Haemoglobinopathies - Sickle Cell Anaemia

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Haemoglobinopathies are groups of genetic diseases that occur as a result of qualitative or quantitative alteration in the haemoglobin molecule present in blood. They can also occur due to structural defect or incomplete synthesis of amino acids in the polypeptide chain.

Today we'll be looking at the most important haemoglobinopathy, the HbS disease also known as Sickle Cell Anaemia

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This disease is caused by substitution of Glutamate with Valine in position 6 of Beta-globin chain.

The presence of this Valine causes polymerization of the haemoglobin which forms a gel like structure. This gel like structure gives rise to a network of fibrous polymers that stiffen and distort the cell, producing erythrocyte with sickle shape. That's why the condition is known as Sickle cell anaemia.

Blockage of the blood vessel(vaso-occlusion) causes pain in the patient. The cells within these vessels are denied oxygen and subsequently, they die. This results in sickle cell patients always losing blood. Loss of blood makes them very susceptible to diseases, and weak at all times. Most times, they die before age 30.

This is something that is very important for kids and parents. To be able to prevent this, we have to know the cause. Sickle cell anaemia as I said before is genetic. What that means is that it is gotten from parents. When carriers of the disease mate, they give birth to a child that is sickle cell.

The implication of this is, no matter how much you love a person, if both your genotypes are AS, it's best you break up with the person. It is also important to know your genotype so you don't mate with the wrong person out of ignorance.

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For those who have the disease already, don't feel too bad. There are still certain things you can do to manage yourself. Some of them are:

• Drink Enough Water: Increased hydration makes red blood cells absorb water, thereby reducing sickling.
• Use aggressive antibiotic treatment as WBC may not be strong enough to fight against diseases.
• Use of Analgesics to reduce pain

Dear parents, teach these things to your children. Kids, please take note of this and heed my advice. It'll be wickedness to be aware of the implications yet go ahead to mate with a person who we're not compatible with just because of love because we'll end up giving birth to a child that'll feel pain all the days of his or her life .

I hope we've learnt something today.