Cystic fibrosis is a chronic, progressive and debilitating disease that affects the lungs, intestines, liver, and pancreas. Cystic fibrosis is caused by a genetic defect that triggers the mucous producing glands of the body to make excessive amounts of abnormally thick and sticky mucus.
Cystic fibrosis results in the clogging of the tubes, ducts and air passages of the body with mucus. This leads to serious problems with digestion and breathing. Cystic fibrosis is eventually fatal, although survival can be significantly extended with early diagnosis and good, consistent medical care. At this time life expectancy is about 36.8 years.
Cystic fibrosis most often affects the lungs and pancreas. In the lungs cystic fibrosis leads to such complications as recurrent lung infections and chronic lung infections due to the clogging of the air passages with mucus.
Cystic fibrosis can also severely affect the pancreas, which produces enzymes that are important to food digestion. In cystic fibrosis, the ducts that carry enzymes from the pancreas to the digestive tract become blocked, leading to problems with digestion, vitamin deficiencies and mineral deficiencies.
Other complications that can occur from cystic fibrosis include diabetes, infertility and rarely, serious liver problems, such as cirrhosis and portal hypertension.
Typically, symptoms of cystic fibrosis affect the respiratory system and the digestive system. However, the symptoms vary between individuals, and not all people will experience symptoms in both body systems.
In healthy lungs, air and oxygen pass through the upper respiratory tract and into the bronchioles and the alveoli in the lungs. The alveoli are tiny hollow sack-like structures where oxygen is absorbed in to the bloodstream. However, the build-up of thick sticky mucus in these air passages due to cystic fibrosis reduces the effectiveness of breathing and the intake of oxygen. This results in shortness of breath and chronic cough. Mucus build-up also encourages the growth of bacteria and viruses, leading to chronic lung infections.
Gastrointestinal symptoms due to abnormal digestion include greasy, foul-smelling feces, dehydration and growth delays in children. The sweat is also excessively salty. For more details on complications and symptoms, refer to symptoms of cystic fibrosis.
Making a diagnosis of cystic fibrosis begins with taking a thorough medical and family history, including symptoms. A physical examination is also performed and includes listening with a stethoscope to the sounds that lungs make during respiration. Lung sounds that may point to a diagnosis of cystic fibrosis include wheezing and decreased lung sounds. Frequent coughing may also be noted. Oxygen levels in the blood may also be tested using a non-invasive pulse oximeter, a device that clips painlessly to a fingertip.
Diagnostic testing includes a test on the sweat, called sweat chloride testing. A high level of salt (sodium chloride) in the sweat is characteristic of cystic fibrosis. A blood test is also available to determine if a person has the defective gene that causes cystic fibrosis.
Other tests are performed to determine the effects of cystic fibrosis on the lungs, digestive system and other areas of the body. These include chest X-ray, pulmonary lung function tests, and tests that can determine liver and pancreas function.
It is possible that a diagnosis of cystic fibrosis can be missed or delayed because symptoms vary between individuals. For more information on misdiagnosis, refer to misdiagnosis of cystic fibrosis.
There is no cure for cystic fibrosis, but it can be treated to minimize symptoms and progression of the disease. For more information on treatment, refer to treatment of cystic fibrosis. ...more »
Cystic Fibrosis: Cystic fibrosis (CF) is the most common fatal genetic disease in the United States today. It causes the body to produce a thick, sticky mucus that clogs the lungs, leading to ... more about Cystic Fibrosis.
Cystic Fibrosis: Cystic fibrosis is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure. More detailed information about the symptoms, causes, and treatments of Cystic Fibrosis is available below.
The types and severity of symptoms of cystic fibrosis vary between individuals. Symptoms may be present at birth or they may not occur for months or even years. Symptoms are related to an excessive build-up of thick mucus in the tubes, ducts and passages of the body. Symptoms most often affect the lungs and digestive system, but not everyone with cystic fibrosis has symptoms ...more symptoms »
Cystic fibrosis is not curable and is eventually fatal. However early diagnosis and consistent compliance with a good treatment program can help to reduce symptoms and complications and can extend life.
There is no way to prevent cystic fibrosis. Good treatments plans for cystic fibrosis include a multifaceted approach that is tailored to the individual case and specific ...more treatments »
A diagnosis of cystic fibrosis may be delayed or missed because the symptoms can vary greatly in different people and mimic symptoms of a wide variety of other conditions, such as pneumonia, bronchopulmonary dysplagia, celiac disease and malnutrition.
Only a thorough evaluation by a physician or licensed health care provider can determine the cause of symptoms. ...more misdiagnosis »
See full list of 77 symptoms of Cystic Fibrosis
See full list of 42 treatments for Cystic Fibrosis
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Prognosis for Cystic Fibrosis: The predicted median age of survival for a person with CF is 37 years. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50. Lung transplantation is often necessary as CF worsens
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Cystic fibrosis (CF) is the most common fatal genetic disease in the United States today. It causes the body to produce a thick, sticky mucus that clogs the lungs, leading to infection, and blocks the pancreas, stopping digestive enzymes from reaching the intestines where they are required to digest food. (Source: Genes and Disease by the National Center for Biotechnology)
Cystic fibrosis (CF) is a chronic, progressive, and frequently fatal genetic (inherited) disease of the body's mucus glands. CF primarily affects the respiratory and digestive systems in children and young adults. The sweat glands and the reproductive system are also usually involved. (Source: excerpt from NHLBI, Facts About Cystic Fibrosis: NHLBI)
A congenital metabolic disorder affecting the exocrine glands, inherited as an autosomal trait. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production which causes obstruction of passageways (including pancreatic and bile ducts, intestines, and bronchi). The sweat sodium and chloride content are increased. Symptoms usually appear in childhood and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather. -- 2003 - (Source - Diseases Database)
The most common congenital disease; the child's lungs and intestines and pancreas become clogged with thick mucus; caused by defect in a single gene; there is no cure - (Source - WordNet 2.1)
Cystic Fibrosis is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Cystic Fibrosis, or a subtype of Cystic Fibrosis,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Cystic Fibrosis as a "rare disease".
Source - Orphanet
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