All 50 states require newborn screening for cystic fibrosis, and pediatricians play an important role when a newborn is diagnosed. The positive screens from your state laboratory will include referral options for confirmatory testing and specialty care at an accredited Cystic Fibrosis Center or CF center affiliate in your area. Pediatricians remain the patient’s "medical home," help to coordinate this specialty care, and remain in close communication with the patient, families, and specialists at a CF center as these infants grow through childhood, adolescence, and into adulthood.
Optimal outcomes come from assessment and treatment by a multidisciplinary team at an accredited CF center. Encourage your CF patient and families to be seen there regularly. It is the same strategy you would use for someone with a chronic condition such as severe asthma, diabetes, juvenile arthritis, or inflammatory bowel disease. You want those specialists on board to help you take optimal care of the child.
By Dr. Lucille Lester
Monitoring at least every 3 months is recommended and is called "expectant" or "proactive management" for an individual with CF. A more thorough assessment occurs at least annually.
Pediatricians are essential, in particular, when patients live far from a CF center. We have patients who live a 3-hour drive from our center at the University of Chicago. In some states, patients may live hundreds of miles from a center and only can be seen for specialty care once per year.
There are some specialist outreach efforts as well for children who live in extremely remote, rural areas. For example, clinicians in Seattle fly to Anchorage, Alaska to monitor and treat children with CF.
If a sick child with CF comes as a new patient to your office and the records are not available, you should always feel free to call the closest CF center to speak to one of the specialists there and eventually refer them for care. Physicians at CF centers work in collaboration with primary care physicians who are the "go to" professionals, often treating other members of a CF child’s family.
Communication and Assessment Key in CF Treatment
Consultation and referral are important. Pediatricians tend to be very, very busy and see 20-40 patients a day in their office for a wide range of indications, including earaches, stomach aches, and well-child exams. But if you are taking care of a sick child with cystic fibrosis who is just not getting better with antibiotic or other treatment, definitely communicate with a CF care provider.
Many pediatricians we know well in the Chicago area will call us up and say, "I have so-and-so in my office. They are coughing a little bit more. I know you sent a letter before saying I should try this particular antibiotic. Is there anything else I should do at this point?" They stay in close touch, and we always have a center physician or nurse available to speak with them.
Regular assessment by a pediatric pulmonologist is part of the CF center care. If a child has an increased cough and they have certain bacteria such as Pseudomonas, we aggressively treat them so they don’t get worse. We monitor lung function and obtain frequent respiratory cultures.
In addition, up to 90% of these children and adolescents develop digestive problems and may benefit from consultation with a gastroenterologist. Some patients develop sinus-related problems and referral to an ENT may be warranted. Accredited centers are required to provide these specialists, as well as dieticians, social workers, and respiratory therapists, who are experienced and knowledgeable about CF and who are involved in providing a care plan for children with CF.
Continued Testing for Young CF Patients
Pediatricians play an important role in the facilitation of regular testing of children and adolescents with cystic fibrosis. At a minimum of once a year, they require blood work and chest x-rays. Many patients do their annual visits in the summer because testing takes almost a whole day. The lab work and chest x-ray copy (on a CD) can then be brought to CF physicians on the day of that "annual" visit. Depending on the patient’s insurance, it may be less expensive and more convenient for the family for the pediatrician to coordinate this annual testing at the local community hospital. Lung function testing typically needs to be done at the center.
We also now do an oral glucose tolerance test for CF-related diabetes in any patient older than 10 years. That often can be done locally as well. Patients with cystic fibrosis may develop a specific type of diabetes. Most patients with CF have pancreatic insufficiency, which results in problems with digestion and the need to take medication (pancreatic enzymes) with every meal. Over time, scarring of the pancreas results and insufficient insulin may be produced.