34th Annual Convention Sickle Cell Disease: Meeting Unmet Needs An Interactive and Accredited Educat

1
34th Annual ConventionSickle Cell Disease
Meeting Unmet NeedsAn Interactive and
Accredited Educational Conference

• PowerPoint Presentations
• Hyatt Regency Dallas at Reunion
• Dallas, Texas
• September 27 30, 2006

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Sickle Cell Disease Academic Skills Development
in Middle Childhood Relative Role of Biomedical
Socio-Environmental Factors

• Kenia Johnson, M.A. Jeffrey Schatz, Ph.D.
  Catherine McClellan, Ph.D. Eve Puffer, M.A.
• Department of Psychology, University of South
  Carolina
• Carla Roberts, M.D.
• Department of Pediatrics, University of South
  Carolina

3
Background Information

• Specific socio-environmental factors ( e.g.,
  parent involvement) linked to higher
  academic/cognitive achievement in early grade
  school.
• Children w/ (SCD) shown to experience
  difficulties in their cognitive and academic
  functioning.
• Biomedical factors (i.e., pre-term delivery, more
  severe SCD) shown to place children at risk for
  developing cognitive and academic impairments.

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Study Objective

• To conduct a preliminary investigation into the
  predictability of early psychosocial factors on
  the academic outcomes of school-aged children
  with SCD.
• To investigate the unique influence of SCD on
  childrens cognitive/academic development.

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Hypothesis

• Both family psychosocial and biological factors
  will contribute significantly to explained
  variance in academic outcomes in children with
  SCD.
• Family psychosocial factors will contribute the
  most to explained variance in academic outcomes
  in children with SCD.
• 3. Biological factors will contribute uniquely
  to the explained variance in academic outcomes in
  children with SCD.

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Method

• Participants were recruited during biannual visit
  to CRS Sickle Cell Clinics.
• N 38 children w/ SCD
• M age 6.3 (range 4.6 8.4)

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Sample Characteristics (n)
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Measures

• Socio-environmental Measures
• Family Involvement Questionnaire (FIQhome-based
  involvement)
• Cohens Perceived Stress Scale
• Family Income
• Biomedical Medical Measures
• Preterm Birth
• Hermatocrit (Hct)
• Academic Skill Measures
• Letter-word Identification subscale (WJ-IV)
• Applied Problems (WJ-IV)

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Results

• Analysis
• Correlation
• Socio-environmental, biomedical, academic
  variables
• Multiple Regression
• Two separate models for each academic outcome
• Hierarchical model to decompose unique v. shared
  variance

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Correlation

• Letter-word Identification
• Preterm birth (r -.409, p lt .05)
• HCT (r .352, p lt .05)
• Applied Problems
• Preterm birth (r -.334, p lt .05)
• HCT (r 328, p lt .05)

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Regression

• Multiple Regression full model was significant
  for L-W ID, but not for App. Prob. (R2 .316, F
  2.87, p lt .05.)
• Hierarchical Regression 1 entered socio-envl
  factors first into equation ? ? R2 .231
• Hierarchical Regression 2 entered biomedical
  factors first into equation ? ? R2 .035

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Summary
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Conclusions

• Both socio-environmental and biomedical factors
  contribute to reading skills in young children w/
  SCD.
• Biomedical factors may exert more influence
  during this stage of development.

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Future Implications

• Continued research investigating impact of
  preterm birth on development in SCD.
• Continued research on impact of disease severity
  to academic outcomes.
• Inclusion of biomedical factors in interventions
  to improve academic outcomes in children w/ SCD.

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The association of oral hydroxyurea therapy
on cognitive functioning in children with sickle
cell disease

• Eve S. Puffer, B. S.
• Jeffrey Schatz, Ph. D.
• Carla Roberts, M. D.

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Purpose of the Study

• To conduct a preliminary examination of
• potential cognitive benefits of oral hydroxyurea
• therapy (OHT) for children with Sickle Cell
• Disease (SCD)

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Rationale Cognitive Effects of SCD

• Cerebral Vascular Accidents (CVA)
• Overt ischemic stroke (Bernaudin et al., 2000
  Cohen et al., 1994 Craft et
• al., 1993 Schatz et al., 1999 Wang et al.,
  2001).
• Silent infarctions (Armstrong et al., 1996
  Schatz et al., 2001 Steen et al., 2003 Wang et
  al., 2001)
• Cognitive decrements without CVA
• General IQ measures
• Crystallized ability/Verbal Comprehension
• Short-term memory
• Processing Speed
• Attention / Executive functioning
• (Brown et al., 1993 Steen et al., 1999
  Bernaudin et al., 2000 Wang et al. 2001 Schatz
  et al. 2002 Schatz et al., 2004)

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Cognitive decrements without CVA

• Potential Causal Factors
• Illness Severity / Reduced school attendance
• Psychosocial variables (e.g. SES)
• Structural or functional brain abnormalities
  associated with hematologic consequences of SCD,
  such as severe anemia.
• Severe anemia associated with cognitive
  decrements (Bernaudin et al, 2000 Brown et al.,
  1993 Steen et al., 1999, 2003)
• Hypotheses
• Chronic cerebral hypoxia
• Increased metabolic needs, insufficient
  nutritional resources for development

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Potential Brain Effects of SCD

• Structural (Steen et al., 1999, 2003)
• Tissue composition
• Volumetric brain growth (Steen et al., 2005?)
• Functional
• PET Localized metabolic abnormalities (Powars et
  al., 1999)
• Perfusion MRI abnormalities (Kirkham et al.,
  2001)
• Near-infrared spectroscopy (NIRS) low brain
  oxygenation levels related to symptom severity
  (Raj et al., 2004)

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Transfusions Cognitive Benefits

• Transfusion therapy
• Cognitive benefit for children with elevated TCD
  velocities (Kral et al., 2003)
• Related to improved brain oxygenation in children
  with severe SCD symptoms (Raj et al., 2004)
• Related to improved ERP performance in adult
  dialysis patients (Grimm et al., 1990 Pickett et
  al., 1999 Stivelman, 2000 )

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OHT and Cognition Potential Links

• OHT Treatment Effects
• Increases fetal hemoglobin (HbF)
• Reduces anemia
• Reduces thrombocytosis
• Reduces pain
• Prevents vaso-occlusions
• (Steinberg, 1999 Rosse et al., 2000)
• Potential mechanisms for cognitive benefits
• Direct brain effects Increased blood and oxygen
  flow to cortex
• Indirect effects Improve energy/endurance for
  cognitive tasks

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Study Design

• Study 1
• Between-groups analysis
• Youth receiving OHT vs. Control group
• Study 2
• Within-groups analysis on changes in cognitive
  scores over time pre-post design
• Youth who received OHT before post-test vs.
  Control group

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Cognitive Measures

• Test Administered
• WJ-R Oral Vocabulary
• WJ-R Spatial Relations
• WISC-III Digit Span
• WJ-R Visual Matching
• DKEFS Word Fluency
• Global Cognitive Index

• Cognitive Domain
• Verbal Comprehension
• Fluid Reasoning
• Short-term memory
• Processing Speed
• Long-term Retrieval
• Global cognitive ability

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Participants

• Participants
• Ages 6-21
• Diagnosis of SCD
• Served at Palmetto Health Richland or
• SC Childrens Rehabilitative Services
• No history of overt stroke or developmental
  disorders

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Study 1 Hypothesis

• Children receiving OHT will score significantly
  higher than control group on measures of
• Verbal Comprehension
• Processing Speed
• Short-term memory
• Global cognitive ability

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Study 1 Statistical Analyses

• Hypothesized effects
• 4 one-way between groups ANCOVAs on
• Oral Vocabulary (Verbal Comprehension)
• Visual Matching (Processing Speed)
• Digit Span (Short-term Memory)
• Global Cognitive Index
• Exploratory analyses
• 2 one-way between groups ANCOVAs on
• Spatial Relations (Fluid Intelligence)
• Word Fluency (Long-term Retrieval)

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Study 1 Sample Characteristics
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Study 1 Statistical Analyses (cont.)

• Covariates
• Age
• Income category
• days hospitalized
• Additional covariates for ANCOVA on Oral
  Vocabulary
• Mean parental education
• Historical hematocrit levels

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Study 1 Results
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Study 1 Results
p lt .05 p lt .01
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Study 1 Summary of Results

• Children on OHT showed better cognitive
  performance than controls in some domains after
  statistically controlling for several other
  factors.
• The Study 1 hypothesis was partially supported
• Children receiving OHT scored higher on
• Oral Vocabulary (Verbal Comprehension subtest)
• Global Cognitive Index
• Exploratory Analyses
• Children receiving OHT scored higher on Spatial
  Relations (Fluid Reasoning)

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Study 2 Purpose and Hypothesis

• Purpose To examine whether OHT status was
  related to changes in cognitive performance over
  time.
• Hypothesis A time by group interaction effect
• OHT group will show more improvement in cognitive
  scores at post-test than the control group on
• Verbal Comprehension
• Processing Speed
• Short-term memory
• Global cognitive ability

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Study 2 Statistical Analyses

• Hypothesized effects
• 4 one-way mixed factor ANOVAs on
• Oral Vocabulary (Verbal Comprehension)
• Visual Matching (Processing Speed)
• Digit Span (Short-term Memory)
• Global Cognitive Index
• Exploratory analyses
• 2 one-way mixed factor ANOVAs on
• Spatial Relations (Fluid Intelligence)
• Word Fluency (Long-term Retrieval)

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Study 2 Sample Characteristics
Statistically significant differences between
groups p lt .05, p lt.01
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Study 2 Results

• OHT (n 10) No OHT (n 10)
• Pre
  Post Pre
  Post
• M SD M SD
  M SD M SD

GCI 88.46 6.65 93.00
10.28 89.22 3.99 89.92 6.46
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Study 2 Results
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Study 2 Summary of Results

• Significant time by group interaction effect on
  Oral Vocabulary (Verbal Comprehension) scores
• Notable, but non-significant, medium-sized
  effects of time by group interaction on
• Visual Matching (Processing Speed)
• Digit Span (Short-term Memory)
• Global Cognitive Index
• Conclusion OHT may act to reverse some cognitive
  decrements

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Conclusions

• OHT may have some cognitive benefits for children
  with SCD.
• Current results provide strongest evidence for
  the benefit of OHT on Oral Vocabulary
  performance.
• Study 2 results more specifically suggest that
  OHT may act to reverse some cognitive decrements.

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Limitations and Future Research

• Limitations
• No random assignment
• Small sample Low power, limited generalizability
• Future Research
• Larger, more representative samples
• Randomization
• More extensive test batteries
• Broader range of cognitive tasks
• Achievement measures

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Future Research

• Investigation of potential mechanisms
• Does OHT reverse anemia-related brain effects of
  SCD?
• Does OHT impact cognitive performance by
  improving illness symptoms, pain, or energy
  level?
• Longitudinal studies
• Cognitive effects of OHT throughout development
• Potential preventative cognitive benefits of OHT
• Potential variation in cognitive effects of OHT
  across children of varying ages, illness
  severity, cognitive trajectories, etc.

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Eventual Implications

• More informed treatment decisions
• Increased information on the range of benefits of
  hydroxyurea
• Potential intervention for reversal, and possible
  prevention, of some cognitive deficits associated
  with SCD

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Sickle Cell Disease Health-Related Quality of
life Questionnaire Development Project

• SCDAA 34th Annual Convention
• September 2006

Marsha J. Treadwell, PhD Roger Levine,
PhD Corrina Moucheraud, MPH Ellen Werner, PhD
43
Background

• Clinical knowledge for treating adults with SCD
  has not kept pace with increased life spans
• Documentation is lacking about the impact of SCD
  and its treatments on health-related quality of
  life (HRQOL)
• Such documentation is essential for developing
  and evaluating the most effective treatments

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Meeting Unmet Needs - 2002

• Health-related Quality of Life defined
• A multidimensional assessment of the patients
  perception of his/her functioning in society, of
  the ability to function without pain, of being
  accepted, and of having intact relationships

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Proposed Domains 2002 HRQOL Working Group

• Burden of treatment
• Support systems
• Living arrangements
• Discrimination
• Stigmatization
• Use of spirituality
• Social relationships
• Coping strategies and skills

• Pain
• Mental health
• self-esteem
• depression
• feeling credible
• Financial status
• Access to care
• Educational attainment
• Employment opportunities

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Main Objective

• Develop a way to include reliable and valid HRQOL
  outcome measurement in clinical studies of
  therapy for adults with SCD

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Specific Aims

• Determine best general HRQOL categories and
  questions to include in a SCD-HRQOL measure for
  adults
• Determine SCD-specific categories and domains to
  include
• Evaluate reliability and validity of patient
  responses to these questions
• Develop the best ways to give the measure
• Evaluate how useful the measure is

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Who We Are

• Business Relationship
• Contract between NHLBI and the American
  Institutes for Research (AIR)
• Childrens Hospital Research Center Oakland
  (CHRCO) subcontracts to AIR
• Investigators
• Ellen Werner, PhD San Keller, PhD Roger Levine,
  PhD Marsha Treadwell, PhD Elliott Vichinsky, MD
• Staff
• Corrina Moucheraud, Maureen Maurer (AIR), Tunisia
  McFadden (CHRCO)

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National Advisory Board

• Wally Smith, MD, Chair
• Richmond, VA
• Lennette Benjamin, MD
• Bronx, NY
• Pat Corley, RN
• Los Angeles, CA
• Peter Grams, MSW
• Los Angeles, CA
• Carlton Haywood, MA
• Baltimore, MD

• J. Hoxi Jones
• Houston, TX
• Eric Kirkwood
• Kansas City, KS
• Kwaku Ohene-Frempong, MD
• Philadelphia, PA
• Kristy Woods, MD
• Winston-Salem, NC

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National Advisory Board

• Met in April and August 2006
• Mission to bring expertise and experience with
  sickle cell disease and the sickle cell community
  to bear on the elicitation and refinement of the
  domains of SCD-HRQOL
• Board reviews and makes recommendations about the
  design of data collection protocols, interview
  and focus group guides, instruments, survey
  procedures, recruitment procedures, preliminary
  analyses, final product and dissemination
  strategies

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Methods

• Literature review to establish potential domains
• Qualitative data collection
• Key Informant Interviews
• Hour-long telephone interviews with doctors,
  nurses, PAs and NPs, social workers, etc.
• Critical Incident Interviews
• Hour-long telephone interviews with adults with
  SCD
• Focus Groups
• Two-hour in-person groups, same-gender groups
  with adults with SCD

52
Research Objectives Methodologies
53
Key Informant Interviews

• Goal
• 20 interviews
• 12 incidents per interview
• Diversity across
• Professions,
• Geography

• Reality
• 15 interviews
• Average of 19 incidents per interview

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Key Informant Interviews
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Key Informant Interview Demographics
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Critical Incident Interviews

• Goal
• 30 interviews
• 15 incidents per interview
• 15 females, 15 males
• Geographic diversity

• Reality
• 35 interviews
• Average of 16 incidents per interview
• 20 females, 15 males

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Example Critical Incident

• CM-01-11
• Critical Incident Form
• What was the situation?
• Ive had pain episodes happen at the worst
  times, like before an event youve been planning
  for weeks. Maybe you get stressed, or its cold,
  but then youre wiped out.
• What happened? What did (person) do?
• Once, I had an episode right before Mothers
  Day. I felt like crap because my mom was sitting
  in the hospital, crying because she couldnt do
  anything for me. Happy Mothers Day. Its just
  awful.
• What was the result?
• Having an episode will put a dent in my life for
  months. Theres the medicine to take, withdrawal
  to go through, depression to get past. Its a
  pain in the ass. I wish there was a warning.
• IF NOT OBVIOUS How does this make the respondent
  feel (bad/good)?

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Critical Incident Interviews
59
Critical Incident Interview Demographics
60
Focus Groups

• Goal
• 12 groups
• 9 incidents per group
• 6 groups with females, 6 groups with males
• Diversity across
• Population density
• Proximity to CSCCs

• Reality
• 11 groups
• 9 per group (max)
• Average of 15 incidents per group
• 6 groups with females, 5 with males

61
Focus Groups
62
Recruitment Methods

• Through Advisory Board members
• Through the Sickle Cell Community Advisory
  Council (Oakland, CA)
• On the SCDAA website
• Through SCDAA member chapters and
• Via Key Informant Interviewees.

63
Sickle Cell Community Advisory Council (Oakland,
CA)

• Pilot focus group in Oakland
• Helped recruit participants
• Focus group in Sacramento
• Helped recruit participants
• Identified location
• Assisted with development and refinement of
  research materials (protocol, consent form,
  flyer, etc.) and review of draft taxonomy

64
SCDAA website

• Posting to patient forum (6/15)
• Follow-up FAQ posting (7/11)

65
(No Transcript)
66
(No Transcript)
67
SCDAA Member Chapters

• Wrote letter to Dr. Willarda Edwards and Ms.
  Sonya Ross at SCDAA
• Had follow-up teleconferences
• With their OK, began contacting local chapters
  initial list included

• Massachusetts (Boston)
• Pennsylvania (Harrisburg)
• Alabama (Birmingham Mobile)
• Mississippi (Jackson)
• North Carolina (Fayetteville Greensboro)

• Virginia (Norfolk)
• Louisiana (Alexandria)
• Illinois (Chicago)
• Indiana (Gary)
• Washington (Seattle) and
• New Mexico (Albuquerque)

68
SCDAA Member Chapters
69
SCDAA Member Chapters

• Can attribute at least
• 18 CIIs (of 33)
• 1 focus group (of 11)
• to SCDAA member chapter participation!

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Focus Groups

• Generation of critical incidents
• Prioritization of candidate domains
• Experiences with surveys

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Focus Groups Moderators

• Sickle cell knowledge/experience
• Trained in focus group skills and elicitation of
  critical incidents
• Focus group moderators
• Shawn Bediako, PhD
• Stephanie Coakley, MSW
• Calvin Moore, MDiv, MA
• Marsha Treadwell, PhD

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All Data Collection
73
Next Steps

• Year 2
• Complete qualitative data analysis
• Finalize taxonomy
• Create and revise item pool (cognitive testing)
• Create paper and pencil interview form
• Years 2 and 3
• Field test protocol
• Create and test beta system
• Finalize and disseminate final SCD-HRQOL
  measurement system

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TEACHING GRADE SCHOOLERS ABOUT SICKLE CELL
DISEASE AND TRAIT

• Eileen Murray BA, Laura McVittie, Pamela Orren
  PhD,
• Marsha Treadwell PhD
• Sickle Cell Disease and
• Newborn Screening Program
• September 2006

The Talking Drums Project
75
Background

• Genetics concepts are technical and advances in
  the area rapid
• To reinforce understanding, genetics concepts
  should be
• Introduced in early grades, then reiterated and
  built upon at higher grade levels
• Taught in a context rather than in isolation
• Teaching about sickle cell disease as a model
  genetic disease can reinforce learning about
  genetics and raise awareness about the disease

76
Partnership With a Purpose

• The Talking Drums Project uses multi-component
  interventions to increase awareness about sickle
  cell disease and trait in communities most
  affected
• Hall of Health Museum promotes wellness and
  individual responsibility for health by improving
  childrens understanding of how the body works

The Talking Drums Project
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Hall of Health grant

• The Hall of Health grant is partnership with
  Childrens Hospital Oakland Research Institute
  (CHORI), focused on diseases an disorders
  prevalent in community, as well as groundbreaking
  research at CHORI

78
Goals

• Provide background for 5th graders about genetics
• Introduce role of genetics in health
• Illustrate genetics concepts using sickle cell
  disease and trait as exemplar
• Increase awareness about sickle cell disease and
  trait for children, parents and teachers

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Methods

• Genetics background material
• Unit includes 5 lessons
• Dynamic hands-on
• Guest speakers
• Games and experiments
• Challenges and enriches 5th grade curriculum

80
Methods

• Sickle cell class covered
• Information about sickle cell
• Scientific concepts
• Life management skills
• Debunk common misconceptions
• Very basic pathophysiology
• Inheritance patterns

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Sample Activities
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Participants

• 160 children in five different 5th grade classes
• Evenly split gender
• Two schools in Oakland, with significant
  proportion of African American and Latino
  students. Both K-5th grades
• Fruitvale makeup 34 Latino, 36 African
  American.
• Hoover makeup 71 African American

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Evaluation

• Independent observation and ratings
• Observation
• Student teacher and outside evaluator reported
  what students learned from guest speakers
• Raters responded that children understood how a
  person lives with sickle cell and about sickle
  cell anemia
• Material rated about right. Lesson content was
  8.8 out of 10.

84
Evaluation

• Evaluated pre and post- tests
• N45
• Students improved with sickle cell disease
  questions
• Significant improvement in sickle cell genetics
  knowledge
• Pre-test mean 8.6 out of 20
• Post-test mean 11.8 out of 20
• t (88 df) -4.57, p lt 0.0001

85
Results
86
Future directions

• Plan for 2007 spring class
• Plain language for questionnaires
• Real-life scenarios for PUNETT squares
• Develop material and course content for other
  grade schools, middle and junior high level
• Create age-appropriate companion activity
  booklets

87
Using Peer Educators to Increase Sickle Cell
Awareness, Detection, and Prevention

• Delores Williams-Edwards
• Executive Director
• Southern Regional Sickle Cell Association
• Bridgeport, Connecticut

Partially Supported by DHHS/MCHB (HRSA- 05-027)
88
Acknowledgements

• Donzella Thompson, RN
• Southern Regional Sickle Cell Association
• Marylin Camacho, MD
• Warren High School Health Careers Magnet
  Coordinator
• Roger Thrall, PhD
• Hospital for Special Care
• Robin Leger, PhD, RN
• University of Connecticut Medical School
• Victoria Odesina
• Citizens for Quality Sickle Cell Care
• Coretta Jenerette, PhD, RN
• Yale University School of Nursing

89
Sickle Cell Trait

• 2 million or 1 in 12 Americans have Sickle Cell
  Trait
• Unaware of having Sickle Cell Trait
• Never tested
• Dont know the results of prior testing
• Consequences of having Sickle Cell Trait are
  manifested during
• Extreme physical activities
• Severe pressure or altitude changes

90
Sickle Cell Trait and Offspring

• It is important to identify and educate
  individuals with Sickle Cell Trait so that they
  will be aware of the risks and consequences of
  having children with Sickle Cell Disease or
  Sickle Cell Trait

91
SCOPE Sickle Cell Outreach Peer Education

• SCOPE was developed to
• Awareness about Sickle Cell Trait
• Provide education
• Offer testing of hemoglobinapathy disorders to at
  risk youth of Connecticut

92
SCOPE Methods to Increase Awareness

• Peer Educators
• Participate in high school assembly programs
• Collaborate with AHEC programs
• Participate in community health fairs
• Peer Educators are effective because
• Well received by their peers
• Communicate information to peers using
  appropriate youth culture
• Work can result in scholarship opportunities

93
SCOPE Methods to Increase Education

• Potential Peer Educators
• Receive certification by participating in a 3 day
  Peer Education Hemoglobinapathy Training Course
• Certified students are able to educate their
  peers about Sickle Cell Trait
• Are able to assist in the integration of Sickle
  Cell education into high school health courses
  and Health Magnet School programs

94
SCOPE Methods to Increase Testing

• Negotiated collaboration with local hospital
  laboratory
• Cost effective
• Timely results
• Implemented community-based hemoglobinopathy
  testing at
• High schools, college campuses, churches, health
  fairs
• Individuals who test positive are contacted by a
  certified hemoglobinopathy counselor

95
SCOPE Results

• Almost 200 high school and college students have
  been educated
• 95 students tested
• 10 positive for Sickle Cell Trait
• 1 positive for Sickle Cell Disease

96
Future of SCOPE

• Continue to train SCOPE Peer Educators
• SCOPE Peer Educators
• Break cultural barriers
• Generational barriers of age

97

• SCOPE Peer Educators are a vital link in
• BREAKING THE SICKLE CYCLE
• QUESTIONS????????????????????

98
A Sickle Cell Management System for Tribal
Populations in IndiaPioneering a Clinical and
Community-Based Model of Disease Management for
Indias Most Neglected Citizens

• 2006 Sickle Cell Disease Association of America
  Convention
• September 28th, 2006
• Dallas, TX

Hari Prabhakar President, Tribal India Health
Foundation (www.tihf.org) Operations Director,
TIHF/GAH Sickle Cell Disease Center
(Gudalur) Hari.Prabhakar_at_jhu.edu
99
An Introduction to the Tribal Populations of India

• Tribal populations classified as adivasis
  (indigenous people) during the colonial period
• 100 million persons enumerated as members of
  Scheduled Tribes (around 10 of Indias
  population)
• Prior to external forces, tribal populations had
  self-regulating economic and political systems
• Greatly dependent on forest for daily needs,
  including food, shelter, instruments, medicine,
  and clothing
• National development leading to the displacement
  of tribals from their native lands and
  relinquishing their natural resources
• Limited knowledge of tribals combined with social
  castigation has led to stigmatization of the
  populations as a whole

100
The Health Profile of Tribal India

• Few functional healthcare centers in tribal
  areas, leading to gaping health disparities as
  compared to metropolitan populations
• Non-governmental organizations as limited sources
  of healthcare
• Prevalence of infectious diseases such as
  malaria, tuberculosis, meningitis, polio, and
  cholera
• Malnutrition and gastrointestinal disorders,
  along with vitamin and protein deficiencies
• Certain tribal groups such as the Onges, Jarawas,
  and Shompens facing extinction due to endemic
  diseases, venereal diseases, and unusually low
  sex ratio

101
The Burden of Sickle-Cell Disease in Tribal India

• Large population, presence of malaria, and
  practice of consanguineous marriage practices as
  causal factors of the high prevalence of genetic
  disorders among tribals
• Sickle-cell disease among tribal populations has
  surged to the forefront as a critical public
  health problem among tribal groups
• Perplexing and deadly genetic disease with 50 of
  patients dying before age 20
• Prevalence of heterozygotes (HbAS) calculated to
  be over 20
• Sickle gene among tribals in South India first
  described by Brittenham, et al. in early 1950s,
  and among the Irula tribe of the Nilgiri hills
• Virtually all studies carried out regarding
  tribal populations and sickle-cell anemia have
  recommended that genetic health services be
  integrated into existing tribal health services,
  though few healthcare systems have implemented
  these recommendations

102
Project Objective

• Combining research with immediate clinical and
  community-based interventions to combat the
  spread of sickle-cell anemia in the Nilgiris
  District via a strong genetic health system
• Screening, Treatment, and Education as main focus

103
Project Setting Gudalur Adivasi Hospital

• Gudalur Adivasi Hospital in the Nilgiri Hills of
  Tamil Nadu
• Four-tiered framework revolving around the base
  hospital and comprising of doctors, nurses
  (tribals), village-based health workers
  (tribals), and health animators (tribals)
• Caters to a tribal population of over 20,000 in
  291 surrounding villages
• Tribes Paniyas (60), Bettikorumbas (20),
  Kattunaickens (10), Mullakurumbas (5),
  Irulas/Kotas (5)
• Prevalence of sickle-cell anemia high as 17.4
  with 17 carrier prevalence among the tribes
• Sickle prep test and hemoglobin electrophoresis
  test as the primary means of detecting
  sickle-cell anemia
• Initiated the TIHF/GAH Sickle Cell Disease Center
  

104
Preliminary Step Community Investigation and
Sensitization

• Active discussion in Tamil with the hospital and
  village-based healthcare staff, along with
  community leaders, on the proposed interventions
  prior and during the implementation process
• Comprehensive participation of the tribal
  community during the intervention process as
  critical in identifying and overcoming societal
  barriers and unforeseen repercussions such as
  stigmatization and noncompliance
• Empowers target population to take an active role
  in their healthcare
• A large majority of healthcare interventions in
  tribal areas overlook this integral component,
  often with deleterious results

105
Community Services of the SCD Center
106
Community Interventions

• Creation and issuing of genetic health cards for
  every patient who has or will undergo a
  sickle-cell screening test (neonatal
  screening/hemoglobin electrophoresis) at the
  Center (Not color coded due to stigmatization)
• Field screening
• Central and secure online database of screening
  results in Nilgiri area to prevent test
  duplication-in collaboration with partner
  organizations
• Genetic counseling programs in progress

107
Clinical Services of the SCD Center
108
Clinical Interventions

• Neonatal screening program at Center
• Penicillin Prophylaxis
• Hydroxyurea
• Pneumococcal Immunization (23-conjugate vaccine,
  as 7-valent not available in India)
• Emphasis on compliance

109
New Sickle Cell Disability Scheme

• Periodic treatment for sickle cell disease
  requires patients coming to the hospital on a
  monthly basis for lab tests and medication
• Often, costs of transportation and food prevent
  patients from coming to the hospital, leading to
  frequent crises
• Sickle cell disability fund provided to needy
  patients to cover the cost of roundtrip bus fare
  and lunch at the Center
• Patients apply for aid at the field-based area
  centers and funds disbursed to eligible patients
• Hopefully increases compliance to HU

110
Project Reflections

• Pilot phase 1 year to determine sustainability
  with existing 46 patients
• Without immediate action, millions of tribals
  throughout India will continue to suffer from the
  physiological and social consequences of
  sickle-cell disease
• Pioneers and pilots a genetic health program that
  combines community sensitization with preventive
  and curative measures in a holistic and
  culturally acceptable framework
• Potential to serve as a standard model for
  genetic health care systems for underserved
  populations throughout India

111
Economics of Sickle-Cell Disease Management per
Patient

• Cost of
• Screening Rs. 55 (1/patient)
• 23-Conjugate Pneumococcal Vaccine Rs. 891 (20)
• Penicillin prophylaxis for 1 year Rs. 720 for
  ages 2-5, Rs. 320 for ages 0-2 (15)
• Hydroxyurea 500 mg/day Rs. 2500/year
  (60/patient)
• Outpatient Care Rs. 400/year (9)
• Hospitalization Rs. 3000/year (9)
• Sickle-Cell Disability Fund, Rs. 10,000 total
  (200)