Sickle-cell anemia is a genetic blood disorder caused by an autosomal recessive trait where the normal hemoglobin A is replaced with abnormal hemoglobin S. Symptoms include anemia from the breakdown of red blood cells, frequent pain from blockages in blood vessels, jaundice from liver damage, and limited growth from low oxygen levels. Treatments involve folic acid, pain medications, and procedures for complications, but there is no cure. It predominantly affects those of African descent, and while carriers are resistant to malaria, full sickle-cell anemia can have severe symptoms.

1. Sickle-Cell Anemia

2. Symptoms
• Anemia: The shriveled blood cells of an individual with this disorder may
break apart, causing a shortage in red blood cells, thus providing little
oxygen to the body for usage. Called anemia, this oxygen deprivation often
results in fatigue for the individual.
• Frequent Internal Pain: Because of their irregular shape, sickled blood
cells may become caught in certain circulatory passages, causing pain
through the restriction of blood to chest and the abdomen. This blockage
may also occur in the hands and feet, which are known to swell in sickle-cell
patients.
• Jaundice: The livers of afflicted individuals become overwhelmed by the
collapse of weakened blood cells, causing extensive damage to the liver
and the eventual yellowing of the skin.

3. • Damaged vision: The sickled blood cells, awkward in shape, often clog
the blood vessels that connect to the retina, causing optical damage.
• Limited Growth: The scarcity of oxygen caused by sickle-cell anemia is
detrimental to healthy human growth.

4. Causes
• Normal hemoglobin is classified as Hemoglobin A, while sickle-cell
patients produce Hemoglobin S.
• Sickle-cell anemia is caused by an autosomal homozygous
recessive trait. Thus, each parent’s genotype must contain at least
one recessive allele to produce affected offspring.
• The presence of only one allele is also potentially harmful, as
carriers of sickle-cell anemia produce both types of hemoglobin, and
thus often experience minor symptoms in environments of limited
oxygen.

6. Treatment
• Unfortunately, there is no method to prevent ailment.
• Folic Acid: Because the sickle cells are weak and prone to shattering, folic acid, a
nutrient vital in blood cell production, is constantly taken by sickle-cell patients.
• Drugs: During a sickle-cell crisis, a period in which body parts ache due to blood
flow blockage, pain medications are often required to ease the intense pain, and in
some cases, narcotics, like Hydroxyurea, are taken into the blood stream.
• Treatments for Possible Complications:
– Dialysis or kidney transplant for kidney disease
– Drug rehabilitation and counseling
– Gallbladder removal
– Hip replacement
– Partial exchange transfusion for acute chest syndrome
– Surgery for eye problems
– Transfusions or surgery for strokes

7. Screening
• Hemoglobin Electrophoresis: Once blood has been drawn, an blood
analysis is performed to determine the concentration of different hemoglobin
molecules as percentages of the total hemoglobin count.
• Normal Levels:
• Hgb A1: 95% to 98%
• Hgb A2: 2% to 3%
• Hgb F: 0.8% to 2%
• Hgb S: 0%
• Hgb C: 0%
• Sickle-Cell Test: In this test, blood is extracted to determine if an
individual possesses abnormal hemoglobin, labeling them either carriers
(sickle-cell trait individuals) or affected (sickle-cell anemia individuals).
• Complete Blood Count Test: Patients are screened for blood-cell
counts, sizes, concentration, and hemoglobin content.

9. Sickle Cell Anemia: The Population
• Millions of people around the globe are thought to have the sickle-cell
anemia.
• How Many: 1 in every 5000, totaling over 70,000, United States citizens
have the condition.
• Who: Sickle-Cell Anemia predominantly affects African-Americans.
• Where: The region of the world most detrimentally affected by this disease
is northern Africa, where one-third of inhabitants carry the gene.
– Fast Fact: A genetic mutation has allowed sickle-cell carriers to be resistant to
malaria.
• Society: Fortunately, there is no discrimination or stereotyping of sickle-
cell patients in society

10. Famous People with Sickle-Cell Anemia
• Miles Davis
• Paul Williams (The Temptations)
• T-Boz (TLC)
• Georgeanna Tillman (The Marvelettes)

11. Bibliography
• "Sickle cell anemia." Mayo Clinic medical information and tools for healthy living -
MayoClinic.com. Mayo Clinic. 25 Feb. 2009
<http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324>.
• "Sickle-cell disease -." Wikipedia, the free encyclopedia. 25 Feb. 2009
<http://en.wikipedia.org/wiki/Sickle-cell_disease>.
• "Medical Encyclopedia: Sickle cell anemia." National Library of Medicine - National
Institutes of Health. 25 Feb. 2009
<http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm>.
• "What is Sickle Cell Disease." Sickle Cell Disease Association of America -- SCDAA
Home. 25 Feb. 2009
<http://www.sicklecelldisease.org/about_scd/>.
• "Sickle cell disease." Genetics Home Reference - Your guide to understanding genetic
conditions. 25 Feb. 2009
<http://ghr.nlm.nih.gov/condition=sicklecelldisease>.