Cystic Fibrosis

Common Name(s)

Cystic Fibrosis

Cystic fibrosis (CF) is an inherited condition that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections. These infections can cause chronic coughing, and wheezing. Over time, mucus buildup and infections can lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. Mucus can also block the digestive tract and pancreas, leading to digestive problems. CF is caused by mutations in the CFTR gene and is inherited in an autosomal recessive fashion.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Cystic Fibrosis" for support, advocacy or research.

Boomer Esiason Foundation

The Boomer Esiason Foundation is a dynamic partnership of leaders in the medical and business communities joining with a committed core of volunteers to heighten awareness, education and quality of life for those affected by cystic fibrosis, while providing financial support to research aimed at finding a cure.

Last Updated: 15 Feb 2013

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Center for Jewish Genetics

The Center is dedicated to gathering and disseminating knowledge about Jewish genetic disorders and hereditary cancers. Its mission is to educate and serve health care professionals, clergy and the Jewish community.

Last Updated: 26 Dec 2012

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Cystic Fibrosis Foundation

Our mission is to assure the development of the means to cure and control Cystic Fibrosis and to improve the quality of life for those with the disease.

Last Updated: 19 Mar 2013

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Cystic Fibrosis Research, Inc.

Cystic Fibrosis Research, Inc. exists to fund research, to provide educational and personal support and to spread awareness of cystic fibrosis, a life-threatening genetic disease.

Last Updated: 5 Mar 2013

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General Support Organizations

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How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

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 "Cystic Fibrosis" for support, advocacy or research.

Boomer Esiason Foundation

The Boomer Esiason Foundation is a dynamic partnership of leaders in the medical and business communities joining with a committed core of volunteers to heighten awareness, education and quality of life for those affected by cystic fibrosis, while providing financial support to research aimed at finding a cure.

http://www.esiason.org

Last Updated: 15 Feb 2013

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Center for Jewish Genetics

The Center is dedicated to gathering and disseminating knowledge about Jewish genetic disorders and hereditary cancers. Its mission is to educate and serve health care professionals, clergy and the Jewish community.

http://www.jewishgenetics.org

Last Updated: 26 Dec 2012

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Cystic Fibrosis Foundation

Our mission is to assure the development of the means to cure and control Cystic Fibrosis and to improve the quality of life for those with the disease.

http://www.cff.org

Last Updated: 19 Mar 2013

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Cystic Fibrosis Research, Inc.

Cystic Fibrosis Research, Inc. exists to fund research, to provide educational and personal support and to spread awareness of cystic fibrosis, a life-threatening genetic disease.

http://www.cfri.org

Last Updated: 5 Mar 2013

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General Resources

CF in the Classroom (English and Spanish)

A guide/manuel for parents, teachers, school administrators, and the healthcare team.

Updated 19 Oct 2012

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CFRI newsletter

Newsletter issued 3 times a year, addressing CF education, research, inspiration, and new information on medications and therapies.

Updated 19 Oct 2012

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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 "Cystic Fibrosis" returned 4987 free, full-text research articles on human participants. First 3 results:

Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure.
 

Author(s): Ashley G Henderson, Camille Ehre, Brian Button, Lubna H Abdullah, Li-Heng Cai, Margaret W Leigh, Genevieve C DeMaria, Hiro Matsui, Scott H Donaldson, C William Davis, John K Sheehan, Richard C Boucher, Mehmet Kesimer

Journal: J. Clin. Invest.. 2014 Jul;124(7):3047-60.

 

The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow depends on the relative mucin concentration of the mucus layer compared with that of the periciliary layer; ...

Last Updated: 3 Jul 2014

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Lung clearance index in adults with non-cystic fibrosis bronchiectasis.
 

Author(s): Sherif Gonem, Alys Scadding, Marcia Soares, Amisha Singapuri, Per Gustafsson, Chandra Ohri, Simon Range, Christopher E Brightling, Ian Pavord, Alex Horsley, Salman Siddiqui

Journal:

 

Lung clearance index (LCI) is a measure of abnormal ventilation distribution derived from the multiple breath inert gas washout (MBW) technique. We aimed to determine the clinical utility of LCI in non-CF bronchiectasis, and to assess two novel MBW parameters that distinguish between ...

Last Updated: 4 Jun 2014

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Viability of Pseudomonas aeruginosa in cough aerosols generated by persons with cystic fibrosis.
 

Author(s): Luke D Knibbs, Graham R Johnson, Timothy J Kidd, Joyce Cheney, Keith Grimwood, Jacqueline A Kattenbelt, Peter K O'Rourke, Kay A Ramsay, Peter D Sly, Claire E Wainwright, Michelle E Wood, Lidia Morawska, Scott C Bell

Journal: Thorax. 2014 Aug;69(8):740-5.

 

Person-to-person transmission of respiratory pathogens, including Pseudomonas aeruginosa, is a challenge facing many cystic fibrosis (CF) centres. Viable P aeruginosa are contained in aerosols produced during coughing, raising the possibility of airborne transmission.

Last Updated: 9 Jul 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 "Cystic Fibrosis" returned 512 free, full-text review articles on human participants. First 3 results:

Treatment of pseudomonas and Staphylococcus bronchopulmonary infection in patients with cystic fibrosis.
 

Author(s): Rashmi Ranjan Das, Sushil Kumar Kabra, Meenu Singh

Journal:

 

The optimal antibiotic regimen is unclear in management of pulmonary infections due to pseudomonas and staphylococcus in cystic fibrosis (CF). We systematically searched all the published literature that has considered the evidence for antimicrobial therapies in CF till June 2013. ...

Last Updated: 3 Feb 2014

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Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases.
 

Author(s): Adriana Haack, Giselle Gonçalves Aragão, Maria Rita Carvalho Garbi Novaes

Journal: World J. Gastroenterol.. 2013 Dec;19(46):8552-61.

 

Cystic fibrosis (CF) causes chronic infections in the respiratory tract and alters the digestive tract. This paper reviews the most important aspects of drug treatment and changes in the digestive tract of patients with CF. This is a review of the literature, emphasizing the discoveries ...

Last Updated: 31 Dec 2013

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Systematic review of the dry powder inhalers colistimethate sodium and tobramycin in cystic fibrosis.
 

Author(s): Lesley Uttley, Sue Harnan, Anna Cantrell, Chris Taylor, Martin Walshaw, Keith Brownlee, Paul Tappenden

Journal: Eur Respir Rev. 2013 Dec;22(130):476-86.

 

This systematic review evaluated evidence for two dry powder formulations, colistimethate sodium and tobramycin, for the treatment of chronic Pseudomonas aeruginosa in cystic fibrosis, as part of the UK national recommendation process for new technologies. Electronic bibliographic ...

Last Updated: 2 Dec 2013

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Ivacaftor (Kalydeco) and Insulin in Cystic Fibrosis (CF)
 

Status: Recruiting

Condition Summary: Cystic Fibrosis Related Diabetes; Cystic Fibrosis

 

Last Updated: 30 Jul 2014

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Metabolic Efficiency of Combined Pancreatic Islet and Lung Transplant for the Treatment of End-Stage Cystic Fibrosis
 

Status: Not yet recruiting

Condition Summary: Cystic Fibrosis; Diabetes Related Cystic Fibrosis

 

Last Updated: 5 Mar 2012

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Ghrelin in Cystic Fibrosis
 

Status: Recruiting

Condition Summary: Cystic Fibrosis

 

Last Updated: 26 Jul 2010

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