SICKLE CELL ANEMIA - Sickle Cell Ministries

ADELINE OLANIRAN

SICKLE CELL ANEMIA

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 don’t move easily through blood. They’re 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 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.

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.

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 don’t 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.

Inheritance of Sickle Cell Anemia

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.

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.



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.



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.


Why Anemia?

Anemia is a condition in which a person’s blood has a lower than normal number of red blood cells, or the red blood cells don’t 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 don’t last very long. Sickle cells die faster than normal red blood cells, usually after only about 10 to 20 days. The bone marrow can’t 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 1-2% 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 doesn’t 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-Food SyndromeSplenic CrisisInfectionsAcute Chest

SyndromeDelayed growth and

puberty in childrenStrokeEye problem

PriapismGallstoneUlcers on the legsPulmonary Arterial

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

TreatmentsEffective treatments are available to

help relieve the symptoms and complications of sickle cell anemia, but in most cases there’s 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 padsHydroxyurea, Folic AcidBlood Transfusions

New Treatments and Medicines

Bone marrow transplantsGene therapyNew 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, don’t 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 don’t smoke

Regular medical checkups and treatment are important

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http://www.webmd.com/video/sickle-cell-miracle

SICKLE CELL ANEMIA - Sickle Cell Ministries

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