VOCABULARY

Sickle cell related vocabulary words and terminology.

A

B

C

D

E

F

G

H

I

J

K

L

M

N

O

P

Q

R

S

T

U

V

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A

 

Acute Chest Syndrome (ACS)

A lung-related complication which results from an infection or sickle cells becoming trapped within lungs. ACS is the leading cause of death in sickle cell disease.

 

Anemia

A condition in which an individual has fewer red blood cells, which causes them to feel tired and weak.

 

Aneurysm

A bulging weakness within an artery wall, which fills with blood.

 

Aplastic Anemia Crisis

A condition where the bone marrow stops producing new blood cells.

This is usually caused by parvovirus B19, which is a viral infection.

 

Avascular Necrosis

The death of bone tissue due to lack of blood flow. This most commonly occurs in within the hips and/or shoulders of those with sickle cell disease.

 

 

B

 

Blood Clot

Blood that thickens and forms clumps inside your veins.

 

Bone Marrow

Flexible tissue inside of bones, which produces new blood cells.

 

 

C

 

Carrier

An individual who has one normal gene and one mutation gene.

In terms of sickle cell a carrier has a normal hemoglobin gene and

an abnormal hemoglobin gene.

 

Complication

An adverse development of a secondary disease or condition because

of the primary disease or condition.

 

Congestive Heart Failure

A condition where the heart is unable to pump blood as well as it should, which results in the lack of blood and oxygen needed for body functions.

 

Crisis

See 'Pain Episode'

 

 

D

 

Dactylitis (Hand Foot Syndrome)

Characterized by painful swelling in hands and feet. This is caused by blocked blood circulation.

 

DNA (Deoxyribonucleic Acid)

A molecules that carries all genetic information about you within each cell.

 

 

E

 

Erythrocyte

Red blood cell (RBC).

 

Exchange Transfusion

A procedure that removes patient's blood and replaces it with donor blood. As a cycle of blood is withdrawn through a catheter, a fresh cycle of donor blood is pumped through another catheter.

 

 

F

 

 

G

 

Gallstones

When sickle red blood cells breakdown they release a substance called bilirubin. Because sickle cell breakdown at a faster rate, bilirubin turns to bile at a faster rate, which collects withing the gall bladder and causes gallstones.

 

Gene

A unit of hereditary information. Each gene contains a set of instructions. Genes are passed down from parents to child and are responsible for the characteristics that make you.

 

Genetic

Inherited through genes, from parents.

 

Genetic Counseling

Counseling for prospective parents in regards to probabilities and statistics of passing on genes to future children.

 

 

H

 

Hematuria

Blood in urine.

 

Hemoglobin

The protein in the red blood cells responsible for carrying oxygen from the lungs to the rest of the body. Along with giving blood its color, hemoglobin also plays a role in maintaining the shape of the red blood cells.

 

Hemoglobin Electrophoresis

A blood test that detects the different types of hemoglobins and can help diagnose disorders such as sickle cell.

 

Hemoglobinopathy

A genetic condition that produces an abnormal structure within the hemoglobin (Hb) molecule. Hemoglobin variants include: Thalassemia, Hb S (sickle cell), Hb C, Hb D-Punjab, Hb O-Arab, and many others.

 

Hepatomegaly

Enlargement of the liver caused by build up from blood cells.

 

Heredity

The passing on of genetic characteristics.

 

Hospital-Acquired Infection (HAI)

An infection that is acquired by a patient within a hospital setting. Common HAIs include: severe pneumonia, urinary tract, surgical site, and bloodstream infections. These infections can be fatal.

 

Hypoxia

The reduction of oxygen supply within tissues. Hypoxic conditions cause the red blood cells to sickle, which can lead to vaso-occlusion and hemolysis.

 

 

I

 

Infection

When bacteria or viruses invade the body, which results in illness or disease. Infants and children with sickle cell are more susceptible to infections, which can be fatal if untreated.

 

Iron Overload

A serious condition that develops when the body absorbs too much iron. This can happen over many years and can result in iron buildup within organs. Those who are transfusion-dependent receives excess iron from each transfusion.

 

 

J

 

Jaundice

The yellowing of the eyes or skin. Jaundice occurs because sickle cells die at a more rapid rate. As a result it is harder for the liver to filter them out. Bilirubin from these cells build up in the system and cause the yellow color.

 

 

K

 

Kidney Failure

(aka renal failure) A condition where the kidney stop working properly and loses the ability to remove waste.

 

L

 

Liver Failure

Loss of liver function due to a other complications such as Iron Overload.

 

M

 

Moyamoya

A disorder in which artery walls at the base of the brain thicken, resulting in narrow arteries. This restricts and even blocks blood flow.

 

Mutation

A rare change within the DNA of a gene. Mutations can be passed down from generation to generation.

 

 

N

 

Newborn Screening

A public health program which screens infants shortly after birth to detect a wide variety of conditions that aren't apparent at birth.

 

O

 

 

P

 

Pain Episode/Crisis

Pain ranging from moderate to severe that occurs as a complication of sickle cell. Pain episodes can occur in any part of the body.

 

Pica

Characterized as cravings and/or ingesting non-edible substances (i.e. paper, chalk, foam, dirt). Pica is caused by nutrient deficiency or anemia.

 

Pneumonia

A lung infection caused by bacteria or virus, which inflames air sacs and can fill with fluid.

 

Priapism

Painful erection of the penis.

 

Pulmonary Hypertension

A lung disorder where arteries become narrowed making it harder for blood to flow. This causes high blood pressure within the arteries.

 

 

Q

 

 

R

 

Renal Medullary Carcinoma

A rare form of kidney cancer found patients with sickle cell trait.

 

Renal Papillary Necrosis

A condition in which, part(s) or all of the renal papillae stop functioning. Renal papillae are the openings of the urine collecting ducts.

 

S

 

Splenic Sequestration

Characterized as the sudden enlargement of the spleen due to an overload of red blood cells being trapped within the spleen. This can cause a decrease in hemoglobin, and if gone unnoticed, can be fatal. This is more common in infants and young children.

 

Stroke

The sudden interruption of the blood flow to the brain caused by a blockage within the arteries. In sickle cell, sickle red blood cells clump to the walls of the arteries which cause them to clump together, which narrows the vessel.

 

 

T

 

Trait

An inherited characteristic.

 

U

 

Urinary Tract Infection

An infection that can occur in any part of the urinary tract system including kidneys, ureters, bladder, or urethra.

 

V

 

Vaso-occlusive Crisis

Caused by the sickle-shaped red blood cells obstructing blood flow through capillaries. This can result in severe pain, organ damage, and necrosis.

 

 

W

 

 

X

 

 

Y

 

 

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