Sickle cell disorder (aslo known as Sickle cell Anemia) is a common genetic condition due to a haemoglobin disorder. It is one the most inherited conditions which primarily affects people from African, Caribbean or Asian backgrounds and some from Middle Eastern or Mediterranean backgrounds. Globally, there are more carriers (i.e. healthy people who have inherited only one mutant gene from one parent) of thalassaemia than of sickle-cell anaemia, but the high frequency of the sickle-cell gene in certain areas leads to a high rate of affected newborns.
The easiest way to explain it is that Sickle cell anaemia is a serious condition where the red blood cells become shaped like a ‘C’ or are ‘sickle-shaped’. Normal red blood cells are smooth, round and move easily through the blood vessels carrying oxygen to different parts of the body. Sickle-shaped cells are sticky and stiff so don’t move around the blood vessels easily and form clumps which block up the blood vessels. If blood vessels become blocked they can easily damage, become painful and lead to infection.
Sickle-cell anaemia can be prevented. Couples at risk of having affected children can be identified by inexpensive and reliable blood tests; chorionic villus sampling from nine weeks of pregnancy can be performed for prenatal diagnosis. - World Health Organisation (WHO)
According to the World Health Organisation (WHO) the frequency of the trait in countries such as Ghana and Nigeria is 15% to 30% of all children born annually whereas in Uganda it shows marked tribal variations, reaching 45% among the Baamba tribe in the west of the country. The Nigerian Medical Association (NMA) regards its prevalence as a direct consequence of ignorance and emotion as it affects two out of every hundred children born.
Babies born with Sickle cell Anemia may not show any signs until they are around four to six months old. The typical symptoms are fatigue, pale skin and nail beds, jaundice and shortening of breath. But then it usually gets worse! At some point in their lives children with sickle cell anaemia will experience sudden episodes of pain when the sickle cells become clumped and block up blood vessels. These are known as sickle cell crises and can happen at any time. Some children have only one a year and others can have as many as fifteen. The pain can either be acute or chronic, with acute pain lasting for hours, sometimes days and chronic pain lasting for days or months. Obviously both of these types of pain will be immensely draining and upsetting for a child so is this preventable?
There are many ways to prevent having a child with this condition but the most important one is -Information.
As much as love is important in a union, don't be blinded by it. If you and your partner have decided to take it to the next level then you both need to know if either of you are carrying the trait and the risks invovled.
Nigeria has the largest reservoir of the sickle cell gene in the world. One in every 4 Nigerians or 25% of the entire population of the country has the sickle cell trait (Sickle cell carrier *healthy carriers - Hb AS). Although Nigeria shares this characteristic with other neighbouring countries, its large population of about 160 million sets it apart with regard to the number of persons affected. In numerical terms, a staggering 40 million Nigerians have the trait. This is more than the entire population of any other African country except Zaire.
- The Sickle Cell Foundation Nigeria.
If like a large population of carriers, you are already in a union and pregnant, you will be able to find out the haemoglobin genotypes of your unborn child within 3 months of pregnancy, so that timely and adequate information, support and counseling can be given to them.
Contact your local health provider as soon as you can.
Useful Links
The Sickle Cell Foundation Nigeria
Sickle-cell anaemia Report by the Secretariat WHO
