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Diseases » Cystic Fibrosis » Summary
 

What is Cystic Fibrosis?

What is Cystic Fibrosis?

Cystic fibrosis is a chronic, progressive and debilitating disease that affects the lungs, intestines, liver, and pancreas. Cystic fibrosis is ...more »

  • 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.
  • 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
  • Cystic Fibrosis: 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

Cystic Fibrosis: Introduction

Types of Cystic Fibrosis:

Types of Cystic Fibrosis:

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

Broader types of Cystic Fibrosis:

How many people get Cystic Fibrosis?

Prevalance of Cystic Fibrosis: 1 per 31,000 Asian American newborns suffer from cystic fibrosis in the US, genetics Home Reference website
Prevalance Rate of Cystic Fibrosis: approx 1 in 31,000 or 0.00% or 8,774 people in USA [about data]
Incidence (annual) of Cystic Fibrosis: 2,500 babies annually USA; 1 in 3,000 Caucasian babies
Incidence Rate of Cystic Fibrosis: approx 1 in 108,800 or 0.00% or 2,500 people in USA [about data]
Prevalance of Cystic Fibrosis: Every year, 1,000 children with cystic fibrosis (CF) are born in the United States. One in 3,000 Caucasian babies have the disorder, making CF one of the most common lethal genetic diseases in Caucasians. Overall, there are 30,000 Americans with CF, and an estimated 8 million people carry one copy of the defective gene that causes the disease. (Source: excerpt from Cystic Fibrosis Research Directions: NIDDK)

Who gets Cystic Fibrosis?

Patient Profile for Cystic Fibrosis: Symptoms usually within 1 year; rarely symptoms wait till adolescence; mostly affected from birth.

Race Profile for Cystic Fibrosis: Mostly caucasian

How serious is Cystic Fibrosis?

Prognosis of 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
Complications of Cystic Fibrosis: see complications of Cystic Fibrosis
Prognosis of Cystic Fibrosis: Improved therapy has transformed CF from a disease characterized by death in early childhood to a chronic illness, with most patients living to adulthood. But despite this progress, there still is no cure for the disease and most patients eventually succumb to infections of the airways and lung failure. (Source: excerpt from Cystic Fibrosis Research Directions: NIDDK) ... On the average, individuals with CF have a lifespan of approximately 30 years. (Source: excerpt from NHLBI, Facts About Cystic Fibrosis: NHLBI)
Deaths for Cystic Fibrosis: 485 deaths (NHLBI 1999)

What causes Cystic Fibrosis?

Causes of Cystic Fibrosis: see causes of Cystic Fibrosis
Causes of Cystic Fibrosis: CF affects tissues that produce mucus secretions, such as the airway, the gastrointestinal tract, the ducts of the pancreas, the bile ducts of the liver and the male urogenital tract. Normal mucus forms a gel-like barrier that plays an important role in protecting the cells lining the inside surfaces of these tissues. In the lung, mucus also transports dust and other particles out of the airway and helps to prevent infection. CF alters the chemical properties of mucus; instead of protecting tissues from harm, the abnormal mucus obstructs the ducts and airways, causing tissue damage. (Source: excerpt from Cystic Fibrosis Research Directions: NIDDK)
Risk factors for Cystic Fibrosis: see risk factors for Cystic Fibrosis

What are the symptoms of Cystic Fibrosis?

Symptoms of Cystic Fibrosis: see symptoms of Cystic Fibrosis

Complications of Cystic Fibrosis: see complications of Cystic Fibrosis

Onset of Cystic Fibrosis: Symptoms of CF appear in infancy and childhood

Can anyone else get Cystic Fibrosis?

Inheritance: see inheritance of Cystic Fibrosis

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Cystic Fibrosis: Testing

Diagnostic testing: see tests for Cystic Fibrosis.

Misdiagnosis: see misdiagnosis and Cystic Fibrosis.

How is it treated?

Treatments for Cystic Fibrosis: see treatments for Cystic Fibrosis
Alternative treatments for Cystic Fibrosis: see alternative treatments for Cystic Fibrosis
Prevention of Cystic Fibrosis: see prevention of Cystic Fibrosis
Research for Cystic Fibrosis: see research for Cystic Fibrosis

Society issues for Cystic Fibrosis

Hospitalizations for Cystic Fibrosis: 12,000 (NHLBI 1999)

Hospitalization statistics for Cystic Fibrosis: The following are statistics from various sources about hospitalizations and Cystic Fibrosis:

  • 0.07% (9,211) of hospital consultant episodes were for cystic fibrosis in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
  • 96% of hospital consultant episodes for cystic fibrosis required hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
  • 49% of hospital consultant episodes for cystic fibrosis were men in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
  • 51% of hospital consultant episodes for cystic fibrosis were women in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
  • 42% of hospital consultant episodes for cystic fibrosis required emergency hospital admission in England 2002-03 (Hospital Episode Statistics, Department of Health, England, 2002-03)
  • more statistics...»

Organs Affected by Cystic Fibrosis:

Organs and body systems related to Cystic Fibrosis include:

Name and Aliases of Cystic Fibrosis

Main name of condition: Cystic Fibrosis

Class of Condition for Cystic Fibrosis: genetic autosomal recessive

Other names or spellings for Cystic Fibrosis:

CF, cystic fibrosis of pancreas, fibrocystic disease of pancreas, mucoviscidosis, Mucovoidosis

Mucoviscidosis Source - Diseases Database

CF, Fibrocystic disease of the pancreas, Pancreatic fibrosis, Mucoviscidosis
Source - WordNet 2.1

CF, Mucoviscidosis
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Cystic Fibrosis: Related Conditions

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

 

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