Regional Epidemiology of Sickle Cell Disease Patients: Identifying Trends, Hotspots, and Data Deficiencies
- Denby Williams
- 17 minutes ago
- 4 min read
In the United States alone, 100,000 individuals suffer from sickle cell anemia, more commonly known as sickle cell disease. Out of this population, around 90,000 are of African American Descent; it is hypothesized that the gene mutation responsible (HBB) was acquired due ot the environmental defense mechanism of fighting against malaria, most commonly from West Africa. The immune system response of those with the sickle hemoglobin trait was more effective at destroying the infected red blood cells, thus creating a natural selection circumstance over time. Shifting the focus back to the United States, those with sickle cell disease frequently experience healthcare inequalities based on socioeconomic status and geographical location. Below is a comprehensive rundown of future trends, hotspots, and target interventions.
When observing the case distribution of sickle cell disease by state, Florida, Georgia, New York, Texas, and Maryland show the highest rate of prevalence, with Florida having documented 13,886 SCD. This, however, differs from a majority of states in the Midwest/West (excluding California) - many of the cases are concentrated within the Eastern region. Looking more specifically, the southern and central parts of Florida, Broward and Miami-Dade, show 14.3% and 14.6% of the SCDD population, respectively. Coming in second is Polk and Orlando county, with 13.5-13.6%. Historically speaking, Georgia has seen a high concentration in the Metro region of Clayton, Cobb, DeKalb, Fulton, and Gwinnett; the total number in the entire state is 10,396. However, the prevalence rate drops significantly when moving into the rural regions. For New York state, 80% of SCD residents (9,372 of the 11,715) reside in New York City. Unlike Florida, Georgia, and New York, Texas shows cases throughout the state, not differing by county, with 9,416 being the total. With Maryland's population size being much smaller than the previous states, only about 5,812 cases are present. Across all of these states, SCD shows ubiquity among the younger population, particularly in the ages of newborn to 15.
Over the past four decades, the mortality rate for sickle cell disease has decreased significantly. "The crude mortality rate was 1.1 per 100,000 Black individuals, which was significantly lower than the period 1999-2009 (crude rate 1.2 per 100,000, p < 0.0001) (Figure 1B)." (Karkosa, McGann 2023). Despite this, the mortality rate for the age group of 15-19 years compared to 20-24 years was much higher. When you observe the mean age of death, life expectancy has been extended by four years. - specifically from 40 to 44 for females, and 38 to 41 for males. Despite the noncommunicable diseases associated with SCD patients, the primary cause of death is unknown, closely followed by cardiovascular disease. While the overall mortality rate is decreasing, there needs to be more emphasis on the care needed for SCD patients during the transitional phase of pediatric to adult care. If you notice in Figure 2, the overall peak resides at ages 25-54. This differs from the gradual lowering to 75-84.
In many SCD patients, vaso-occlusive crises are quite common. It is a painful complication where the red blood cells block the flow of blood vessels, resulting in tissue damage. Although the main triggers for vaso-occlusive crises are dehydration, stress, and infection, if mild, symptoms can be treated with over-the-counter medication. Given how easy these incidents can happen, the majority of hospitalizations and emergency visits are composed of this. The socioeconomic inequalities become present once it is known that certain states, like Georgia, only permit a number of visits within a specified period. The out-of-pocket costs put a strain on those already in an economically disadvantaged situation.
Targeted interventions for SCD are typically lifestyle behaviors; this primary prevention includes drinking plenty of water, avoiding high altitudes, and exposure to low oxygen levels. Infections can be incredibly harmful for SCD patients, so ensuring hand washing, safe food preparation, and vaccines is a must. With secondary prevention, having consistent contact with your eye doctor, neurologist, and hematologist is essential for the prevention of comorbidities. Continuing to tertiary prevention, medications such as Hydroxyurea, L-glutamine, and Crizanlizumab work on a fundamental basis of protein building and binding within the blood cells. Stem cell and blood transplants also work to minimize organ damage. In 2023, the FDA approved two gene cell therapies - CASGEVY and LYFGENIA -, which work as the 'faulty cells', so to speak, are replaced with healthy stem cells provided by the therapy. These 'faulty cells' are eliminated through rounds of chemotherapy.
When observing SCD, many of the problems presented are primarily from healthcare inequalities among the African American and Hispanic populations. To adequately address this disease, community efforts throughout the southeast and urban regions will need to be implemented to reduce morbidity and mortality rates. Despite this, biotechnology and early prevention techniques show exponential progress. The future for Sickle Cell Disease shines bright.
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