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Cystic Fibrosis

What is Cystic Fibrosis?

Cystic Fibrosis, or CF, is a genetic disease that affects the exocrine glands. These glands control the production of sweat, digestive fluids, and mucus. Cystic Fibrosis causes the body to produce thick mucus that blocks airways, prevents normal digestive functions, and leads to bacterial infections.

How is Cystic Fibrosis Tested?

People with CF sweat out excessive amounts of salt. The most common test for the cystic fibrosis disease is a simple sweat test.

Life Expectancy of a Cystic Fibrosis Patient:

Over that last few decades, huge advances in technology and CF treatments have been made in the cystic fibrosis community. In the 1980’s, the average life expectancy of a CF patient was 14 years. In 2015, the average life expectancy of a cystic fibrosis patient is 37 years.

Cystic Fibrosis Facts:

  • Cystic Fibrosis is a recessive gene mutation, meaning both parents must carry the defective gene for their children to have CF.
  • Advances in newborn screening help diagnose cystic fibrosis at birth.
  • CF patients produce heavy mucus that builds up and damages many organs in the body.
  • CF is most commonly found in the Caucasian population of the United States.
  • CF Symptoms and Common Complications:
    • Cystic Fibrosis-Related Diabetes (CFRD)
    • Chronic sinus and lung infection
    • Gastroesophageal Reflux Disease (GERD)
    • Constant coughing; asthma, wheezing, and shortness of breath
    • Bone Disease (fracture, osteopenia or osteoporosis) 

Cystic Fibrosis Symptoms by Organs

CF Lung Symptoms:

Mucus buildups make it easier for bacteria to grow, this causes lung and sinus infections. The heavy mucus in the lungs makes it increasingly difficult to breathe and sustain normal respiratory function. Daily breathing treatments and antibiotics are used to break down the mucus and fight bacteria growth.

CF Digestive Symptoms:

Mucus can block tubes, plug air ducts, and even stop digestive enzymes from reaching the stomach. The pancreas needs these enzymes to break down food. Without them, the body cannot properly absorb key nutrients, minerals, and proteins.

Sweat Glands:

Cystic Fibrosis patients are easily dehydrated because their bodies sweat out excessive amounts of salt. Replacement therapy is common for CF patients because salt is essential in sustaining muscle function and nerve cell health.

IV Solutions’ Cystic Fibrosis Medications

IV Solutions provides limited distribution medications and devices for cystic fibrosis patients. These medications include: Cayston®, Bethkis®, Creon®, TOBI®, TOBI® Podhaler, Altera® Nebulizer System, Altera® Handset, and more. See full list.