Abstract
Background
Sickle cell anemia (SCA) is a significant, under recognized contributor to global childhood mortality, especially in sub-Saharan Africa. Early diagnosis is critical to enable timely access to care and education, before severe and life-threatening complications develop in the first year of life. Unfortunately, such early and comprehensive care remains largely unavailable for many infants across Africa. In an attempt to reduce the high early mortality associated with SCA, an infant SCA clinic was developed and implemented in the capital city of Luanda, Angola. We describe the early experiences and successful outcomes for infants enrolled in this clinic.
Methods
Infants were enrolled in the clinic if the diagnosis of SCA was made in the first year of life. The clinic was established in the major public pediatric hospital in Angola, Hospital Pediátrico David Bernardino (HPDB). The vast majority of enrolled infants were diagnosed by newborn screening at local maternity hospitals, while some were diagnosed due to clinical suspicion or known family history. Initial clinic visit included intake of demographics such as contact information, family history, and details of basic housing conditions. A dried bloodspot was collected and the diagnosis of SCA was confirmed by isoelectric focusing. All families received sickle cell education and confirmed infants received penicillin prophylaxis (125 mg by mouth twice per day), pneumococcal vaccination series (Prevnar-13), and an insecticide-treated mosquito net for malaria prophylaxis.
Results
In the first twenty months of the HPDP Infant SCA clinic, 301 infants were enrolled. Eighty-one percent (244/301) were identified through the associated newborn screening program, while the remaining fifty-seven infants presented due to clinical symptoms or known family history. Families live in the urban and poverty-stricken Luanda. The average household has 6.4 people with 2.8 people per bedroom. Only 34.2% of families reported access to water within their household. Despite poverty and difficult housing situations, continued follow-up was extraordinarily high at 97.3%. After the initial visit, only eight families (2.7%) chose not to follow-up – four preferred faith or traditional healing techniques, and four chose follow-up care at a private clinic. With a concentrated effort to track and enable timely follow-up care, there were zero babies truly “lost to follow-up.” For the 167 babies who are now at least one year of age, the calculated infant mortality rate (under 1 year of age) is 6.6%, which compares favorably to the nationally reported infant mortality rate for all children (9.8%). Upon reviewing the 11 deaths, in nearly all cases the families sought appropriate medical care as instructed, and most deaths were likely preventable if appropriate and timely emergency care were available in the community.
Conclusions
Early mortality associated with SCA can be significantly reduced through early diagnosis and access to care and education, even in countries with few health resources such as Angola. These experiences with a newborn clinic in the urban city of Luanda demonstrate that simple, lifesaving care is feasible and that follow-up and survival is excellent. Although the survival in this Angolan cohort was even better than the national infant mortality rate, the few deaths illustrate gaps in the understanding of emergency SCA care among the healthcare community. It is critical to include education and training of healthcare professionals at all levels of care in any national strategy, so that children with SCA can be promptly triaged and adequately treated for emergent and life-threatening complications.
Disclosures:
No relevant conflicts of interest to declare.
Prediction of atopy in the first year of life using cord blood IgE levels and family history
