United States Sickle Cell Disease Population and Current Programs

1
United States Sickle Cell Disease Population
andCurrent Programs

• Kathryn Hassell, M.D.
• Associate Professor of Medicine
• Director, Colorado Sickle Cell Treatment and
  Research Center
• University of Colorado Health Sciences Center
• Denver, CO

2
Key Issues for Consideration

• Optimal planning of programs and services for
  sickle cell disease requires
• Characterization of the affected population
• Number of affected individuals
• Location and types of utilized services/facilities
  
• Nature and number of providers
• Identification of the scope of current programs
• National sickle cell programs
• Comprehensive Sickle Cell Centers
• Sickle Cell Disease Clinical Research Network
• Sickle Cell Disease Treatment Demonstration
  Program

3
Sickle Cell Disease Population

• The actual size is unknown
• Published estimates range from 55-80,000
• Estimation techniques
• (Affected population) x (Birth prevalence)
• (Number of infants) x (Life expectancy)
• (Hospitalizations) x (Ratio patientshospitalizati
  ons)

4
Challenges to Population Estimation

• At-risk populations
• Often only African-American census used
• Misses other populations, e.g. Hispanic
• Birth prevalence
• Universal screening only recently adopted data
  often derived from single states no up-to-date
  central data source
• Misses immigrants
• Life expectancy unknown
• Published estimates based on select populations
• 45 years for HbSS 65 years for HbSC
• Centralized data (CDC) based on death
  certificates

5
Distribution of Age at Death SCD
Mean age of death 1979 28 years 2004 38
years Sickle cell disease identified at
underlying cause of death
http//wonder.cdc.gov
6
Distribution of Age at Death SCD
http//wonder.cdc.gov
7
SCD Population Estimates
METHOD
ESTIMATE
8
Johns Hopkins
9
Location and Sites of Utilization

• National/centralized information limited to
  health care activity
• Hospital admissions (n113,098 in 2004)
• Primary Source is HCUP NIS, KID, SID
• Assumes accurate coding at discharge
• ED and ambulatory visits difficult to determine
• Represents episodes of care rather than patient
  numbers
• However, each episode represents an individual in
  need of sickle cell expertise

10
Sickle Cell Patient Hospitalizations 2004
gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500
Extrapolated from SID
11
Comparison of Admissions for Children with SCD
vs.All Admissions in Children 2003
12
Comparison of Admissions for Children with SCD
vs.All Discharges in Children 2003
13
Comparison of Admissions for Adults with SCD
vs.Admissions for Adults Aged 18-64 2003
14
Comparison of Admissions for Adults with SCD
vs.Admissions for Adults Aged 18-64 2003
15
Summary of Utilization (Hospitalizations)

• Hospitalized SCD patients are
• Often from metropolitan areas in the South and
  Northeast with lower median household income
• Seek care at teaching hospitals
• As or more likely to have some kind of insurance,
  but more often Medicaid
• However,
• 25-30 are in the Midwest and West
• 20-35 receive care at small, non-teaching
  hospitals
• 10 live outside metropolitan areas

16
Sickle Cell Disease Health Care Providers

• There is no centralized information about the
  number or type of providers
• Often assumed but undemonstrated that most
  children receive sickle cell care
• Knowledgeable adult providers are lacking
• ASH Sickle Cell Disease Listing
• 243 adult heme/onc physicians in 46 states
• Sickle Cell Adult Provider Network
• 95 additional sickle cell physicians

17
National Sickle Cell Disease Programs

• Comprehensive Sickle Cell Centers Program
• NIH/NHLBI
• 10 Comprehensive Research Centers (Coordinating
  Center) located in 11 states
• 110 million over 5 years
• Sickle Cell Disease Clinical Research Network
• NIH/NHLBI
• 8 Phase II/III Clinical Research Centers
  (Coordinating Center) located in 8 states
• 36 million over 5 years
• Sickle Cell Disease Treatment Demonstration
  Project
• HRSA
• 4 Networks (Coordinating Center) located in 4
  states
• 8 million over 4 years

18
Current Comprehensive SC Centers

CSCC site
CSCC satellite
gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500
19
Top 10 States for SCD Admissions
estimated by (population) x (birth
prevalence) based on applications to NHLBI
programs
CSCC
20
SC Clinical Research Network Sites
SCDCRN site
gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500
21
Top 10 States for Admissions of SCD Patients
estimated by (population) x (birth
prevalence) based on grant applications to
NHLBI programs
SCDCRN
22
SCD Treatment Demonstration Program Sites
SCDTDP site
gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500
23
Top 10 States for Admissions of SCD Patients
estimated by (population) x (birth
prevalence) based on grant applications to
NHLBI programs
SCDTDP
24
Current National Program Sites

gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500
25
Known Sickle Cell Centers/Providers
Independent sites
Sickle cell Providers (ASH/SCAPN)
gt10,000 1,000-2,499 5,000-9,999
500-999 2,500-4,999 lt500