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Glossary

Acute Chest Syndrome (ACS)
ACS is caused by obstruction of the blood vessels in the lungs or by an infection namely pneumonia. ACS is a life threatening condition, early detection and treatment are essential.

Anemia
Individuals with Sickle cell diease have fewer red blood cells than the norm because their red blood cell are destroyed more rapidly than usual. The average red blood cell lives approximately 120 days. In those with sickle cell diease, the red blood cells live only 10 to 3o days thereby producing anemia. This shorten life span of the red blood cell, which may make eyes yellow and urine darker. The individual may also experience increased fatigue and paleness in color.

Aplastic Crisis
This occurs when the body stops making new red blood cells usually caused by an infection. This is a life-threatening condition and must be treated immediately with blood transfusion and treatment of any accompanying infection. This is most often seen in early childhood, nonetheless, may occur at any age.

Avascular Necrosis
When blood flows to the hip joint is slowed by sickle cells, the bone in the joint becomes flat and crooked and the hip cannot move easily and without pain. Walking puts pressure on the joint and causes more damage to it. (This condition also occurs in the shoulder and knee joint).

Cardiac Disorder
The chronic anemia state of individuals with SCD causes the enlargement of the heart and heart murmurs are common.

CBC
Abbreviation for Complete Blood Count which includes red and white blood cells counts and hemoglobin levels.

Dactylitis
Painful swelling of the feet and hands during the first several years of life of children with sickle cell anemia.

Fetal Hemoglobin (HbF)
The hemoglobin that is normally made in the fetus. The presence of increased levels of fetal hemoglobin within the red blood cells of people with sickle cell disease decrease the severity of the disease.

Gallstones
Gallstones are common and related to the increased breakdown of hemoglobin in the destroyed red blood cells. Removal of the gallbladder is sometimes necessary in many individuals by early adulthood.

Hb
Abbreviation for Hemoglobin.

Hemoglobin
The red substance in red blood cells that carries oxygen.

Kidney
Frequent urination and bed-wetting is a common problem found in young children. This is due to the kidney's inability to maximally cncentrate urine. There is also an increase incidence of urinary tract infections along with the effects of "sickling" process could lead to kidney damage.

Leg Ulcers
Leg ulcers are common in adults and are seen on the lower 1/3 of the leg. They can painful, recurrent and are difficult to heal.

Oxygenation
The binding of oxygen. (i.e. Hemoglobin takes up oxygen from the lungs and transfer it to the tissues).

RBC
Abbreviation for Red Blood Cells.

Red Blood Cell
The cell that contains hemoglobin.

Sickling
The formation of sickle hemoglobin crystals within the RBC which distorts its normal round shape.

Spleenic Sequestration
Young children may have episodes of blood pooling in the spleen, which may be life threatening. Often, the spleen is dysfunctional by 6 to 9 months of age and has shrunk by adulthood. If young children continue to have this problem, surgery may be required.

Visual Changes
There is an increased risk for retinal changes and increased intracocular pressure. An annual check up by an ophthalmologist is recommened in individuals above 8 years of age for early identification and management of problems.

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