1910
• Sickle cells were first explained by Janes B. Herrick.
• He was cardiologist and a professor of medicine.
1922
• Verne Mason – a medical patient at Johns Hopkins Hospital- first named the disease.
1927
• Hahn and Gillespie showed that sickling of the red cells was related to low oxygen.
History Continued…
1948
• Itano and Pauling discovered that hemoglobin from patients with sickle cell was different from the type of hemoglobin found in normal people.
1956
• The disease became the first genetic disorder whose molecular basis was known.
1984
• Bone marrow transplantation in a child with sickle cell disease produced the first reported cure of the disease.
1955
Hydroxyurea became the first (and only) drug proven to prevent complications of sickle cell disease in the Multicenter Study of Hydroxyurea in Sickle Cell Anemia.
What is Sickle-Cell anemia?
Type of anemia
Anemia- condition where the body lacks enough healthy red blood cells. Red blood cell count is unusually low.
Inherited Blood
Disorder
Blood cells are normally “disks” and
look like donuts.
Have hemoglobin proteins that carry oxygen
from the lungs.
Sickle shaped cells are those that are affected
Blood cells that contain sickle hemoglobin
are crescent/ sickled shaped.
What is this like visually?
What happens?
Sickle cells obstruct the blood flow in blood vessels of limbs and organs.
This leads to
Pain, severe infections, and organ damage
The Symptoms include…
• the result of a high red blood cell breakdown rate. This is shown through a yellow tint of the skin and eye-whites.
Jaundice
• due to the clotting. Periods of such pain are called pain crises. This may also come with a fever, cough, and difficulty in breathing.
Muscle and organ pain
More Symptoms
Bone Pain
Delayed Puberty/Growth
Fatigue
Paleness
Rapid Heart Rate
Ulcers
Excessive Thirst
Poor eyesight
Frequent urination
Strokes
How do you get Sickle Cell Anemia?
First off, there are two cases of having Sickle Cells:
Sickle Cell trait- you carry the gene for sickle hemoglobin, but you do not have it.
Sickle Cell Anemia- you inherit both recessive alleles for the sickle hemoglobin gene and you are born with it.
Chromosome that codes for Sickle Hemoglobin is…
Chromosome 11 carries the gene HBB that is associated with Sickle Cell Anemia.
It is an autosomal recessive genetic disorder.
Carriers have the genotype SA (sickle cell trait)
Those who have S.C.A. have SS.
Living with Sickle Cell AnemiaMost people with sickle cell anemia can still lead perfectly normal lives. However, symptoms of the disease such as episodes of severe pain known as “crisis”, chronic pain, fatigue and frequent hospitalization may hinder a sickle cell patient’s life.
For the family, it may be a very painful thing emotionally because of the frequent hospitalizations and the hospital bills that would accumulate from that and also because of the daily pain that the patient would have to go through.
How is Sickle Cell Anemia treated?
Treatments for sickle cell include medicine, blood transfusions and specific other treatments for complications that may arise.
Mild crises can be treated with simple things like over the counter medicines, however, more painful crises usually result in hospitalization where the patient will be given drugs such as
acetaminophen, non-steroidal anti-inflammatory drugs (NSAIDs), and narcotics to ease the pain.
Blood transfusions are usually used to treat worsening sickle cell symptoms such as worsening anemia or enlargement of
the spleen. Regular blood transfusions can be dangerous though since there can be a dangerous buildup of iron in the
blood which leads to an increased risk of infection.
Other examples of types of treatment: Leg ulcers - treated with cleansing solutions and medicated creams or ointmentsGallbladder– surgery to remove the stones.
Fun Facts
• 1 in 400 African Americans is born with Sickle Cell Anemia, affecting more Americans than Cystic Fibrosis and Phenylketonuria do.
1
• Normal red blood cells live for 120 days, but sickle-cells live 10-20 days.
2
• S.C.A. is most common to those with heritages from Africa, India (BLESSAN), the Caribbean, the Middle East .
3
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