Cystic Fibrosis

Team Philosophy

Each team member is passionate about providing our pediatric patients with the most comprehensive and aggressive preventive care possible. We work from a philosophy that maintaining excellent health as a young patient affords them the best opportunity for longevity, better quality of life, and opportunities to benefit from new therapeutic discoveries emerging from CF research.

Meticulous monitoring

We adhere to the practice guidelines of the National Cystic Fibrosis Foundation, which promote preventive care and close follow-up of all patients. We feel it is our duty to provide thorough and complete oversight of our patients’ health status:

• Quarterly visits
• Quarterly Spirometry
• Quarterly sputum cultures
• Yearly chest x-rays
• Yearly blood testing

Asymptomatic Infants/Toddlers

We recognize the importance to closely monitor infants and toddlers who have been diagnosed with CF but have not yet developed symptoms. Research has shown that children have evidence of disease activity long before overt symptoms develop:

• Bronchial Lavage shows inflammation from birth
• High Resolution Computerized Tomography (HRCT) of the chest reveals bronchial wall thickening and early bronchiectasis
• Infant pulmonary function tests show airway obstruction

Beginning treatment at an early age — even before symptoms start — yields many benefits, including improved adherence when symptoms worsen in the future.

Aggressive Outpatient Care

We maintain a philosophy that aims at keeping children healthy and out of the hospital.

• Meticulous monitoring
• Treatment with Pulmozyme®
  • Decreases inflammation
  • Decreases exacerbations
  • Increases lung function
  • Excellent safety profile
• Immediate commitment to Chest Physiotherapy- improves adherence and improves the child’s overall health.
• With first-time culture of Pseudomonas aeruginosa
  • 3 months of inhaled TOBI® plus oral ciprofloxacin for 3 weeks
  • If these measures fail to eradicate the bacteria, the child can be given IV antibiotics as an outpatient
  • Despite these measures, experience shows that most children with CF will develop permanent colonization with Pseudomonas — Chronic TOBI can be used as a suppressive therapy

New Therapeutic Modalities

As an academic institution connected to ongoing research, we are always aware of new treatment methods as they appear.

• Tri-weekly Azithromycin
  • Anti-inflammatory properties and affects Pseudomonas binding
  • Excellent results showing prolongation of life in panbronchiolitis
  • Three large studies (Europe, Australia, US) in CF patients showing improvement in lung function and reduction in exacerbations.
  • Recent recommendations by the CF Foundation for all patients with positive Pseudomonas cultures

Growth Expectations

We believe that all 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:

• Increased outpatient visits
• Oral supplementation
• Early gastrostomy placement
• Plans for calorimetry monitoring in clinic
• Surveillance for liver disease
• Surveillance for CF related Diabetes

CF Exacerbations

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

• Review health maintenance being done, or not done, with suggestions for improvement.
• Oral antibiotics and/or inhaled antibiotics
• Outpatient I.V. antibiotics will take the place of oral/ inhaled antibiotics when necessary
• Close monitoring of spirometry will determine continued need for therapy with the goal of maintaining a child at his or her best
• Continued failure to improve spirometry or other concerning trends such as weight lose, poor appetite, fatigue, sinusitis will lead to hospital admission

KU Practice Compared Nationally

The University of Kansas Cystic Fibrosis clinic compares quite favorably to programs across the nation, with more patients treated successfully as outpatients and better usage of preventive treatments.

	University of Kansas	National Cystic Fibrosis Foundation
Proportion hospitalized	28.6%	32.2%
Proportion on home IV	19%	13.8%
Median length of hospital stay	6 days	9 days
Median home IV use	17 days	11 days
Pulmozyme usage	81.1%	64.9%
TOBI usage	69.9%	64.3%

Cystic Fibrosis Education

As in everything, knowledge is power. We understand the importance of education for our patients and families. We also strive to remain abreast of the latest advances in treatment.

• Patient Education
  • Respiratory Therapist
  • Registered Dietician
  • Nurse Practitioner
  • Social Worker
• CF Team commitment to Self Education
  • Most Team members attend the annual National CF Meeting

Cystic Fibrosis Experience

• CF Nurse Practitioner – 30 years as RN, 22 in CF
• Registered Dietician – 27 years as RD, 12 in CF
• Respiratory Therapist – 20 years as RT, 5 in CF
• Social Worker – 28 years as SW, 4 in CF
• Medical Doctors – combined experience of 40+ years
• Every patient is seen by M.D., not by NP alone.
• Continuity of care available in the same institution as patient becomes adult.

Success of Outpatient Care

We are fully convinced that close contact with our patients by phone and our accessibility to patients for questions is the key to our success.

• Our small intimate program allows for personalized care.
• CF Nurse Practitioner/M.D. is available 24 hours a day.
• Patients who fail to keep appointments are called and encouraged to follow up.

In short, we are here to partner with you for the care of your child.

The KU Cystic Fibrosis Clinic:

Location: Main Pediatric Clinic, HC Miller Building
Office Phone: 913-588-6377
Appointment Phone: 913-588-6381
For after-hours emergencies, please call the hospital operator at 913-588-5000 and ask for the Pediatric Cystic Fibrosis staff on call.
The toll-free number for the KU Medical Center is 1-800-332-4199

The KU Cystic Fibrosis Center Staff:

• Mitzi Scotten, M.D.
• Adrienne Liebergen, ARNP.