By: Mavis Offei Acheampong
Research on sickle cell anemia is on the front burner, because of new drug therapies, cures through stem cell transplantation and the promise of gene therapy. Approximately 5% of the world’s population carries trait genes for this haemoglobin disorder – sickle cell.
Ghana’s Navrongo Health Research Centre in the Upper East Region is therefore adding up to research studies on sickle cell in Ghana.
The two-year study will involve screening of children under five years and also right from birth in the Kassena Nankana District in the Upper East Region to know their sickle cell status.
Sickle cell disease (SCD), is an inherited condition caused by abnormal haemoglobin that damages and deforms the gene in the red blood cells, and this results in a person becoming anaemic.
Haemoglobin SS, SC, SB+ (beta) and SB 0 (Beta-zero) are the four main types of sickle cell anaemia that causes different mutations in the genes. A person will be born with sickle cell disease only if, two of these genes are inherited, meaning one from the mother and another from the father.
A person who inherits just one gene is healthy and not anaemic, but said to be a “carrier” of the disease. But a person who has both is said to be Sickle Cell anaemic, or has the Sickle Cell trait, commonly known as SS.
Periodic episodes of extreme pain, are a major symptom of sickle cell anaemia. This condition can cause organ damage, stroke and even death. However, doctors say people with sickle cell anaemia can live normal lives if they stay healthy, hydrated and with moderate exercising per their strength.
The Study Clinician for the Sickle Cell Project at the Navrongo Health Research Centre, Dr. Lucy Osei Ababio, said Sickle Cell is not a death sentence. She said there are interventions and more people to living to show that one can live a normal life.
“People diagnose with sickle cell should understand what the condition is all about, the follow up clinic, the interventions and adhere to the medication given. With that they should be able to live normal lives without complications and issues” she said.
In Africa, sickle cell disease (SCD) is a major public health problem with over 200,000 babies born per year with the condition. In Ghana, approximately 15,000 (2%) of Ghanaian new-borns are diagnosed with SCD annually. From studies, most people with sickle cell disease (SCD) in Ghana often go undiagnosed until age 4 and during a pain crisis.
These findings reflect the importance of new-born screening programmes, which are lacking throughout the country. For this reason, the two-year study being conducted by researchers at the Navrongo Health Research Centre in the Upper East Region, for the Kassena Nankana District, is to help identify the sickle cell status of children from age zero, instead of 8 months, as has been the case all along.
A screening of the blood sample is done after a parent’s consent using the gazelle that gives results within 8 minutes. Unlike the HP electrophoresis that takes hours the new test with the gazelle is instant and the sickle scan takes less than one minute at the clinic.
Dr Lucy Ababio said the fact that these children do not have to wait till complications sets in before they are tested to find them out and put on medications, they are picked up immediately they are born.
“Sickle cell is seen as a silent killer. The child can be fine, may not have crisis but complications are silent and by the time it comes up it is too late. So we are able to get them early at a time when they are healthy and put them on medication and interventions given to them to live normal lives. We follow up on them when they don’t attend the clinic through home visits. We don’t want to lose them because they refuse to attend the clinics. Before we started the clinic most of the children were in hiding. We had lost most of the children to complications and it was heartbreaking to see that”
About 10 communities with 6000 children within the Kassena Nankana District in the Upper East Region are expected to be rolled onto the study. With the high rate of sickle cell patients in the Communities within the Kassena Nankana District in the Upper East Region, Study Clinician for the Sickle Cell project at the Navrongo Health Research Centre Dr. Lucy Osei Ababio was optimistic the project will help reduce the disease burden in the region. She said parents will no longer travel to the south for treatment.
“Before we started the clinic, sickle cell parents will have to go to Accra and Kumasi for the clinic and medication since most of the testing is done in either Korle Bu Teaching Hospital or Komfo Anokye in the Ashanti Region.
Parents have to borrow or contribute before they can take their children to the south. But now the Navrongo Health Research Centre (NHRC) through the study is supporting us to buy the medication for the children under the project.
The hydroxyurea comes in the form of capsules which was for children and older adults and parents have to pay for it and this last them between one to three months but with the presence of the clinic Novartis has brought us a new type which is dispersible which can be given to children as early as 9 months when we start them on hydroxyurea so these children will not have to pay for the drugs go to Accra for drugs.
The hydroxyurea capsule is on the National Health Insurance (NHIS) and we will still appeal if the dispersible ones can also be on the National Health Insurance (NHIS) so that every other child who may not have been on the study even after the study can have the medication to be saved.
She said there is now an overall improvement in the health of the children with less crisis. Currently, there are sponsors who are providing the logistics needed for the testing and needs government to support the communities even after the study.
Stem cell or bone marrow transplants are the only cure for sickle cell disease now, but this is not done very often because of the significant risks involved.
Current treatment options for Sickle Cell focus largely on best supportive care, including blood transfusions and pain medication.
Researchers believe outcomes of the Kassena Nankana District study will propel duty bearers and Government to further introduce it widely throughout the country to prevent mortality in children under five with Sickle Cell disease.
People often consider sickle cell anaemia to be a fatal condition, but recent advancements in care means that it is possible to survive it into adulthood.