People with CF may undergo multiple medical procedures to manage lung infections, digestive issues, and other complications.
Cystic fibrosis (CF) is a genetic condition that leads to the production of thick, sticky mucus, which can cause complications in multiple organs, including the sinuses, lungs, intestines, and liver.
As a result, people with CF often undergo a variety of medical procedures to manage symptoms, prevent complications, and maintain quality of life.
Endoscopy is a minimally invasive procedure that allows doctors to visualize the interior of the body using a flexible tube with a camera and light source. In CF, doctors commonly use an endoscopy for:
- Nasal and sinus evaluation: Doctors can assess the nasal passages and sinuses for inflammation, blockages, or infection.
- Bronchoscopy: A specific type of endoscopy that examines the airways and lungs. Doctors use it to collect samples, remove mucus plugs, or assess lung health.
Lavage refers to the washing out of a body cavity, typically performed during an endoscopic procedure to remove mucus or debris and improve function.
- Sinus lavage: This procedure flushes out thick mucus from the sinuses to relieve congestion, reduce infections, and improve airflow. For more severe cases, it can be done using saline rinses at home or during surgery.
- Bronchoalveolar lavage (BAL): This procedure, performed during a bronchoscopy, involves introducing saline into the airways to collect fluid samples for testing bacteria, fungi, or viruses. It can also help remove excess mucus from the lungs, reduce infection risk, and improve breathing in people with CF.
Nasal and sinus surgery, typically performed through endoscopic sinus surgery (ESS), is used to treat chronic sinus infections and nasal blockages common in people with CF.
The thick mucus buildup associated with CF can lead to recurring sinus infections, inflammation, and nasal polyps. When medications such as antibiotics, nasal rinses, and steroids are no longer effective, a medical professional may recommend surgery.
ESS helps clear blocked sinuses, remove polyps, and improve drainage, providing relief from symptoms like congestion and sinus pain.
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The results showed that, on average, participants had a significant improvement in their sinus symptoms after surgery. However, those with a certain type of bacteria (pseudomonas) in their sinus culture showed less improvement.
People with CF often experience reduced appetite, malabsorption, and higher energy demands due to lung infections and damage, making it challenging to meet their nutritional needs.
When regular meals and oral supplements are insufficient, enteral tube feeding becomes necessary.
This method delivers high calorie nutrition either through a nasogastric tube (inserted through the nose) or a gastrostomy tube (surgically placed into the stomach). A medical professional will frequently administer tube feeding overnight to promote growth and maintain overall health.
Research shows that feeding tube insertion can lead to the following benefits:
- increased body weight
- improved height and height velocity
- enhanced BMI
- increased body fat and lean tissue mass
- improved biochemical markers of nutritional status
- slower decline in lung function
- improved lung function
- fewer infections and hospital admissions
- increased physical activity
- well-tolerated by participants (nasogastric and gastrostomy feeding)
However, tube feeding can be expensive and may affect self-esteem or cause minor complications like tube dislodgement, nausea, or site infections.
Bowel surgical interventions help address blockages, cancer risks, and other complications related to CF’s impact on the digestive system.
Here are several reasons why people with CF may need bowel surgery:
- Distal intestinal obstruction syndrome (DIOS): A common complication in CF in which thick mucus and undigested food block the intestines. One 2022 study found that intestinal obstruction was the most common reason for surgery in adults with CF (26% of cases).
- Meconium ileus: A type of bowel obstruction in newborns with CF caused by thick, sticky meconium.
- Biliary issues: Problems with the gallbladder or bile ducts can arise, sometimes requiring surgical treatment.
- Gastroesophageal reflux disease (GERD): Severe GERD that doesn’t respond to medication or lifestyle changes might need surgery to manage symptoms. A 2020 study of children with CF found that anti-reflux surgery improved symptoms in 59% of people and helped slow the decline in lung function while reducing flare-ups or lung infections.
CF causes thick mucus buildup, infections, and scarring in the lungs, which can eventually lead to end stage lung disease.
Doctors may consider transplants when treatments are no longer effective, lung function drops to about 30%, and the disease progresses to an advanced stage — but before it reaches end stage.
About 17% of all lung transplants are for people with CF.
Delaying the transplant too long can reduce options, increase the risk of complications, or lead to death while waiting for a donor.
CF can affect bile flow, resulting in conditions such as cirrhosis (advanced liver scarring) and portal hypertension (high blood pressure in the liver’s veins).
Liver disease affects about 5% to 10% of people with CF, typically manifesting around the age of 10 or 11. CF-related liver disease (CFLD) is recognized as the third leading cause of death among people with CF.
When CFLD progresses to severe complications — such as cirrhosis, portal hypertension, or life threatening issues like variceal bleeding — a liver transplant may be necessary to save the person’s life.
Healthcare professionals often consider transplants when liver function deteriorates significantly and other treatment options can no longer manage the complications.
CF is a complex condition that affects multiple organs, including the lungs, intestines, and liver.
To manage complications, people with CF may undergo airway clearance therapies, gastrointestinal surgeries, and transplants (like liver or lung).
While the need for multiple treatments can be challenging, advances in care are improving outcomes and helping people with CF live longer and healthier lives.