Sickle Cell Anemia
The red blood cells coursing through your veins deliver critical oxygen to tissues and
organs throughout your body.
o Sickle
... [Show More] Cell Anemia disrupts this important process by distorting normally flexible,
disc-shaped red blood cells into stiff crescents that get stuck inside of blood
vessels.
o The resulting blockages lead to pain (massive pain) and organ damage.
o Sickle Cell Anemia affects about 70,000 people in the United States—mostly
those with ancestors who lived in Africa, the Mediterranean (occasionally), India,
or the Middle East within the last thousand years.
o Occurs primarily in African Americans.
o Occurrence of 1 in every 375 infants born in the United States.
"Sickled" red blood cells can clog small blood vessels, preventing oxygen from making it
to certain parts of the body. In the short term, this can cause excruciating episodes of
pain called "crises." If the small blood vessels in the hands or feet are blocked, swelling
will occur. This is sometimes the first sign of Sickle Cell Anemia in infants.
o When you see some swelling in the hands and feet this will be one of the first
indications in infants that they do have sickle cell anemia.
Comes from areas in the world where malaria is common, individuals with sickle cell
trait tend to have survival advantage over those without trait.
Autosomal recessive disorder:
o 9% of African Americans are carriers (have sickle cell trait)
o 40% among native Africans are carriers
Most common ways of getting the disease:
o If both parents have trait, each offspring will have one in four chances (25%
chance) of having the disease.
o If one parent has sickle cell disease and the other has sickle cell trait, each of
their children will have a 50 percent chance of having sickle cell disease and a 50
percent chance of having sickle cell trait.
o If one parent has sickle cell disease and the other does not, all of their children
will have sickle cell trait, but none of them will have sickle cell disease.
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There are the normal RBC which flow freely, when they start sickling they start
interrupting the normal flow, and this interruption is what causes all the pain, such as
abdominal pain, swelling of the hands and feet, ulcers…
There is no cure except a possible bone marrow transplant.
Supporting care/ prevent sickling episodes.
o Make sure always of oxygen being quick and fluid.
Frequent bacterial infection may occur because of immunocompromise.
Bacterial infection is leading cause of death in young children with sickle cell disease.
Strokes in 5% to 10% of children with the disease.
The spleen, which helps filter bacterial infections from the blood and acts as the recycle
center for old red blood cells, tends to destroy the misshapen sickle cells faster than
normal red blood cells. Sickle cells only live for about 14 days, while normal red blood
cells can live up to 120 days. This leads to a chronic lack of oxygen-carrying red cells in
the blood which we know as anemia. Sickle cells can also damage the child’s spleen,
increasing the risk of certain potentially life-threatening bacterial infections.
Precipitating factors that lead to sickle cell crisis
o Anything that increases the body’s need for oxygen or alters transport of oxygen
o Trauma
o Infection, fever (we want to keep infection rate low because the more infection
we have the bigger the chance of getting a sickle cell crisis)
o Physical and emotional stress
o Increased blood viscosity caused by dehydration
o Hypoxia
The severity of the symptoms of sickle cell disease can vary greatly depending on the
specific genetic type and even within those of the same type. Each child may experience
symptoms differently and it is very difficult to predict.
Acute exacerbation that vary on severity and frequency
o Types:
Vaso-oclussive thrombotic [Pain crisis (also called vaso-occlusive crisis)]
Most common type of crisis; very painful
Stasis of blood with clumping of cells in microcirculation ->
ischemia -> infarction
Signs: fever, pain, tissue engorgement
With a sickle cell crisis you always hydrate first and then you use
oxygen.
occurs when the flow of blood is blocked to an area because the
sickle-shaped cells have become stuck in the blood vessel
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Pain can occur anywhere but most often occurs in the bones of
the arms, legs, chest and spine.
Painful swelling of the small bones of the hands and feet
(dactylitis) can occur in infants and toddlers.
Priapism results from sickling that occurs in the penis. This results
in a painful and unwanted erection.
Any interruption in blood flow to the body can result in pain,
swelling, dysfunction and possible death of the surrounding tissue
not receiving adequate blood and oxygen.
Splenic sequestration (pooling):
A lot of the sickle red cell blood cells become trapped in the
spleen, the spleen enlarges (is also painful) and gets damaged and
does not work as it normally would the person is more likely to
have a serious life-threatening infection with certain types of
bacteria and they can become septic and die.
Sickle cells can block the exit of blood from the spleen resulting in
pooling of sickle-shaped cells in the spleen, causing a sudden
worsening of the anemia.
Life threatening: death can occur within hours when this happens
suddenly and is considered an emergency, and this will also cause
sudden and severe anemia, symptoms of sudden weakness, pale
lips, rapid breathing, excessive thirst, belly pain, and rapid
heartbeats.
Blood pools in the spleen
A severe episode of sequestration requires surgical removal of the
spleen.
Aplastic crisis:
Diminished production and increased destruction of RBCs.
Triggered by viral infection or depletion of folic acid
Signs include profound anemia, pallor
Bone marrow suddenly stops producing red blood cells, which
results in sudden and severe anemia. During this time, the patient
will often feel tired, have pale skin, and be short of breath.
Blood transfusions might be done to treat an aplastic crisis. After a
few days, the bone marrow usually recovers on its own. And red
blood cell production returns to its usual rate.
Chest syndrome (ACS- Acute Chest Syndrome)- is a noninfectious
vaso-occlusive crisis of the pulmonary vasculature commonly seen
in patients with sickle cell anemia. It is characterized by a new
infiltrate on a chest x-ray.
o when sickle-shaped cells stick together and block the flow
of oxygen in the vessels in the lungs
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