Pediatric 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 Cystic Fibrosis Center in Maine. We are part of the Therapeutic Development Network, so we are able to provide on-site access to research studies working toward finding a cure.
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, a baby born today can expect to live into adulthood with good care. People with CF can have serious breathing problems due to 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.
Newborn screenings for cystic fibrosis
Every baby born in Maine is offered newborn screening for CF using the newborn blood spot, so that babies can be identified and treated as early as possible. 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.
Cystic fibrosis symptoms
Most newborns with CF have trouble gaining weight in the first few weeks and months of life and have oily, frequent stools. They may not have breathing issues until a few months of age. Sometimes symptoms are not picked up in childhood, because they can be different from person to person. Common symptoms in young children can include:
- Salty sweat or salty skin
- Blocked small intestine at birth
- Diarrhea that doesn’t go away; often this is oily and foul smelling
- Chronic constipation and belly pain
- Poor weight gain
- Breathing problems
- Wet cough that doesn’t go away
- Chronic severe sinus infections
Later CF symptoms
Later cystic fibrosis 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, particularly 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.
Cystic fibrosis treatment
Treatment to avoid lung problems includes chest physical therapy (various inhaled therapies to thin mucus and inhaled antibiotics to treat bacteria and exercise). The majority of patients need to take pancreatic enzymes for normal growth. There also are 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 four times a day, exercise and consultation with the CF team and other specialists as needed.
Maine Medical Center is the only accredited teaching and research CF Center in Maine. Maine Medical Center also has an affiliate center that we oversee at Eastern Maine Medical Center to serve northern Maine. CF patients are seen and hospitalized at a state-of-the-art facility at The Barbara Bush Children’s Hospital at Maine Medical Center or in the MMC adult unit.
We have won national recognition from the CF Foundation for our work in quality improvement to improve outcomes for children with CF. Our outcomes can be seen on the CF Foundation website at www.cff.org
Experienced care from a multidisciplinary team of CF providers
Patients are seen at least four times per year and monthly in the first year of life. 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 four board-certified pediatric pulmonologists and two board-certified adult pulmonologists. Our goal is to provide cystic fibrosis patients and families access to research studies and expert care with compassion for the challenges this disease creates.