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 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 of closely monitoring 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. 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 with:

• Meticulous monitoring
• Treatment with Pulmozyme®
• Immediate commitment to Chest Physiotherapy — improves adherence and improves the child's overall health
• As an academic institution connected to ongoing research, we are always aware of new treatment methods as they appear.

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, including:

• 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 well-defined treatment plan tailored to the needs of your child, including:

• Review health maintenance being done, or not done, with suggestions for improvement
• Oral antibiotics and/or inhaled antibiotics
• Outpatient IV antibiotics will take the place of oral/inhaled antibiotics when necessary
• Close monitoring of spirometry will determine continued need for therapy
• Continued failure to improve spirometry or other concerning trends such as weight loss, poor appetite, fatigue, sinusitis will lead to hospital admission

Success of Outpatient Care

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.

• 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