CF is an ongoing and progressive disease, and studies are showing that hidden damage may be happening as early as birth. Having a proactive CF care plan in place can help you manage the disease.
Get involved with a CF Center to be sure that you have access to experts. Locate the CF Center in your area and make an appointment to find out more. You will be able to get aCF Care Team, which may improve the quality of your CF care.
It's possible that your CF management plan will have multiple aspects. Here are some examples of things that might be a part of your plan: * Oral antibiotics, which may be swallowed * Intravenous (IV) antibiotics, which may require a hospital stay * Inhaled antibiotics, which may be an aerosol or mist * Airway Clearance Techniques (ACTs), which may include postural drainage and percussion, as well as special equipment, such as a vest * Inhaled medications, which may require a nebulizer. Nebulizers vary, so you should talk to your CF Care Team about which is FDA approved for use with your medicine.
Indication and Usage
Daily administration of Pulmozyme® (dornase alfa) Inhalation Solution along with standard therapies is indicated in the management of cystic fibrosis patients to improve lung function. In patients with a forced vital capacity (volume of air exhaled with maximum effort and speed) greater than or equal to 40% of predicted, daily administration of Pulmozyme has also been shown to reduce the risk of respiratory tract infections requiring the administration of injectable antibiotics. In our pivotal study, safety and efficacy of daily administration has not been studied in patients beyond 12 months.
Important Safety Information * Pulmozyme should not be used in patients who are allergic to any of its ingredients * Pulmozyme should be used along with standard therapies for cystic fibrosis * When starting Pulmozyme therapy, patients may experience change in or loss of their voice, discomfort in the throat, chest pain, red watery eyes, rash, dizziness, fever, or runny nose * These side effects are usually mild and short-lived * The effect of Pulmozyme on exercise tolerance has not been established in adults and children
Medications

People with cystic fibrosis will have to take medications for the rest of their lives. Some of the common medications are:

Antibiotics: Taken to prevent or cure an infection. Sometimes the antibiotics are taken by mouth, and sometimes they are a liquid that is vaporized and inhaled using a machine called a nebulizer.

Anti-inflammatory: An anti-inflammatory is a drug that reduces irritation and swelling. Anti-inflammatory drugs may be prescribed to CF patients to reduce the swelling in the lungs and breathing passages.

Steroids are one type of anti-inflammatory drug, but they are not used very often in CF treatment anymore because they can cause diabetes or problems with the kidneys and bones. Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are more commonly prescribed in CF treatment.

Enzymes: One of the problems caused by CF is that the thick secretions in the pancreas prevent it from releasing the enzymes the body needs to digest food. If the food is not digested, the body can’t absorb any nutrients. People with CF need to take pills that contain the necessary enzymes before each meal.

Mucolytics: Mucolytics are medicines that help to thin mucus and make it easier to cough out. They are often prescribed to cystic fibrosis patients to help them clear their airways of the thick, sticky mucus that CF causes. Sometimes mucolytics are taken by mouth and sometimes they are inhaled using a nebulizer.

Airway Clearance

An essential part of cystic fibrosis treatment is the use of a variety of techniques to help loosen and remove mucus. Cystic fibrosis patients must have this therapy several times a day. The therapy will involve one or more of these techniques:

Chest Physical Therapy (CPT): This therapy uses a technique called percussion that involves rhythmic tapping of the hands on strategic areas of the chest. With newly diagnosed CF patients, a respiratory therapist or nurse will usually do CPT but parents will be taught how to do it so they can take over therapy at home. A typical CPT session lasts about 30 minutes, and will need to be done up to four times per day.

Vibration: Another technique that is sometimes done along with percussion during CPT is vibration. This is also done with the hands, but as the name implies, a vibrating motion is used rather than a tapping motion.

Postural Drainage: During CPT, the patient is placed in positions that will allow gravity to help the loosened secretions flow out of the lungs. This is known as postural drainage.

Inflatable Therapy Vest: Sometimes, instead of manual CPT, a device called and airway clearance vest will be used. The vest inflates and deflates rapidly to loosen mucus through a gentle squeezing motion. The benefits of the using the vest are that the patient can do therapy without help, and the sessions last only about 20 minutes.

Flutter Device: Also known as a flutter valve, this small hand-held device looks similar to an inhaler. The patient puts the mouthpiece in his or her mouth and exhales forcefully. The device uses the exhaled air to create vibrations of the chest.

Diet

People with cystic fibrosis don’t absorb nutrients properly from the food that they eat, and the work of breathing causes them to burn extra calories. This double whammy causes malnutrition and poor growth. To combat nutrition problems, CF patients require up to 50% more calories than other people their age. Nutritionists create individualized diet plans tailored to the needs of each patient, but usually people with cystic fibrosis can expect to:

Eat 3 meals and 2-3 snacks per day
Eat foods high in protein and fat
Drink nutrition shakes once or twice a day
Take vitamin supplements, especially vitamins A, D, E, and K, which are not stored well in people with CF.
Exercise

Physical activity has both short-term and long-term benefits for people with cystic fibrosis. The immediate benefit is that exercise forces more air through the lungs and helps loosen mucus. The long-term benefit of exercise is that it helps improve cardiovascular health and endurance, which gives the body extra stamina to fight the effects of CF.

Lung Transplantation

When cystic fibrosis progresses and causes severe lung damage, lung transplation may be a treatment option for people who meet the criteria. While lung transplantation is not an option for everyody, it has proven to be quite successful for some CF patients. More than 1600 people with cystic fibrosis have received lung transplants, and about half of them have survived for at least five years after after receiving their new lung.