Sickle Cell Disease in California and Georgia: Death Rate Estimates
This study estimated death rates among people with sickle cell disease (SCD) by matching up data from studies that monitor all people with SCD in a population with state death records. The researchers found higher death rates among people with SCD than previous estimates that used other methods. They also found that death rates of people with SCD between ages 5-74 years are higher than those of African Americans in California and Georgia or those of people in the general population in these states. This study provides the most accurate picture of rates of death in people with SCD to date.
About This Study
The National Institutes of Health and Centers for Disease Control and Prevention funded the Registry and Surveillance System for Hemoglobinopathies (RuSH) project, which was a population-based monitoring study that collected data on people with SCD in the states of California and Georgia from 2004-2008. These types of studies monitor large groups of people and collect information about how their health changes over time.
The information collected by RuSH was matched up (linked) with state death record files to determine how many people with SCD in these two states and at what ages they died during the 2004-2008 time period. The researchers then compared these death rates to those of African Americans and to the general population in the corresponding states.
By using multiple diverse sources of data, the researchers were able to gather information about people who may not have been included in previous studies, such as those who received care outside of sickle cell care centers or those who may have had limited or no access to regular medical care. Additionally, by linking multiple sources of data, this study is the first to provide information on death among people with SCD who died from causes not specific to SCD, which is a patient group that is missing from studies of death records alone. Read more about the sickle cell disease mortality study hereexternal icon.
Sickle Cell Disease is a group of genetic (inherited) red blood cell disorders. Healthy red blood cells are round and they move easily through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky, and they look like a C-shaped farm tool called a “sickle.” The sickle cells die early, which causes a constant shortage of red blood cells. They can also get stuck and clog the blood flow when they travel through small blood vessels. These blockages can cause pain and other serious problems, such as infection, lung-related complications, and stroke
- The researchers found that 615 people out of 12,143 people with SCD who lived in California and Georgia died during 2004-2008.
- Among people with SCD, the average age of death was about 43 years for females and 41 years for males. About 1 in 6 deaths occurred in those under 25 years of age and nearly half of all deaths occurred in those over 44 years of age.
- People with SCD in California and Georgia between the ages of 5-74 were more likely to die than either African Americans or people in the general population of similar age in these states.
- Of the 615 people with SCD who lived in California and Georgia, nearly half did not have SCD listed as a cause of death on their death certificate. Therefore, using death records only, these people would not have been counted in studies of death among people with SCD.
Critical Gaps and Future Directions
Many challenges exist for researchers that work to accurately estimate the number of deaths among people with SCD. Not everyone who has SCD dies of a cause related to SCD, so the death reports of those individuals might not include the fact that they had SCD. Without this information in the death record, it becomes challenging to estimate the number of people with SCD who have died. Because the researchers who conducted the study described here used multiple sources of information, they were able to gain a more accurate picture of age at death among people with SCD. Further research using multiple sources of data from additional states, spanning additional years, is necessary in order to better understand how and when people with SCD die.
Paulukonis ST, Eckman JR, Snyder AB, Hagar W, Feuchtbaum LB, Zhou M, Grant AM, Hulihan MM. Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004-2008external icon. Public Health Reports 2016 131:367-375.