Cystic Fibrosis

Multidisciplinary Care for Adult and Pediatric Patients with Cystic Fibrosis

Loyola Medicine provides comprehensive, multidisciplinary care for cystic fibrosis (CF) for both adults and children. If you or a loved one is concerned that recent symptoms are due to cystic fibrosis, you want an accurate diagnosis as quickly as possible. Our dedicated team will determine what is causing your symptoms and deliver the highest quality of care. 

Loyola’s pulmonologists are leaders in their field, providing the most advanced medical and surgical treatments for patients with cystic fibrosis. Your Loyola healthcare team will first explore conservative approaches to treating your cystic fibrosis, including lifestyle changes and medical management. If your condition cannot be well controlled in these ways, lung transplant surgery may be recommended.

Why Choose Loyola for Cystic Fibrosis Treatment?

Loyola’s compassionate team of specialists treats both children and adults with cystic fibrosis. Our program, which is accredited through the Cystic Fibrosis Foundation, delivers the highest standard of care available. Our pulmonologists, surgeons, advanced practice nurses, nurses, respiratory therapists, dietitians, genetic counselors, social workers and psychologists will help you manage this condition. 

In the event that a lung transplant is recommended, Loyola’s cystic fibrosis team works closely with our widely regarded transplant team to provide collaborative care. Loyola’s transplant surgeons are highly skilled in minimally invasive surgical approaches. Your pulmonologist will work closely with Loyola’s Transplant Center to provide you the most advanced care for your lung transplant surgery.

According to the International Society for Heart & Lung Transplantation, Loyola’s lung transplant program is among the top 15 percent in the world in volume, which leads to better outcomes. Our one-month and three-year patient survival rates are above the national average, as are our one-month and three-year graft survival rates.

Loyola also has a program that pairs transplant candidates with post-transplant mentors, which our patients find to be extremely beneficial in navigating this condition. 

What is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic condition that affects about 30,000 adults and children in the United States. It disrupts the normal function of the digestive and respiratory systems and other organs.

Cystic fibrosis causes the body to produce an abnormally thick mucus, which clogs the lungs and provokes serious lung infections. It also leads to obstructions in the pancreas and interrupts an enzymatic process that helps the body break down food and absorb much-needed nutrients. CF also can affect the sweat glands and the reproductive system in men. Many advances have been made for this life-altering disease, helping patients live better and longer lives.

In newborns, symptoms of cystic fibrosis include:

  • Growth delays
  • Lack of bowel movements in the first 24 to 48 hours
  • Low weight gain
  • Salty-tasting skin

In adults, symptoms of cystic fibrosis include:

  • Bouts of pancreatitis
  • Chronic cough
  • Clubbed fingers
  • Excessive gas, bloating and a distended belly
  • Fatigue
  • Frequent bouts of pneumonia
  • Infertility (in men)
  • Loss of appetite
  • Malnutrition
  • Mucus buildup in lungs and sinuses
  • Nasal congestion from polyps
  • Severe constipation
  • Sinus pain from infection or polyps
  • Stools that are pale, contain mucus or float
  • Weight loss

How is Cystic Fibrosis Diagnosed?

Your pulmonologist will take a detailed personal and medical history and conduct a thorough exam. One or more of the following tests may be ordered for you or your child:

  • Chest X-ray
  • CT scan (computed tomography)
  • DNA testing
  • Fecal fat test (to check for steatorrhea, or greasy stools)
  • Immunoreactive trypsinogen test (often used in newborn screening)
  • Pancreatic function test
  • Pulmonary function test
  • Secretin stimulation test
  • Spirometry test
  • Sweat chloride test (often used for infants, sometimes referred to as the sweat test)
  • Upper GI and small bowel series

A blood test is also conducted on all newborns to check for a variety of illnesses, including cystic fibrosis. This early screening will allow parents start treatment earlier and delay some of the damage caused by cystic fibrosis.

How is Cystic Fibrosis Treated?

It is highly recommended that patients seek treatment through medical centers that have been accredited with the Cystic Fibrosis Foundation, such as at Loyola. 

Follow-up and medical monitoring are essential in treating cystic fibrosis. Several therapies and mucus thinners are used to clear mucus buildup; and because infections can be serious, antibiotics likely will be part of your treatment. You may need one or more of the following:

  • Airway mucus-clearing devices
  • Antibiotics to treat or prevent sinus and lung infections
  • Chest physiotherapy
  • CV catheter ports
  • DNase enzyme therapy
  • High-protein, high-calorie diet
  • Hypertonic saline solutions
  • Inhaled medicines
  • Kalydeco (an FDA-approved medication shown to help patients with a genetic mutation of CF)
  • Lung exercise therapy
  • Oxygen therapy
  • Pancreatic enzymes to help with absorption of fats and nutrients
  • Percutaneous gastrostomy (PEG)
  • Stool softeners
  • Vaporized medications to help clear mucus from deep in the airways
  • Vitamin supplements
  • Yearly flu vaccine
  • Yearly pneumococcal polysaccharide vaccine

In some cases, lung transplant surgery may be considered the best next option. Loyola’s lung transplant team is widely recognized for treating the most difficult cases with excellent outcomes. Our Transplant Center has performed more than 825 lung and heart-lung transplant surgeries since 1988 and is among the top 15 percent in the world in volume, which leads to better outcomes. Learn more about lung transplant at Loyola.

Ongoing Clinical Trial and Research to Advance Cystic Fibrosis Treatment

Loyola is conducting research today that will lead to the treatments of tomorrow. Our lung transplant program is the only one in the state of Illinois to receive funding from the National Institutes of Health for lung transplant research.

We have 19 research programs underway, including clinical trials for idiopathic pulmonary fibrosis, LAM, cellular mechanisms in acute lung injury, breathing control, respiratory muscle function, mechanisms of patient-ventilator interactions and the effect of alcohol on the lungs. Loyola’s program also has created a blood bank to assist with future research. 

Loyola patients will also be granted access to the latest medications and therapies through our clinical trials