The Cystic Fibrosis (CF) Center at the University of Florida is accredited by the Cystic Fibrosis Foundation. The Foundation accredits and provides partial funding for interdisciplinary pediatric and adult cystic fibrosis care centers nationwide. Staffed by dedicated professionals, these centers provide specialized CF care. Each center undergoes a thorough review by the CF Foundation’s Care Center Committee before it receives accreditation and funding. This review for accreditation occurs every year.
Care centers also participate in CF clinical research and work to educate healthcare professionals and families about CF and the care people living with the disease require. Data from people with CF is collected in the national CF Foundation Patient Registry, which serves as a rich resource for CF health care providers and CF researchers to track the health of people living with CF in the United States.
What is Cystic Fibrosis (CF)?
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive systems of about 30,000 children and adults in the United States and 70,000 people worldwide. CF is caused by a recessive gene, which means that a child must inherit two copies of the defective CF gene—one from each parent—to have the disease. It is most often found in Caucasians, but it can be found in people of all races. Because of a defective gene and the protein it produces, the body manufactures unusually thick, sticky mucus. This mucus clogs the lungs and can lead to life-threatening lung infections. It also can obstruct the pancreas and block the release of sufficient pancreatic enzymes to help the body break down and absorb food. Other systems can be affected as well.
The treatment of CF depends upon the severity of symptoms and the organs involved. Most people with CF must take pancreatic enzyme supplements with every meal to absorb enough calories and nutrients to grow and stay healthy. They also must eat a high-calorie, high-fat diet. People with CF also perform daily airway clearance therapy to help clear mucus from the lungs. Other types of treatments include antibiotics to fight lung infections and drugs to thin the mucus and improve lung function.
CYCTIC FIBROSIS FOUNDATION
CF clinics are held on Tuesdays in the Shands Medical Plaza located at 2000 Archer Road, on the second floor in the pediatric specialties clinics. These special CF clinics are staffed by the interdisciplinary team of physicians, nurses, nutritionists, respiratory care, social work, and clinical psychology.
Patients are scheduled carefully based on their respiratory cultures to prevent transmission of resistant organisms. This scheduling is called “cohorting” and each patient will be assigned to an appropriate clinic by a color designation: white, orange, blue, red, or green. When calling to schedule an appointment, knowledge of your cohort will assure correct scheduling for you/your child. Return appointments are made at each clinic visit. If you need to change your appointment, you should call (352) 265-8250 and tell the scheduler which CF clinic you need.
Every effort is made in the CF clinic to prevent the transmission of organisms between patients. Clinic appointments are made according to the cohort plan as described in the scheduling section above. This prevents individuals with very resistant respiratory organisms from being in the clinic at the same time as patients who have organisms less difficult to treat. In addition, all patients should request a mask at the check-in desk when they arrive and everyone should use the hand disinfectant located outside the rooms upon entering and leaving. The pulmonary function testing room and all patient rooms are disinfected between patients.
Guidelines for Hospital Admissions
People with CF are usually admitted to the hospital when treatment with oral and inhaled antibiotics has failed to get lung function back to their baseline (best PFT’s they can get) or get their cough and/or weight under control. Experience has taught us that rest, intensive airway clearance, high quality and high-calorie diet along with IV antibiotics are necessary to get the best gains in health. To accomplish the best results, the healthcare team and the patient/family work in partnership. The patient’s part of this includes being in their room at times when treatments are scheduled, getting a good night’s sleep so they can be rested and be able to participate in their care during the day, doing their part to maintain good infection control, eating lots, and sharing the details of their progress with the physician team. The healthcare team will design and implement a plan of care, communicate the plan with the family, and be open to input if there are aspects of the plan that the family is uncomfortable with.
Infection control in the hospital is extremely important to protect everyone. We know that people with CF can transmit organisms to each other through coughing or by contact with organisms on a surface. These organisms are then carried by the hand to the mouth and into their lungs. There are also patients in the hospital units who have “suppressed immune systems” making their natural defenses too weak to resist pathogens that could make them dangerously ill.
Thus, infection control in the hospital is taken very seriously. If you are asked to remain in your room it means that you are a risk to other people. When you are allowed to leave your room, you must always disinfect your hands before leaving and again on your return and wear a mask until you are off the hospital unit. You may even be asked to change into a clean set of clothing before leaving the room. This is to protect you as well as others.
- Cystic Fibrosis Foundation
- CF at School: Educating school personnel about CF
- Living with Cystic Fibrosis
- Therapies for Cystic Fibrosis
- CF School letter
- CF School Medication Authorization form
- Blank School Medication Authorization form
- CFF educational materials
- Florida statute – Child self-administering enzymes