Pediatric Cystic Fibrosis

Each member of our cystic fibrosis, or CF, team is passionate about providing your child the most comprehensive and aggressive preventive care possible. We take a highly personalized approach and will partner with you for the care of your child. In addition to physicians and nurses, your child's multidisciplinary care team may include:

As an academic medical center connected to ongoing research, we learn of treatment advances as they are developing. Our patients are often among the first to benefit from these new therapeutic discoveries.

Mom holding her baby at doctor's appointment.

Providing your child care that is needed

Helping young patients maintain excellent health affords them the best opportunity for longevity and improved quality of life. We believe our patients should be able to achieve a normal height and weight. Any children who are felt to be at risk receive early nutritional intervention, including:

We also recognize the importance of closely monitoring infants and toddlers who have been diagnosed with CF but have not yet developed symptoms. Research shows that children display evidence of disease activity long before overt symptoms develop. There are many benefits to beginning treatment at an early age — even before symptoms start — including developing habits that can help maintain your child's health as the condition advances.

Despite the best efforts of parents and the healthcare team, children with CF do get worse at times. Using the resources of the entire CF team, we will create a well-defined treatment plan tailored to the needs of your child that includes:

  • Review of health maintenance being done, or not done, with suggestions for improvement
  • Oral antibiotics and/or inhaled antibiotics
  • Outpatient IV antibiotics taking the place of oral/inhaled antibiotics when necessary
  • Close monitoring of spirometry to determine continued need for therapy

Continued failure to improve spirometry or other concerning trends, such as weight loss, poor appetite, fatigue or sinusitis will lead to hospital admission.

Exceeding national standards of care

Our specialists adhere to the practice guidelines of the National Cystic Fibrosis Foundation. You can be assured that we will provide thorough and complete oversight of your child's condition through:

Need help making an appointment?

Call 913-588-1227 or request an appointment online.

Patient care

Our patient-centered approach allows us to provide the best possible care for children with cystic fibrosis, in both our inpatient and outpatient facilities. Learn more about how we can help you provide the best care possible for your child.

  • Pediatric clinic services
  • Pediatric same-day sick clinic
  • Pediatric inpatient services

Diagnosis

If you are a primary care provider needing to schedule a sweat test, please call 913-588-6364 or fax your request to 913-588-6167.

Diagnosis of cystic fibrosis

  • Newborn screening occurs in all 50 states. This blood test, often known as a newborn screen, is performed in the first 2 weeks of a child’s life. The newborn screen includes screening for cystic fibrosis. If the newborn screen comes back positive for possible CF, a sweat chloride test is done to either rule out or confirm a diagnosis. The sweat test will measure the amount of salt in a child’s sweat. This is often the only test that is needed for diagnosis. However, your child may have other tests to evaluate his or her condition, including:
    • Stool evaluations to check for absorption of fat.
    • DNA tests to confirm diagnosis, if needed.
    • Sweat Chloride Test

Outreach clinic

Learn about our outreach clinic located in Pittsburg, Kansas.

Family Advisory Council

The pediatric cystic fibrosis team provides a Family Advisory Council. The mission of the council is to develop activities to ensure that we meet the needs of our patients and families by providing the education and support necessary for success in caring for their children.

The council is led by our patient family members and caregivers. CF staff support the group’s overall goals and listen to ideas on how we can best serve the needs of our families. Your voice and participation is essential to the continued growth and quality of the pediatric CF program.

If you are interested in becoming a member of the Family Advisory Council, contact Lyndsay Marriott at 913-588-6320 or by email.

Pharmacy

Your CF pharmacist

As a part of your cystic fibrosis care team, your CF pharmacist helps you understand the benefits and possible side effects of your medications. By assuring that your dose and medication timing are right for you and your lifestyle, your CF pharmacist can help make sure you are getting the maximum benefit from your medications. Your CF pharmacist is available in clinic, by email or phone for any medication related questions or issues.

We offer services to help you stay on track with your treatment plan:

  • Free shipping with tracking numbers
  • Pharmacy patient advocate to assist you with insurance, billing and medication payment assistance questions
  • We can contact you each month before your refills are due or set you up for automatic refills
  • We can send a text message when medications are ready to be picked up or have been shipped
  • Free parking with validation and medication pick-up from the pharmacy

Research

Pediatric clinic services

Our cystic fibrosis team is passionate about providing patients the most comprehensive and aggressive preventive care possible. We take a highly personalized approach, and partner with you to care for your child. Our work is guided by the philosophy that maintaining excellent health as a young patient affords the best opportunity for longevity, improved quality of life and opportunities to benefit from new therapeutic discoveries emerging from CF research.

Care standard

Our care center is accredited by the National Cystic Fibrosis Foundation. Our team follows care guidelines put forth by the CF Foundation, which include:

  • Clinic visits every 3 months for patients 2 years and older. Patients under age 2 are seen more frequently, per CF Foundation care guidelines.
  • Quarterly throat/sputum cultures
  • Quarterly spirometry
  • Annual chest X-rays
  • Annual blood testing
  • Annual mental health screening for of-age patients and caregivers
  • Quarterly visits with our social worker, dietitian and respiratory therapist

Aggressive preventive care

We recognize the importance of closely monitoring infants and toddlers who have been diagnosed with CF, but have not yet developed symptoms. Research shows that children display evidence of disease activity long before overt symptoms develop. There are many benefits to beginning treatment at an early age, even before symptoms start, including developing habits that can help maintain your child's health as they grow older.

Pediatric FAQs

  • A sweat test may be requested by the physician when a patient has symptoms of CF, a family history of CF or when a newborn has a positive screening test. During the sweat test, sweat is stimulated and a small amount is collected from the forearm. The amount of salt in the sweat is measured in the clinical laboratory.
  • Two electrodes containing the sweat stimulating drug pilocarpine are placed on your child's forearm or thigh for 5 minutes. The stimulation causes the sweat glands to produce sweat. This process can cause a mild tingling feeling during the stimulation, but is not painful. A collection device is placed to collect the sweat for 30 minutes. Once the sweat has been collected, it is taken to the lab for testing.
  • It takes approximately 1 hour to complete the sweat test collection.
  • Yes, parents stay with the child. The test is not painful. It helps if children have something to keep them busy (such as a pacifier, bottle, special toy or book) during the test.
  • Results are ready about 1 hour after the sweat test. A CF provider will see you in the clinic immediately following the sweat test. Test results are also sent to your doctor.
  • No, diet will not affect the sweat test results.
  • Your child should keep taking medicines as prescribed.
  • For questions, call the pediatric cystic fibrosis care center at 913-588-6364 or 913-588-6320.

Respiratory therapy

Our respiratory therapist attends every clinic visit. The visit with your RT includes inhaled medication review, chest physiotherapy compliance and review of airway clearance techniques.

Pulmonary function testing will be performed at each clinic visit beginning at age 5. PFTs measure how well your child’s lungs are working. Changes to the airflow within the lungs is measured with values called FEV1 and FVC. FEV1 is the forced expiratory volume of air that is forcefully exhaled in 1 second. FVC is the forced vital capacity, which is the total volume of air that is forcefully expelled. Monitoring these values for significant changes can alert your child’s care team to illness or damage to the lungs.

*Important note: Our clinic follows the infection control guidelines of the Cystic Fibrosis Foundation. Therefore, in the outpatient setting, PFTs are performed in the clinic at the start of the visit, and in the inpatient setting, PFTs are done in the patient’s hospital room.

Testimonials

"The staff are fantastic. They're cheerful, they ask questions, and make sure that I understand what's going on. They're quick, they get me in on time almost always, and they seem to care individually about my children."

"They are very knowledgeable about CF and how best to help my son. They are prompt and caring. They know him."

"I like that the team takes a personal interest in my child, and that my child is not just a number to them. They truly care about her and, by extension, the rest of the family. They take the time to talk to her on her level and me on mine."

We are here to partner with you for the care of your child. Close contact with our patients by phone and our accessibility to patients for questions is the key to our success.

Appointments and referrals

The cystic fibrosis clinic for pediatric patients occurs Tuesday afternoons, as well as 1-2 Fridays every month (usually the second and/or fourth Fridays of the month).

To schedule an appointment, call 913-588-6364 or 913-588-6320. If you need to reschedule or cancel an appointment, please notify our team 24 hours prior to your appointment.

Fax records to 913-588-6167.

Pediatric inpatient unit

Despite the best efforts of caregivers and the healthcare team, children may develop illnesses. Using the resources of the entire CF team, we will create a well-defined treatment plan tailored to the needs of your child that includes:

  • Review of health maintenance being done with suggestions for improvement
  • Oral antibiotics and/or inhaled antibiotics
  • Outpatient IV antibiotics taking the place of oral/inhaled antibiotics when necessary
  • Close monitoring of spirometry to determine continued need for therapy

Continued failure to improve spirometry or other concerning trends, such as weight loss, poor appetite, fatigue or sinusitis, will lead to hospital admission.

Pediatric same-day sick clinic

We offer same-day appointments for illness or injury that does not warrant a trip to the Emergency Department, but that still may need urgent attention. Call 913-588-6320 if you believe that your child may need to be seen by a physician that day.

To learn more about our CF Center, email our CF Nurse Coordinator, Lyndsay Marriott.