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Cystic Fibrosis

Cystic Fibrosis: Introduction

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, ... 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.

Cystic Fibrosis: Symptoms

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 ...more symptoms »

Cystic Fibrosis: Treatments

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 symptoms, the ...more treatments »

Cystic Fibrosis: Misdiagnosis

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 »

Symptoms of Cystic Fibrosis

Treatments for Cystic Fibrosis

  • Respiratory treatments - for treating the lung and respiratory tract problems:
    • Voluntary coughing
    • Respiratory therapy - daily chest and back percussion
    • Percussion treatments
    • Flutter device
  • more treatments...»

Home Diagnostic Testing

Home medical testing related to Cystic Fibrosis:

Wrongly Diagnosed with Cystic Fibrosis?

Cystic Fibrosis: Related Patient Stories

Cystic Fibrosis: Deaths

Read more about Deaths and Cystic Fibrosis.

Alternative Treatments for Cystic Fibrosis

Alternative treatments or home remedies that have been listed in various sources as possibly beneficial for Cystic Fibrosis may include:

Types of Cystic Fibrosis

  • Over 500 different cystic fibrosis mutations exist
  • Delta F508 mutation cystic fibrosis
  • R117H mutation cystic fibrosis
  • more types...»

Curable Types of Cystic Fibrosis

Possibly curable types of Cystic Fibrosis include:

  • Pneumothorax related to cystic fibrosis
  • Nasal polps related to cystic fibrosis
  • Chronic sinusitis related to cystic fibrosis
  • Meconium ileus related to cystic fibrosis
  • Intussusception related to cystic fibrosis
  • Undescended testis associated to cystic fibrosis
  • more types...»

Rare Types of Cystic Fibrosis:

Rare types of Cystic Fibrosis include:

  • Nasal polyps related to cystic fibrosis
  • Pancreatic insufficiency related cystic fibrosis
  • Sinusitis related cystic fibrosis
  • Bronchiectasis related cystic fibrosis
  • Intussusception related to cystic fibrosis
  • more types...»

Diagnostic Tests for Cystic Fibrosis

Test for Cystic Fibrosis in your own home

Click for Tests

Cystic Fibrosis: Complications

Review possible medical complications related to Cystic Fibrosis:

Causes of Cystic Fibrosis

  • Genetic mutation. Cystic fibrosis is one of the most common life-shortening genetic diseases
  • more causes...»

More information about causes of Cystic Fibrosis:

Disease Topics Related To Cystic Fibrosis

Research the causes of these diseases that are similar to, or related to, Cystic Fibrosis:

Cystic Fibrosis: Undiagnosed Conditions

Commonly undiagnosed diseases in related medical categories:

Misdiagnosis and Cystic Fibrosis

Mild worm infections undiagnosed in children: Human worm infestations, esp. threadworm, can be overlooked in some cases, because it may cause only mild...read more »

Chronic digestive conditions often misdiagnosed: When diagnosing chronic symptoms of the digestive tract, there are a variety of conditions that may be misdiagnosed. The best known, irritable...read more »

Intestinal bacteria disorder may be hidden cause: One of the lesser known causes of diarrhea is an imbalance of bacterial in the gut, sometimes called intestinal imbalance. The digestive system contains a variety...read more »

Antibiotics often causes diarrhea: The use of antibiotics are very likely to cause some level of diarrhea in patients. The reason is that antibiotics kill off not only "bad" bacteria, but can also kill the "good...read more »

Food poisoning may actually be an infectious disease: Many people who come down with "stomach symptoms" like diarrhea assume that it's "something I ate" (i.e. food poisoning). In fact, it's more likely...read more »

Mesenteric adenitis misdiagnosed as appendicitis in children: Because appendicitis is one of the more feared conditions for a child with abdominal pain, it can be over...read more »

Blood pressure cuffs misdiagnose hypertension in children: One known misdiagnosis issue with hyperension, arises in relation to the simple equipment used to test blood pressure. The "cuff" around the arm to measure blood pressure...read more »

Celiac disease often fails to be diagnosed cause of chronic digestive symptoms: One of the most common chronic digestive conditions is celiac disease, a malabsorption disorder with a variety of...read more »

Children with migraine often misdiagnosed: A migraine often fails to be correctly diagnosed in pediatric patients. These patients are not the typical migraine...read more »

Chronic digestive diseases hard to diagnose: There is an inherent difficulty in diagnosing the various types of chronic digestive diseases. Some of the better...read more »

Chronic lung diseases hard to diagnose: Some of the chronic lung diseases are difficult to diagnose. Even the well-knowns conditions such as asthma or ...read more »

Cystic Fibrosis: Research Doctors & Specialists

Research related physicians and medical specialists:

Other doctor, physician and specialist research services:

Hospitals & Clinics: Cystic Fibrosis

Research quality ratings and patient safety measures for medical facilities in specialties related to Cystic Fibrosis:

Choosing the Best Hospital: More general information, not necessarily in relation to Cystic Fibrosis, on hospital performance and surgical care quality:

Cystic Fibrosis: Rare Types

Rare types of diseases and disorders in related medical categories:

Latest Treatments for Cystic Fibrosis

Evidence Based Medicine Research for Cystic Fibrosis

Medical research articles related to Cystic Fibrosis include:

Click here to find more evidence-based articles on the TRIP Database

Cystic Fibrosis: Animations

Prognosis for Cystic Fibrosis

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

Research about Cystic Fibrosis

Visit our research pages for current research about Cystic Fibrosis treatments.

Clinical Trials for Cystic Fibrosis

The US based website ClinicalTrials.gov lists information on both federally and privately supported clinical trials using human volunteers.

Some of the clinical trials listed on ClinicalTrials.gov for Cystic Fibrosis include:

Statistics for Cystic Fibrosis

Cystic Fibrosis: Broader Related Topics

Cystic Fibrosis Message Boards

Related forums and medical stories:

User Interactive Forums

Read about other experiences, ask a question about Cystic Fibrosis, or answer someone else's question, on our message boards:

Article Excerpts about Cystic Fibrosis

Genes and Disease by the National Center for Biotechnology (Excerpt)

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)

NHLBI, Facts About Cystic Fibrosis: NHLBI (Excerpt)

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)

Definitions of Cystic Fibrosis:

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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