One in 10 Bahraini residents may carry the abnormal sickle cell gene, and 1 in 100 may be affected by sickle cell disease.
Despite being one of the most prevalent diseases in Bahrain, sickle cell disease (SCD) does not get due attention because of low awareness levels among public. Dr Shruti Prem, Consultant Hematology at Bahrain Specialist Hospital has called out for the need to increase public education on this disease that can be prevented through prenatal and pre-marital screening.
One in 10 Bahraini residents may carry the abnormal sickle cell gene, and 1 in 100 may be affected by SCD. The high frequency of this abnormal gene in the Bahraini population means that more than 200 babies with SCD can be expected to be born in Bahrain every year. If both parents have sickle cell trait or carry the abnormal sickle cell gene, there is a 1 in 4 chance that their child will have sickle cell disease which can be prevented by prenatal diagnosis.
Patients with SCD have an abnormal haemoglobin (a protein in red blood cells that carries oxygen to the body). While regular blood cells are round in shape and move through the vessels smoothly, the abnormal hemoglobin makes the red blood cells stiff and sticky, changing their shape and making the movement through blood vessels difficult. These sickle cells hinder the flow of oxygen and damage tissues.
Dr Shruti advised that children suffering from anemia since young age, or those who have episodes of unexplained severe pain, infection, fever, or swelling in hands and feet should consult a haematologist to rule out SCD. Strokes, lung damage, and blindness can occur more frequently in SCD than in normal people and lung damage is an important cause of death in SCD patients.
The pain of sickle cell crises is among the most unbearable pain syndromes in medicine. Dr Shruti emphasized that every patient suffering from sickle cell disease should be given an effective and individualized pain management strategy in order to decrease the suffering from this chronic disease. Regular follow-up with a hematologist is important to manage complications from this disease such as chronic organ damage which can develop later in life.
For patients suffering from sickle cell disease, Dr Shruti suggested regular check-ups at least once in three to six months, and emphasized the need for staying well hydrated, maintaining a healthy lifestyle, being up to date with immunizations, and avoiding extreme temperatures, stress, and heavy exertion to decrease the frequency of pain crises.
Patients can also be started on a medication called Hydroxyurea after consultation with a hematologist to decrease complications of SCD. Quitting or avoiding smoking is also very important as lung damage is frequently encountered in sickle cell disease. Newer treatments with gene editing using CRISPR technology have recently been pioneered successfully in SCD and now offer hope for a cure to people suffering from this chronic disease.
Dr. Shruti Prem Sudha
Consultant Hematologist
Bahrain Specialist Hospital
Email: shruti.sudha@bsh.com.bh