What is hydroxyurea?

Normally, our red blood cells are flexible and round-bodied which makes them easily pass through the smallest of blood vessels to supply enough oxygen to the body tissues.

In sickle cell anaemia, the red blood cells become rigid, sticky and sickle-shaped, which causes them to block blood vessels leading to failure among others to supply oxygen. They are then broken down earlier than their lifespan; say in about 10 to 20 days instead of the usual 120 days for men and 110 days for women, leading to anaemia.

Hydroxyurea is also known for treating blood cancers (leukaemia) and skin conditions such as psoriasis but lately, it is being used to treat sickle cell anaemia, although on prescription. Each sickle cell patient requires individualised treatment depending on how severe the disease is, complications and associated disease conditions. 

Faetal haemoglobin made up of two alpha and two gamma protein unit chains is the major haemoglobin in faetal red blood cells during pregnancy but by six to 12 months after the birth of the child, it is almost completely replaced by adult haemoglobin with two alpha and two beta chains.

In sickle cell anaemia, the beta chains, the glutamic amino acid is replaced by Valine, leading to the likelihood of sickle and lead symptoms in times of low blood oxygen content.  

The faetal haemoglobin has no Beta chains and may not have a likelihood of sickle when the blood oxygen levels are low.

Hydroxyurea increases the amounts of foetal haemoglobin and, therefore, helps in the prevention of sickling and related symptoms.

Today, as a result of better preventive measures or other treatments, a person with sickle cell disease can live longer. Please keep in touch with your doctor or sickle cell clinic for effective care of your offspring.