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What Is Cystic Fibrosis?

Cystic fibrosis is a genetic disease that causes recurring lung infections and makes it harder to breathe over time. People with cystic fibrosis have mutations in the cystic fibrosis transmembrane conductance regulator gene (or CFTR gene), which causes the CFTR protein to work incorrectly so it does not move chloride to the cell surface. As a result, cells are unable to attract water to their surfaces, and mucus inside the body becomes sticky and thick.

The thickened mucus causes a variety of problems throughout the body. In the lungs, mucus builds up, clogging airways and trapping bacteria and germs, which eventually leads to inflammation, infections and potential respiratory failure. The buildup of mucus in the pancreas and liver can also cause significant digestive problems, as well as impeding proper digestion and causing malnutrition and poor growth. It can also block the bile duct, which can lead to liver disease. In men, cystic fibrosis can even cause infertility. Because of the progressive nature of cystic fibrosis, symptoms increase over time, so it is essential that cystic fibrosis treatment begins as early as possible. 

What Causes Cystic Fibrosis?

Cystic fibrosis is a genetic disease caused by a CFTR gene mutation. There are more than 1,700 known mutations of cystic fibrosis. A person must have inherited one copy of the defective CFTR gene from each parent. People with just one copy of the gene are called carriers. They can pass the gene on to their children, but they will not develop the disease themselves. However, even if two people who each have copies of the gene have children, it does not guarantee that the person will develop cystic fibrosis.

Here are the facts. If two cystic fibrosis carriers have a child: 

  • There is a 25% chance the child will have cystic fibrosis.
  • There is a 50% chance the child will be a carrier.
  • There is a 25% chance the child will not be a carrier.

How to Treat Cystic Fibrosis

Cystic fibrosis varies widely from patient to patient and a variety of factors contribute to the way the symptoms manifest. This can include the age of the patient, their overall health and how far the disease has progressed thus far. As a result, cystic fibrosis treatment will vary, too, depending on the unique aspects of each person’s disease and symptoms. 

What is the treatment for cystic fibrosis? Cystic fibrosis treatment depends on the patient and their unique symptoms and circumstances. The cystic fibrosis treatment guidelines recommend health care teams work hand-in-hand with patients and families in order to customize the cystic fibrosis treatment each patient receives. So, when a patient asks, “What is the treatment for cystic fibrosis for me?” the answer will be slightly different for each patient.

Cystic Fibrosis Treatment Options

Cystic fibrosis treatment options aim to help patients live as full a life as possible while helping to slow the progression of the disease and avoid infection. Here are the cystic fibrosis treatment options that are likely to be considered and used in a variety of different combinations.

Airway clearance techniques (ACTs)

These techniques help patients loosen and clear the sticky, thick mucus that accumulates in the airways and lungs. All of these techniques include coughing or huffing, which helps move the mucus out of your airways, after which it must be spit out and discarded. It is important that patients do not swallow the mucus they cough up. 

  • Active cycle of breathing: This gets air behind the mucus. First, breathing control is employed, wherein the patient gently breathes in through their nose, relaxing their airways. Next, the patient inhales deeply and holds their breath for 3 seconds, which helps expand the chest and gets air behind the mucus so it can move up into the larger airways. Finally, the patient huff-coughs to move the mucus from the larger airways out of the body. 
  • Airway oscillating device (AOD): Similar to a PEP device, resistance helps to open the airways, and the device’s vibrations help loosen mucus so it is more easily moved and can be coughed or huffed out. 
  • Autogenic drainage: First, the patient takes shallow breaths to help detach mucus from the airways. Next, the patient holds their breath for 3 seconds, allowing mucus to collect. FInally, the patient employs a hard huff-cough to move the mucus out of the airways. 
  • Chest physical therapy: In this technique, another person uses cupped hands and percussion on the patient to loosen mucus in the airways. The patient lays in a position that allows gravity to assist with draining mucus from the airways. The patient can then huff or cough the mucus out of their lungs. 
  • High-frequency chest wall oscillating vest: The vest compresses the chest in and out to create airflow which, in conjunction with the vibrations of the vest, helps loosen mucus from the airway walls so it can be expelled.
  • Positive expiratory pressure (PEP) device: This device uses resistance to open the airway, which helps get air behind the mucus. This helps loosen it, making it easier to move from the smaller airways to the larger ones and be huffed or coughed up. 

CFTR modulators

These medications help correct the malfunctioning CFTR protein, but are only effective with certain specific mutations. 

Inhaled medicines

Inhaled medicines help open the airways and thin the mucus, and can include bronchodilators, inhaled antibiotics and mucus thinners. 

Oxygen Therapy

Oxygen therapy is a cystic fibrosis treatment when blood oxygen levels decline. While oxygen therapy does not treat the disease itself, it does help to improve quality of life for many patients. 

Pancreatic enzyme supplements

These help the body absorb nutrients to help counteract the risk of malnutrition. 

Cystic Fibrosis and Oxygen Therapy

Cystic fibrosis treatment research continues to focus on developing life-saving therapies for the disease, with the hopes of creating newer cystic fibrosis treatment that can help even more patients. However, oxygen therapy remains a vital aspect of cystic fibrosis treatment options for many patients who struggle with getting sufficient oxygen, especially in later stages of the disease. If you have cystic fibrosis, talk to your doctor about how oxygen therapy can help you breathe easier. For questions about whether a portable oxygen concentrator is right for your cystic fibrosis treatment, contact Inogen today. 

Additional Sources

https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/

https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/

https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/Airway-Clearance-Techniques/

https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Medications/CFTR-Modulator-Therapies/


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