Cystic Fibrosis

Healthcare providers usually diagnose cystic fibrosis in babies. But about 1 in 50 people with CF don’t receive a diagnosis until adulthood.

Maybe you’ve dealt with coughing and repeated sinus infections your whole life but never knew why. Maybe you were told you had asthma or a chronic cough as a child, but as you’ve gotten older, you’ve felt worse.

No matter what brought you here, we’ll help figure out what’s going on. You can trust we’ll find the answers you’ve been looking for so you can begin the treatment that’s right for you.

What to expect at your first visit

You may wonder how your first appointment with us will be any different from all the others you’ve had over the years. It’s easy to give up hope that you'll feel better when you’ve gotten your hopes up so many times before. We understand how you might be feeling, and that’s why the first thing we’ll do is learn about you.

We want to know about the road you’ve been on and what it’s been like for you. If you’ve been diagnosed with CF, we’ll talk to you about your treatment plan and how it’s going. If you suspect you have CF but haven’t been diagnosed, we’ll talk about your symptoms and any diagnoses you’ve received elsewhere.

To diagnose CF, we’ll need to do some tests. One test that’s very helpful is a sweat test. It measures the amount of chloride (salt) in your sweat. Higher levels can be a sign of CF. This test isn’t painful and doesn’t use a needle.

Other tests you might need include:

• Genetic testing to look for gene variants (DNA changes) that cause CF. This involves taking a sample of your blood.
• Chest X-rays to see if mucus is blocking your airways and lungs.
• Sinus X-rays to look for too much mucus, bone damage or too many nasal polyps in your sinuses.
• Sputum (phlegm) culture to check for bacteria in your spit.
• Pulmonary (lung) function tests to see how well your lungs are working.
• Nasal potential difference (NPD) test to measure the electricity generated when ions cross your nasal tissue.
• Intestinal current measurement (ICM) test, which involves having a rectal tissue biopsy to check for chloride.

We’ll talk about your test results and what they mean for your treatment plan. 

Meet Our Cystic Fibrosis Team

Experts from many different specialties work together to give you the best possible care, no matter your age or the stage of your CF. You’ll have a handpicked team of providers on your side. Your team may include:

• Pulmonologists.
• Pediatric pulmonologists.
• Transplant pulmonologists.
• Cardiothoracic surgeons.
• Gastroenterologists.
• Rheumatologists.
• Otolaryngologists.
• Endocrinologists.
• Infectious diseases.
• Respiratory therapists.
• Fertility specialists.
• High-risk obstetrics.
• Social workers.
• Psychologists.
• Genetic counselors.
• Physical therapists.
• Nurse practitioners.
• Physician assistants.
• Research coordinators.
• Financial counselors.

We treat all stages of CF. Whether you have early-stage lung disease or you’re a lung transplant recipient (or somewhere in between), we’re here for you. We also specialize in treating the most challenging CF-related airway infections.

Here are just some of the ways we can treat your CF.

Techniques and devices for clearing your airways

We’ll teach you special ways to cough and breathe that’ll help clear out the mucus that blocks your airways. Our physical therapists can also help you with chest physical therapy, where you move into certain positions that loosen and drain out mucus.

Your providers might also recommend devices that fit into your mouth or therapy vests that use vibrations to loosen the mucus.

Medications

Medications can sometimes be used to treat or prevent lung infections, help you clear out mucus and prevent complications. Your provider might prescribe:

• Antibiotics to treat or prevent lung infections.
• Inhaled bronchodilators to open and relax your airways.
• Inhaled medicine to thin mucus and make it easier to get rid of.
• Medications that treat CF if you have certain gene changes.
• Pancreatic enzymes to help you digest food.
• Stool (poop) softeners if you’re constipated.

We may also see if you’re a candidate for some of the latest therapies, called cystic fibrosis transmembrane conductance regulator (CFTR) modulators.

CFTR modulators focus on the root cause of cystic fibrosis — genetic changes that stop your body from making the CFTR protein. When that protein doesn’t work right, your body makes mucus that’s too thick and sticky.

CFTR modulators help the protein work better, which can ease your symptoms. And they’ve proven to be a game-changer.

But modulators only work for people with certain types of genetic mutations (changes) as the underlying cause of CF. If that’s not you, there’s still hope on the horizon. We’re continuing to research medications that’ll help even more people with CF in the future.

Surgeries

If you need surgery to treat CF or its complications, you’re in good hands. We’re experienced in a range of surgeries, including:

• Sinus surgery to help your sinuses clear out mucus better.
• Placement of a feeding tube to help you get enough nutrients (percutaneous endoscopic gastrostomy or PEG).
• Surgery to remove blockages in your small intestine.
• Lung transplantation.

Hearing you may need new lungs can be unsettling. But if you do, our team will walk you through the process one step at a time. We work closely with Cleveland Clinic’s lung transplant program, which is a national leader in transplants for people with CF. We’re prepared to get you through the transplant process and help you recover.

Follow-Up Care

CF is a lifelong condition, and we’ll be with you every step of the way. We’ll see you for regular follow-ups and keep a close eye on how you’re doing.

Our team-based approach means experts from many different specialties will guide your CF treatment and handle any related medical conditions that come up, like diabetes or osteoporosis. We know your treatment needs may change over the years, and our experts will adjust your plan if needed.

Taking the Next Step

Maybe you’ve been on your CF journey for a while. Or maybe you’re on a journey that doesn’t have a name yet. The experts at Cleveland Clinic are ready to meet you wherever you’re at. Together, we’ll envision a tomorrow that’s better than yesterday. And we’ll provide the care you need to help make that vision a reality.