Cystic fibrosis (CF) is a disease that causes the body to make thick, sticky mucus. The thick mucus blocks airways, damages the lungs, and makes it hard to breathe. It can also lead to lung and sinus infections.
The thick mucus also blocks tubes in the digestive system, making it hard for fluids to move from the pancreas to the intestines. Fluid made by the pancreas helps break down food in the intestines. When the tubes between the pancreas and intestines are blocked, it’s harder for your child to digest and absorb food.
Cystic fibrosis can affect all races and ethnic groups, but it is seen mostly in white people with northern European ancestors.
There is no cure for cystic fibrosis. It is a life-long disease that gets worse over time. Many people with CF live into their mid 30s. With early screening and treatment, and ongoing research for management of the problems caused by CF, life expectancy may increase for children born after 2000.
Cystic fibrosis is inherited, which means that it is passed from parents to children through their genes. Genes are inside each cell of your body. They contain the information that tells your body how to develop and work. When you have CF, changes in the genes change the way the body makes mucus and uses salt and water.
To have CF, a child must inherit 1 CF gene from each parent. If a child gets a CF gene from one parent, but not the other, he or she is a carrier of the disease but does not have the disease. If both parents have the cystic fibrosis gene, each child has a 25% chance of having CF.
Cystic fibrosis affects people in different ways and to different degrees. Often children start having symptoms soon after birth. Other children may have a milder form of the disease that doesn't show up until they are teenagers or young adults.
Your child may have some, but not all of these symptoms:
Most children with CF are diagnosed before they are a year old. Newborn screening blood tests include a test for cystic fibrosis. A positive blood test needs to be confirmed with a sweat test or genetic test.
The goal of treatment is to slow down the progress of the disease and help your child lead as normal a life as possible. Treatment for CF includes:
Diet and Exercise
Your child may need to eat more calories to grow normally, keep a good weight, have energy, and fight infections. This may mean giving a baby high-calorie breast milk or formula. For a toddler or older child it means increasing the amount of fat and protein in the diet. Milk products are an easy way to add calories. For example, you can give your child whole milk, use extra butter on foods, or add extra cheese to pizza or casseroles.
Encourage your child to exercise according to your healthcare provider’s recommendations. Exercise can help loosen mucus and make the heart and lungs stronger.
You and your child will learn ways to clear your child’s airways. Some techniques you can do yourself, and some require special equipment or a trained person to help you. The techniques that you or your older child can do include:
Follow the full course of treatment prescribed by your child's healthcare provider. In addition:
Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.