Also called: Also called: CF
Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to problems such as repeated lung infections and lung damage.
The symptoms and severity of CF vary widely. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults.
Although there is no cure for CF, treatments have improved greatly in recent years. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old.
National Heart, Lung, and Blood Institute
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Cystic Fibrosis
(National Heart, Lung, and Blood Institute) - http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_what.html
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Cystic Fibrosis
(Patient Education Institute) - Requires Flash Player - http://www.nlm.nih.gov/medlineplus/tutorials/cysticfibrosis/htm/index.htm
Also available in Spanish http://www.nlm.nih.gov/medlineplus/spanish/tutorials/cysticfibrosisspanish/htm/index.htm -
Genetics Home Reference: Cystic fibrosis(National Library of Medicine) - http://ghr.nlm.nih.gov/condition=cysticfibrosis
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Cystic Fibrosis(March of Dimes Birth Defects Foundation) - http://www.marchofdimes.com/pnhec/4439_1213.asp
Also available in Spanish http://www.nacersano.org/centro/9388_9971.asp
- Cystic Fibrosis(Mayo Foundation for Medical Education and Research) - http://www.mayoclinic.com/print/cystic-fibrosis/DS00287/DSECTION=all&METHOD=print
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New Pig Model of Cystic Fibrosis Lays Groundwork for Better Understanding of Human Disease(09/25/2008, National Heart, Lung, and Blood Institute, National Institute of Diabetes and Digestive and Kidney Diseases) - http://www.nih.gov/news/health/sep2008/nhlbi-25.htm
- Cystic Fibrosis Treatments Improving(09/18/2008, Reuters Health) - http://www.nlm.nih.gov/medlineplus/news/fullstory_69426.html
- Experimental Drug Shows Early Promise against Cystic Fibrosis(08/21/2008, HealthDay) - http://www.nlm.nih.gov/medlineplus/news/fullstory_68376.html
- CF Gene Mutation Testing(American Association for Clinical Chemistry) - http://www.labtestsonline.org/understanding/analytes/cf_gene/test.html
- Sweat Test(Nemours Foundation) - http://kidshealth.org/parent/general/sick/sweat_test.html
- Trypsin Test(American Association for Clinical Chemistry) - http://www.labtestsonline.org/understanding/analytes/trypsin/test.html
- Trypsinogen Test(American Association for Clinical Chemistry) - http://www.labtestsonline.org/understanding/analytes/trypsinogen/test.html
- Building Strength: Therapies for CF(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Therapies/
- Current Treatments (Cystic Fibrosis)(American Association for Respiratory Care) - http://www.yourlunghealth.org/lung_disease/cystic_fibrosis/treatment/index.cfm
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Carrier Screening for Cystic Fibrosis (CF)(March of Dimes Birth Defects Foundation) - http://www.marchofdimes.com/pnhec/159_525.asp
Also available in Spanish http://www.nacersano.org/centro/9246_9642.asp
- Newborn Screening for Cystic Fibrosis(Cystic Fibrosis Foundation) - http://www.cff.org/AboutCF/Testing/NewbornScreening/
- Nutrition and Cystic Fibrosis: Changes through Life(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/NutritionThroughLife/Nutrition%20and%20CF%20-%20Changes%20Through%20Life%2002-06.pdf
- Nutrition for Your Child with Cystic Fibrosis (Four to Seven Years)(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/treatments/Therapies/Nutrition/ForChild/Nutrition%20-%20For%20Your%20Child%20w%20CF%2002-06.pdf
- Nutrition for Your Infant with Cystic Fibrosis (Birth to One Year)(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/treatments/Therapies/Nutrition/ForInfant/Nutrition%20-%20For%20Your%20Infant.pdf
- Nutrition for Your Toddler with Cystic Fibrosis (One to Three Years)(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/treatments/Therapies/Nutrition/ForToddler/Nutrition%20-%20For%20Your%20Toddler.pdf
- Nutrition: Bone Health and Cystic Fibrosis(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/treatments/Therapies/Nutrition/BoneHealth/Nutrition%20-%20Bone%20Health%20and%20Cystic%20Fibrosis.pdf
- Nutrition: School, Enzymes, and Sports for the Child with Cystic Fibrosis(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/AtSchool/SchoolEnzymes/Nutrition%20-%20School%20Enzymes%20Sports.pdf
- Pancreatic Enzyme Replacement in People with Cystic Fibrosis(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/EnzymeReplacement/Nutrition%20-%20Pancreatic%20Enzyme%20Replacement%2002-06.pdf
- Pancreatic Enzyme Supplements: Food and Drug Administration Requirements(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Therapies/Nutrition/Enzymes/
- Airway Clearance Techniques(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Therapies/Respiratory/AirwayClearance/
- Home Oxygen Therapy(American Association for Respiratory Care) - http://www.yourlunghealth.org/healthy_living/living/home_oxygen_therapy/
- Hypertonic Saline Therapy for Cystic Fibrosis: Is It Right for You?(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Therapies/Respiratory/HypertonicSaline/
- Ibuprofen: Information for Patients and Families(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Therapies/Respiratory/Ibuprofen/
- Introduction to Postural Drainage & Percussion(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/treatments/Therapies/Respiratory/PosturalDrainage/An%20Introduction%20to%20Postural%20Drainage%20and%20Percussion%201-2006.pdf
- Oxygen Therapy(American Thoracic Society) - Requires Flash Player - http://www.thoracic.org/sections/education/patient-education/patient-education-materials/patient-information-series/oxygen-therapy.html
- Respiratory: Which Nebulizer for Which Drug?(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/LungHealth/WhichNebulizer/WhichNebulizerForWhichDrug.pdf
- Allergic Bronchopulmonary Aspergilliosis in Cystic Fibrosis (ABPA)(Cystic Fibrosis Foundation) - http://www.cff.org/LivingWithCF/StayingHealthy/Germs/ABPA/
- Living with CF at School(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/LivingWithCF/AtSchool/
- Managing Cystic Fibrosis-Related Diabetes (CFRD): An Instruction Guide for Patients & Families(Cystic Fibrosis Foundation) - Large PDF file - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/Diabetes/CFRD%20Manual.pdf
- Respiratory: Stopping the Spread of Germs(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/StoppingTheSpread/StoppingSpreadGerms.pdf
- Respiratory: What You Should Know about Germs(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatYouShouldKnow/WhatYouShouldKnowAboutGerms.pdf
- Risks of Smoking for People with Cystic Fibrosis(Cystic Fibrosis Foundation) - http://www.cff.org/LivingWithCF/StayingHealthy/LungHealth/Smoking/
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Cystic Fibrosis(Patient Education Institute) - Requires Flash Player - http://www.nlm.nih.gov/medlineplus/tutorials/cysticfibrosis/htm/index.htm
Also available in Spanish http://www.nlm.nih.gov/medlineplus/spanish/tutorials/cysticfibrosisspanish/htm/index.htm
- What Is Cystic Fibrosis?(Dolan DNA Learning Center) - Requires Flash Player - http://www.yourgenesyourhealth.org/cf/whatisit.htm
- Day-to-Day: Know Your Health Insurance Coverage(Cystic Fibrosis Foundation) - Links to PDF - http://www.cff.org/UploadedFiles/LivingWithCF/Insurance/Know%20Your%20Health%20Insurance%2002-06.pdf
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ClinicalTrials.gov: Cystic Fibrosis(National Institutes of Health) - http://clinicaltrials.gov/search/open/condition=%22Cystic+Fibrosis%22
- Clinical Research(Cystic Fibrosis Foundation) - http://www.cff.org/research/ClinicalResearch/
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Genetics Home Reference: Cystic fibrosis(National Library of Medicine) - http://ghr.nlm.nih.gov/condition=cysticfibrosis
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Learning about Cystic Fibrosis(National Human Genome Research Institute) - http://www.genome.gov/page.cfm?pageID=10001213
- Drug Development Pipeline(Cystic Fibrosis Foundation) - http://www.cff.org/treatments/Pipeline/
References and abstracts from MEDLINE/PubMed (National Library of Medicine)
- Article: Ignoring the nonsense: a phase II trial in cystic fibrosis. - http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=pubmed&dopt=AbstractPlus&list_uids=18722009&tool=MedlinePlus
- Article: Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense... - http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=pubmed&dopt=AbstractPlus&list_uids=18722008&tool=MedlinePlus
- Article: Newborn screening for cystic fibrosis: a lesson in public health... - http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=pubmed&dopt=AbstractPlus&list_uids=18718257&tool=MedlinePlus
- Cystic Fibrosis -- see more articles - http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=search&db=pubmed&term=cystic+fibrosis[majr]+AND+english[la]+AND+humans[mh]+AND+(jsubsetaim[text]+OR+jsubsetk[text]+OR+jsubsetn[text]+OR+guideline[pt]+OR+clinical+trial[pt]+OR+patient+education+handout[pt]+OR+systematic[sb])+NOT+(editorial[pt]+OR+case+reports[pt]+OR+letter[pt])&doptcmdl=summary&cmd_current=Limits&pmfilter_EDatLimit=last+1+Year&tool=MedlinePlus
- Cystic Fibrosis Foundation - http://www.cff.org/
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National Heart, Lung, and Blood Institute - http://www.nhlbi.nih.gov/
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National Institute of Diabetes and Digestive and Kidney Diseases - http://www.niddk.nih.gov/
- Cystic Fibrosis(Nemours Foundation) - http://kidshealth.org/kid/health_problems/heart/cystic_fibrosis.html
- Cystic Fibrosis: Diet and Nutrition(Nemours Foundation) - http://kidshealth.org/kid/nutrition/diets/cf_diet.html
- Cystic Fibrosis(Nemours Foundation) - http://kidshealth.org/teen/diseases_conditions/digestive/cystic_fibrosis.html
- Cystic Fibrosis: Diet and Nutrition(Nemours Foundation) - http://kidshealth.org/teen/nutrition/diets/cf_diet.html
- Lung Transplantation - http://www.nlm.nih.gov/medlineplus/lungtransplantation.html
- Genetics/Birth Defects - http://www.nlm.nih.gov/medlineplus/geneticsbirthdefects.html
- Lungs and Breathing - http://www.nlm.nih.gov/medlineplus/lungsandbreathing.html
The primary NIH organization for research on Cystic Fibrosis is the National Heart, Lung, and Blood Institute - http://www.nhlbi.nih.gov/