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Sickle Cell Disorder And How It Concerns You (Part 2)

By Ini Akpan
01 October 2023   |   11:40 am
‘Hey, you, come here!’ Frank, a JSS 2 student of Howard Community School intoned loudly. ‘Come here quickly!’ His command was directed at Cyprian, a 14-year-old SS1 student who looked much like a 10-year-old JSS1 student in stature. It was not uncommon for him to be mistaken for a junior student, and he continued strolling…

‘Hey, you, come here!’ Frank, a JSS 2 student of Howard Community School intoned loudly. ‘Come here quickly!’
His command was directed at Cyprian, a 14-year-old SS1 student who looked much like a 10-year-old JSS1 student in stature.

It was not uncommon for him to be mistaken for a junior student, and he continued strolling to his dormitory. Soon enough, the uncouth junior student would realise his mistake.

Delayed puberty and stunted growth is a common phenomenon in persons living with sickle cell disease. In this article, we will take a look at the second key difference between persons living with SCD and those who do not.

The first key difference, which we discussed last week, is the shape of their red blood cells (RBCs). The second key difference is the rate at which these red blood cells die off.
If you are a healthy human being without the sickle cell disorder, your RBCs usually last for about 120 days before dying off. Also, your body produces about two million (2,000,000) red blood cells are produced every second to replenish old ones.

If you have sickled blood cells, however, the lifespan of your RBCs is about 10 to 20 days. Notice the huge difference.

In a person living with SCD, the sickled blood cells are usually dying off faster than the body can produce new cells. This means that this person often has a shortage of red blood cells. This is why the stable packed cell volume (PCV) in an adult sickle cell ‘warrior’ can be as low as 15 per cent instead of 35.5 per cent to 44.9 per cent for females and 38.3nper cent to 48.6 per cent for males.

This low blood volume is what is usually referred to as anaemia. According to WHO, ‘Anaemia is a condition in which there is a reduced number of red blood cells or the haemoglobin concentration within the red blood cells is lower than normal.

Haemoglobin is needed to carry oxygen and if you have too few or abnormal red blood cells, or not enough haemoglobin, there will be a decreased capacity of the blood to carry oxygen to the body’s tissues.’
Sickle cell anaemia is different from other forms of anaemia and is often lifelong. It leads to a number of complications including:
Tiredness
Irritability
Dizziness and light-headedness
Fast heart rate
Difficulty breathing
Jaundice (yellow colour to the skin, lips and whites of the eyes)
Insomnia
Slow growth and delayed puberty, etc.
While anaemia is very common in SCD, it is not ideal and here are some tips to help you boost your haemoglobin levels:
1. Eat foods rich in folate such as leafy green vegetables such as pumpkin leaves and spinach (‘green’), citrus fruits (like oranges), beans, bananas, avocado, beef liver, cereals, rice and eggs.
2. Eat foods rich in proteins because they aid cell regeneration.
3. Take food supplements like folic acid and Vitamin B Complex under your doctor’s supervision. Your haematologist might also prescribe hydroxyurea to help your body produce more foetal haemoglobin.
4. Protect yourself from blood depletors like malaria and infections.
5. Blood transfusions when necessary.
Please note that taking iron supplements is not recommended for people with sickle cell disease. This type of anaemia is not caused by too little iron in the blood, it is caused by not having enough red blood cells. In fact, taking iron supplements could harm a person with sickle cell disease because the extra iron builds up in the body and can cause damage to the organs.

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