The universal screening programme is part of a thrust by the government to develop a strong framework for the early detection, diagnosis and treatment of persons suffering from the debilitating condition. SCD is one of the priority conditions that have been included in the government’s Strategic and National Action Plan for the prevention and control of non communicable diseases (NCDs) 2013 to 2018.
Universal newborn screening for SCD is strongly advocated by experts in the area, including global leader in SCD and Director of the Sickle Cell Trust, Professor Graham Serjeant. The expert, who is the foremost authority on the clinical manifestations and care of patients with SCD, diagnosed over 300 patients at birth while screening 100,000 babies at the Victoria Jubilee Hospital between 1973 and 1981.
He said the findings from the original Jamaican study proved that newborn screening is essential in preventing many of the complications associated with SCD.
“We learned and continue to learn a huge amount about the disease (from that cohort study) . . . Newborn screening helps in the prevention of early death caused by sudden enlargement of the spleen. The Jamaican study showed that by teaching the parents how to detect this complication in their children and what to do about it, deaths could be prevented,” Serjeant pointed out.
The screening programme, being implemented by the Ministry of Health, involves collaboration with the Sickle Cell Unit (SCU) at the University of the West Indies (UWI); National Health Fund (NHF); Sickle Cell Support Foundation; and the Sickle Cell Trust.
The SCU will be carrying out the screenings through a J$10 million (US$83,755) grant from the NHF.
Director of the SCU, Professor Jennifer Knight-Madden, said testing pregnant women and newborns is the first phase of the initiative.
She explained that the focus is on newborns “for the simple reason that the highest chance of dying from sickle cell disease is during the first three years of life.”
SCD is a recessive genetic blood disorder, resulting from inheriting abnormal haemoglobin genes from both parents. It is characterized by the red blood cells assuming an abnormal, rigid, sickle shape. This decreases the flexibility of the cells, blocking blood flow to the limbs and organs, which can cause pain, serious infections, organ damage, stroke and there is an increased risk of death. More than 10 per cent of Jamaicans have the sickle cell gene and the condition affects one in every 150 newborns.
Children who are not diagnosed for SCD, are at risk of experiencing preventable SCD complications, including stroke, and Knight-Madden said six per cent of children between the ages of five and 15, who have the disease are at high risk of having a stroke.