1. Sickle Cell Disease Registry in Arkansas
Leigh Ann Wilson, L.C.S.W. at the University of Arkansas for Medical Sciences, Pooja Motwani M.D. at the University of Arkansas for Medical Sciences and Donna Ezell R.N. at the University of Arkansas for Medical Sciences
Division of Hematology, Adult Clinical Sickle Cell Program at the University of Arkansas for Medical Sciences, Little Rock, AR
Dept. or
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BACKGROUND
CONCLUSIONS
Collection of data in the registry began in March 2015 and as of March 2017 there were 106 patients enrolled. The state-wide registry is a good start for gathering information on adults living with sickle cell disease but has several deficits. The registry only includes patients seen at the University of Arkansas Medical Sciences and it only captures services provided at UAMS, Baptist Health and other services are based on patient report. Since patients are enrolled during hospitalizations at UAMS or clinic appointments at UAMS it is not representative of patients throughout the state.
The state of Arkansas did not have a way of tracking patients with sickle cell disease outside of the newborn screening. It is unclear the number of people living in Arkansas with sickle cell disease as well as any information about them. The Arkansas Department of Health has been conducting Newborn Screenings of all Arkansas births since 1988 and includes screening for Sickle Cell Disease. Through this, we know that approximately 25 babies are born every year in Arkansas with Sickle Cell Disease. Given the life expectancy of those with the most severe form of the disease is in the mid-forties, a rough estimate of the total number of people with the disease in Arkansas is 1300.
A Sickle Cell Disease Registry was established to learn more about the adults living in Arkansas Where do they live? Where do they receive care? How long are they living? The purpose of the registry is to help healthcare providers learn more about the adult population and improve the care they are receiving.
RESULTS
Gender: 61 females, 45 males
State of Birth: 82 born in Arkansas, 24 Born outside of Arkansas
Type of Insurance: Medicaid-61, Medicare- 26, private insurance-17, 2 without insurance
Employment: employed full time-18, employed part time-10, unemployed-14, students-17, disabled- 47
Education level: less than high school education- 16, high school diploma- 25 , some college- 48, college graduate-12, post graduate-5
Reproductive: 68 have children, 38 do not have children
Patients with a family member with sickle cell disease: 82
Diagnosis: SS Disease-56, SC Disease -27, Beta Thalassemia Disease- 21, O Arab Disease-2
Patients received prescriptions for Hydroxyurea: 98
Patients received prescriptions for Exjade: 27
Patients that consented to blood and urine collection: 90
Complications: Acute Chest-42, Pulmonary Hypertension- 6, Priapism- 22, Chronic Renal Failure/proteinuria avascular necrosis- 5, Stroke/Transient Ischemic Attack, Seizures- 9, Iron Overload- 25, Gallstones/Cholelithiasis, Cholecystitis- 17, Splenomegaly, Splenicsequestration, Hypersplenism- 15, Leg Ulcers- 2, Osteomyelitis- 3, Retinal Disease-18, Avascular Necrosis 12
METHODS
Patients are identified for participation by the Sickle Cell Team. Patients have the option to participate in the data collection as well as provide a one-time blood and urine sample. At the time consent is completed, patients provide information about their past healthcare utilization, complications, demographic information, SC Diagnosis, and complete a Quality of Life Scale. Patient’s healthcare utilization, labs, medications and complications are monitored by the Sickle Cell Team through medical chart review and updated yearly. A patient’s tissue sample is collected at the time of their next lab appointment, if the patient agreed to blood and urine collection. Their sample is then stored for future research. .