Know Your Sickling Status To Prevent Complications – Pediatrician

Dr Audrey Frimpong-Barfi, Paediatrician at the Tema General Hospital, has urged Ghanaians to know their sickling status to help avoid future complications.

Sickling is a blood-related condition where the haemoglobin inside red blood cells sticks or clumps together, causing the cell to become fragile, rigid, and crescent—or sickle.

Dr Frimpong-Barfi, explaining the difference between blood group and sickle cell said, "sickle cell disease is an inherited red blood cell disorder that changes the normal shape of the red blood cell to look like a crescent shape farm tool called sickle, given its name sickle cell”.

“Blood group refers to the type of markers found on the surface of the red blood cells,” Dr Frimpong-Barfi told the Ghana News Agency in an interview in Tema and explained that everyone has a blood group, others may have a sickle cell with it and others do not.

Dr Frimpong-Barfi, who is also the head of Paediatrician Department at the Tema General Hospital, said a person with ‘AS’ is a sickle cell carrier (sickle cell trait)

She noted that “if two ‘AS’ should get married, the likelihood of these couple having a baby with sickle cell was 25 per cent; 50 percent chance for AS and another 25 percent for AA for every pregnancy”.

Dr Frimpong-Barfi further noted that it was vital for everyone to know their sickle cell status and not just for sickle cell as there were other types of sickle cell diseases.

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

Sickle Cell Anaemia (SS): When a child inherits one substitution beta globin genes (the sickle cell gene) from each parent, the child has Sickle Cell Anaemia (SS).

Populations that have a high frequency of sickle cell anaemia are those of African and Indian descent.

Sickle Haemoglobin- C Disease (SC): Individuals with Sickle Haemoglobin-C Disease (SC) have a slightly different substitution in their beta globin genes that produces both haemoglobin C and haemoglobin S.

Sickle Haemoglobin-C disease may cause similar symptoms as sickle cell anaemia but less anaemia due to a higher blood count level. Populations that have a high frequency of Sickle Haemoglobin-C disease are those of West African, Mediterranean and Middle Eastern descent.

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.

Populations that have a high frequency of Sickle Beta Thalassemia are those of Mediterranean and Caribbean descents.

Sickle Haemoglobin-D Disease: Through research, haemoglobin D, which is a different substitution of the beta globin gene, has been found to interact with the sickle haemoglobin gene.

Individuals with Sickle Haemoglobin-D disease (SD) have moderately severe anaemia and occasional pain episodes. Populations that have a high frequency of Sickle Haemoglobin-D disease are those of Asian and Latin American descents.

Sickle Haemoglobin-O Disease: Haemoglobin O, another type of substitution in the beta globin gene, also interacts with sickle haemoglobin. Individuals with Sickle Haemoglobin- O disease (SO) can have symptoms of sickle cell anaemia.

Populations that have a high frequency of Sickle Haemoglobin-O disease are those of Arabian, North African and Eastern Mediterranean descent.

She said, however, that, there was also another type called Thalassemia - an inherited blood disorder that affects the way the body makes haemoglobin.

According to Dr Frimpong-Barfi, instead of just testing for sickling, it was preferable to do Haemoglobin electrophoresis which can test for HbS and the other types such as C, B, D and E, the sickling test only shows S.

"When persons with AS and AC give birth, the child can be SC," she said.

Dr Frimpong-Barfi said, it was not just enough testing for sickling before marriage that mattered but rather undertaking haemoglobin electrophoresis, and stressed, even with that, the Haematologist would say it is not enough when it comes to testing for Thalassemia and the other forms.

According to her, sometimes, some parents get confused when their children have sickle cell because the husband may be AS and the wife AA but it could be as a result of S beta thalassemia behaving like SS.

Dr Frimpong-Barfi advised that immediately a child was born, it was very necessary for sickle cell test to be conducted on the child, because early detection would mean early management and most of the complications would be taken care of.

"I am happy the Government is trying to introduce newborn screening for sickle cell as a national project and it is now being rolled out and soon it would be scaled to quite a number of places in Ghana," she said.

She added that, there were a lot of medications coming up and currently in Ghana, hydroxyurea had been introduced and this would help reduce number of times crises occur, admissions and the number of times they get blood transfusion.