Sickle cell disease

Common Name(s)

Sickle cell disease, Sickle cell anemia, Hb SS disease

Sickle cell anemia (Hb SS) is a rare genetic condition. Hb SS affects the number or shape of the red blood cells in the body. Normally, the red blood cells are round and donut-shaped. They bring the oxygen we breathe from our lungs to different parts of our body. Those with Hb SS make some red blood cells that are crescent or sickle shaped rather than a round, donut shape. These unusually shaped cells do not last as long as normal red blood cells and die off. This can lead to anemia (a low number of red blood cells). The sickle or crescent shaped cells also tend to get stuck in blood vessels. When this happens, some parts of the body may not get enough blood. If red blood cells are not able to move through the blood vessels, parts of the body cannot get enough oxygen. Therefore, it is very important for our bodies to have an adequate number of red blood cells.

Those affected by Hb SS may develop signs and symptoms a couple of months after birth. The characteristic features of this condition may include pale skin, sleeping longer or more often, tiredness, difficulty breathing, pain or swelling in the hands or feet, and cold hands or feet. If untreated, this disorder can lead to organ failure and death. Many of these complications can be prevented with early detection and lifelong management with appropriate medications, fluids, and blood transfusions. Many babies are screened for Hb SS at birth so that treatment can begin early, however the conditions included in newborn screening differ from state to state. For more information, visit Baby’s First Test. Hb SS is an autosomal recessive trait. A genetic counselor can help you understand the genetics. Also talk with your doctor and specialists to decide on the best treatment plan. Support groups are also a good source of information.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Sickle cell disease" for support, advocacy or research.

Citizens for Quality Sickle Cell Care-SCDAA-Northern, CT Chapter, Inc.

To ensure the availability and accessibility of quality and comprehensive medical care and support services for children and adults in northern Connecticut with Sickle Cell Disease and other related disorders.

Last Updated: 13 Jan 2014

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North Alabama Sickle Cell Foundation, Inc.

North Alabama Sickle Cell’s mission is to aid and enhance the lives of those affected by Sickle Cell and associated disorders through the provision of supportive services, education and advocacy.

Last Updated: 16 Jan 2014

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Sickle Cell Disease Association of America, Southern Connecticut, Inc.

The Sickle Cell Disease Association of America Southern Connecticut Inc. mission is to provide education,screening, counseling,and support services to persons affected with sickle cell disease and the trait.

Last Updated: 3 May 2014

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William E. Proudford Sickle Cell Fund, Inc.

Our mission is to support sickle cell awareness, education, state-of-the-art treatment and research, and to bring hope to families affected by this devastating disease.

Last Updated: 14 Jan 2014

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The Baton Rouge Sickle Cell Anemia Foundation, Inc.

The Foundation is proud of its ongoing efforts to meet and exceed its established mission by bringing innovative and creative programs (1) to provide financial and advocacy supportive services to persons diagnosed with sickle cell disease; (2) to provide treatment support to enhance the quality of life for those suffering from sickle cell disease; and (3) to educate the general public about sickle cell disease.

Last Updated: 5 Mar 2013

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American Sickle Cell Anemia Association

ASCAA is an organization that provides quality and comprehensive services through diagnostic testing, evaluation, couseling, and supportive services to individuals and families at-risk for sickle cell disease.

Last Updated: 11 Feb 2013

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Children's Sickle Cell Foundation, Inc.

The mission of the Children’s Sickle Cell Foundation, Inc. (CSCF) is to provide support for children with sickle cell disease (SCD) and their families to help them face the educational, social and economic challenges caused by the disease. Our commitment to sickle cell disease education and community outreach, and support for sickle cell disease research are natural extensions of this mission. CSCF is the sole provider of community based programs for children with SCD in Western Pennsylvania. Some of the programs offered by CSCF are the Family, Educational Support and Learn2Swim.

Last Updated: 11 Feb 2013

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Sickle Cell Disease Association of America, Inc-Eastern NC Chapter

To improve the quality of life for persons and families affected by sickle cell disease and related genetic disorders

Last Updated: 20 Feb 2013

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Sickle Cell Disease Association of America, Inc.

Our mission is to help improve the quality of life for persons/families affected by the sickle cell syndrome and related genetic disorders.

Last Updated: 22 Jan 2013

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Sickle Cell Disease Association of Illinois

Our primary mission is to enhance the quality of life for people with sickle cell disease and their families. To effectively achieve this goal, the Sickle Cell Disease Association of Illinois (SCDAI) acts as an advocate for improved health care and services for individuals with sickle cell by educating and informing the community through outreach programs.

Last Updated: 4 Oct 2013

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Sickle Cell Disease Foundation of California

To provide education and life-enhancing programs and services to individuals with sickle cell disease, to broaden public awareness about sickle cell disease and to promote medical research and education to ultimately find a cure.

Last Updated: 27 Mar 2013

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werathah

To provide support and health education to patients and their families with genetic and congenital disorders

Last Updated: 1 May 2013

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General Support Organizations

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How do you compare to others with this condition?

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Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Sickle cell disease" for support, advocacy or research.

Citizens for Quality Sickle Cell Care-SCDAA-Northern, CT Chapter, Inc.

To ensure the availability and accessibility of quality and comprehensive medical care and support services for children and adults in northern Connecticut with Sickle Cell Disease and other related disorders.

http://www.cqscc.org

Last Updated: 13 Jan 2014

View Details
North Alabama Sickle Cell Foundation, Inc.

North Alabama Sickle Cell’s mission is to aid and enhance the lives of those affected by Sickle Cell and associated disorders through the provision of supportive services, education and advocacy.

http://sicklecellna.org

Last Updated: 16 Jan 2014

View Details
Sickle Cell Disease Association of America, Southern Connecticut, Inc.

The Sickle Cell Disease Association of America Southern Connecticut Inc. mission is to provide education,screening, counseling,and support services to persons affected with sickle cell disease and the trait.

http://www.scdaaofsouthernct.org/

Last Updated: 3 May 2014

View Details
William E. Proudford Sickle Cell Fund, Inc.

Our mission is to support sickle cell awareness, education, state-of-the-art treatment and research, and to bring hope to families affected by this devastating disease.

http://www.wepsicklecell.org

Last Updated: 14 Jan 2014

View Details
The Baton Rouge Sickle Cell Anemia Foundation, Inc.

The Foundation is proud of its ongoing efforts to meet and exceed its established mission by bringing innovative and creative programs (1) to provide financial and advocacy supportive services to persons diagnosed with sickle cell disease; (2) to provide treatment support to enhance the quality of life for those suffering from sickle cell disease; and (3) to educate the general public about sickle cell disease.

http://brscaf.org

Last Updated: 5 Mar 2013

View Details
Logo
American Sickle Cell Anemia Association

ASCAA is an organization that provides quality and comprehensive services through diagnostic testing, evaluation, couseling, and supportive services to individuals and families at-risk for sickle cell disease.

http://www.ascaa.org

Last Updated: 11 Feb 2013

View Details
Logo
Children's Sickle Cell Foundation, Inc.

The mission of the Children’s Sickle Cell Foundation, Inc. (CSCF) is to provide support for children with sickle cell disease (SCD) and their families to help them face the educational, social and economic challenges caused by the disease. Our commitment to sickle cell disease education and community outreach, and support for sickle cell disease research are natural extensions of this mission. CSCF is the sole provider of community based programs for children with SCD in Western Pennsylvania. Some of the programs offered by CSCF are the Family, Educational Support and Learn2Swim.

http://www.cscfkids.org

Last Updated: 11 Feb 2013

View Details
Logo
Sickle Cell Disease Association of America, Inc-Eastern NC Chapter

To improve the quality of life for persons and families affected by sickle cell disease and related genetic disorders

http://www.sicklecelleasternnc.org

Last Updated: 20 Feb 2013

View Details
Sickle Cell Disease Association of America, Inc.

Our mission is to help improve the quality of life for persons/families affected by the sickle cell syndrome and related genetic disorders.

http://www.sicklecelldisease.org/

Last Updated: 22 Jan 2013

View Details
Sickle Cell Disease Association of Illinois

Our primary mission is to enhance the quality of life for people with sickle cell disease and their families. To effectively achieve this goal, the Sickle Cell Disease Association of Illinois (SCDAI) acts as an advocate for improved health care and services for individuals with sickle cell by educating and informing the community through outreach programs.

http://www.scdai.org

Last Updated: 4 Oct 2013

View Details
Sickle Cell Disease Foundation of California

To provide education and life-enhancing programs and services to individuals with sickle cell disease, to broaden public awareness about sickle cell disease and to promote medical research and education to ultimately find a cure.

http://www.scdfc.org/

Last Updated: 27 Mar 2013

View Details
werathah

To provide support and health education to patients and their families with genetic and congenital disorders

http://www.werathah.com

Last Updated: 1 May 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Sickle cell disease" returned 1189 free, full-text research articles on human participants. First 3 results:

Proteinuria in patients with sickle cell disease.
 

Author(s): Ahmed M Alkhunaizi, Adil A Al-Khatti

Journal: Saudi J Kidney Dis Transpl. 2014 Sep;25(5):1038-41.

 

Proteinuria is a complication of sickle cell nephropathy that can progress to renal insufficiency and end-stage renal disease. The magnitude of proteinuria among patients with sickle cell disease (SCD) has been reported with variable prevalence. The aim of this study was to determine ...

Last Updated: 6 Sep 2014

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Elevated sphingosine-1-phosphate promotes sickling and sickle cell disease progression.
 

Author(s): Yujin Zhang, Vladimir Berka, Anren Song, Kaiqi Sun, Wei Wang, Weiru Zhang, Chen Ning, Chonghua Li, Qibo Zhang, Mikhail Bogdanov, Danny C Alexander, Michael V Milburn, Mostafa H Ahmed, Han Lin, Modupe Idowu, Jun Zhang, Gregory J Kato, Osheiza Y Abdulmalik, Wenzheng Zhang, William Dowhan, Rodney E Kellems, Pumin Zhang, Jianping Jin, Martin Safo, Ah-Lim Tsai, Harinder S Juneja, Yang Xia

Journal: J. Clin. Invest.. 2014 Jun;124(6):2750-61.

 

Sphingosine-1-phosphate (S1P) is a bioactive lipid that regulates multicellular functions through interactions with its receptors on cell surfaces. S1P is enriched and stored in erythrocytes; however, it is not clear whether alterations in S1P are involved in the prevalent and debilitating ...

Last Updated: 4 Jun 2014

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Pediatric hematology providers on referral for transplant evaluation for sickle cell disease: a regional perspective.
 

Author(s): Bethany Mikles, Monica Bhatia, Suzette O Oyeku, Zhezhen Jin, Nancy S Green

Journal: J. Pediatr. Hematol. Oncol.. 2014 Oct;36(7):566-71.

 

Hematology referral for evaluation is a key step for hematopoietic stem cell transplantation for sickle cell disease (SCD). Pediatric SCD providers in the US Northeast (New York-Mid-Atlantic and New England regions) were surveyed anonymously for perspectives and practices regarding ...

Last Updated: 24 Sep 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Sickle cell disease" returned 119 free, full-text review articles on human participants. First 3 results:

The glomerulopathy of sickle cell disease.
 

Author(s): Kenneth I Ataga, Vimal K Derebail, David R Archer

Journal: Am. J. Hematol.. 2014 Sep;89(9):907-14.

 

Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria ...

Last Updated: 15 Aug 2014

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Asthma management in sickle cell disease.
 

Author(s): Esteban Gomez, Claudia R Morris

Journal: Biomed Res Int. 2013 ;2013():604140.

 

Asthma is a common comorbid factor in sickle cell disease (SCD). However, the incidence of asthma in SCD is much higher than expected compared to rates in the general population. Whether "asthma" in SCD is purely related to genetic and environmental factors or rather is the consequence ...

Last Updated: 10 Dec 2013

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Consequences and management of iron overload in sickle cell disease.
 

Author(s): John Porter, Maciej Garbowski

Journal: Hematology Am Soc Hematol Educ Program. 2013 ;2013():447-56.

 

The aims of this review are to highlight the mechanisms and consequences of iron distribution that are most relevant to transfused sickle cell disease (SCD) patients and to address the particular challenges in the monitoring and treatment of iron overload. In contrast to many inherited ...

Last Updated: 9 Dec 2013

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Hematopoietic Stem Cell Transplant for Sickle Cell Disease
 

Status: Not yet recruiting

Condition Summary: Sickle Cell Disease; Sickle Cell Anemia; SCD

 

Last Updated: 21 Aug 2014

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Gene Transfer for Patients With Sickle Cell Disease
 

Status: Recruiting

Condition Summary: Sickle Cell Disease; Sickle Cell Anemia

 

Last Updated: 11 Jul 2014

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Sickle Cell Disease - Stroke Prevention in Nigeria Trial
 

Status: Recruiting

Condition Summary: Sickle Cell Anemia; Sickle Cell Disease; Stroke

 

Last Updated: 10 Dec 2014

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