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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 disparity

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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
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

What is sickle cell anemia sca
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.

Sickle cell syndromes
Sickle Cell Syndromes

  • Sickle cell trait

  • Sickle cell anemia

  • Hemoglobin SC

  • Hemoglobin SE

  • Sickle/Thal anemia

  • Sickle cell HPHF


  • 2 copies of the gene for Hb (each parent)

  • HbS –Recessive

    • S=Sickle

    • A=Normal

    • 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 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.

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.


  • 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.

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

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 tissuedeath

    • 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

Serious complications
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

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

Three common types of

Sickle Cell Disorders

  • Sickle Cell Anemia

    • Sickle hemoglobin (HbS) + Sickle hemoglobin (HbS)

    • Most Severe – No HbA

Other sickling disorders
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
“‘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.

“of all forms of inequality,

injustice in health care is

the most shocking and


Martin Luther King, Jr

Health disparities iom
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 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.

Evidence of racial and ethnic disparities in healthcare
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)‏

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 Preferences


The Operation of Healthcare

Systems and the Legal and

Regulatory Climate


Quality ofHealthCare



Discrimination: Biases and

Prejudice, Stereotyping, and


Populations withEqual Access to Health Care

Summary of findings
SUMMARY OF FINDINGS 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 care.

Disparities in the clinical encounter the core paradox
Disparities in the Clinical Encounter: The Core Paradox with Equal Access to Health Care

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 paradox1
Disparities in the Clinical Encounter: The Core Paradox with Equal Access to Health Care

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’

Summary of recommendations
SUMMARY OF RECOMMENDATIONS with Equal Access to Health Care


  • 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
Sickle cell disease history/awareness with Equal Access to Health Care

  • 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: by putting sickle cell

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)


Treatment of Sickle Cell Pain by putting sickle cell

Fostering Trust and Justice

William T. Zempsky, MD

JAMA. 2009;302(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
The Sickle Cell Pain Believer by putting sickle cell

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 - 18:00 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.

Prevalence With Sickle Cell Disease

  • 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