

This disease has a great effect on the liver. This effect of SCD on the liver manifests as liver dysfunction often referred to as sickle cell hepatopathy. It occurs predominantly in patients with SCD and to a lesser extent in patients with HbC diseases and HbS Thalassemia. This liver dysfunction encompasses a range of hepatic pathology arising from the primary SCD process and complications of its treatment.

The primary disease process that may lead to liver dysfunction includes anemia, suckling of red cells in the Sinusoid, swollen kuffer cells, fibrin deposits and healing thrombosis leading to obstruction of blood flow in the liver. Consequences of treatment such as blood transfusion and antibiotics therapy can also lead to liver dysfunction complications of multiple blood transfusion like iron overload, acute and chronic infection with hepatitis B and C are also important causes of liver dysfunction in SCD. Third generation Calphalosporins are known to sometimes crystallize in the gallbladder leading to choleithiasis.

However, you can do several things that can keep Sickle cell crisis from occurring: 1) Don't drink a lot of alcohol 2) Don't smoke. If you do smoke, quit 3) Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluids 4) Drink at least eight 12-ounce glasses of water a day during warm weather 5) Reduce or avoid stress. Talk to your doctor if you're depressed or have problems with your family or job 6) Treat any infection as soon as it occurs. When in doubt, see your doctor 7) Wear warm clothes outside in cold weather and inside in air-conditioned rooms during hot weather. Also, don't swim in cold water 8) Try to be positive about yourself 9) Tell your doctor if you think you might have a sleep problem, such as snoring or if you sometimes stop breathing during sleep 10) If you have another medical condition, like diabetes, get treatment and control the condition 11) If you are pregnant or plan to become pregnant, get early prenatal care and 12) Only travel in commercial airplanes. If you have to travel in an unpressurized aircraft, talk to your doctor about extra precautions.

Most institutions in Nigeria lack day care settings for management of sickle cell crisis, however, this high mortality disease can be managed with antibiotics, blood transfusion also known as stem cell transplant. Bone marrow transplant is ultimately the only known cure for Sickle cell disease, but finding a donor is often difficult and the procedure could be costly and sometimes life threatening complications may be involved.

New SCD carriers can be avoided through premarital screening and genetic counselling, prenatal diagnosis, preconceptional diagnosis and implantation of normal embryos after in vitro fertilisation, and in utero therapy using stem cell transplantation. Prevention of the disease through carrier identification and genetic counselling remains the only realistic approach to reduce the impact of the disease and allows better use of available resources in the low-income countries where the condition is most prevalent. Programmes of population screening and genetic counselling can have a major impact on the birth rate of children with SCD and other genetic diseases. The prospective control of SCD by heterozygote detection through premarital screening, which is vital to the identification of the couples at risk, is of utmost importance. The success of such a programme to a large extent depends on the SCD knowledge in the community, the understanding of the full consequences of having a sickler child, and people's attitude towards genetic screening and counselling.
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Publication:Daily Independent
Date:Jul 23, 2021
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