Most people are diagnosed with cystic fibrosis before they are 1 year old. After a child is diagnosed, a team of health professionals will build a treatment plan based on the child's specific health problems. Treatment is different for everyone but usually involves a combination of medicines and home treatment. Home treatments include getting rid of mucus, eating healthy foods, and exercising to help prevent infections and complications. It can be challenging to follow a treatment plan, but doing so will help your child live a longer, healthier life.

The best treatment available is generally found at cystic fibrosis multidisciplinary specialist centers. These centers address the medical, nutritional, and emotional needs of people who have cystic fibrosis. There are more than 100 of these centers in the United States. You can locate one by contacting the Cystic Fibrosis Foundation.

Many people with cystic fibrosis and their families need emotional support to help them live with this life-shortening genetic disease. Support groups, counseling, and educating yourself about the disease can be very helpful.

Initial treatment

Usually, cystic fibrosis causes problems with both the respiratory and digestive systems, although sometimes it causes problems only in one or the other. Other parts of the body may also be affected. Tests such as a stool analysis, sputum culture, or lung function tests can help your doctor know how serious the disease is and how it is affecting your child's body.

Your doctor will ask you about your child's immunizations and schedule any necessary shots. Children with cystic fibrosis should have all the recommended shots in addition to pneumococcal and flu shots. For more information on the recommended schedule of immunizations for children, see the topic Immunizations.

Your doctor will want to make sure that your child is eating properly and is gaining weight and growing at a normal rate. He or she will record your child's weight, height, and head size in order to keep track of how your child is developing over time.

Your doctor may also talk to you about different therapies used to treat cystic fibrosis. These include:

Respiratory therapy. Respiratory therapy refers to any treatment that slows down lung damage and improves breathing. The focus of this therapy is on reducing infection and getting rid of mucus to keep the lungs healthy. Medicines used in respiratory therapy include:

• Bronchodilators (such as albuterol or salmeterol), which are used to make breathing easier. Bronchodilators may also make it easier to cough up mucus.
• DNase (such as Pulmozyme), which is used to thin mucus in the lungs.
• Mucolytics (such as Mucomyst), to thin mucus in the lungs and also in the intestines. These are not used very much, because they can irritate the lungs.
• An inhaled saltwater solution (hypertonic saline), sometimes used to help clear mucus from the lungs. It is low-cost, and it may help reduce inflammation in the airways.^{2, 3}