Cystic Fibrosis
Meet the Team
Parents; you are the most important team members!
You are your child's caregiver. The cystic fibrosis (CF) team is here to support you and provide you with the resources necessary for your child to grow up with the brightest future.
The CF team members are physicians, nurse practitioners, nurses, social workers, nutritionists, respiratory therapists, physical therapists, child life specialists, psychologists, pharmacists, genetic counselors, care managers, and integrative medicine specialists.
We are also your coaches, cheerleaders, and fans, rallied around three central goals:
- To give the best possible care.
- To keep your child in the best possible health for new therapies.
- To see your child graduate to an adult CF center with normal lung function and normal nutritional status.
More information about the CF team:
At Children's, a multidisciplinary team experienced in pediatrics focuses on compassionate care and thorough management of your child's health care needs.
John McNamara, M.D.
Medical Director
Michael Pryor, M.D.
Associate Medical Director
Christopher Steffen
Specialty Care System Clinic Manager
Mary Sachs, RN, CNP
Coordinator
During your visits, you may meet some or all of these specialists:
Pediatric Pulmonologist and Certified Nurse Practitioner
A pediatric pulmonologist is a doctor who specializes in the treatment of respiratory systems in children. A certified pediatric nurse practitioner is an advanced practice nurse with additional education and training in pediatrics and children with special needs.
These are the physicians and nurse practitioners who manage your child's CF care. Working closely with your child's primary care physician, they perform histories and physical examinations, order diagnostic tests, make diagnoses, order treatments, and direct the CF care your child receives.
You will see these specialists during CF clinic visits and during hospitalizations. They develop treatment plans in collaboration with other team members. In addition, they help maintain your child's health, anticipate health needs, and provide education and family support.
CF Coordinator
The CF coordinator is an advanced practice nurse who helps coordinate your child's care and education during the hospital stay. The coordinator teaches you and your child about CF and helps inform other team members about your questions and concerns. In addition, the coordinator directs our research activities.
Care Manager
While in the hospital, the care manager helps your child's primary care nurse coordinate your child's stay. The care manager can address insurance issues and will help you find medical resources and obtain home medical equipment upon discharge.
Chaplain
Chaplains offer you and your family spiritual support. They help you and your child adjust to life with a chronic illness by looking at what brings you hope and resiliency. Chaplains also are available to assist with religious or spiritual needs you may have around your child's illness.
Child Life
Child life staff help minimize your child's stress while in the hospital. They help your child understand and cope with treatments. They also support your child's growth and development with age-appropriate activities.
Exercise Physiologist
The exercise physiologist complement your child's treatment by improving health, fitness, energy, and performance through exercise. At the Kohl's PowerCubed Exercise Medicine Center, staff develop an exercise plan for your child and evaluate outcomes.
- Learn more about the Kohl's PowerCubed Exercise Center at Children's
- See our Patient/Family Education handout about CF exercises
Genetic Counselor
The genetic counselor helps you understand how CF runs in families. The counselor also arranges genetic testing and interprets these tests for you and your family.
Hospital Patient Care Nurse
Children's registered nurses are experts in helping patients and their families learn about CF and their medications, and in providing the care patients need. CF patients range from infants to young adults. Children's nurses understand the different needs of each age group. Your child's nurse will work with you to make sure you have the information you need to care for your child at home.
Integrative Medicine Program
Staff in Children's Integrative Medicine program include pediatricians, psychologists, nurses and other health professionals who blend the best of conventional and complementary therapies. Services are provided both inpatient and outpatient, and include assessment, treatment and consultative services. Therapies may include biofeedback, imagery/self-hypnosis, massage, healing touch, acupoint stimulation, psychotherapy, and mind body skills groups. Integrative medicine services will help your child develop self-management skills and stress-management strategies. Children with symptoms of anxiety, stress, pain, sleep problems and adjustment issues related to CF may also benefit from Integrative Medicine services.
Nutritionist
Nutritionists teach you and your family about the importance of nutrition for a child with CF. They will provide information and counseling about infant formulas, vitamins, high-calorie and high-protein diets, pancreatic enzyme supplements, and dietary supplements.
Pharmacist
Pharmacists review and dispense all medications. While at Children's, a clinical pharmacist monitor your child daily for drug effectiveness and interactions, and is available to address your medication questions and concerns.
- Learn more about pharmacy services at Children's
- See our Patient/Family Education handout about CF medicines
Physical Therapist
Physical therapists will teach you and your family about the benefits of exercise. During a hospital stay they provide you child with age appropriate physical activities. They create a program for your child to help improve strength, flexibility, and aerobic capacity in the hospital and at home. They can also help your child become involved in community activities such as team sports.
Psychologist
Psychologists will help you and your child understand and cope with the demands of a chronic illness and adjust to a medical routine. They also can help by evaluating emotional reactions and learning or attention problems. Psychologists will assist with behavioral management for children of all ages.
Pulmonary clinic nurse
The pulmonary clinic nurse coordinates your child's outpatient care. This includes addressing your concerns and questions when you call the clinic and helping with appointment scheduling. The pulmonary clinic nurse also provides you with information about CF during clinic visits.
Respiratory Care Practitioner
Respiratory care practitioners will perform all airway clearance treatments, aerosol therapy, and pulmonary function testing while your child is in the hospital. They will work closely with you to help you learn the skills to administer respiratory therapy treatments for your child at home.
Social Worker
Social workers help you get the emotional support you and your child need during hospitalizations, clinic visits, and at home. The social worker also helps answer questions you may have about the financial aspects of your child's care.
Other Affiliated Specialists
Sometimes your child may need the expertise of other specialists if she/he develops complications of CF related to other body systems. At Children's we coordinate with experts in the following fields:
- Ear, Nose, and Throat (ENT)
- Gastroenterology (GI)
- Endocrinology
- Genetics
Outcomes
In 2009, Children's cystic fibrosis program was designated a Therapeutic Development Network Center. Since this designation, more than 60 percent of Children’s patients with cystic fibrosis annually participate in one or more research studies.
- Safe
- Timing
- Efficient
- Effective
- Patient-Family Centered
- Equitable
These six areas for measuring quality of care were developed by the Institute of Medicine and are widely used by health care organizations. If you have a question about Children's data, we encourage you to speak to your physician. We are happy to talk to you about program outcomes and the prognosis for your child.
Safe
Interpretation: None of our patients reported second hand smoke exposure in 2009.
What are we doing to improve: We continue our smoking cessation efforts and have a certified smoking cessation specialist on staff.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 cystic fibrosis patients in National Cystic Fibrosis Registry
Interpretation: Children's influenza immunization rate is well above the national average, with over 80 percent of patients being immunized.
What are we doing to improve: All patients are encouraged to receive yearly flu vaccines and are educated about it's importance of preventing disease.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 cystic fibrosis patients in National Cystic Fibrosis Registry
Timely
Interpretation: Cystic fibrosis is a chronic, lifelong disease that requires regular treatment to maintain health. It is recommended that children with cystic fibrosis see their provider at least four times, have at least one respiratory culture, and two pulmonary function tests per year. Children's is slightly above the national average in meeting this recommendation.
What are we doing to improve: We are working on new strategies through our EMR to improve communication and follow-up.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 cystic fibrosis patients in National Cystic Fibrosis Registry
Efficient
Interpretation: Oral glucose tolerance test (OGTT) is used to screen patients for cystic fibrosis-related diabetes. Glucose screening in nondiabetic cystic fibrosis patients is recommended yearly for patients 14 years and older. Children's has the second highest screening rates for diabetes, with two-thirds of our patients receiving the recommended screening.
What are we doing to improve: We are participating in a leadership and learning collaborative sponsored by the CF Foundation to improve outcomes of patients with CF-related diabetes.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 CF patients in National Cystic Fibrosis Registry
Interpretation: Children's length of stay for patients with pulmonary exacerbations is the same as the national average.
What are we doing to improve: We are examining several strategies to reduce hospital length of stay including home IV therapy.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 CF patients in National Cystic Fibrosis Registry
Effective
Interpretation: Lung function is measured by FEV1, or Forced Expiratory Volume over one second. A higher FEV1 rate is related to better lung function. Children's performs better than the national average.
What are we doing to improve: We are currently involved in a quality improvement project to ensure our patients are on CF Foundation approved therapies.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 cystic fibrosis patients in National Cystic Fibrosis Registry
Interpretation: Body Mass Index (BMI) is an indicator of nutritional status that is calculated using a child's height, weight, age and gender. In patients with cystic fibrosis (CF), a higher BMI is related to better lung function. Patients with a BMI above the 50th percentile range have the best pulmonary outcomes. Most of Children's patients have BMI's above the 50th percentile.
What are we doing to improve: We encourage families to be seen in the clinic every three months to identify nutritional problems. We also have a psychologist as part of the cystic fibrosis team to help families with feeding and nutrition issues.
Data source: National Cystic Fibrosis Registry
Benchmark data: 25,638 cystic fibrosis patients in National Cystic Fibrosis Registry
Patient-family centered
Interpretation: Most families said they had complete trust and confidence in their provider. Eighty-seven percent said they were involved in decisions about their child's care as much as they wanted.
What are we doing to improve: Children's CF team is dedicated to involving patients and their families in their care by surveying families annually. We are also in the process of developing a parent advisory council for patients with CF.
Data source: Family Survey
Benchmark data: Not available
Equitable
This measure is still in process.
Cystic Fibrosis
Cystic fibrosis
The Cystic Fibrosis (CF) Center at Children's of Minnesota diagnoses and treats children in all stages of CF. Our dedication to family-centered care and education helps children and their families learn to live with CF.
CF care is a leading programs at Children's, called a cornerstone program. These are major programs meeting rigorous criteria for excellence, including clinical research, use of advanced technology, and evidence-based practice.
Care at Children's for patients with CF ranks among the top 10 programs nationally in key outcomes measured by the National Cystic Fibrosis Registry. Children's provides a continuum of care through coordinated inpatient and outpatient services, from diagnosis through long-term follow-up. The Cystic Fibrosis Center of Children's provides state-of-the art comprehensive care for children with CF.
Our team approach to care
Meet other affiliated specialists
Outcomes and quality measures
What is Cystic Fibrosis?
CF is a chronic, progressive, life-limiting genetic disease that affects about 30,000 people in the United States. An altered gene causes cells to produce mucus that is thicker and stickier, resulting in blocked ducts which affects the function of certain organ systems.
New therapies and treatments are constantly improving life expectancy and quality of life for people with CF. In fact, 40 percent of people with CF are adults.
What is cystic fibrosis?
How is cystic fibrosis diagnosed?
What is a genetic disease?
Learn about newborn screening
- Children's CF Team created a newborn screening video for parents. To view, click here.
"Understanding newborn screening for cystic fibrosis" brochure
Ongoing Cystic Fibrosis Care
The Cystic Fibrosis Center at Children's provides state-of-the art care to monitor every aspect of your child's cystic fibrosis. Care may include scheduled annual vistis; or, sometimes children with CF need to be admitted to the hospital when they are sick.
Learn more hospital and clinic visits:
Clinic visits
Hospital visits
Helpful resources when visiting Children's
L.I.F.E. with Cystic Fibrosis Education Sheets:
L.I.F.E. stands for Lifetime Involving Fitness and Exercise. The Cystic Fibrosis disease process can cause negative changes in the musculoskeletal system, therefore, fitness and exercise aids in preventing a decline in this system. Listed below are suggestions of age-appropriate exercise activities that focus on areas that are affected by CF.
Infants
Ages 1-3
Ages 4-6
Ages 7-12
Age 13+
Children's wins award at cystic fibrosis conference
At the North American Cystic Fibrosis Conference in Baltimore Oct 21-23, 2010, Children's was recognized with a Quality Care Award Recognizing Outstanding QI Processes and Accomplishments for sustained commitment to quality improvement resulting in improved outcomes. We were also awarded an Outstanding Partnership Award for our center's work with the Minnesota Chapter of the Cystic Fibrosis Foundation, where John McNamara, MD, serves as a member of the Board of Directors.
