Cystic Fibrosis Services
At our St. Vincent Center, we have an adult CF specialist and a pediatric CF specialist. They are responsible for many aspects of your care, including regular physical examinations, managing medications and other treatments, and ordering/analyzing appropriate lab/diagnostic tests. They provide in-hospital and outpatient care and will refer you to other medical specialists if/when necessary.
Medical Nutrition Therapy
Achieving optimal nutrition status is imperative for patients with Cystic Fibrosis to maintain good lung health. A registered nutritionist or dietician will be available to help determine a medical nutritional therapy that ensures that you receive the nutrients and calories you need. The CF team dietician will monitor your growth patterns (height and weight), your ideal body weight, and body mass index (BMI) very closely at every visit to help identify ways to help you meet your nutritional goals and to make sure that growth patterns/weight stability is on target for your age. A nutrition plan of care is tailored for each patient to help nurture success. The plan of care includes the following.
- Adjust/monitor pancreatic enzymes to minimize gastrointestinal symptoms.
- Develop methods to increase nutrient density of foods (including use of nutrition supplements and tube feedings) to optimize growth.
- Identify/manage the nutrition plan of care for patients who develop Cystic Fibrosis Related Diabetes.
The Cystic Fibrosis Dietitian, along with the other members of the Cystic Fibrosis Team, is committed to providing the best nutrition plan of care for each patient.
Social Work and Case Management
Many families experience stress when a member has a chronic illness. This stress is usually multifactorial and caused by dynamics such as concern for the health of their loved one, financial issues, worry, or not having enough time in the day to get everything done. The CF team social worker/case manager:
- is available to assist families in managing this stress by partnering with them to find solutions
- is present at clinic visits, during hospital stays, and weekdays by phone to provide resource information, emotional support, answer questions about life issues, and make appropriate referrals
- helps in the transition process from dependence on caregivers to independence in CF care, goal-setting, and education and/or career planning
- works with the team to transition those patients moving from the pediatric care team to the adult care team as they grow from teens to young adults
- performs routine psycho-social assessments in order to assist the team in providing comprehensive care to those who have been affected by cystic fibrosis
The CF team Respiratory Therapist will help you find ways to fit your chest physiotherapy, exercise and aerosolized treatments into your daily routine. The respiratory therapist will be available to teach you various modes of mucus clearance, introduce and trial any additions or changes to your prescribed nebulized medications, and review the best way to maintain your respiratory equipment.
The Respiratory Therapy Department provides a full range of cardiopulmonary therapeutics for children, adolescents, and adults in both inpatient and outpatient settings. The department is staffed 24-hours a day. Services include:
- Mechanical ventilation
- Noninvasive monitoring
- Oxygen administration
- Aerosolized drug delivery
- Postural drainage/percussion and vibration
- Other airway clearance techniques
- Cardiopulmonary function testing
- Cardiopulmonary rehab
- Referral requirements
People with cystic fibrosis will require medications to help keep their lungs healthier, aid the digestion of food to enhance nutrition and treat infections along with medications used for other reasons. The cystic fibrosis pharmacist focuses on each person’s medications. All medications are reviewed for appropriateness, proper dose, drug interactions, and possible side effects. The pharmacist may recommend a new medication or a change in a current medication. Pill swallowing tips may be offered.
Education is an important role of the pharmacist. The more a person knows about the medication they are taking, the more likely they are to take it properly and get the maximum benefit from it. The person with cystic fibrosis should know the name and dosage of their medications, why the medication is prescribed, how it works and what to do if side effects occur. This is a family affair until the person with CF can handle their medications on their own.
The CF Center pharmacist may also help with antibiotic selection and dosing and monitors and interprets drug levels when necessary. The pharmacist educates the entire pharmacy staff at St. Vincent Hospital about the care of persons with cystic fibrosis. This includes information about available medications and treatments and those that are in the development pipeline.
Genetic counselors at St. Vincent Hospital are experienced members of the CF Center team. The genetic counselor’s role in our CF Center often starts when a baby has a CF newborn screen that indicates he/she may have CF. After a positive (abnormal) CF newborn screen, further diagnostic testing includes a sweat test, which can rule out or confirm a diagnosis of CF. Find more information about CF newborn screening, here.
Genetic counselors work with families in the CF Center to help them understand genetic information in a personally meaningful way. Genetic counselors provide support and resources for families throughout many stages of their lives. We address questions about genetic testing, CF inheritance, and likelihood for other children or relatives to have CF or to be a CF carrier. We also coordinate genetic testing for families and help them gain access to relevant clinical trials. The genetic counselors are dedicated to quality CF care.