Sickle Cell Disease Key Performance Indicators, United States and Jamaica

Future directions in performance indicators include readmission rates, access to pain management medications such as Dilaudid through patient-controlled analgesia pumps, access and efficacy of gene therapy and stem cell transplant, wait times during acute crises, and aspects of social determinants of health data, such as access to transportation to health care facilities or racial discrimination in pain management practices (Chaturvedi & DeBaun, 2016; Oyeku & Faro, 2017; Ramsay et al., 2021). Implementing and documenting the above KPIs is challenged by limited infrastructure and financial resources for even the current, fundamental indicators outlined in Table 1 (Shravya et al., 2023). Until then, early childhood mortality is as high as 90% in some parts of Sub-Saharan Africa (Chaturvedi & DeBaun, 2016).

Best Practices

High-income countries benefit from increased life expectancy in their residents with SCD, largely due to access to financial, clinical, technological, human, and pharmacological resources. Yet, limitations in access to resources can still result in improved outcomes, as evidenced by the Jamaica case study. Jamaica, overall, lacks many of the infrastructure components of the United States but reports a similar life expectancy for its sickle cell population. By implementing a low-cost universal newborn screening, universal vaccination for influenza and pneumonia, connection to a medical home for management (even without access to advanced specialty care available at the Sickle Cell Unit in Kingston or the University Hospital of the West Indies), and rigorous patient and family education on identifying triggers and spleen self-exams, they have managed to develop key best practices that can be translated globally (Serjeant et al., 2018).

In Jamaica, universal newborn screening results in a series of interventions that precipitate improved outcomes, including addition to a national disease registry (which the US does not have, even though it conducts universal newborn screening), connection to a hematology-trained care team, and Doppler cranial studies to assess for the likelihood of stroke later in life (Oyeku & Faro, 2017).

Before the widespread adoption of hydroxyurea as a prophylactic treatment to prevent acute chest syndrome and strokes in patients with SCD, the “gold standard” in high-income countries such as Canada, the United Kingdom, and the United States, was to conduct routine blood transfusions to maintain hemoglobin levels. Jamaica, which could not implement similar approaches due to cost and infection prevention concerns, limited the routine use of blood transfusions to prior to surgical procedures or during vaso-occlusive crises (Chaturvedi & DeBaun, 201

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