What Is Cystic Fibrosis?

Cystic Fibrosis: Understanding the Signs, Symptoms, and Treatments

There are few conditions that elicit strong feelings of both fear and confusion. People may know that cystic fibrosis (CF) is a serious medical condition, but they may struggle to accurately identify what it means, what its symptoms are, and the long-term prognosis.

Whether you have a family member with the condition or you are just interested in learning more about CF getting moving from uninformed to well-informed can be a trying process but gaining more knowledge is always valuable.

Here are the basic pieces of information you need to become aware of CF.

What Is Cystic Fibrosis?

Cystic fibrosis is a lung disorder that causes lung infections that persist over time. CF is a genetic, progressive condition meaning:

  • The condition is inherited from parents with the disease or parents who carry the disease. Parents with a mutated CF gene are more likely to have a child with CF. When both parents have the gene, there is a 25 percent chance their child will have CF, a 50 percent chance their child will be a carrier and a 25 percent chance their child will not have CF or be a carrier.
  • Over time, the condition will worsen. Often, treatments can slow the progression of CF, but eventually, the person will succumb to the disease. The average age of survival is around 40. Though this number is troubling, the life expectancy has grown tremendously in the last 60 years.

CF causes an excessive amount of mucus in and around the lungs, pancreas, and other organs throughout the body. The thick and sticky substance in the lungs results in the airways being clogged and full of bacteria, which leads to infections, respiratory damage, and chronic breathing troubles in the long-term.

In the pancreas, the excessive mucus hinders needed enzymes from being released. Without the enzymes, the body cannot break down food and absorb its nutrients.

Screenings and blood tests can identify parents that carry the genetic mutation before or during pregnancies. Most people with CF are diagnosed with the condition before the age of two.

Facts on Cystic Fibrosis

There are more than 70,000 in the world diagnosed with CF with about 30,000 of those living in the U.S. Each year, there are about 1,000 more people diagnosed and treated for the disease. The average age of people with CF is increasing. Currently, more than 50 percent of all people with CF are over the age of 18.

What Are the Symptoms of Cystic Fibrosis?

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These people living with CF will have symptoms commonly associated with the condition including:

  • Skin with a salty taste
  • Consistent coughing with phlegm
  • Frequent lung problems like pneumonia and bronchitis
  • Trouble breathing due to wheezing and shortness of breath
  • Inability to grow taller and gain weight despite eating habits
  • Bowel movements that are greasy or bulky
  • Infertility in males

Since the condition is progressive, these cystic fibrosis symptoms may start small and slowly before developing into more pressing concerns as the person ages. Each person’s journey will be unique, but the complications are unavoidable.

Cystic Fibrosis Treatment Options

The treatment options for cystic fibrosis have the goal of improving the individual’s daily quality of life while adding to their overall quantity of life. For many, treatments are conducted daily including:

  • Airway clearance. Because one of the main problems from CF is thick mucus in the lungs, this treatment helps to clear it up. One way to establish airway clearance is with the use of a special vest. When the person puts on the vest, the device inflates and vibrates the chest at a special frequency, which loosens and thins the problematic mucus from these areas. With the mucus reduced, breathing becomes easier.
  • Inhaled medications. While the vest works from the outside, these inhaled medications work from the inside to minimize the impact of the mucus and prevent or treat harmful infections. Medications are usually liquids that are transformed into mists through a tool called a nebulizer.
  • Enzyme supplements. Since the CF prevents proper enzyme regulation, people with CF will often utilize supplements to boost the action of the enzymes in the body. With these medications, more nutrients can be absorbed during meals and snacks to improve overall health.

Within the last few years, medication breakthroughs have added another treatment to the CF toolkit. Called CFTR modulators, these new medications target and treat the origin of CF – a defective protein called CFTR.

As more and more people use these medications in conjunction with their other treatments, they could add years or decades on their lifespan. This is an exciting and optimistic time in terms of CF treatments.

It’s true that CF is a devastating and life-changing diagnosis, but the world of CF is changing. Where people in the past living with CF faced many hardships paired with a short life expectancy, things are changing now.

Now, people with CF are accomplishing the dreams they didn’t know was possible. With the time and attention paid to CF, the next breakthrough could be right around the corner.

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