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Advances in CF

Cystic fibrosis (CF) management has come a long way in a short period of time. It is always important to stay up to date on the latest advances and maintain health as best as possible.

CF Milestones

Over the years, the research and medical communities have learned more about CF, including its cause. As a result of extensive research, advancements in diagnosis and management of the disease have helped improve the care of people with CF.

  1. Today

    Advances in science, medicine, and the care of people with CF continue to be made.

  2. 2015

    The median predicted survival for people with CF is 42 years.

    For the first time, there are more adults than children living with CF.

    Median predicted survival numbers are based on data relating to all people in the CFF Patient Registry.

    Median predicted survival means that in this year, half the people with CF were expected to live longer and half were expected to live less than this age.

    Nearly 60% of CF diagnoses are detected by newborn screening.

  3. 2012

    Medicines that modulate activity of the CFTR protein are introduced.

  4. 2002

    The median predicted survival for people with CF is 31 years.

    Median predicted survival numbers are based on data relating to all people in the CFF Patient Registry.

    Median predicted survival means that in this year, half the people with CF were expected to live longer and half were expected to live less than this age.

  5. 1997

    Inhaled antibiotics that target bacteria in the lungs become available.

  6. 1993

    Mucolytics, medicines that help thin out the mucus in the lungs, are introduced.

  7. 1992

    The median predicted survival for people with CF is 29 years.

    Median predicted survival numbers are based on data relating to all people in the CFF Patient Registry.

    Median predicted survival means that in this year, half the people with CF were expected to live longer and half were expected to live less than this age.

    Function of the CFTR protein as a channel that allows chloride passage is discovered.

  8. 1990

    Anti-inflammatory medicines are first studied in people with CF to help reduce inflammation caused by pulmonary exacerbations.

  9. 1989

    The cystic fibrosis transmembrane conductance regulator (CFTR) gene is identified as the gene responsible for the defective CFTR proteins that cause CF.

  10. 1988

    A vest is introduced to make chest physical therapy at home easier.

  11. 1985

    The median predicted survival for people with CF is 25 years.

    Median predicted survival numbers are based on data relating to all people in the CFF Patient Registry.

    Median predicted survival means that in this year, half the people with CF were expected to live longer and half were expected to live less than this age.

  12. 1978

    Coated pancreatic enzymes that help with nutrient absorption are introduced.

  13. 1964

    The first comprehensive CF treatment plan is developed, focusing on scarring in the lungs, infections, and pancreatic issues.

  14. 1961

    The Cystic Fibrosis Foundation forms a care center network.

  15. 1959

    Sweat chloride testing becomes a standard procedure for diagnosing CF.

  16. 1955

    The median predicted survival for people with CF is 5 years.

    Median predicted survival numbers are based on data relating to all people in the CFF Patient Registry.

    Median predicted survival means that in this year, half the people with CF were expected to live longer and half were expected to live less than this age.

    Scientists study the use of pancreatic enzymes in CF.

  17. 1953

    Extra salt loss in sweat in people with CF is connected to problems at the cellular level.

  18. 1949

    Scientists begin to think that CF is caused by a defect in a single gene and single protein.

  19. 1946

    Scientists use antibiotics to treat lung infections in people with CF.

  20. 1938

    First comprehensive medical report on CF is written.

To learn more about available treatments to help manage symptoms and treat CF, talk to your care team.

Help for Your Disease Management Routine

CFDaily is a free iPhone® app for people with CF. This app is designed especially for people with CF and their caregivers to help manage their treatment routines. Visit CFDailyApp.com to learn more.

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Measuring Disease Impact

Some tests may detect progression in the lungs earlier than FEV1

One of the most common tests used to measure CF progression is a pulmonary function test. People with CF may know it better as FEV1, or forced expiratory volume in 1 second. The FEV1 test is usually done after age 6.

But FEV1 does not always find the beginnings of permanent lung damage and disease progression. There are other tests that can show progression in the lungs as well.

Computed tomographic (CT) scans

A CT scan is an x-ray technique that shows much greater detail by using multiple images of a body part, put together by a computer, to form a detailed image. Since it takes a photo of the lungs, damage may be visible early on.

Magnetic resonance imaging (MRI)

An MRI produces images of specific body parts, similar to x-rays and CT scans, but without the exposure to radiation.

Lung clearance index (LCI)

LCI is a test that is sometimes used during clinical trials of CF medicines. It uses a specific technique to measure how much oxygen is getting to all the different parts of the body when someone breathes. It is considered to be more sensitive than the FEV1 test, so it may catch progression sooner.

CF Fact or Fiction CF fact CF Fact or Fiction CF Fact

Other tests 
besides FEV1 can be used in order 
to detect early lung damage.

Because FEV1 doesn't always find progression when it begins, healthcare providers may also look to other tests to assess lung function.

FACT or FICTION

Other Measures of CF

A variety of other tests fill out the rest of the CF picture

There are also other tests that allow care teams to determine the impact of CF on the body.

Sinus endoscopy
  • A small camera is used to look at the sinuses
  • Assesses the extent of disease in the sinuses
Sweat chloride levels
  • Sweat is collected, weighed, and chloride concentration is measured
  • Helps to diagnose someone with CF
Bacterial culturing
  • A throat or sputum culture or bronchoscopy is used to collect bacteria from the lungs
  • Identifies what kind of bacteria are present and the best medicine to treat them
Immunoreactive trypsinogen (IRT)
  • A dried spot of blood is analyzed for IRT, usually in the first few days of life
  • Determines how well someone's pancreas works. IRT is found in higher amounts in someone with CF
Fecal elastase-1
  • A single stool sample is measured for the pancreatic digestive enzyme elastase-1
  • Assesses how well the pancreas is working. If elastase-1 is low, it usually means the pancreas isn't working well
Blood sugar testing
  • A finger stick that uses a needle or a continuous glucose monitor tests insulin levels in the blood
  • Helps people with CF keep their insulin levels in check and control cystic fibrosis-related diabetes (CFRD)
Transaminase levels
  • A blood sample is tested for elevations of certain liver enzymes called transaminases
  • Measures the amount of transaminases because they increase as liver disease advances
Dual energy x-ray absorptiometry (DXA)
  • A special kind of x-ray with low radiation takes a picture of the bones
  • Measures bone mass
Palpation
  • A test done by healthcare providers in which they press on the liver to determine size, tenderness, or masses
  • Helps healthcare providers identify signs of liver disease

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