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Sickle Cell Anemia & Health Disparities


Sickle Cell Anemia & Health Disparities Everardo Cobos, MD Hematology Division Summer Seminar on Health Disparities * * * * * * * * * * Evidence of Racial and Ethnic ... – PowerPoint PPT presentation

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Title: Sickle Cell Anemia & Health Disparities

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

Sickle Cell Anemia Health Disparities outline
  • Review of sickle cell anemia
  • Brief review of health disparities
  • Interplay between sickle cell anemia and health
  • 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.
Red blood cells Going through Vessels
Sickle Cell Syndromes
  • Sickle cell trait
  • Sickle cell anemia
  • Hemoglobin SC
  • Hemoglobin SE
  • Sickle/Thal anemia
  • Sickle cell HPHF

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

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
  • 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.

Sickle Cell Gene
Severe Malaria
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.
Hemoglobin Haplotype
  • As populations migrated, the sickle cell-mutation
    spread to other Mediterranean areas, further into
    the Middle East and eventually into the Western
  • 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

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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
  • 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

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

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
  • Marked by severe pain in the affected region and
    by weakened bone that may flatten and collapse

Serious Complications
  • Leg ulcers
  • Seen in patients older than 10 years of age
  • Resistant to therapy and cause significant
  • 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

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
  • 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

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

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
  • 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
Health disparitiesIOM
  • 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
  • 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.

Evidence of Racial and Ethnic Disparities in
  • 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.,

Black and White Differences in Specialty
Procedure Utilization Among Medicare
Beneficiaries Age 65 and Older
Differences, Disparities, and Discrimination
Populations with Equal Access to Health Care
Clinical Appropriateness and Need Patient
The Operation of Healthcare Systems and the
Legal and Regulatory Climate
Quality of Health Care
Discrimination Biases and Prejudice,
Stereotyping, and Uncertainty
Populations with Equal Access to Health Care
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
Disparities in the Clinical Encounter The Core
  • 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?

Disparities in the Clinical Encounter The Core
  • 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
  • Stereotyping evidence suggests that physicians,
    like everyone else, use these cognitive

  • 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.

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

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."
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//
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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 . . .
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.
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
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  • 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
  • 1/50 Asians are carriers for Sickle Cell
  • 1/100 Greeks are carriers for Sickle Cell

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