What Is Cystic Fibrosis?

If you or someone you love has been diagnosed with cystic fibrosis, you may be wondering, “What is cystic fibrosis, exactly?” Cystic fibrosis (CF) is a progressive, genetic disease that causes mutations in the cystic fibrosis transmembrane conductance regulator gene, also known as the CFTR gene. These mutations cause the CFTR protein in the cells to stop working correctly, which makes it unable to move chloride to the surface of the cell. Without chloride, which attracts water to the surface of the cell, excess, thick mucus begins to form inside the lungs, pancreas and other organs. When this mucus forms in the lungs, it blocks the airways, making breathing difficult, and trapping germs and bacteria inside the lungs. The trapped germs and bacteria often lead to respiratory infections and inflammation, eventually damaging the lungs. Cystic fibrosis also affects the gastrointestinal tract, and can even cause infertility in men. It is a serious, life-threatening disease that requires treatment.

Cystic Fibrosis Symptoms

It is helpful to be familiar with cystic fibrosis symptoms if it runs in your family, though the severity of the symptoms varies from person to person. Here are the most common cystic fibrosis symptoms:

  • Allergies year round
  • Chronic coughing: Either dry or coughing up mucus
  • Constipation
  • Excessive sweating
  • Frequent, greasy, large bowel movements with a foul odor
  • Frequent sinus infections due to thicker mucus 
  • High blood sugar symptoms, like frequent thirst and urination
  • Intestinal blockage
  • Poor growth
  • Recurring pancreatitis
  • Recurring respiratory infections and chest colds
  • Salty-tasting skin
  • Shortness of breath
  • Wheezing that does not respond to typical asthma therapies

Lung function can seem fairly normal in childhood with some patients with increasing symptoms over time, often beginning in the teen years.

Cystic Fibrosis Causes 

Know your cystic fibrosis facts. Cystic fibrosis is not contagious and cannot be contracted. The only cystic fibrosis causes are genetic and require inheriting two abnormal CF genes. Sometimes cystic fibrosis occurs without a known family history because anyone with a single abnormal CF gene is a carrier. If two carriers have a child, chances are:

  • 25% that the child will have CF
  • 50% that the child will be a carrier
  • 25% that the child will not be a carrier or have CF

About 30,000 people in the United States are living with CF and about 1,000 new cases will be diagnosed each year. More than 75% of those with the disease are diagnosed by age 2. While it is an inherited disease, there are also risk factors that can affect the severity of the disease. Here are some cystic fibrosis facts and risk factors to consider. 

  • Age: Cystic fibrosis symptoms get worse with age, with lung function declining a bit each year.
  • Environment: Exposure to secondhand smoke and other lung irritants can worsen your symptoms and make your disease progress faster.
  • Exercise: It is important to get regular exercise to keep lungs healthy.
  • Genes: Other modifier genes can affect cystic fibrosis symptoms and outcomes.
  • Nutrition: People with cystic fibrosis require a high-calorie diet to maintain weight or grow, and they should be very conservative when it comes to alcohol consumption.
  • Type of CF gene mutation inherited: CF gene mutations are divided into five classes (Class I, II, III, IV or V) based on the extent of the CFTR damage; Classes I-III are generally more severe cases, while Classes IV and V are often milder. 

If you are diagnosed with cystic fibrosis, follow your doctor’s recommendations in order to stay as healthy as possible. 

What Is Cystic Fibrosis Treatment Like?

There is no cure for cystic fibrosis, so it is essential to stay as healthy as possible. Your health care providers will assess the severity of your cystic fibrosis, as well as assessing your overall health, before choosing your ideal treatment plan.

In most cases, cystic fibrosis treatment includes the following:

  • Airway clearance therapy: ACT therapy shakes or vibrates the mucus in the airways manually, with the help of a VEST device or with a portable “flutter” device, enabling the patient to cough up the mucus and clear their airway
  • Antibiotics: Treats bacteria growth in the mucus
  • Anti-inflammatory medication
  • Mucus-thinning medication: Helps mucus to be cleared with ACT, usually provided via inhaler or nebulizer
  • Oxygen therapy: Helps improve oxygenation in the body
  • Pancreatic enzyme replacement therapy: Helps the body properly absorb the nutrients from food
  • Salt replacement

How Inogen Can Help

If your doctor decides that supplemental oxygen will help ease your cystic fibrosis symptoms, ask if a portable oxygen concentrator is right for your oxygen therapy. Inogen’s portable oxygen concentrators are small and lightweight, allowing you to get your oxygen treatments at home or on the go. Supplemental oxygen has been shown to help cystic fibrosis patients sleep better and tolerate exercise better, so a portable oxygen concentrator could be used for both purposes. Speak with your doctor and contact Inogen today to find out more about how our products can help you breathe better. 

Sources

https://www.cochrane.org/CD003884/CF_additional-oxygen-in-the-treatment-of-cystic-fibrosis


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