Sickle Cell Disease: Causes, Symptoms, Management, Prevention!

Sickle Cell

Sickle Cell Anaemia is a very common medical condition in our environment. It has been documented that at least 25% of Nigerians (50 million) has the gene for SCA & about 2-3% (6 million persons) have the disease in a country of 200 million people. So you can see that it is actually very common.

Table Of Content

What is Sickle Cell Disease

This is a medical condition where by there is production of abnormal red blood cells by the body. These cells cannot withstand stress like normal red blood cells, and hence, they crumble under stress.
When this happens, it leads to destruction of these abnormal cells, causing Anaemia (known simply as Low Blood in the body). This anaemia is known as Sickle Cell Anaemia.
Medically means a decrease in RBC/Hb values below normal.

What Causes Sickle cell Disease

The cause of sickle cell disease is due to a defect in the production process of the Red blood cells, where one protein (valine) takes the place of another one (glutamic acid) during the production of the red blood cells.

Just imagine that you are building your family house, and after the drawings and costing. You told the builder to use Aluminum roofing sheets so as to last long (like 20 years) & prevent roof leak. The builder now used Iron roofing sheets instead & after just 3 years of sun & rain, the iron roof cannot withstand the stress & it rusts & start leaking.

When this happens, what do you do? You have to remove the rusted (bad) roofs.

This is exactly what happens in the case of Sickle cell Disease, due to this usage of the wrong protein by the body in the production of the Red blood cells. Instead of the cells to last for up to 120 days like normal cells, these cells which are abnormal then crumble under stress, and when that happens, they are destroyed by the body & lasts less than 90 days.

Normal Red Cells & Sickle Cells
Normal Red Cells & Sickle Cells. Image Credit: MinorityHealth

This destruction of the cells now cause the symptoms of Anaemia (low blood) that we see, & blood is very important in the body to move food, oxygen round the body, and also remove wastes (dirts) that the body does not need. The blood is basically the transport system of the body, so when the amount/volume of blood in your body drops, your body would not be able to function optimally & you would need to do something about it.

What Can make Me have Sickle Cell Disease

Genetics/Family: Sickle cell Disease runs in the blood. It is a hereditary disease. Unlike most hereditary diseases that you can get from just one parent, this one is passed from both father & mother. The mother & father has to carry the genes that cause the disease, before the child can have it. This is why we said, it is very important for prospective couples to run their genotype before marriage. Click here to read on that & see who you can marry to avoid giving birth to children with sickle cell disease.

What can trigger Sickle Cell Crisis

Anything that causes stress to the child can trigger crisis in a person with sickle cell. Examples includes;

  1. Malaria: infection with parasites that causes malaria attacks can trigger crisis in a person with SCA.
  2. Infection: presence of bacteria causing any other infection can trigger crisis.
  3. Fever: which can be from any source apart from malaria can also trigger crisis in a person with SCA.
  4. Dehydration: not taking enough water can also cause crisis.
  5. Physical exertion: when they overwork & get tired, even from sport activities, they can have a crisis.
  6. Extreme weather: Very hot or very cold weather can cause them to have crisis.
  7. Emotional disturbances: just like in Asthma, emotional disturbances such as grief or the loss of someone can trigger crisis.

So, now you see that they need a lot of love & care right?

How do I know a Sickler (Symptoms)

Most persons having this medical condition are called sicklers, due to the fact that they seem to fall sick often & are always having frequent visitations to the hospital. However, it is not right to address them as such. They are normal individuals just like you & I, with a special health condition.
Persons having this condition would show the following often;

  1. Their hands or eyes would usually appear pale or white as if they have no blood in them (due to the destruction of the red blood cells).
  2. They would have yellowing of the eyes, known as jaundice.
  3. They can have very painful swelling of the fingers.
  4. The can also have painful swelling of the back of their hands and feet. A condition known as Hand & foot syndrome.
  5. They are going to be prone to having infections.
  6. You might notice that they have a large fore head, and that their upper & lower teeth does not exactly align (one comes out more than the other).
  7. They would tend to be feeling pain in their bones or abdomen.
    Among other things.
  8. They can have Sustained painful penile erection.

They are also at the risk of developing stroke due to blockage of vessels.

Diagnosis: The diagnosis of sickle cell Disease can be done in different ways, but the best is usually with a test known as Haemoglobin Electrophoresis. You would have to go to the hospital & a doctor would request the test.

What do I do for a person with Sickle cell (Management)

A person with this condition is special & should be handled as such. They would need to visit the hospital regularly for check ups.
They would need to be seen by a doctor who would access their condition & determined if they would need drugs or not.
Treatments are usually given only during crisis.

For those not in crisis: the target would be to prevent crises by;

  • Avoiding their exposure to cold weather and high places.
  • They should take fluid occasionally to prevent dehydration.
  • They would need Folic acid as well as medications to prevent malaria.
  • Vaccination is important like pneumococcal and Hib vaccines that prevent meningitis and other diseases as well as Hepatitis B vaccine.
  • Also, it is essential to carry out proper Health education and genetic counseling especially to prospective couples.
  • Finally, regular check up & follow-up.

Prevention of Sickle Cell Disease

  1. Genetic counseling: is a very important step to prevent sickle cell disease, as the genes that cause the disease is inherited from both father & mother. Hence if one doesn’t have the gene, you cannot have it. Read more about that here.
  2. Prenatal diagnosis: babies in the womb can be tested & identify if they have the genes for SCA, and in countries where it is legal, Selective abortion can be done.

Help spread the knowledge about Sickle Cell Disease & let’s reduce the incidence in our nation.

Authors

  • Obasi Chinedu David attended the College of Medicine, University of Nigeria Teaching Hospital, in ituku-ozalla, Enugu state, Nigeria.

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