The Best Ways to Monitor Cystic Fibrosis

Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. In people with CF, the body produces unusually thick and sticky mucus that:

  • clogs the lungs and leads to life-threatening lung infections;
  • blocks the pancreas and stops natural enzymes from helping the body break down and absorb food.

There is no cure for CF, but treatments have improved dramatically in recent years. With early diagnosis and aggressive treatment, people with CF are living longer and healthier lives.

What is cystic fibrosis?

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick and sticky mucus that:

  • clogs the lungs and leads to life-threatening lung infections;
  • blocks the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with CF lived to attend elementary school. Today, advances in research and medical treatments have dramatically improved the quality and length of life for people with CF. Many people with the disease now live into their 30s, 40s and beyond.

What causes cystic fibrosis?

Cystic fibrosis is caused by a defective gene that a person inherits from each parent. The defective gene controls the movement of salt and water in and out of cells. In people with CF, the gene causes the body to produce unusually thick and sticky mucus. The mucus:

  • clogs the lungs and leads to life-threatening lung infections;
  • blocks the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the past, most children with CF died before reaching elementary school age. Today, advances in research and medical treatments have dramatically improved the quality and length of life for people with CF. Many people with the disease now live into their 30s, 40s and beyond.

How is cystic fibrosis diagnosed?

Cystic fibrosis is usually diagnosed in childhood, but it can be diagnosed at any age. A diagnosis of CF can be made through a simple sweat test or a genetic test.

How is cystic fibrosis treated?

There is no cure for CF, but treatments have improved dramatically in recent years. With early diagnosis and aggressive treatment, people with CF are living longer and healthier lives.

Treatment for CF focuses on:

  • preventing and treating lung infections;
  • clearing the mucus from the lungs;
  • preventing and treating malnutrition;
  • managing other CF-related complications.

Most people with CF receive treatment at a specialized CF care center. A team of CF specialists will work with you and your family to create a treatment plan that meets your individual needs.

What are the complications of cystic fibrosis?

The thick mucus that clogs the lungs and blocks the pancreas can lead to a number of serious complications, including:

  • lung infections;
  • respiratory failure;
  • pneumonia;
  • bronchiectasis;
  • malnutrition;
  • diabetes;
  • liver disease;
  • infertility;
  • osteoporosis.

People with CF are also at increased risk for developing certain types of cancer, including:

  • bronchial adenocarcinoma;
  • nasal squamous cell carcinoma;
  • intestinal adenocarcinoma.

What is the life expectancy for someone with cystic fibrosis?

The median age of survival for people with CF is now 37 years. This is up from 20 years in the early 1980s. With early diagnosis and aggressive treatment, people with CF are living longer and healthier lives.

How can I prevent cystic fibrosis?

There is no way to prevent CF. However, if you have a family member with CF, you can be tested for the defective gene. If you are a carrier of the defective gene, you can have genetic counseling to discuss your risk of having a child with CF.

What is the outlook for someone with cystic fibrosis?

The outlook for people with CF has improved dramatically in recent years. With early diagnosis and aggressive treatment, people with CF are living longer and healthier lives. The median age of survival for people with CF is now 37 years. This is up from 20 years in the early 1980s. However, CF is still a progressive and life-threatening disease. People with CF typically have a shorter life expectancy than the general population.

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