Sickle Cell Anemia

By: Chandler Dixon

Other Names Include:

    • Sickle cell disease

    • Hb SS disease

Causes of the disorder:

  • Sickle Cell Anemia is a rare genetic condition which is the result of mutant beta globin which causes sickling of the hemoglobin.

  • In other words the regular shape of the red blood cells in the body which are supposed to be round and doughnut- shaped, are mutated into a sickle or crescent moon-like shape.

Chromosomes Affected:

  • Sickle Cell affects the hemoglobin-Beta gene (HbSS) on chromosome 11

  • Which affects the number/shape of the red blood cells in the human body

Prenatal Tests:

  • Testing of the father is recommended prior to the pregnancy or at the first doctors visit. If the baby’s father has the Sickle Cell trait then amniocentesis (a process used to obtain a small sample of amniotic fluid) is one of the ways to determine if the fetus will have the trait for the disease.

  • Women who have the disease however, have to have more frequent prenatal visits to monitor the fetus’s health and well being.

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Symptoms:

  • Research has shown that some of the symptoms of Sickle Cell Anemia include: cough, night sweats, pale skin, longer sleeping pattern, tiredness, abdominal pain, poor appetite, swelling in the hands or feet, fatigue, organ failure, death, and most commonly with this disease aches and pains in the legs and joints.

Population Affected:

  • Approximately 1 in every 500 African Americans are born with Sickle Cell Disease

Candidates for Sickle Cell Anemia:

  • Most common among people with African, Spanish, Mediterranean and Indian ancestry

How Sickle Cell is Inherited:

  • It is an autosomal recessive trait
  • Not inherited from the sex chromosomes

Medical Assistance of an Affected Child:

  • As the child gets into the teen years, assistance includes:

  • Help with confidence with body image since problems occur with delayed sexual maturity

  • Learning ways to cope with fear of addiction because of the strong pain medicines

  • Learning ways to prevent lining with uncertainty of the disease because of the unpredictable pain and damage to the body.

  • Often blood tests

  • Often checkups

Long-Term Outlook For The Child:

  • Able to live into the mid 40’s or beyond

  • Possible emotional issues

  • Frequent body pain

  • Needs to visit doctor regularly

  • Frequent vaccines

Treatments/ Cures:

A lifetime treatment of certain medications, fluids, and blood transfusions can help treat and possibly cure this disease

Prevention:

  • Sickle Cell Disease cannot be prevented, if parents of an offspring have the trait then there is a chance that their child could be born with Sickle Cell Disease also.

Children and Sickle Cell Anemia:

Individuals with this disease are able to have children, however there is a good chance they will be affected by the disease.


Chances they will be affected by the disease:

  • If only one parent has the disease then each pregnancy has a 50% chance of producing a child with the disease.


  • If both parents have the disease then the chances are:
    25 percent chance of producing a child with sickle cell disease (SS).

  • 25 percent chance of producing a child with neither sickle cell trait nor sickle cell disease.

  • 50 percent chance of producing a child with sickle cell trait.

Current Research:

  • Scientists are investigating a way to cure the disease with gene therapy, Nitric Oxide, and Decitabine and Butyrate.

Cure/ New Treatment On The Way?

There is a cure developing to the disease, which involves bone marrow and stem cell transplantation that has worked on a few patients; however, it is very expensive, so not a lot of patients have access to the cure.


So technically, there is not yet a common cure for the disease

Sickle Cell Anemia in America:

2 million americans carry one gene for the sickle cell trait.

Works Cited:

Anne, Stumpf M. "Sickle Cell Anemia." OMIM. John Hopkins University, 13 June 1999. Web. 30 Apr. 2015.



Children's Hospital of The King's and Daughters. "Sickle Cell Disease and Pregnancy." Children's Hospital of The King's Daughters Health System. Children's Hospital of The King's Daughters, 16 Oct. 2014. Web. 01 May 2015.


Duke Medicine. "Sickle Cell Disease." US News. U.S.News & World Report, 28 Jan. 2010. Web. 01 May 2015.



Helmsley, Harry B. "Sickle Cell Disease." Disease Info Search. Genetic Alliance, 13 Jan. 2014. Web. 1 May 2015.



National Heart, Lung, and Blood Institute. "Living With Sickle Cell Anemia." - NHLBI, NIH. National Institute of Health, 28 Sept. 2012. Web. 01 May 2015.