Adult Cystic Fibrosis
At MaineHealth, our specialists work with a multi-disciplinary team to offer the most up-to-date services for diagnosing and treating cystic fibrosis. We have an adult and pediatric CF Center at Maine Medical Center in Portland. We are part of the Therapeutic Development Network so we are able to provide on site access to multi-center research studies through the CF Foundation toward a cure.
Genetic screening is offered to all families detected by screening to have CF or to be a CF carrier.
Many pregnant mothers are screened for CF genes but it is still recommended that their babies have CF newborn screening.
Our experienced CF team consists of a nurse practitioner, two social workers one of whom is funded by the CF Foundation to work exclusively on the mental health of our patients and families. We also have a nurse coordinator, nutritionist, respiratory therapist, pharmacist, research coordinator and physical therapist all of whom attend the National CF Conference annually to keep up to date. . We have two board certified adult pulmonologists.
Our goal is to provide CF patients and families with the most advanced care and access to research studies.
What is cystic fibrosis?
Cystic fibrosis, or CF, is an inherited disease passed down in families. Both parents have to carry the gene and carriers are asymptomatic. There is no cure for cystic fibrosis but through the work of the CF Foundation new therapies are being developed for this disease.
People with CF can have serious breathing problems due to problems with the mucus in their lungs. They also have problems with the pancreas requiring that they take enzymes to digest their food. Other organs can be affected as well.
Cystic fibrosis symptoms can vary
Sometimes symptoms are not picked up in childhood, because they can be different from person to person.
Later symptoms include:
Coughing up mucus or bloody mucus
Shortness of breath with exercise
Growths (polyps) in the nose or sinuses
Rounded or flattened fingers
Infertility, especially in males
Diagnosing cystic fibrosis
All babies in Maine are screened for cystic fibrosis at birth, if the family agrees to do the test. The screening tests for high levels of a certain enzyme. A laboratory test called a Sweat Test (iontophoresis) measures how much salt is in the sweat and is required to make a diagnosis of CF. Patients with CF have a high level of salt in their sweat. The test can only be done at an accredited laboratory.
Treatment to avoid lung problems includes chest physical therapy and various inhaled therapies to thin mucous and inhaled antibiotics to treat bacteria and exercise. The majority of patients need to take pancreatic enzymes for normal growth. There are also new therapies that can treat the cellular defect and can be taken in pill form that offer great promise for CF patients.
Many CF patients have to be hospitalized for several weeks for intravenous antibiotic therapy to treat lung infections. During this time they receive chest physical therapy 4 times a day, exercise and consultation with the CF team and other specialists as needed