AS MENTIONED before sickle cells may block the flow of blood through vessels resulting in tissue damage. Any organ in the body can be affected. However, the common tissues affected include:
Lung - pain in chest with shortness of breath (acute chest syndrome)
Bone - pain episodes (arms, legs, chest and abdomen)
Brain - stroke
Penis - priapism (painful prolonged erection)
Kidney - frequent urination, including bed-wetting, proneness to dehydration, and blood in urine
Skin - leg ulcers.
Sickle cell disease may also cause damage to other organs including the spleen and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections such as pneumococcus and salmonella.
In addition, patients may often complain of being easily tired due to the anaemia, and yellowness of eyes.
DIAGNOSIS AND TREATMENT
Treatment for patients with sickle cell disease starts with early diagnosis, preferably in the new-born period. In Jamaica, infants born to mothers at the Victoria Jubilee Hospital, University Hospital of the West Indies and Spanish Town Hospital are routinely tested at birth to see if they have sickle cell disease. This screening programme is a collaborative venture between the Ministry of Health and the Sickle Cell Unit of the University of the West Indies, Mona. Briefly a sample of the infant's blood from the umbilical cord is placed on special paper and this is then sent to the Sickle Cell Unit at the University of the West Indies, Mona, for testing. At the Sickle Cell Unit testing is done using a technique called haemoglobin electrophoresis. The mothers of infants diagnosed with sickle cell disease are then contacted.
If an individual was not tested at birth then the diagnosis can be determined by having a blood test done. An haemoglobin electrophoresis done on this sample will reveal the diagnosis.
EARLY DIAGNOSIS
It is important to make the diagnosis as early as possible as it allows health care workers to counsel and advise parents about good health practices to reduce the risk of complications as well as to identify problems early. An important health care practice that is taught to the child's caregiver is instructions on how to feel for the spleen.
Children with sickle cell disease may get a complication called acute splenic seques-tration. In this complication the spleen becomes swollen because blood becomes trapped in the spleen. This is a serious complication which may result in death but is easily treated once identified early. Also children with sickle cell disease less than four years need to be given penicillin prophylaxis to prevent infections by the pneumococcus bacteria. At age four years they should be given pneumococcal vaccination.
There is currently no universal cure for sickle cell disease. However, treatment of complications often includes antibiotics for infections, pain management, intravenous fluids, and blood transfusions as appropriate. Additionally, a drug called hydroxyurea is often used to treat the severe form of the disease.
