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Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of CF treatment include:
The basic daily care program varies from person to person, but usually includes pulmonary therapy (treatments to maintain lung function) and nutritional therapy (a high-calorie, high-fat diet with vitamin supplements). Your doctor may also prescribe oral doses of pancreatic enzymes to help improve food digestion. Oral or inhaled antibiotics may be needed occasionally to treat lung infections and mucolytic medication (a mucus-thinning drug) is sometimes given to keep mucus fluid and flowing.
CF can also cause complications. When this happens, treatments may require a stay in our medical center. Fortunately, medical technology has come a long way so that treatments are more effective. The following help you understand the kinds of treatments available and their benefits.
Airway clearance is a form of therapy that is used by people with Cystic Fibrosis (CF) to help them breathe easier. Airway clearance helps to loosen the thick, sticky mucus in the airways (breathing tubes), allowing it to be coughed up and out of the lungs.
Airway clearance helps keep the airways clear of mucus. This leads to less infections and better lung function.
Antibiotics are used in CF to reduce the amount of bacteria in the lungs. They may be prescribed to treat an "exacerbation" or illness or they may be prescribed on a chronic basis. Antibiotics can be oral, inhaled or intravenous, meaning they're delivered to the body through an "IV."
Common antibiotics used to treat CF:
Sometimes we may need to treat fungal infections in the lung. They can be used in addition to antibiotics or may be used alone. Antifungals can be oral or IV (intravenous).
Antifungals used in CF:
Research supported by the Cystic Fibrosis Foundation has led to other respiratory treatments that are now considered "standard therapies" for many people battling cystic fibrosis.
For example, ibuprofen, which is an anti-inflammatory, was found by CF Foundation-supported researchers to benefit people with cystic fibrosis. It can slow the rate at which lung function usually declines for some people with CF.
Ibuprofen is an anti-inflammatory and non-steroidal agent (does not upset hormonal balance), most often prescribed to treat arthritis, fever, menstrual symptoms and pain. Ibuprofen was originally developed as a prescription drug called Motrin®. Today, ibuprofen is available over-the-counter and by prescription by several different manufacturers and is sold as Advil®, Nuprin® and Motrin-IB®, among others.
Ibuprofen isn't right for every person with CF. Some people with certain conditions should not take ibuprofen as it may cause unwanted side-effects.
The dose you/your child will need to take is very different from over-the-counter ibuprofen. Your doctor will perform a specific blood test to determine the right dosage. Too little an amount has no benefit, while too much could carry very high risk.
It is also important to know that the benefits of ibuprofen won't be felt right away, nor does it cure CF. What it does do is begin to slow the rate of lung damage in people with CF. Over a long period of use, lung function has been shown to improve and infections appear to be reduced. It may take as long as four years to begin to see real improvement, and the drug will need to be taken over the course of many years.
Nutrition needs change with age – especially for people with cystic fibrosis. Dietitians at cystic fibrosis care centers offer specially tailored dietary programs for each stage of life.
The Cystic Fibrosis (CF) Clinic has a team of dietitians to help you/your child with nutrition needs. During your clinic visit, the pulmonologist may ask the dietitian to see you/your child to provide nutrition recommendations that can help manage your/your child's condition.
Our dietitians can:
Children and teens with cystic fibrosis need extra calories to grow and develop. Everyone with CF, no matter their age, needs good nutrition to stay strong against lung infections and other challenges. Occasionally, patients need to take nutrition through a tube ("tube-feeding") to provide extra calories that help the body grow and stay strong.
Body Mass Index, or BMI, is a calculation used to assess how healthy an individual's weight is relative to their height. Click here for more information and to find out your BMI.
Almost half of all people with cystic fibrosis in the United States today are adults. they're facing diseases most often associated with the middle-aged or elderly. These diseases include CF-related diabetes and brittle bones (osteoporosis or osteopenia). By working closely with CF care centers, good nutrition can help to prevent or lessen the impact of these and other health problems.
The guidelines that follow can help you prepare for discussions with your dietitian, and also offer helpful tips and meal planning:
Chances are, most people you know are trying to keep pounds off. But you need more calories and nutrients than they do just to keep the pounds on. That's because CF makes it hard for the body to absorb fat and nutrients. But keeping your weight – and sometimes increasing it – is key to fighting infection and keeping your lungs and body strong.
We've put together this section to make it easier for you to "eat on the go." It turns out that small changes in your routines can make a big difference for your weight.
The following resources offer more information about adult nutrition. They can also help you develop questions to discuss with your dietitian.
If your child sees a doctor in the CF Clinic, and you would like to contact the dietitian, please call: 682-885-7496.
The best way to take control of CF is to arm yourself with lots of healthy information. The links below can get you started:
Vitamins are needed for the normal growth, function, and health of our bodies. People with CF need extra vitamins for good health.
Minerals help with normal growth, function and maintenance of good health.
Enzymes are necessary for good digestive health.
When you have CF, your pancreas, just like your lungs, also produces thick mucus. This mucus blocks the release of the enzymes your body needs to digest your food and absorb nutrients. Read up on the importance of enzymes and then talk with your CF care team to determine the supplements for you.
Exercise used to be discouraged for people with cystic fibrosis (CF) because it was thought that overexertion would increase breathing problems. Now, we know that the opposite is true. Studies have shown that regular physical activity provides many benefits to people with cystic fibrosis:
Did you know?
Aerobic exercise that makes you breathe harder can help loosen the mucus in your airways so you can cough it up. Exercise also helps improve your overall physical condition.
Increase lung capacity
Of course, that doesn't mean you should just run right out and start a vigorous routine. Each person is different, and so is each case of CF. It is very important that you work with your health care team to create a plan that works for you. And make sure that you discuss what is and isn't working during your clinic visits. That way, the team can adjust your exercise program to make sure that it is giving you the most benefit possible.
The general rule of thumb is that to receive the most benefit, exercise routines should include 20-30 minutes of aerobic activity three times per week. However, any amount of exercise is better than no exercise at all and exercise routines should be adjusted according to each person's level of tolerance.
There are a few extra precautions that people with CF should take when getting active to get the most from your exercise and prevent potential setbacks, among them are:
It's hard to think that something as tiny as a germ could cause really big problems, but they do.
Some germs (like the viruses that cause colds, the flu and RSV) affect people with and without CF. But when people with CF get a respiratory virus, they may get sicker because of their lung disease. Other germs (like the bacteria pseudomonas and B. cepacia) are more common and cause more harm in people with CF than in people who don't have CF.
Germs spread fast!
When a person sneezes, particles can fly out the nose at up to 40 mph. And they can land on things about 15 feet away. Always use a tissue when you sneeze, and avoid sick people who might sneeze on you.
Why do people with CF get more and worse lung infections? The altered gene that causes CF leads to an error in how salt moves in and out of the cells in the lungs. This causes thick, sticky mucus. Germs stick, stay and grow well in this mucus in the lungs. White blood cells in the body attack the germs, which can also hurt the lungs. The airways swell because of inflammation and make more mucus. More germs grow, and the cycle continues.
Germs are everywhere: the air, soil and people. They can also be found on equipment that touches mucus or mucus membranes (such as your mouth), like nebulizers—which is why it's very important to clean them. You should also do the following:
Several different types of inhaled medications are used in treating Cystic Fibrosis. They are:
Inhaled medications may come in an inhaler, a dry powder device or may need a compressor to deliver the medication to the lungs. Your respiratory therapist will instruct you how to take the medication, which nebulizer cup to use and any special cleaning instructions.
Your doctor will discuss with you which medications are best for you/your child. It is important to take these medications as instructed.
Colymicin is a medication that treats Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).
Committing to your treatment plan with Colymicin may improve lung function, decrease the concentration of Pseudomonas aeruginosa in your sputum or throat culture, and reduce the length of hospital stays. For more information, contact your Cook Children's CF care team or visit the Cystic Fibrosis Foundation at www.cff.org.
TOBI is an inhaled medication, by nebulizer or dry powdered inhaler, that treats Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).
Committing to your treatment plan with TOBI may improve lung function, decrease the concentration of Pseudomonas aeruginosa in your sputum or throat culture, and reduce the length of hospital stays. For more information, contact your Cook Children's CF care team, visit the Cystic Fibrosis Foundation at www.cff.org, or learn more at www.tobitime.com.
Pulmozyme® is used to thin and loosen the mucus in your lungs.
Pulmozyme may help delay CF progression. For more information, contact your Cook Children's CF care team or visit the Cystic Fibrosis Foundation at www.CFF.org.
Hypertonic saline (HTS) is extra salty water inhaled twice a day with your nebulizer. It is used to improve airway clearance by making you cough.
Hypertonic saline is one more "tool" in CF care. It isn't meant to replace other treatments. For more information, contact your Cook Children's CF team or visit the Cystic Fibrosis Foundation at www.CFF.org.
Cayston is an inhaled antibiotic for the treatment of Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).
For more information, contact your Cook Children's CF care team or visit the Cystic Fibrosis Foundation at www.CFF.org.
Sometimes, in severe cases of lung disease, the CF care team may discuss the option of a lung transplant. This can be very scary to the person with CF and the family. The team usually sits down with you long before a transplant is needed to make sure that you have time to gather all the facts, learn about the procedure and understand the risks involved in having, or not having. We want you to have all the necessary information so that you can weigh your options and make the decision that is right for you/your child and your family.
If you're considering a transplant, your doctor will refer you to a lung transplant center for evaluation. While you're there, the medical team will explain the overall process—testing, waiting for a donor match, the surgery, any risks involved and survival statistics. They will also go over the recovery process and short- and long-term care of the new lungs, including the medications that will need to be taken for the rest of your/your child's life.
While each center has specific criteria they use to determine transplant candidates, there are some general things they will be looking at:
Potential medical tests may include:
The results from the full evaluation will go before a Medical Review Board to be reviewed. The Board can decide a few things:
If you're approved for a transplant, you will be placed on a waiting list for a donor match. The donor is the person donating the lungs. In order for the transplant to be successful, there must be a medical and biological match. The medical team considers many things, including: blood type, height, size of the chest, etc. The better the match, the more likely your body won't reject the new lungs.
The immune system in the human body is designed to protect cells and organs from any foreign material that isn't part of the original body such as germs or transplanted organs. After your transplant, your immune system will naturally react by trying to reject your new lungs. To protect your lungs, you will be given immunosuppressive medications to stop your immune system from attacking your new lungs. You will need to take these drugs for the rest of your life.
Lung transplant does not cure CF. Your new lungs will be disease-free, but this won't prevent or improve problems in other parts of your body caused by CF. Even though your transplant won't cure you, it can help you live a longer life.
If you have been told your child might have CF, we know you have lots of questions. We can help. To schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-885-6299. After hours and on weekends/holidays, call 682-885-4000 and ask the operator to page the pulmonologist on call.