Cystic Fibrosis

Treatment Overview

After a child is diagnosed, a team of health professionals will build a treatment plan based on the child's specific health problems. Following a treatment plan will help your child live a longer, healthier life.

Your child will likely have ongoing respiratory therapy, digestive therapy, and treatment with medicines such as antibiotics. Regular medical care, home treatment such as postural drainage, and taking steps to reduce infection can help people with cystic fibrosis lead relatively normal lives.

The best treatment available is generally found at cystic fibrosis care centers. These centers address the medical, nutritional, and emotional needs. You can find one by contacting the Cystic Fibrosis Foundation at www.cff.org.

Doctor visits and immunizations

Regular visits with the team of health professionals involved in your child's care are important. Your doctor will want to make sure that your child is eating properly and is gaining weight and growing at a normal rate. The doctor will record your child's weight, height, and head size in order to keep track of how your child is developing over time.

Lab 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 will schedule any shots that are needed. Children with cystic fibrosis should have all the recommended shots, plus pneumococcal shots. To learn more, see the topic Immunizations.

Respiratory therapy

Respiratory therapy is 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 may be used to control the amount and thickness of mucus.

Other ways to help remove mucus from the lungs involve certain types of movements, coughing, or exercises known as airway clearance techniques.

Cystic Fibrosis: Helping Your Child Cough Up Mucus

People with severe lung disease may need to use oxygen at home.

Oxygen Therapy: Using Oxygen at Home

Digestive therapy

This treatment works to replace certain digestive enzymes, to make sure the body absorbs all the vitamins and minerals it needs, and to prevent or treat intestinal blockages. Digestive therapy involves:

• Digestive enzyme replacement therapy (such as with Creon or Pancreaze), to help the intestines absorb nutrients from food.
• Nutritional therapy to help replace lost nutrients. This may include taking vitamins; eating high-calorie, high-fat foods; drinking nutritional drinks; getting fed through a tube in the stomach; and, in some cases, receiving intravenous nutrient supplementation.
• Preventing intestinal blockages with stool softeners (to avoid constipation) and enemas.

Treating complications

Serious cystic fibrosis problems or complications occur when the respiratory system or digestive system becomes damaged. Most people who have complications will need to stay in the hospital. Treatment for complications may include medicines or surgery, depending on the person's age and symptoms.

The doctor may do tests, such as a chest X-ray, to know what kinds of problems your child is having.

Other treatments for complications from cystic fibrosis may include:

• Blood transfusions and medicines to treat the bleeding (embolization therapy), if your child is coughing up large amounts of blood. Coughing up small amounts of blood is normal for people who have cystic fibrosis. But coughing up large amounts of blood can be life-threatening.
• Placement of a semipermanent intravenous (IV) tube to give your child antibiotics frequently without having to place a line in the vein each time.

Home care for cystic fibrosis

Home treatment is very important. It can make a person with cystic fibrosis feel better and live longer. Here are some things you can do at home, or help your child do, to help prevent more serious health problems like lung infections:

• Don't smoke. And avoid secondhand smoke.
• Use airway clearance techniques, such as postural drainage and chest percussion.
• Eat nutritious, high-calorie foods.
• Exercise.
• Drink plenty of fluids.
• Add salt to foods, especially during hot weather.
• Get all recommended vaccines and practice good hygiene. Also keep clean any breathing equipment you use for your treatment.

As children with cystic fibrosis get older, it is important for them to learn how to help care for themselves. Even though it can be hard to follow a treatment plan every day, there are many benefits of home treatments. Skipping a treatment may not make a person feel worse right away. But it raises the chances of having more serious problems later.

Getting support

Handling the challenges of caring for a child who has cystic fibrosis can be difficult. Take good care of yourself, physically and emotionally, so that you can give your child the best care possible.

Many people with cystic fibrosis and their families need emotional support to help them live with this genetic disease. Support groups, counseling, and education about the disease can be very helpful not only for people who have cystic fibrosis but also for their families.

It is also important to talk about the kind of medical procedures you want or don't want for yourself or for your child.

Research for new treatments

Medical researchers are looking at gene transfer therapy. It involves introducing healthy genes into the lung cells of people who have cystic fibrosis.

Researchers are also investigating protein repair therapy, or protein assist therapy. This treatment involves taking medicines that help the defective protein work more normally to allow a small amount of salt and water to move out of cells.

Gene transfer and protein repair therapies are in the experimental, developmental stages. Talk to your doctor about clinical trials for these and other new treatments being studied.