The sickle cell disease — not a mysterious one

Monday, May 12, 2008 (Daily Graphic, Pg 27)
By Lucy Adoma Yeboah

SIXTEEN-year-old Master Gideon Asante was forced to abandon school at secondary level because both his teachers and schoolmates continually made fun of him for often complaining of ill health, a situation he felt too stressful to bear. Gideon was a sickle cell patient who often had crisis because conditions in the boarding school was not good for his health.
In another scenario, Maame Afia Asantewaa was considered a witch among the people she lived with in Ntowkrom. The reason was that she lost four out of the six children she had when they were very young. The accusation continued until one of the surviving children was tested and found to be suffering from the Sickle Cell disease, a disease which probably might have claimed the lives of all the four children who may not have had proper care.
The Sickle Cell disease is neither a new disease nor is it a mysterious one. It is not caused by evil spirits and it is certainly not a curse. The disease is not contagious. It does not change into another disease later in one’s life.
The Sickle Cell disease is not a condition one grows out of, yet it is not a disease that must kill in childhood. It is not a disease of only people of Africa and it is also certainly not a disease of only black people.
Due to the seriousness of the disease in mostly Africa, there are a number of groups that try to educate people on the issue and among them is an advocacy group called the Federation of Associations of Sickle Cell Societies with its headquarters in Nigeria.
As the group celebrates the annual Africa Day of Sickle Cell on May 10 this year, it has called on all Ghanaians, as well as the government and institutions, to be committed to Ss alleviating the plight of Sickle Cell patients.
According to the Sickle Cell Disease Association of America, the disease is an inherited blood disorder that affects red blood cells. People with the Sickle Cell disease have red blood cells that contain mostly haemoglobin “S”, an abnormal type of haemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.
It states that when sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications. There is currently no universal cure for the Sickle Cell disease.
Types of Sickle Cell Disease
There are several types of the Sickle Cell disease. The most common are: Sickle Cell Anaemia (SS), Sickle-Hemoglobin C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.
What is Sickle Cell Trait?
The Sickle Cell trait (AS) is an inherited condition in which both haemoglobin ‘A’ and ‘S’ are produced in the red blood cells, always more ‘A’ than ‘S’. The Sickle Cell trait is not a type of Sickle Cell disease. People with Sickle Cell traits are generally healthy.
Inheritance
Sickle Cell conditions are inherited from parents in much the same way as blood type, hair colour and texture, eye colour and other physical traits. The types of haemoglobin a person makes in the red blood cells depend on what haemoglobin genes the person inherits from his or her parents. Like most genes, haemoglobin genes are inherited in two sets; one from each parent.
If one parent has Sickle Cell Anaemia and the other is Normal, all of the children will have sickle cell trait.
If one parent has Sickle Cell Anaemia and the other has Sickle Cell Trait, there is a 50 per cent chance (or one out of two) of having a baby with either the Sickle Cell disease or a sickle cell trait with each pregnancy.
When both parents have the Sickle Cell Trait, they have a 25 per cent chance (one out of four) of having a baby with sickle cell disease with each pregnancy.
A simple blood test followed by a laboratory technique called Haemoglobin Electrophoresis will determine the type of haemoglobin one has.
When you pass an electric charge through a solution of haemoglobin, distinct haemoglobin move different distances, depending on their composition. This technique differentiates between normal haemoglobin (A), Sickle haemoglobin (S), and other different kinds of haemoglobin (such as C, D, E, etc.).
Medical Problems
Sickle Cells are destroyed rapidly in the body of people with the disease causing anaemia, jaundice and the formation of gallstones.
The Sickle Cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism (painful prolonged erection). It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes Sickle Cell disease patients, especially young children, easily overwhelmed by certain bacterial infections.
Treatment
Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn and includes penicillin prophylactics, vaccination against pneumococcus bacteria and folic acid supplementation.
Treatment of complications often include the use of antibiotics, pain management, intravenous fluids, blood transfusion and surgery, all backed by psycho-social support. Like all patients with chronic disease, patients are best managed in a comprehensive multi-disciplinary programme of care.
Discovered and studied during the first half of the 20th century, Sickle Cell disease is still not very well known and appreciated by the population of Africa where it constitutes the number one genetic disease.
The disease is considered the number one cause of mortality among those less than five years of age and also tops the list, although it is not counted among the six childhood killer diseases.
Sickle Cell is a major cause of absenteeism and low productivity, and causes a lot of harm to the development of countries where it renders unproductive, their productive labour force.
Due to its serious progression and debilitating character, the morbidity that it brings in its wake, makes it a very serious public health problem.
Of every million children born in Ghana, no less than 30,000 inherit an abnormal gene relating to haemoglobin formation from both parents resulting in the disease. More than 20,000 of this number are likely to suffer from the sickle cell disease. – (F.I.D. Konotey-Ahulu-The Sickle Cell Disease Patient, 1991).
In Ghana, the Sickle Cell disease is considered by the public as a rheumatic disease, recognised in various languages and is described based on the grinding bone pain experienced by the patients.
Some of the local names are ‘twetweetwe’ in Ga, ‘sasabro’ in Twi and ‘Nudui” in Ewe.
Some of the untruths that are making the rounds are that only black people have Sickle Cells; that patients never live beyond the age of 20; that sickle cell patients have a low Intelligent Quotient ( IQ) and are therefore incapable of higher education. Another untruth is that people with Sickle Cell traits stand the danger of having ‘sickle cell crisis’ when they fly in unpressurised aircraft and that sickle cell diseased women cannot have children.
Impact
According to health experts, by the nature of the disease, hospitalisation is quite frequent if parents and patients do not have any knowledge of the management of the disease at home.
Families living with the disease are mostly in the low-income group and therefore, struggle to meet the high cost of admissions and treatment. Sickle Cell patients are not given any considerations at all in the payment of medical and laboratory bills and because they cannot afford these high bills, they are not sent to hospitals until their condition becomes very critical.
For those who have to be transfused, which is a common treatment option, it is a major problem accessing blood.
In view of the above, there is the need for well set-out health centres in at least all regional capitals to cater for the medical and psycho-social needs of sickle cell patients. They need to get some relief from this painful disease.