Cystic Fibrosis

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 Reference mucus Opens New Window, eating Reference healthy foods Opens New Window Reference Opens New Window, 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. You can locate one by contacting the Cystic Fibrosis Foundation at www.cff.org.

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 Reference respiratory Opens New Window Reference Opens New Window and Reference digestive Opens New Window Reference Opens New Window systems, although sometimes it causes problems only in one or the other. Other parts of the body may also be affected. 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 Reference immunizations Opens New Window and will schedule any shots that are needed. Children with cystic fibrosis should have all the recommended shots in addition to pneumococcal shots. For more information, see the topic Reference 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:

• Reference Bronchodilators (such as albuterol or salmeterol), which are used to make breathing easier. They may also make it easier to cough up mucus.
• Reference DNase (such as Pulmozyme), which is used to thin mucus in the lungs.
• Mucolytics (such as acetylcysteine), 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 Reference inflammation Opens New Window in the airways.^{Reference 1, Reference 2}

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

• Reference Postural drainage and chest percussion, to help your child cough up mucus from the lungs. For specific instructions, see:

  Reference Reference Cystic Fibrosis: Helping Your Child Cough Up Mucus.

• Deep breathing exercises, to help your child breathe out completely and strengthen the muscles used for breathing.
• Directed cough to help clear mucus by breathing and coughing in specific ways.
• Reference Exercise. Aerobic exercise can improve how well the lungs work. Ask your doctor about what kinds of exercise your child should do.
• Reference Alternate methods of airway clearance, such as using a positive expiratory pressure (PEP) device, high-frequency chest compression vest, or Flutter valve. These methods use mechanical equipment to help clear mucus from the lungs.

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

• Digestive Reference 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 Reference intravenous Opens New Window nutrient supplementation.
• Preventing intestinal blockages with stool softeners (to avoid constipation) and Reference enemas Opens New Window.

Antibiotics. These medicines, which kill bacteria that cause infections, are often used to treat cystic fibrosis. Some Reference antibiotics may be prescribed to help prevent infections. Others may be prescribed to help fight infections.

Ongoing treatment

Most ongoing treatment for cystic fibrosis focuses on controlling and reducing problems or complications in the Reference respiratory and digestive systems Opens New Window Reference Opens New Window.

Your child is likely to continue with respiratory therapy, digestive therapy, and antibiotics.

People with severe lung disease may need to use oxygen at home. Regular visits with the team of health professionals involved in your child's care are also important.

Reference Reference Oxygen Therapy: Using Oxygen at Home

Doctors may do certain tests to help find out what kinds of problems your child is having. These tests may include:

• A Reference throat culture or Reference sputum culture, to identify what kind of bacteria are present. This test helps your doctor prescribe the best type of antibiotic.
• Reference Lung function tests Opens New Window, to see how healthy the lungs are.
• A Reference stool analysis, to see how well the digestive system is working.
• Reference Blood sugar (glucose) tests, to check for Reference diabetes Opens New Window.

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 his or her chances of having more serious problems later increase.

Treatment if the condition gets worse

Serious cystic fibrosis problems or complications occur when the Reference respiratory system or Reference digestive system becomes damaged. Reference Bronchiectasis Opens New Window, which is caused by long-lasting airway inflammation, is common. 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.

Some of the tests that help the doctor know what kinds of problems your child is having include:

• A chest Reference X-ray Opens New Window, to look for signs of lung infection or other problems.
• A Reference CT scan Opens New Window, to identify any serious disease in the lungs, pancreas, or other organs.

If complications develop, one or more of the following medicines may be needed:

• Reference Antibiotics, to treat infection
• Anti-inflammatories to reduce inflammation
• Reference Bronchodilators and Reference anticholinergics, to open airways in the lungs
• Medicine to control the amount and thickness of mucus and to reduce the chance of infection

Sometimes surgery is needed to treat complications of cystic fibrosis. Procedures may include:

• Chest tube drainage and possible Reference thoracoscopy, to treat a collapsed lung (Reference pneumothorax Opens New Window).
• A Reference lung transplant.
• Repair of an Reference intestine that has collapsed Opens New Window inside itself or the removal of a Reference bowel obstruction Opens New Window.
• Removal of Reference nasal polyps Opens New Window or Reference endoscopic Opens New Window sinus surgery.
• Abdominal (belly) or perineal (anal) surgery for Reference rectal prolapse Opens New Window Reference Opens New Window, in rare cases.

Other treatments used to treat complications from cystic fibrosis may include:

• Reference Blood transfusions Opens New Window 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 feeding tube into your child's stomach.
• Placement of a semipermanent intravenous (IV) tube in order to give your child antibiotics frequently without having to place a line in the vein each time.

Palliative care

As your condition gets worse, you may want to think about Reference palliative care Opens New Window. Palliative care is a kind of care for people who have illnesses that do not go away and often get worse over time. It is different from care to cure your illness, called curative treatment. Palliative care focuses on improving your quality of life—not just in your body, but also in your mind and spirit. Palliative care can be combined with curative care.

Palliative care may help you manage symptoms or side effects from treatment. It could also help you cope with your feelings about living with a long-term illness, make future plans around your medical care, or help your family better understand your illness and how to support you.

If you are interested in palliative care, talk to your doctor. He or she may be able to manage your care or refer you to a doctor who specializes in this type of care.

For more information, see the topic Reference Palliative Care.

End-of-life issues

It is important to talk about the kind of medical procedures you want or do not want for yourself or for your child.

For specific information on end-of-life issues, see the topic Reference Care at the End of Life. For more information on how to handle emotions associated with death, see the topic Reference Grief and Grieving.

Reference Support groups, Reference counseling Opens New Window, and education about the disease can be very helpful not only for people who have cystic fibrosis but also for their families.

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