Section 1 | Incidence and Prevalence

Sickle Cell Disease

Approximately 100,000 Americans live with SCD. There is no precise estimate of SCD global incidence. Current estimates indicate that worldwide close to 300,000 people are born with SCD each year. Measuring a more precise prevalence of the disease would be helpful in ensuring all patients are receiving adequate care. Without surveillance data that track the true prevalence of SCD in the U.S. and territories, understanding where patients live and where they are receiving care — or if they even seek care — is unknown. Better surveillance is imperative to identify areas of concern and address gaps in care.

Sickle Cell Trait

While people with SCT do not have the symptoms or illness of SCD, they carry the sickle cell gene and, if their partner also carries the sickle cell gene, there is a chance their children may have sickle cell disease. Partnership with the local and state newborn screening programs is important to ensure that comprehensive counseling is conducted so that people can make informed decisions about family planning.

More than 1.1 million newborn babies were born with sickle cell trait (SCT) in the United States (Benson & Therrell Jr, 2010). • This means one in nearly 70 babies have SCT (Kato et al., 2018) • 1 in 13 Black or African-American babies is born with SCT. • More than 100 million people live with SCT around the world.

ABOUT SCD TYPES

Hemoglobin SS disease is the most common type of sickle cell disease. It occurs when a person inherits copies of the hemoglobin S gene from both parents. This forms hemoglobin known as Hb SS. As the most severe form of SCD, individuals with this form also experience the worst symptoms at a higher rate (Healthline, 2019). In addition to the complications described in the main report, listed here are additional severe complications.

• SCD also causes damage to kidneys, the liver, and other organs. • One-third of male adolescents and young men with SCD suffer from painful, prolonged erections (priapism), which can result in scarring, deformation, and impotence (Idris et al., 2020). • Girls and women with SCD may have delayed puberty, more intense pain before and during menstruation, difficulty getting pregnant, or complications during pregnancy (Andemariam & Browning, 2013; Ghafuri et al., 2017; Kuo & Caughey, 2016; Stimpson et al., 2016). • More information about acute and chronic complications from SDC can be found in the National, Heart, Lung and Blood Institute Evidence-Based

Management of Sickle Cell Disease: Expert Panel Report, 2014.

Minimizing physical symptoms and disease progression of SCD is important to patients; to achieve this, healthcare costs for patients with SCD are substantial. In 2016, it was estimated that the average person living with SCD accrued nearly $1 million in total lifetime healthcare costs, with annual costs of more than $30,000 for adults (Novartis, 2019). This is before adding two new essential medications, Crizanlizumab and Voxelotor, whose prices — although potentially covered by patients’ insurance — range from $7,00010,000 a month (BioPharma Dive, 2019; CVS Health, 2020). Addressing this issue is especially important given that SCD is a lifelong condition that impacts several health areas.