How cystic fibrosis affects your body over time

Cystic fibrosis (CF) is a lifelong, progressive genetic condition that can affect nearly every system in your body. As you age, your CF symptoms and complications may increase – especially if you’re not proactively treating and monitoring it.  

“While CF is often associated with the lungs, it’s truly a multi-system condition. Early detection of complications in other organs can greatly improve quality of life and outcomes,” says pulmonologist Catherine T. Shoff, DO, director of the Geisinger adult CF program. 

Learning how CF manifests in your body over time can help you, your caregivers and healthcare providers stay a step ahead of the disease. 

What is cystic fibrosis? 

Cystic fibrosis is a genetic disorder that affects the CFTR protein, which plays a crucial role in maintaining the balance of salt and water on certain surfaces of the body.

“Mutations in the CFTR gene cause the body to produce thick, sticky mucus that can clog airways and trap bacteria,” Dr. Shoff explains. 

CF causes  

CF is one of the most common life-shortening genetic diseases in the U.S. According to the Cystic Fibrosis Foundation, more than 30,000 people in the U.S. have CF, with about 1,000 new cases diagnosed each year.  

The cause? “It’s genetic,” says Dr. Shoff. “It’s caused by inheriting 2 faulty CFTR genes — 1 from each parent.” 

How CF affects your lungs  

Lung complications are the most recognized hallmark of CF. Thick mucus starts building up early in life, even before symptoms become noticeable.  

While various CFTR mutations impact the CFTR protein differently, the outcome in the lungs is the same: mucus that is difficult to clear and damaging over time, says Dr. Shoff. 

Infections and inflammation 

The sticky mucus traps bacteria, leading to frequent lung infections and ongoing inflammation. These infections can be severe and recurring, increasing the risk of permanent damage. 

Lung damage  

Repeated episodes of infection, called pulmonary exacerbations, can scar and damage the lungs. This damage reduces airway elasticity and lung function, which becomes more difficult to reverse as CF progresses.  

Loss of lung function 

Bronchiectasis, a condition where airways become abnormally widened, is common in CF and contributes to the gradual loss of lung function. Other serious complications include: 

• Hemoptysis (coughing up blood) 
• Pneumothorax (collapsed lung) 
• Advanced lung disease
• Respiratory failure 

Preventing lung damage with CF 

“Preventive care is essential,” says Dr. Shoff. Airway clearance techniques, regular physical activity, appropriate use of antibiotics and staying on top of routine lung function tests can help preserve lung health for as long as possible. She adds, “Many people with CF with specific mutations are now eligible for new CFTR modulator therapies, which help correct the dysfunctional protein made by the CFTR gene and restore function.” 

How CF affects your digestive system   

While CF is often thought of as a lung disease, it can also affect your digestive tract, including the pancreas, liver and intestines. 

Pancreas issues 

The pancreas may become blocked by thick mucus, leading to: 

• Pancreatic insufficiency, where digestive enzymes can’t reach the intestines 
• Pancreatitis, inflammation of the pancreas
• Cystic fibrosis-related diabetes (CFRD), a unique form of diabetes common in adults with CF 

Liver issues  

Cystic fibrosis-related liver disease is a leading cause of morbidity in CF. It may present as: 

• Fat accumulation in the liver 
• Hepatitis
• Cirrhosis, which may lead to liver failure in severe cases 

Intestinal issues  

CF can thicken digestive fluids, causing: 

• Chronic constipation 
• Acute conditions such as meconium ileus in newborns or distal intestinal obstruction syndrome (DIOS) in older patients
• An increased risk of colorectal cancer in adults with CF 

How CF affects your kidneys 

CF does not directly impact kidney function, but certain risk factors increase the likelihood of developing chronic kidney disease (CKD), particularly in adults ages 25 – 35. These risk factors include: 

• Long-term use of nephrotoxic medications, such as aminoglycoside antibiotics 
• Diabetes (CFRD)
• Kidney stones, which are more prevalent in people with CF 

How CF affects your sinuses 

CF affects mucus production in the sinuses as well. When this mucus becomes thick and stagnant, it leads to: 

• Chronic sinus infections or sinusitis
• Nasal polyps, which can obstruct breathing and impair smell 

Sinus pressure and inflammation can significantly affect quality of life, so it’s an important area to manage your CF symptoms.  

How CF affects your ears 

CF itself doesn’t harm hearing, but long-term use of life-saving medications can. People with CF often need aminoglycoside antibiotics to treat lung infections. Unfortunately, these medications may cause up to 20% hearing loss in people exposed long term. Get regular hearing screenings if you’ve ever been on these treatments. 

How CF affects your bones 

If you have CF, your bone health may be compromised due to poor nutrient absorption, particularly of vitamin D and calcium. Inflammation from ongoing infections also contributes to reduced bone density. 

• Up to 26% of adults with CF have osteopenia
• Severe cases may develop into osteoporosis
• Weakened bones are more prone to fractures

Routine bone density screening and supplementation, often starting in childhood, are standard parts of CF care. 

Cystic fibrosis life expectancy  

CF care has advanced significantly in recent years, and people with CF are living longer, fuller lives than ever before. 

Dr. Shoff notes, “We’re now seeing most patients with CF reaching adulthood and even older age, which was unheard of just a few decades ago. This opens up new challenges — and new opportunities — for managing CF across the lifespan.” 

Proactive management, regular screenings and collaboration with a multidisciplinary care team at an accredited CF care center are crucial to maintaining health and preventing complications. 

As research continues, more therapies and approaches are becoming available to help people with CF live well at every stage of life. 

Next steps:

Learn about lung care at Geisinger
What does an air quality alert mean?
Bronchitis vs. pneumonia: What's the difference?