Sickle Cell Anemia & Health Disparities

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Sickle Cell Anemia Health Disparities

• Everardo Cobos, MD
• Hematology Division
• Summer Seminar on Health Disparities

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Sickle Cell Anemia Health Disparities outline

• Review of sickle cell anemia
• Brief review of health disparities
• Interplay between sickle cell anemia and health
  disparity
• Increase awareness of racial, ethnic, gender,
  geographic disparities in health care among the
  general public and key stakeholders, and increase
  health care providers awareness of disparities

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What is Sickle Cell Anemia (SCA)?

• First described in Chicago in 1910 by James
  Herrick as an inherited condition that results in
  a decrease in the ability of red blood cells to
  carry oxygen throughout the body

Sickle red blood cells become hard and
irregularly shaped (resembling a sickle) Become
clogged in the small blood vessels and therefore
do not deliver oxygen to the tissues. Lack of
tissue oxygenation can cause excruciating pain,
damage to body organs and even death.
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Red blood cells Going through Vessels
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Sickle Cell Syndromes

• Sickle cell trait
• Sickle cell anemia
• Hemoglobin SC
• Hemoglobin SE
• Sickle/Thal anemia
• Sickle cell HPHF

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• Genetics
• 2 copies of the gene for Hb (each parent)
• HbS Recessive
• SSickle
• ANormal
• Genetic disease NOT a black disease

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Some Genetic History

• The error in the hemoglobin gene results from a
  genetic mutation that occurred many thousands of
  years ago in people in parts of Africa, the
  Mediterranean basin, the Middle East, and India.
• A deadly form of malaria was very common at that
  time
• Malaria epidemics caused the death of many
• In areas where malaria was a problem, children
  who inherited one sickle hemoglobin gene and who,
  therefore, carried the sickle cell trait - had a
  survival advantage.
• Unlike the children who had normal hemoglobin
  genes, they survived the malaria epidemics they
  grew up, had their own children, and passed on
  the gene- for sickle hemoglobin.

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Sickle Cell Gene
Severe Malaria
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Sickle Hemoglobin Haplotype Distribution in
Africa. The three major ßs-globin haplotypes
found in Africa are shown. The distributions
represent the highest concentrations. The genes
are expressed at lower frequency outside the
highlighted zones.
Sickle Hemoglobin Haplotype Distribution in the
Middle East and India. The ßs-globin haplotype
found in the Middle East and India are shown. The
haplotypes are identical in the two areas. The
gene probably originated in India and was carried
to the Persian Gulf area by trade and migration.
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Hemoglobin Haplotype
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History

• As populations migrated, the sickle cell-mutation
  spread to other Mediterranean areas, further into
  the Middle East and eventually into the Western
  Hemisphere.
• In the United States and other countries where
  malaria is not a problem, the sickle hemoglobin
  gene no longer provides a survival advantage.
• Instead, it may be a serious threat to the
  carrier's children, who may inherit two abnormal
  sickle hemoglobin genes and have sickle cell
  anemia.

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Medical Complications

• kidney damage and
• loss of body water in urine
• painful erections in men (priapism)
• blood blockage in the spleen or liver
  (sequestration)
• eye damage
• low red blood cell counts (anemia)
• delayed growth

• pain episodes
• strokes
• increased infections
• leg ulcers
• bone damage
• yellow eyes or jaundice
• early gallstones
• lung blockage

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Serious Complications

• Infectious complications
• Prominent early in life
• Leading cause of morbidity and mortality
• Great improvement in the prognosis related to
  newborn screening for sickle cell disease,
  vaccination for childhood illnesses, the use of
  prophylactic antibiotics, and aggressive
  diagnosis and treatment of febrile events
• Acute splenic sequestration
• Episodes of rapid increase in splenic size and
  decrease in hemoglobin
• Potential source of morbidity and mortality early
  in life for children with sickle cell anemia and
  at any age for those with Hb SC disease and
  sickle thalassemia

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Serious Complications

• Strokes
• Up to 15 of children may have overt or silent
  strokes during childhood
• Chronic transfusion therapy reduces the
  recurrence rate of overt stroke which may
  approach 75 without intervention
• Bone disease
• Early risk is primarily from osteomyelitis
• Infectious usually painful inflammatory disease
  of bone often of bacterial origin and may result
  in bone tissue death
• Avascular necrosis of the femur and humerus
• Death of bone tissue due to disrupted blood
  supply
• Marked by severe pain in the affected region and
  by weakened bone that may flatten and collapse

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Serious Complications

• Leg ulcers
• Seen in patients older than 10 years of age
• Resistant to therapy and cause significant
  morbidity
• Ophthalmic complications
• Proliferative retinopathy, vitreous hemorrhage,
  retinal detachment
• Priapism
• Distressing complication that occurs at all ages
• Difficult to treat
• Causes a high incidence of impotence
• Chronic Anemia
• Associated with fatigue, irritability, jaundice,
  pain, delayed puberty, leg sores, eye problems,
  gum disease

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Serious Complications PAIN Recurrent Pain
Episodes or Sickling Crises

• Occur at any age but appear to be particularly
  frequent during late adolescence and early adult
  life
• Unpredictable
• Red Blood Cells get stuck in the small veins and
  prevent normal blood flow
• Characterized by severe pain in the back, chest,
  abdomen, extremities, and head
• Highly disruptive to life
• Most common reasons for individuals to seek
  health care

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Three common types of Sickle Cell Disorders

• Sickle Cell Anemia
• Sickle hemoglobin (HbS) Sickle hemoglobin (HbS)
• Most Severe No HbA

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Other Sickling Disorders

• Other types of Hb combine with sickle Hb
• Hemoglobin S-C disease
• Sickle hemoglobin (HbS) (HbC)
• Hemoglobin S-Beta thalassemia
• Beta thalassaemia gene reduces the amount of HbA
  that can be made
• Sickle hemoglobin (HbS) reduced HbA
• Milder form of Sickle Cell Disorder than sickle
  cell anemia

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Sickle Cell Anemia It's Not a Black Disease

• One of the common misconceptions is that only
  black people get sickle cell, but this is not the
  case.
• There are many different ethnicities that can
  have sickle cell.

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of all forms of inequality, injustice in health
care is the most shocking and inhumane Martin
Luther King, Jr
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Health disparitiesIOM

• STUDY CHARGE
•  
• Assess the extent of racial and ethnic
  differences in healthcare that are not otherwise
  attributable to known factors such as access to
  care (e.g., ability to pay or insurance
  coverage)
• Evaluate potential sources of racial and ethnic
  disparities in healthcare, including the role of
  bias, discrimination, and stereotyping at the
  individual (provider and patient), institutional,
  and health system levels and,
• Provide recommendations regarding interventions
  to eliminate healthcare disparities.

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Evidence of Racial and Ethnic Disparities in
Healthcare

• Disparities consistently found across a wide
  range of disease areas and clinical services
• Disparities are found even when clinical factors,
  such as stage of disease presentation,
  co-morbidities, age, and severity of disease are
  taken into account
• Disparities are found across a range of clinical
  settings, including public and private hospitals,
  teaching and non-teaching hospitals, etc.
• Disparities in care are associated with higher
  mortality among minorities (e.g., Bach et al.,
  1999 Peterson et al., 1997 Bennett et al.,
  1995)?

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Black and White Differences in Specialty
Procedure Utilization Among Medicare
Beneficiaries Age 65 and Older
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Differences, Disparities, and Discrimination
Populations with Equal Access to Health Care
Clinical Appropriateness and Need Patient
Preferences
Non-Minority
The Operation of Healthcare Systems and the
Legal and Regulatory Climate
Difference
Quality of Health Care
Minority
Disparity
Discrimination Biases and Prejudice,
Stereotyping, and Uncertainty
Populations with Equal Access to Health Care
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SUMMARY OF FINDINGS
Racial and ethnic disparities in health care
exist and, because they are associated with worse
outcomes in many cases, are unacceptable.   Racial
and ethnic disparities in health care occur in
the context of broader historic and contemporary
social and economic inequality, and evidence of
persistent racial and ethnic discrimination in
many sectors of American life.   Many sources
including health systems, health care providers,
patients, and utilization managers contribute
to racial and ethnic disparities in health
care.  
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Disparities in the Clinical Encounter The Core
Paradox

• How could well-meaning and highly educated health
  professionals, working in their usual
  circumstances with diverse populations of
  patients, create a pattern of care that appears
  to be discriminatory?

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Disparities in the Clinical Encounter The Core
Paradox

• Possibilities examined bias (prejudice),
  uncertainty, stereotyping
• Bias no evidence suggests that providers are
  more likely than the general public to express
  biases, but some evidence suggests that
  unconscious biases may exist
• Uncertainty a plausible hypothesis,
  particularly when providers treat patients that
  are dissimilar in cultural or linguistic
  background
• Stereotyping evidence suggests that physicians,
  like everyone else, use these cognitive
  shortcuts

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SUMMARY OF RECOMMENDATIONS

• GENERAL RECOMMENDATIONS
• Increase awareness of racial, ethnic, gender,
  geographic disparities in health care among the
  general public and key stakeholders, and increase
  health care providers awareness of disparities.

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Sickle cell disease history/awareness

• 1920 described in literature
• 1949 Linus Pauling Sickle Cell Disease A
  Molecular Disease, Science
• 1968-1972 period of political activity/awareness
  for sickle cell disease
• MLK health disparities sickle cell
• A warm december Sidney Portier
• TV movie Bill Cosby
• Marcus Welby, MD

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President Richard Nixon surprised many in his
health message by putting sickle cell disease
into the glowing spotlight of presidential
politics. He said, "There are moments in
biomedical research when problems begin to break
open and results begin to pour in. We believe
that cancer research has reached such a point. A
second targeted disease for constrained research
should be sickle cell anemia. It is a sad and
shameful fact," he said, "that the causes of this
disease have been largely neglected throughout
our history. We cannot rewrite the record of
neglect, but we certainly can reverse it."
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Funding There are huge disparities between the
amounts of federal dollars allocated to sickle
cell when compared to other disorders. For
example, there are three times as many babies
born with SCD than with cystic fibrosis, 8,000
versus 2,500. For every baby born with cystic
fibrosis there are 2,733 research dollars spent
to find a cure in contrast to 345.58 spent for
every baby born with SCD (National Institutes of
Health) Source http//www.nbcchicago.com/brchann
el/FP-HLX-Background-Information-on-Sickle-Cell-Di
sease.htmlixzz0uHEhUidB
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Treatment of Sickle Cell Pain Fostering Trust and
Justice William T. Zempsky, MD
JAMA. 2009302(22)2479-2480.
The following scenario serves as an example of an
emerging literature on injustice in health
care. A 19-year-old man with sickle cell
disease presents to the emergency department with
progressive leg and back pain. His hooded
sweatshirt is pulled over his eyes, he is wearing
headphones, and is singing along to an unheard
tune. His attempts to manage his pain at home
have been unsuccessful and he tells the nurse
that his pain is a 9 on a 10-point scale. The
nurse responds with apparent disbelief and says
"Really?" then sends the patient to the waiting
room where he sits for several hours before
seeing a physician. The patient's request for a
specific dose of morphine is met with doubt and
disdain. This patient is stigmatized as a drug
seeker or abuser, with little consideration for
the complexities of his pain. Justice in this
context is . . .
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The Sickle Cell Pain Believer
I woke up in severe pain. I tried to control
it, but in vain. I went to my primary care
physician Hoping to achieve a remission. He
said, "You are a drug addict, And that's my
verdict. I do not believe what you say. There
will be no prescription today. You did not keep
the terms of our agreement And, hence, there will
be no treatment. I dragged myself to the
emergency room. There I was also met with doubt
and gloom. I had to go through triage And wait
for release from my quayage. Hours passed at a
pace that was slow But my pain continued to grow.
Sleepy, hungry and thirsty, I collapsed. I opened
my eyes with my pain enhanced. They said I had a
seizure, In addition to a high fever. They
rushed me into a stall And treated me with
diminished gall. Finally, I received pain meds
with some relief. This greatly reduced my
grief. They decided to admit me to the
hospital A decision that was most acceptable. I
was happy to come to the end of my ordeal And to
be treated with respect and more zeal. But I am
most thankful to the seizure. It made a doubting
physician a believer.
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Bioethicists Examine Trust Toward Physicians
Among Adults With Sickle Cell Disease Main
Category Biology / BiochemistryArticle Date 04
Jun 2010 - 1800 PDT
Posted February 10, 2010 Hematology
News Access to care, pain relief still problems
for sickle cell patients   Even though expert
panels recommend hydroxyurea therapy and clinical
trial data assure its safety and efficacy,
confounding barriers continue to restrict its
use.
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Prevalence

• It is estimated the up to 80,000 people in
  America have Sickle Cell Disease
• 1/500 African Americans have Sickle Cell Disease
• 1/1000 -4000 Hispanics have Sickle Cell
• 1/12 African Americans are carriers for Sickle
  Cell
• 1/50 Asians are carriers for Sickle Cell
• 1/100 Greeks are carriers for Sickle Cell

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