Sickle Cell Disease Newborn Screening-An Audit of a Twin Island State Pilot Program

Shivon Belle Jarvis; Edda Hadeed; Ketty Lee; Marie-Dominique Hardy-Dessources; Jennifer M Knight-Madden; Claudine Richardson

doi:10.3390/ijns9010014

Sickle Cell Disease Newborn Screening-An Audit of a Twin Island State Pilot Program

Int J Neonatal Screen. 2023 Mar 1;9(1):14. doi: 10.3390/ijns9010014.

Authors

Shivon Belle Jarvis¹, Edda Hadeed², Ketty Lee³, Marie-Dominique Hardy-Dessources⁴, Jennifer M Knight-Madden⁵, Claudine Richardson¹

Affiliations

¹ Paediatric Department, Sir Lester Bird Medical Centre, Michael's Mount, St. John's, Antigua and Barbuda.
² Gambles Medical Centre, Friars Hill Road, St. John's, Antigua and Barbuda.
³ Laboratory of Molecular Genetics and Inherited Disorders of Red Blood Cell, University Hospital of Guadeloupe, Guadeloupe, F.W.I, France.
⁴ Université des Antilles, UFR Médecine/Campus de Fouillolle-Université Paris Cité, Inserm, BIGR, 97159 Pointe-à-Pitre, France.
⁵ Caribbean Institute for Health Research-Sickle Cell Unit, The University of the West Indies, Mona, Kingston 7, Jamaica.

Abstract

The prevalence of Sickle Cell Disease (SCD) within the Caribbean region remains second only to that of West Africa. The Newborn Screening (NBS) Program in Antigua and Barbuda remains heavily dependent on grants, therefore ultimately facing sustainability challenges. Early intervention and implementation of preventative measures post-NBS result in significant improvements in morbidity, quality of life, and survival. This audit reviewed the pilot SCD NBS Program in Antigua and Barbuda from September 2020 to December 2021. A conclusive result was received by 99% of babies eligible for screening, 84.3% of which were HbFA, whilst 9.6% and 4.6% were HbFAS and HbFAC, respectively. This was comparable to other Caribbean countries. Sickle Cell Disease was noted in 0.5% of babies screened, which translates to 1 in 222 live births. Eighty-two percent of mothers were aware of their sickle cell status, compared to 3% of fathers. The importance of instituting a quality improvement team post the initiation of a screening program and the need for a robust public education program have been demonstrated by this audit.

Keywords: Antigua and Barbuda; audit; newborn screening; sickle cell disease.

Grants and funding

This audit received no external funding. CAREST, the European Regional Development Fund, the Interreg Caribbean Program, and AUA were instrumental in the funding that facilitated the SCD NBS pilot program.