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Cystic Fibrosis: Diagnosis, Treatment and a Team Approach

When most people hear “cystic fibrosis” (CF) they think of a lung disease, if they know what CF is at all. While CF is a pulmonary disease, it also impacts other systems of the body. In fact, CF was discovered as a disease involving the gastrointestinal (GI) system. Patients would be so ill with abdominal  issues that they never progressed to the point where pulmonary issues could be identified. As survival has improved through the years, CF’s tie to lung disease became apparent and pediatric pulmonologists have absorbed the bulk of patient care.  However, nutrition and GI issues remain an important part of the disease.

What is Cystic Fibrosis?

CF is an inherited disease that affects males and females from all racial and ethnic groups, but is common in persons of northern European descent.  It is a long-term (chronic), progressive disease caused by defects in a channel that controls salt and water balance in the body. This means that many body organs and systems can be affected by thick, sticky mucus secretions. These include the sinuses, lungs, pancreas, liver, gallbladder, intestines and reproductive organs. Children with CF may experience coughing, wheezing, difficulty with breathing, lung infections, sinus infections, belly pain, trouble with bowel movements, liver disease, poor weight gain and growth, and more.

How is CF diagnosed?

It can be diagnosed through a screening process at birth. If the screening result is abnormal, babies should have a follow-up test called a sweat chloride test where a small sample of sweat is collected and the amount of salt is measured.

It is important that babies with CF are treated in a clinic setting where the only patients are babies and toddlers to reduce the risk of exposure to bacterial organisms that would be present in the lungs of older children with CF. There are also other tests involved in managing CF including GI tests to see if there are problems with digestion, chest x-rays, lung function tests, respiratory tests and DNA tests.

Not all patients with CF are diagnosed at birth, so if your child has chronic breathing, GI, or sinus issues, it is important to talk with your doctor about testing for CF.

How can parents help with care?

  • Ensure your child receives chest physiotherapy (CPT) as prescribed to help remove mucus from the lungs.
  • Provide aerosol breathing treatments when needed.
  • Give antibiotics when prescribed by your child’s physician, even for mild, cold-like symptoms.
  • Keep your child away from cigarette and e-cigarette smoke.
  • Know that sometimes it may be necessary for your child to be hospitalized for IV antibiotic therapy.
  • Ensure your child has proper nutrition. Food can be challenging for patients with CF not only because of some of the symptoms they may have, but also because they need to consume a high amount of calories.

Multiple specialists should discuss your child’s needs, medication and management together to come up with a well-rounded treatment plan with your pediatric pulmonologist. These specialists include:

  • Gastroenterologist
  • Endocrinologist
  • Ear, Nose and Throat Specialist
  • Respiratory Therapist
  • Physical Therapist
  • Pharmacist
  • Audiologist
  • Social Worker
  • Psychologist
  • Child Life Specialist
  • Dietitian
  • Nurses
  • Research Coordinators

This team approach helps to reduce the number of clinic visits and improves how well your child feels. Keeping your child as healthy as possible not only affects how they feel today, it also affects how they will feel in the future. While many people think CF is a childhood disease, because of research and improved clinical care, more and more children with CF are living longer lives.  We have reached an incredible juncture in CF where there are as many adults living with CF as children, a true testament to the hard work of the CF families, clinical teams, and researchers across the country.

If you would like to learn more about the CF program and our team approach to care at Nationwide Children’s Hospital, click here.

Benjamin Kopp, MD
Dr. Benjamin Kopp is an Assistant Professor in the Section of Pediatric Pulmonology and a Principle Investigator in the Center for Microbial Pathogenesis at Nationwide Children’s Hospital. He is avid cystic fibrosis researcher focused on novel therapies for infection. He has also started the pulmonary/sickle cell clinic to provide an integrated medical home for patients with sickle cell lung disease. He is a proud father of 2 children who keep him and his wonderful wife on their toes! Dr. Kopp is a buckeye for life, and loves to talk sports superstitions. Follow Dr. Ben on Twitter @KidsLungDoc for the latest pulmonary research updates and tips on how to manage chronic lung disease.

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