Sickle-cell disease also known as sickle-cell anaemia (or drepanocytosis), is a genetic life-long blood disorder characterised by red blood cells that assume an abnormal, rigid, curved (sickle) shape.
Normal haemoglobin is called haemoglobin A, but people with sickle cell disease have only haemoglobin S, which turns normal, round red blood cells into abnormally curved (sickle) shapes.
Haemoglobin is an iron containing protein that forms about 97 per cent of the red blood cells when dry. Haemoglobin transports oxygen from the lungs or gills to the rest of the body (for example; the tissues) where it releases the oxygen for use by the cells.
Normally, a person inherits two copies of the gene that produces beta-globin, a protein needed to produce normal haemoglobin (haemoglobin A).
A person with sickle cell trait inherits one normal beta-globin gene (A) and one abnormal gene (S) from both parents, respectively. The sickling occurs because of a mutation in the haemoglobin gene.
The sickling shape assumed by the red blood cells decreases the cells' flexibility and results in a risk of various complications.
Some of these complications are decreased immune reactions, leg ulcers, stroke, chronic renal failure, chronic pain, bacterial bone infection, intrauterine growth retardation, spontaneous abortion, and pre-eclampsia in those who become pregnant, among many others.
Life expectancy is shortened in the sickle cell patient. Some studies report an average life expectancy of 42 and 48 years for males and females, respectively in the developed world.
About two per cent of babies born in Ghana every year have sickle cell diseases which need to be managed properly to avoid infant mortality. Without proper medical diagnosis and treatment, most of these children die by the age of five years, with malaria, chest infections and anaemia being the major causes.
The sickle cell child needs to be closely monitored as a neglect of any form can result in crises or complications that will lead to death. Their almost always failing health and sickness puts a lot of stress on their parents and guardians.
Visits to the hospital are a commonplace and very necessary. Medications that they are supposed to take should be given the necessary attention. Sickle cell patients should observe all the rules governing their condition.
Being a life-long condition, a good diet plays a role in their health and well-being. This is because; eating (feeding) is a life-long activity as well.
Every child deserves to be fed well so they grow well and healthy. The child with sickle cell can grow well and be brilliant in school if they are getting the kind of nutrients they need from their food.
Extra calories needed
Children with sickle cell disease use more energy or calories than other children. Some researchers say that they may be using about 20 per cent more calories than other children.
Again, research shows that children with sickle cell disease do not take enough calories. During sickness of all forms, the body needs a lot of energy to heal itself and so do they.
To make for the extra energy need, they should be given snacks in addition to their main meals in order to get more energy.
Snacking on fresh fruits, vegetables, bread, pasta and cereal mixes are acceptable. Snacking on foods that contain a lot of sugar is not acceptable.
Having adequate foods to make for the rise in caloric requirements of the sickle cell child helps solve the problem of delayed growth and stunting.
A diet adequate in proteins is very important. The loss of blood resulting from death of sickle shaped red blood cells leads to anaemia. Protein is needed to rebuild the blood cells. Enough protein is needed for catch up growth as well.
Other nutrients are also very vital and therefore a balanced diet must be consumed by sickle cell patients.