SICKLE CELL ANEMIA

ADELINE OLANIRAN

What is Sickle Cell Anemia?

A serious condition in which red blood

cells can become sickle-shaped Normal red blood cells are smooth and round. They move easily through blood vessels to carry oxygen to all parts of the body. Sickle-shaped cells dont move easily through blood. Theyre stiff and sticky and tend to form clumps and get stuck in blood vessels. The clumps of sickle cell block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessel can cause pain, serious infection, and organ damage.

Normal and Sickled Red Blood Cells in Blood Vessels

Figure B shows abnormal, sickled red blood cells clumping and blocking the blood flow in a blood vessel. The inset image shows a cross-section of a sickled red blood cell with abnormal strands of hemoglobin.

Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin.

Source from http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html

Sickle Cell Anemia vs. Sickle Cell Trait

People who have sickle cell anemia are born with it;

means inherited, lifelong condition. They inherit two copies of sickle cell gene, one from each parent. Sickle cell trait is different from sickle cell anemia. People with sickle cell trait dont have the condition, but they have one of the genes that cause the condition. People with sickle cell anemia and sickle cell trait can pass the gene on when they have children.

If one parent has sickle cell trait (HbAS) and the other does not carry the sickle hemoglobin at all (HbAA) then none of the children will have sickle cell anemia. There is a one in two (50%) chance that any given child will get one copy of the HbAS gene and therefore have the sickle cell trait. It is equally likely that any given child will get two HbAA genes and be completely unaffected.

Inheritance of Sickle Cell Anemia

Source from http://www.sicklecellsociety.org/education/inherit.htm#anchor298279

If both parents have sickle cell trait (HbAS) there is a one in four (25%) chance that any given child could be born with sickle cell anemia. There is also a one in four chance that any given child could be completely unaffected. There is a one in two (50%) chance that any given child will get the sickle cell trait.

Inheritance of Sickle Cell Anemia

Source from http://www.sicklecellsociety.org/education/inherit.htm#anchor298279

If one parent has sickle cell trait (HbAS) and the other has sickle cell anaemia (HbSS) there is a one in two (50%) chance that any given child will get sickle cell trait and a one in two (50%) chance that any given child will get sickle cell anemia. No children will be completely unaffected.

Inheritance of Sickle Cell Anemia

Source from http://www.sicklecellsociety.org/education/inherit.htm#anchor298279

If one parent has sickle cell anaemia (HbSS) and the other is completely unaffected (HbAA) then all the children will have sickle cell trait. None will have sickle cell anemia.

The parent who has sickle cell anemia (HbSS) can only pass the sickle hemoglobin gene to each of their children.

Inheritance of Sickle Cell Anemia

Source from http://www.sicklecellsociety.org/education/inherit.htm#anchor298279

Why Anemia?
Anemia is a condition in which a persons blood has a lower

than normal number of red blood cells, or the red blood cells dont have enough hemoglobin. Hemoglobin is an iron-rich protein that gives blood its red color and carries oxygen from the lungs to the rest of the body. Normal red blood cells last about 120 days in the bloodstream and then die. Their main role is to carry oxygen, but they also remove carbon dioxide (a waste product) from cells and carry it to the lungs to be exhaled. In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells dont last very long. Sickle cells die faster than normal red blood cells, usually after only about 10 to 20 days. The bone marrow cant make new red blood cells fast enough to replace the dying ones. The result is anemia.

Who Is At Risk?
The disease originated in at least 4 places in Africa, Mediterranean countries (such as Turkey, Greece, and Italy), and in the Indian/Saudi Arabian subcontinent. It exists in all countries of Africa and in areas where Africans have migrated. It is most common in West and Central Africa where as many as 25% of the people have sickle cell trait and 12% of all babies are born with a form of the disease. In the United States with an estimated population of over 270 million, about 1,000 babies are born with sickle cell disease each year. In contrast, Nigeria, with an estimated 1997 population of 90 million, 45,000-90,000 babies with sickle cell disease are born each year.

Who Is At Risk?
Most common in

people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.

Who is at Risk?
United States, sickle cell anemia

affects about 70,000 people. Mainly affects African Americans, with the condition occurring in about 1 in every 500 African American births. Hispanic Americans also are affected; the condition occurs in 1 out of every 1,000 to 1,400 Hispanic American births. About 2 million Americans have sickle cell trait. About 1 in 12 African Americans has sickle cell trait.

Signs and Symptoms

Individual signs and symptoms

varies. Some have mild symptoms, others have very severe symptoms and may be hospitalized for treatment Present at birth, many infants doesnt show signs until after 4 months of age Anemia: Fatigue (tiredness), pale skin and nail beds, jaundice, and shortness of breath Pain (Sickle Cell Crisis): Sudden episode of pain throughout the body. Common sites: bones, lungs, abdomen, and joints. Lack of blood flow can cause pain and organ damage.

Complication of Sickle Cell Anemia

Hand-Foot Syndrome Splenic Crisis Infections Acute Chest Syndrome Delayed growth and Priapism Gallstone Ulcers on the legs Pulmonary Arterial

puberty in children Stroke Eye problem

Hypertension (High blood pressure) Multiple Organ Failure

Diagnosis
Early diagnosis is very important for proper

treatment USA: 44 States, District of Columbia, Puerto Rico & U.S. Virgin Islands now test ALL newborn for sickle cell anemia. Other 6 States test done best on request

Treatments
Effective treatments are available to

help relieve the symptoms and complications of sickle cell anemia, but in most cases theres no cure. The goal is to relieve the pain; prevent infections, eye damage, strokes and control complications if they occur. Pain medicine: acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics such as meperidine, morphine, oxycodone, and etc. Heating pads Hydroxyurea, Folic Acid Blood Transfusions

New Treatments and Medicines

Bone marrow transplants Gene therapy New medicine Butyric acid. This is a food additive that may increase normal hemoglobin in the blood. Clotrimazole. This is used now to treat fungus infections. This medicine helps prevent the loss of water
from a red blood cell and can keep the cell from turning into a sickle cell.

Nitric oxide. This may make sickle cells less sticky and keep blood vessels open. People with sickle cell
anemia have low levels of nitric acid in their blood.

Prevention
Identify what can trigger the

Crisis such as stress, avoid extremes of heat and cold weather, dont travel airplane that is not cabin pressurized Maintain healthy lifestyle habits
Eating healthy Avoid dehydration Exercise regularly Get enough sleep and rest Avoid alcohol and dont smoke

Regular medical checkups and

treatment are important

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