Expert Q&A: Reeves on the state of sickle cell disease in Michigan

What is Sickle Cell Disease?

Sickle cell disease (SCD) is the most common inherited blood disorder, causing lifelong illness and premature death. SCD causes the body to produce abnormal hemoglobin, leading to red blood cells that are stiff and “C”- or sickle-shaped. In people who do not have SCD, red blood cells are flexible and disc-shaped, enabling them to flow smoothly through even the smallest blood vessels. But cells that are sickled get stuck and form clumps in the small blood vessels, reducing blood flow and lowering the amount of oxygen that gets delivered to organs and tissues throughout the body. This leads to serious health complications, including episodes of pain (both chronic and acute); anemia; pneumonia and acute chest syndrome; stroke; eye problems; infections; and kidney, liver, and heart disease. The estimated life expectancy of people with SCD in the United States is 45 years – more than 30 years shorter than the general population.

Who does Sickle Cell Disease affect?

Millions of people throughout the world are affected by SCD, including an estimated 100,000 Americans. SCD is most common among people whose ancestors came from places where malaria is common, including Sub-Saharan Africa and Spanish-speaking countries in the Western Hemisphere. In fact, over 90% of people affected by SCD in the United States are Black, Hispanic, or Latino, and close to half of these individuals are enrolled in Medicaid or the Children's Health Insurance Program (CHIP). Due to the critical importance of early detection for effective disease management, all children born in the United States are now tested for SCD through newborn screening.
 

What is the current state of health care for Sickle Cell Disease?

People with SCD require consistent, lifelong access to a variety of healthcare services to maximize their health and quality of life. This includes ongoing visits to specialist clinics, interactions with multidisciplinary teams of providers, and disease-specific screenings and medications to address the full scope of symptoms associated with this disease. SCD treatments largely focus on avoiding pain episodes, managing symptoms, and preventing complications. The need for acute care is also common; in 2022, 55% of people with SCD had an emergency department visit and 38% had one or more inpatient hospital stays. However, despite decades of recommendations and evidence, the quality of care for people with SCD remains severely inadequate. For example, fewer than 10% of children with SCD receive the recommended amount of life-saving antibiotics to prevent infections, and only about one-third undergo screenings to assess their risk of stroke. Less than half of adults with SCD visit a hematologist within a three-year period, even though they should have multiple appointments each year.

What is the Sickle Cell Data Collection Program?

Since 2015, the Centers for Disease Control and Prevention (CDC) Sickle Cell Data Collection (SCDC) Program has been partnering with states throughout the country to collect information about people with SCD to examine long-term trends in diagnosis, treatment, healthcare access, and health outcomes for people with SCD. The SCDC program tracks important data on where people with SCD live, transitions from pediatric to adult care, identification of SCD in Hispanic and Latino populations, understanding older adult health problems unrelated to SCD, and the use of healthcare services. In its 2020 consensus report, Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action, the National Academies of Science, Engineering, and Medicine acknowledged that “robust and well-supported longitudinal data collection systems that include the majority of those living with the disease will provide the information and evidence needed for decision-making and facilitate the evaluation of changes in SCD care.”

Does Michigan have its own Sickle Cell Data Collection Program?

Yes! The Michigan Sickle Cell Data Collection (MiSCDC) Program is one of 16 CDC-funded state data collection programs working to improve the lives of people with SCD. MiSCDC is a population-based data collection system led by the Susan B. Meister Child Health Evaluation and Research Center (CHEAR) at the University of Michigan (U-M) and the Michigan Department of Health and Human Services (MDHHS). U-M/CHEAR is legally authorized to conduct public health surveillance of SCD in Michigan under a Grant of Public Health Authority from MDHHS. MiSCDC consolidates and leverages the statewide SCD data to inform public health policy and program expansions. Data sources include SCD clinics throughout the state of Michigan, newborn screening, Michigan Medicaid, Children’s Special Health Care Services, immunization and vital records, hospital discharge databases, and, most recently, self-reported health status assessments from the Sickle Cell Disease Association of America - Michigan Chapter (SCDAA-MI). CHEAR links, deduplicates, and analyzes these data before sending aggregate information to CDC. CHEAR was a recipient of the Institute for Healthcare Policy and Innovation’s Policy Sprint award in 2022.

The unique MiSCDC partnerships bring together data to identify everyone living with SCD in Michigan and to understand where gaps in healthcare services and outcomes exist. Researchers and policy makers use this information – working together with clinicians, MDHHS, community-based organizations, and those with lived experience – to formulate action plans to close those gaps.

What have we learned so far from MiSCDC surveillance efforts?

The MiSCDC program has played a key role in informing and evaluating policies and programs that aim to improve the lives of the roughly 4,000 Michiganders with SCD. For example, MiSCDC supported the expansion of health benefits for adults with SCD over the age of 21 in Michigan through the Children’s Special Health Care Services (CSHCS) program. This expansion, implemented in October 2021, increased access to care for over 60% of the population of people living with SCD in Michigan. The multisource dataset of MiSCDC played a key role in identifying over 2,500 Michigan adults who would potentially qualify for CSHCS coverage under the new policy, as well as the proportion already covered by the state Medicaid program. This data also informed the state’s cost estimates for the expanded CSHCS policy and helped to target their outreach and educational efforts.

Data from the MiSCDC program also helps monitor important aspects of healthcare quality. SCD clinics throughout the state and MDHHS use this information to create plans and projects aimed at improving the quality of care for this vulnerable population. Through insight gained from MiSCDC, decisions that benefit Michigan residents can be grounded in evidence-based practices and tailored to the specific needs of people with SCD. This leads to better healthcare access, reduced costs, and improved quality of life. Importantly, these Michigan-specific policy learnings can be translated across the United States, resulting in efficient use of resources to improve the health and well-being of all people living with SCD.

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