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.
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.
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.
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:
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.
Along with the 6-foot rule, John Hopkins Medicine recommends that patients and anyone around them should follow these practices
Hopkinsmedicine.org lists several types of airway clearance techniques to remove mucus from the lungs and bronchial tubes, such as:
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.
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.
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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Linda Guerrera has been the Digital Content Manager for Health Products For You since 2022. A recipient of the New York State Broadcasters Award for Outstanding Work in Radio, she spent over ...
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