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: 30 May 2015

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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: 1 Jun 2015

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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 Educational Support and Learn2Swim.

Last Updated: 11 Mar 2016

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Cochrane Collaboration

The Cochrane Collaboration is an international network of healthcare professionals, researchers and consumers preparing, maintaining, and disseminating high quality systematic reviews of randomised control trials. This review group focuses on genetic disorders such as cystic fibrosis (CF), haemoglobinopathies, coagulopathies and inborn errors of metabolism.

Last Updated: 12 May 2015

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Hina Patel Foundation

To increase the awareness in Kern County about Sickle Cell Disease and trait and provide accessible services, including education, testing, and counseling with the ultimate goal of enhancing the well-being of the affected people and their families.

Last Updated: 11 Jun 2015

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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: 1 Jun 2015

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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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Sickle Cell Society

Our vision is to be the most successful sickle cell organisation nationally with a wide network of well-informed, committed and active supporters working at local, national and international levels. We will be better able to empower and assist people with sickle cell disorders to achieve their full economic and social potential.

Last Updated: 23 Jun 2015

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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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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: 30 May 2015

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
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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.

http://www.ascaa.org

Last Updated: 1 Jun 2015

View Details
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 Educational Support and Learn2Swim.

http://www.cscfkids.org

Last Updated: 11 Mar 2016

View Details
Cochrane Collaboration

The Cochrane Collaboration is an international network of healthcare professionals, researchers and consumers preparing, maintaining, and disseminating high quality systematic reviews of randomised control trials. This review group focuses on genetic disorders such as cystic fibrosis (CF), haemoglobinopathies, coagulopathies and inborn errors of metabolism.

http://cfgd.cochrane.org

Last Updated: 12 May 2015

View Details
Logo
Hina Patel Foundation

To increase the awareness in Kern County about Sickle Cell Disease and trait and provide accessible services, including education, testing, and counseling with the ultimate goal of enhancing the well-being of the affected people and their families.

http://www.hinapatelfoundation.org

Last Updated: 11 Jun 2015

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: 1 Jun 2015

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
Logo
Sickle Cell Society

Our vision is to be the most successful sickle cell organisation nationally with a wide network of well-informed, committed and active supporters working at local, national and international levels. We will be better able to empower and assist people with sickle cell disorders to achieve their full economic and social potential.

http://www.sicklecellsociety.org/

Last Updated: 23 Jun 2015

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

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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 1599 free, full-text research articles on human participants. First 3 results:

The impact of the oral condition of children with sickle cell disease on family quality of life.
 

Author(s): Maria Luiza da Matta Felisberto Fernandes, Ichiro Kawachi, Patrícia Corrêa-Faria, Saul Martins Paiva, Isabela Almeida Pordeus

Journal: Braz Oral Res. 2016 ;30():.

 

The aim of this study was to assess the impact of oral conditions of children with sickle cell disease (SCD) on their parents' quality of life (QoL). A cross-sectional study was performed with parents of outpatients suffering from SCD at a hematology referral center in Belo Horizonte, ...

Last Updated: 25 Feb 2016

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Maternal risk behavior and caries incidence in children with sickle cell disease.
 

Author(s): Felipe Fagundes Soares, Maria Cristina Teixeira Cangussu, Maria Isabel Pereira Vianna, Thais Regis Aranha Rossi, Anderson Santos Carvalho, Maria Goretti Silva Brito

Journal: Braz Oral Res. 2016 ;30():.

 

The aim of this study was to analyze the incidence of caries, in relation to maternal risk behaviors and clinical conditions representing different levels of sickle cell disease severity. A total of 295 children aged 6 to 60 months participated in this cohort conducted from August ...

Last Updated: 17 Dec 2015

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The relationship between genotype, psychiatric symptoms and quality of life in adult patients with sickle cell disease in São Paulo, Brazil: a cross-sectional study.
 

Author(s): Érika Bergamini Mastandréa, Fátima Lucchesi, Marcela Mayumi Gomes Kitayama, Maria Stella Figueiredo, Vanessa de Albuquerque Citero

Journal: Sao Paulo Med J. ;133(5):421-7.

 

Health-related quality of life (HRQoL) may be worsened in sickle cell patients due to the presence of psychiatric disorders. The aims of this study were to describe the psychiatric symptoms in Brazilian sickle cell patients and to evaluate the relationship of these symptoms to the ...

Last Updated: 9 Dec 2015

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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 153 free, full-text review articles on human participants. First 3 results:

Environmental determinants of severity in sickle cell disease.
 

Author(s): Sanjay Tewari, Valentine Brousse, Frédéric B Piel, Stephan Menzel, David C Rees

Journal: Haematologica. 2015 Sep;100(9):1108-16.

 

Sickle cell disease causes acute and chronic illness, and median life expectancy is reduced by at least 30 years in all countries, with greater reductions in low-income countries. There is a wide spectrum of severity, with some patients having no symptoms and others suffering frequent, ...

Last Updated: 5 Sep 2015

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Sickle cell disease and H3Africa: enhancing genomic research on cardiovascular diseases in African patients.
 

Author(s): Ambroise Wonkam, Julie Makani, Solomon Ofori-Aquah, Obiageli E Nnodu, Marsha Treadwell, Charmaine Royal, Kwaku Ohene-Frempong,

Journal: Cardiovasc J Afr. ;26(2 Suppl 1):S50-5.

 

Sickle cell disease (SCD) has a high prevalence in sub-Saharan Africa. There are several cardiovascular phenotypes in SCD that contribute to its morbidity and mortality.

Last Updated: 12 May 2015

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A biopsychosocial model for the management of patients with sickle-cell disease transitioning to adult medical care.
 

Author(s): Lori E Crosby, Charles T Quinn, Karen A Kalinyak

Journal: Adv Ther. 2015 Apr;32(4):293-305.

 

The lifespan of patients with sickle-cell disease (SCD) continues to increase, and most affected individuals in high-resource countries now live into adulthood. This necessitates a successful transition from pediatric to adult health care. Care for transitioning patients with SCD ...

Last Updated: 1 May 2015

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Decision Aid for Therapeutic Options In Sickle Cell Disease
 

Status: Recruiting

Condition Summary: Sickle Cell Disease; Sickle Cell Anemia; Hemoglobin SS; Hemoglobin SC; Hemoglobin Beta Thalassemia

 

Last Updated: 25 Jan 2016

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Gene Transfer for Patients With Sickle Cell Disease
 

Status: Recruiting

Condition Summary: Sickle Cell Disease; Sickle Cell Anemia

 

Last Updated: 13 Apr 2016

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A Randomized Trial of LOVAZA in Pediatric Sickle Cell Disease (SCD)
 

Status: Not yet recruiting

Condition Summary: Sickle Cell Disease; HEMOGLOBIN SS; Hemoglobin S Beta-0 Thalassemia; Inflammation; Quality of Life

 

Last Updated: 22 Oct 2010

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