Implementation of hospital-based sickle cell newborn screening and follow-up programs in Haiti

Abstract

One in 120 children are born with sickle cell disease (SCD) in Haiti. However, health care challenges include isolated newborn screening (NBS) activities and lack of transcranial Doppler (TCD) ultrasound to assess stroke risk. The implementation activities of the Comparative Study of Children in Haiti and Miami with Sickle Cell Disease involved both NBS and TCD ultrasound implementations at 4 Haitian clinical sites. We hypothesized that hospital-based newborn SCD screening and follow-up programs would be feasible at Haiti. A traditional NBS laboratory method with dried blood samples was performed at 3 Port-au-Prince sites, and the traditional method plus point-of-care (POC) testing was used at the 2 northern sites. The rate of clinical follow-up for newborns with SCD as the outcome for the NBS intervention was compared with that of the NBS method. The NBS programs identified SCD in 0.77% of 8224 newborns over a 24-month period. In the rural hospital assigned to the combination screening, 56% of newborns identified with POC testing returned for follow-up, compared with 0% when POC was not available (P = .044). Newborns who tested positive for SCD and children aged <6 years with SCD at the clinical sites were eligible for study follow-up. Accrual was successful: 165 participants (mean age, 42 months; 53% males; 93% hemoglobin SS) were recruited and received oral penicillin. TCD ultrasound screening was hampered by poor internet connections and trained staff leaving Haiti, with only 1 active site conducting screening. Despite challenges, the implementation of NBS and sickle cell programs in Haiti is feasible. We are in the process of understanding how to mitigate implementation limitations.

Graphical abstract

Figure 1.

Haiti and the study environment. Port-au-Prince is Haiti’s capital, with 2.9 million inhabitants (2022 data) with a poverty level of 87.6% and 30.3% of the population living under the extreme poverty line. Social factors include street violence and suboptimal literacy rate for Haiti, in general, with 61.7% literacy for individuals aged ≥15 years and 83% literacy for the subgroup of individuals aged 15 to 24 years. SDH, the only pediatric hospital in Haiti, has a 224-bed capacity. The hospital serves 1000 children with SCD, from infancy to age 12 years. The SDH laboratory has a Sebia capillary hemoglobin electrophoresis machine and a Perkin Elmer IEF machine in use for NBS. HUEH does not have capacity for hemoglobinopathy evaluation and outsources samples to SDH. Thirty children with SCD are examined at HUEH. JUH is located at Cap Haitien, Haiti’s second largest city after Port-au-Prince, with 190 000 inhabitants. This program has a nurse coordinator and CHW, serving 30 children with SCD. Located at Milot, a rural town of 25 100 inhabitants, 11 miles south from Cap Haitien, SCH does not have hemoglobinopathy laboratory equipment; >200 children are followed up at SCH. None of the 4 hospitals had a TCD ultrasound machine before the CSHSCD started. Hydroxyurea was purchased from outside vendors (rarely) or supplied by sponsors. Before the CSHSCD, penicillin was administered by intramuscular injection every month as pneumococcal prophylaxis for children aged <5 years.

Figure 2.

CSHSCD network. This figure shows the descriptions, location, NBS methods (laboratory analysis alone or with POC), and expected laboratory flow of the different entities. The University of Miami is the US private University SCD Center. The US state NBS laboratory is in Florida. SCH is the private hospital in rural Haiti, which is supposed to send NBS samples to JUH, the public hospital in medium-sized Haitian city, Cap Haitien. The public general hospital in Port-au-Prince (large city) is HUEH that sends NBS samples to the SDH, the private children’s hospital also in Port-au-Prince.

Figure 3.

POC devices used to identify or quantify hemoglobins. Sickle SCAN (BioMedomics) detects hemoglobins A, S, and C, and Gazelle Hemoglobin Variant (Hemex Health) detects and quantifies different hemoglobins present in the samples.