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7 Tips For Living With Cystic Fibrosis

Cystic Fibrosis primarily affects the lungs and digestive system. It is a genetic disorder of the CFTR gene, which helps water and salt pass through our cells. This defect causes thick, sticky mucous in the lungs, digestive tract, and other organs.

How Does Cystic Fibrosis Affect The Body?

Cystic Fibrosis can cause serious issues in various body systems. The production of thick mucus affects the respiratory system by clogging airways and making breathing difficult. It also causes inflammation, which damages lung tissue and leads to infections caused by trapped bacteria.

The thick mucus can also obstruct the pancreas and prevent digestive enzymes from reaching the small intestine. When this occurs, nutrients are not absorbed properly and can cause malnutrition.

Symptoms of Cystic Fibrosis

Various symptoms are common with CF, and they can vary widely. The most common include coughing, wheezing, and frequent lung infections like pneumonia or bronchitis. Those living with Cystic Fibrosis often have trouble gaining weight, greasy stools, and excess salt in sweat, which makes the skin taste salty.

7 Tips For Those Living With Cystic Fibrosis

1. Medications to Manage CF

In 2019, the FDA approved Trikafta, a drug that combines three medicines which treats the most common type of cystic fibrosis gene mutation affecting 90% of those suffering from CF. Other medications to treat CF according to The Cystic Fibrosis Foundation include:

• Antibiotics - combat bacterial infections
• Bronchodilators - widen airways and help surrounding muscles relax
• Mucus-thinning drugs - to thin mucus and make coughing more productive
• Enzyme supplements to aid digestion

2. Abide By The 6-Foot Rule

Cystic-Fibrosis.com states that CF patients should avoid contact with each other and anyone with a respiratory illness. Sneezing, coughing, or speaking can transmit germs that become trapped in mucus and potentially cause a dire health emergency for those with CF. Even the common cold can cause life-threatening issues. The 6-Foot Rule reduces the risk of dangerous infections by keeping a distance of at least six feet from other CF patients and anyone with any type of respiratory illness, fever, cough or flu.

3. Minimize Infections

Along with the 6-foot rule, John Hopkins Medicine recommends that patients and anyone around them should follow these practices

• Wash hands regularly using antimicrobial soap
• Use alcohol-based hand sanitizer
• Wear a mask
• Don’t touch things in common areas such as waiting rooms. Magazines, pens, toys, etc., are notorious for harboring harmful germs.

4. Airway Clearance Techniques

Hopkinsmedicine.org lists several types of airway clearance techniques to remove mucus from the lungs and bronchial tubes, such as:

• ACBT (Active Cycle of Breathing Technique) - This 3-part therapy works by relaxing the airway, clearing mucus and forcing mucus from the lungs.
• Autogenic Drainage – A breathing therapy that uses various breathing speeds to clear mucus from the lungs
• Chest Percussion Therapy (also known as CPT and Manual Percussion) - Drains mucus from the lungs by using gravity and a clapping motion with a cupped hand.
• Exercise – Being active increases lung functionality and strengthens bones.
• High Frequency Chest Wall Oscillation Vest – An inflatable vest is placed on the patient and attached to a machine which uses high frequency vibrations to help clear mucus from the lungs.
• Huffing Coughs – This technique is used as part of other airway clearance therapies by breathing in and holding the breath and then exhaling assertively. Inhaling loosens mucus from the walls of the lungs and by breathing out with force, mucus is expelled.
• Positive Expiratory Pressure – PEP is an inhalation therapy which uses a device or mask to remove mucus from the airway by creating resistance upon exhalation.

5. Be Aware Of Air Quality

It is vital for CF patients to be aware of the air quality of their surroundings.The National Library of Medicine prepared a report in 2022 citing the impact of air pollution for those suffering with Cystic Fibrosis. It references a study that noted an increased risk of complications of CF and developing respiratory diseases when air quality is poor. There are many weather-related apps and websites that monitor local air quality daily for potentially hazardous conditions for those with compromised lungs.

6. Avoid Second-Hand Smoke

Smoking damages the lungs, period! This includes second-hand smoke. Exposure to any type of smoke, whether it is from a cigarette, vape pen, cigar, hookah, pipe, or even a fire puts lung health at risk.

7. Stay Away From Vaping

It is a myth that vaping is a safe alternative to smoking. Vape gets into the lungs and can cause all sorts of issues. Cysticfibrosis.org states that vaping can cause excess phlegm, shortness of breath, bronchitis and coughing.

While there is no cure for Cystic Fibrosis, life expectancy and complications have been significantly improved by advancements in scientific research. Treatment options focus on improving overall health and well-being, managing symptoms, and preventing any possible complications. Talk to your doctor about medications for Cystic fibrosis and airway clearance techniques. Nutritional support, exercise, and close monitoring by a specialized healthcare team will help improve symptoms and quality of life.

Research into CF continues, exploring new therapies, medications, and approaches aimed at addressing the underlying causes of the disease and improving outcomes for individuals living with CF.

 

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