Cystic Fibrosis Program
Success Story of a Children's Hospital
Kathleen
Miller Children’s Hospital Long Beach Patient Kathleen Ryan Fights To Beat The Odds Against Cystic Fibrosis
Miller Children’s Cystic Fibrosis program is one of the largest in the U.S. and provides comprehensive, multidisciplinary care including evaluation, diagnosis and treatment for children from birth to age 18, with Cystic Fibrosis (CF). The program also provides comprehensive care, including diagnostic testing such as, sweat tests, genetic tests, throat cultures, sputum cultures, blood work and chest x-rays.
Symptoms of cystic fibrosis may resemble other conditions which is why it is always important to consult a specialist for diagnosis. However, each child may experience symptoms differently. Infants born with CF usually show symptoms within the first year. Some children, though, may not show symptoms until later in life.
The following are the most common symptoms for CF:
- Frequent episodes of wheezing
- Frequent episodes of pneumonia
- Persistent cough
- Poor growth
- Clubbing of fingers and toes (the ends of the fingers and toes become enlarged)
- Pneumothorax (the presence of air or gas in the plural cavity causing the lung to collapse)
- Hemoptysis (coughing up blood)
- Abdominal pain
- Skin that tastes like salt
- Greasy or foul-smelling stools
CF Tests & Treatment
There isn’t a cure for Cystic Fibrosis (CF), but tests and treatments are available to help the child and their family members manage the condition. Tests and treatments can also help identify preventative measures that need to be put into place, such as rest, enzyme treatments, medication or even hospitalization.
Sweat Test
The sweat test is done to rule out cystic fibrosis. It also may be used to test people with a family history of cystic fibrosis and for anyone with symptoms of cystic fibrosis.
Pulmonary Function Testing
Pulmonary function testing is a broad range of tests that measure how well the lungs take in and exhale air and how well they transfer oxygen into the blood.
Blood Tests
Blood or cheek scraping cells can be tested for mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Other blood tests can assess infection, and involvement of certain organs.
Chest x-rays
These are diagnostic tests which use invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. This can help determine fluid build up in the lungs.
Sputum cultures
Sputum cultures are diagnostic tests performed on the material that is coughed up from the lungs and into the mouth to determine if an infection is present.
Stool evaluations
These help to measure stool fat absorption.
Enzyme Treatment & Nutrition
Children that have CF need a high-calorie, high-protein diet and are prescribed enzymes to gain weight and grow. Extra snacks at school and high-calorie nutrition supplements may be needed to increase caloric intake. Children with CF need high-fat foods, even though that is generally discouraged to the general population. Also, children with CF need to take their enzymes in a reasonable timeframe both at school and home, that allows them a good amount of time to eat.
Exercise & Activities
Exercise and activities help build muscle and strong bones, strengthen the lungs, clear lung mucus and lower emotional stress for children with CF. Children with CF can and should be included in games and activities. However there are days, where CF can limit how much a child can do. Fluids are crucial during exercise and activities for a child with CF, because they sweat less, lose more salt and create more heat during exercise.
Salt can be replaced by eating salty foods like pretzels or potato chips. Sports drinks are best for children with CF because they have added carbohydrates and salt which give fuel to the muscles and keep blood sugars up.
Approved CCS Center
The Cystic Fibrosis Center at Miller Children’s is a California Children’s Services (CCS) approved Special Care Center (SCC). This means a specially designated multidisciplinary care team is responsible for all care coordination and case management of a patient in this program. Patients typically qualify for the Cystic Fibrosis CCS Special Care Center by medical diagnosis, complexity of their disease and financial status.
Visit the Cystic Fibrosis Center.
Miller Children's Hospital Long Beach
2801 Atlantic Ave., Long Beach, CA 90806
(562) 933-5437
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