Sickle Cell screening programme to commence in SVG

In the upcoming months, a pilot programme founded by a Vincentian doctor to screen newborn babies for sickle cell disease will be implemented.

This was announced by Dr Shelly-Ann Williams (founder), who is a paediatrics fellow in the Division of Neonatal-Perinatal{{more}} Medicine at the Medical University of South Carolina (MUSC).

Dr Williams made the announcement at a symposium held on Saturday, September 6 at the Peace Memorial Hall.

“Our goal is to have all babies tested because we think it’s important to know what the true incidence of sickle cell disease is,” she explained. “We know it is the first step to improve the care of patients with sickle cell disease in St Vincent and the Grenadines,” Dr Williams said.

The programme, which will initially run for a year, will offer free screening of newborn babies for the disease. Blood samples will be sent to a laboratory in South Carolina, and results will be relayed to parents through their local baby clinics.

All babies who are identified as having sickle cell will be seen by clinic doctors, and started on prophylactic antibiotics to prevent infections. Additionally, parents will receive education and counselling on how to cope with the disease.

Dr Williams said: “Screening, coupled with comprehensive care, has been shown to decrease morbidity and mortality of sickle cell disease across many different populations of different ethnicities.”

Experts in the disease say that it mostly afflicts those of African, Caribbean, Arabic, Greek, Italian, Latin American and Indian descent.

Dr L Miguel, who also gave an address at the symposium, said that from January 2009 to April 2014, there were 626 reported cases of sickle cell disease. 418 were females and 208 were males.

In that period of time, there were five deaths (four males, one female), of which 50 per cent were attributed to Acute Chest Syndrome, a complication of the disease.

Dr Miguel pointed out that the actual numbers could have been higher because many people mistake their symptoms for arthritis or other conditions, and would not have sought medical attention.

Regionally, the incidence of sickle cell in newborns is 1:150 in Jamaica, 1:304 in Guadeloupe, 1:200 in St Lucia and 1:238 in Tobago.

Dr Julie Kanter, assistant professor in the Department of Paediatrics at MUSC, in her feature address emphasized the importance of the pilot programme in improving and extending the lives of SCD patients in SVG.

Globally, males with the disease have an expected life span of 53 years, while females have an expected life span of 58 years.

Dr Kanter said that the disease, which is inherited, is much more than a simple disorder of “oddly shaped cells.”

“Sickle cell disease is a complex multi-symptom illness,” she pointed out.

Dr Kanter further explained that given the disease’s complexity, there is no one universal treatment that will work successfully for every patient – rather, several treatment options that patients can combine with medication.

She said that Hydroxyurea – a drug that increases fetal haemoglobin production, which reduces the chance that red blood cells will sickle – is proving to be an increasingly effective treatment in children with three or more flare-ups or crises per year.

Additionally, Dr Kanter said that although it is commonly reported that there is no cure for sickle-cell disease, a bone marrow transplant does cure the condition. However, it is not suitable for every patient.

A bone marrow transplant offers a 85 – 89 per cent chance of a cure, but requires an entire year for the procedure to be administered and for the patient to recover fully. It was discovered by accident when a 10-year old sickle-cell patient received a bone marrow transplant for leukemia, and doctors realised that it has also cured the blood disorder.

Dr Kanter also said that she would be very interested in setting up a sickle cell treatment centre in SVG.

“Sickle-cell patients really shouldn’t be treated in the emergency room. They need a specialised provider who knows exactly how to treat sickle-cell disease.”

The project is being funded through a grant that Dr Kanter received as the director of sickle-cell disease research at the MUSC.(JSV)