Treating Cystic Fibrosis

Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of CF treatment include:

Preventing and controlling lung infections
Loosening and removing thick, sticky mucus from the lungs
Preventing or treating blockages in the intestines
Providing enough nutrition
Preventing dehydration (a lack of fluid in the body)

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 techniques

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.

Why is airway clearance important?

Airway clearance helps keep the airways clear of mucus. This leads to less infections and better lung function.

What are some airway clearance options?

Coughing
Coughing is the most basic form of airway clearance. It helps to clear the mucus from the airways. Although coughing is helpful, it may not be enough to provide good airway clearance.
Huffing
Huffing is a type of cough. It requires that you be able to take a big breath in and then "huff" the air back out of your lungs. Again, although huffing is helpful, it may not be enough to provide good airway clearance.
Active cycle of breathing technique (ACBT)
ACBT is made up of three phases of breathing techniques to help remove mucus from the airways. They are: breathing control, thoracic expansion and forced expiration technique. Breathing control involves normal, easy breathing. Thoracic expansion involves taking big, deep breaths in. Forced expiration technique involves huffing breaths out while keeping your mouth and throat open.
Autogenic drainage
Autogenic drainage involves a series of controlled breaths that move mucus from the small airways to the large airways allowing it to be coughed up and out of the lungs. The controlled breaths are done in three stages. One is done with a little bit of air in the lungs. Another is done with a moderate amount of air in the lungs and the last is done with the lungs almost filled with air. Although this technique can be done anywhere without the help of a second person, it is difficult to master.
Chest physical therapy (CPT)/postural drainage and percussion (PD)
CPT is a common form of airway clearance used by many people with CF. PD uses gravity to move the mucus from the smaller breathing tubes to the larger ones. This is done by assuming various positions to help drain the mucus. Once the mucus is in the large airways, it can be coughed up and out of the lungs. CPT requires that someone claps or pats your chest to help loosen the mucus from the airways. Again, once the mucus is loose, it can be moved to the larger airways and coughing up and out of your lungs. For more details on CPT and PD, including pictures, go to www.cff.org to read "An Introduction to Postural Drainage and Percussion".
Oscillating positive expiratory pressure
Oscillating positive expiratory pressure requires the use of a hand held device that you blow through. When you blow into the device, your airways vibrate. This vibration helps to loosen the mucus from your airways allowing you to cough it up and out of your lungs. This process needs to be repeated several times during one session. Although the technique isn't difficult to perform, you usually need to be at least 5 years of age before you can do it properly. An advantage of this form of airway clearance is that you don't need a second person to help you. Examples of oscillating positive expiratory pressure devices include the Flutter® and Acapella®.
High frequency chest wall oscillation
High frequency chest wall oscillation (often referred to as "the vest") requires the use of an inflatable vest that is attached to a machine. When the machine is turned on, it produces vibrations. These vibrations help to loosen the mucus in your airways allowing you to cough it up and out of your lungs. Although there are many size vests available, you do need to be a certain size before you can effectively use the vest. To find out if the vest is right for you, ask your CF doctor at your next visit. During the visit, we will be able to measure you and find out which vest would work best for you. An advantage of this form of airway clearance is that you don't need a second person to help you. Examples of the vest include the inCourage System™, the SMARTVest™, the HillRom Vest and Afflovest.
Exercise
Exercise is important for everyone, especially those living with CF. Reasons why exercise is important include: 1) lungs function more efficiently, 2) cough is stronger and more effective, 3) airways are cleared more effectively, 4) muscles get stronger, 5) feeling of daily fatigue improves, 6) pain associated with changes in posture from coughing can be prevented or reduced, 7) posture and appearance improve, 8) endurance (being able to continue an activity for a longer period of time) increases, and 9) sense of well-being increases. We recommend that you perform 20 -30 minutes of vigorous activity every day. Because people with CF lose more salt in their sweat when they exercise in the heat, it is important to drink plenty of fluids, especially sports drinks such as Gatorade. In addition, you may be a candidate for salt supplements. At Cook Children's CF Clinic, we encourage a formal exercise study for everyone 13 years of age and up. Part of the exercise study will include meeting with a physical therapist to determine a program that is both good for you and fun.

Antibiotics and antifungals

Antibiotics

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:

Oral: Keflex, Zithromax, ciprofloxacin, Levaquin®, Bactrim, doxycycline, linezolid
Inhaled: TOBI®, TOBI Podhaler®, Bethkis®, Cayston®, colymicin
Intravenous: tobramycin, amikacin, ceftazidime, cefepime, Timentin, Zosyn, Merrem, vancomycin, linezolid

Antifungals

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:

Oral: nystatin, itraconazole, voriconazole, fluconazole
IV: micafungin, Abcelet

Anti-inflammatories

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.

What is ibuprofen?

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.

Can all CF patients take ibuprofen?

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.

Is it okay to buy over-the-counter ibuprofen?

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.

Dietary therapy

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.

Dietitian Services in the CF Clinic

Our dietitians can:

Help set goals for you/your child's weight gain and growth
Help you understand energy, protein and fat intake goals
Provide recipes or suggestions to help reach and maintain a healthy weight
Monitor and make changes to tube feeding plans
Help you decide if you/your child needs vitamin or mineral supplements
Help you assess the safety of herbal supplements
Provide advice on eating habits and patterns for a healthier lifestyle
Help you deal with picky eaters & negative mealtime behaviors
Promote bone health
Help you manage cystic fibrosis-related diabetes
Provide recommendations for sports nutrition
Help you make healthy food choices during pregnancy
Recommend appetite stimulants if needed

Children and teens

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:

Adults

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.

Use these ideas – for "grab ‘n go" breakfasts, mini-meals and snacks.
Work with your CF care team – to get advice on nutrition and keeping a healthy weight.
Check out this great recipe book – get tips and tricks for tasty meals and treats.

The following resources offer more information about adult nutrition. They can also help you develop questions to discuss with your dietitian.

Your pulmonary dietitian team

Esther Giezendanner MPH, RD, LD
Rachel Hemik MS, MBA, RDN, LD
Nikki Sabia RD, LD

Contacting your CF Clinic dietitians

If your child sees a doctor in the CF Clinic, and you would like to contact the dietitian, please call: 682-885-7496.

Additional resources

The best way to take control of CF is to arm yourself with lots of healthy information. The links below can get you started:

Staying Healthy – This page has lots of information, including how nutrition can help a person with CF stay healthy.
Treatments – You'll find information on different therapies, including CF nutritional therapies.
Using Nutrition to Stay Healthy with CF – A CF Education Webcast.
Adult Nutrition – A CF Education Webcast.
Partnering for Care Series: Help Your Dietitian Help You Thrive – A CF Education Webcast.

Vitamins

Vitamins are needed for the normal growth, function, and health of our bodies. People with CF need extra vitamins for good health.

Vitamin A has many roles in health: normal vision, bone and tooth formation, cell function, and immunity. Vitamin A also works to help fight infections and to keep the intestines healthy.
Vitamin D helps to build and maintain strong bones and teeth. Without enough vitamin D, bones can become thin and brittle. People with CF are at risk for bone problems. So, it is important to get the right amounts of vitamin D every day.
Vitamin E is an antioxidant, which means that it protects compounds in the body from combining with oxygen. When compounds become oxidized, they become harmful to the body. Vitamin E helps to keep red blood cells healthy. It also helps to fight infection and maintain the health of the intestines.
Vitamin K is best known for its role in helping blood clot. It also helps to keep bones healthy.

Minerals

Minerals help with normal growth, function and maintenance of good health.

Calcium is an essential element that helps to build strong bones and teeth, as well as maintain nerves and muscles, and is found in dairy products and some green vegetables. Calcium supplements are available.
Iron is another essential element, which means that the body can't make it on its own, and is responsible in blood cells for carrying oxygen from the lungs to the body.
Sodium chloride is also called salt. They are minerals which play an important role in maintaining fluid balance in the body. Because people with CF lose more salt in their sweat than those who don't have CF, they must eat more salty foods, especially during hot, humid weather.
Zinc has many important functions in the body every day, from growth and healing to taste and appetite.

Minerals

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.

Recipes

CF-friendly recipes - The recipes are safe for healthy children and adults and people with cystic fibrosis. For specific recommendations on the right amount of energy, protein, and other nutrients for YOU/YOUR child, always contact your dietitian or doctor. These recipes aren't meant to replace the medical advice of your doctor or nutritional advice of your dietitian.

Exercise

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

Increase strength and endurance
Increase energy
Increase life expectancy
Improve airway clearance, which helps prevent respiratory infections
Increase bone density

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.

So how do you get the most benefit?

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.

Play it safe

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:

Talk with your physical therapist – creating the right plan for your particular needs can help to ensure that you not only get a health workout plan, but one that is fun!
Avoid dehydration – CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. Thus, you will want to stay hydrated by replacing fluids during and after your workout. Your doctor may also recommend a high-salt diet, snacks or salt supplements to maintain the balance of minerals in your blood.
Replace calories – Exercise increases the body's energy requirements, which are already quite high in people with cystic fibrosis. In order to prevent weight loss, it is important for people with CF to eat enough calories to replace the calories burned in exercise. In addition to your doctor and physical therapist, your exercise plan should be discussed with your dietitian who will recommend appropriate dietary additions. Our dietitians also have many, many recipes that they can provide you with, and you'll find additional recipes here.
Airway clearance – Many people with cystic fibrosis find that doing airway clearance treatments prior to physical activity helps to increase their exercise tolerance. As an added bonus, the exercise itself could loosen more secretions so it might be necessary to do another airway clearance treatment after the workout. Exercise also seems to help improve airway passages over the long term. In fact, in some cases, if you exercise regularly, you may be able to cut back on your CPT. However, you should check with your doctor first.

Infection prevention

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.

Attack germs before they attack you

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:

Keep vaccines up to date – they help prevent illness. And children aren't the only ones who need them. Parents, family members, caregivers and even close friends do too, because if they're around you or your child without protection, they could unknowingly spread viruses.
Use tissues. When people cough or sneeze without a tissue, the germs can travel up to 6 feet. Used tissue should also been discarded in a trash can right away.
Clean, disinfect and air-dry nebulizers to kill germs.
Don't share eating utensils, cups, soda cans or bottles.
Keep your hands clean. Always clean hands after shaking hands with someone else, after you cough, sneeze or blow your nose, anytime you touch automatic teller machines (ATMs), handrails, elevator buttons, public telephones, grocery carts other peoples' pens. You should also clean your hands when you're at the doctor's office, especially in the waiting room or lab, or after touching door knobs. If your hands are visibly dirty, use soap and water, otherwise, use a hand gel.
Avoid sick people – people with CF need to take extra care to avoid friends and family members who are ill. Even exposure to a common cold can cause uncommon problems. Family members living in the same home as someone with CF should also try to avoid sick people as well so that germs aren't sneaking in the door.

Take hand gel with you wherever you go. To clean your hands with hand gel:

Put a nickel-sized amount in the palm of one hand.
Rub gel on all parts of your hands, fingers and nails.
Rub your hands together until dry.
Do not rinse or dry your hands with a towel.

Hand washing steps:

Wet hands with warm water.
Apply soap. Liquid antibacterial soap is best.
Rub hands together to lather.
For 20 seconds, scrub back and front, up to the wrist and between fingers.
Clean under nails.
Rinse in warm running water.
Use a clean towel to dry hands. Paper towels are best
Using the paper towel, turn off the faucet and open the door.

5 ways to prevent infections

Clean your hands
Keep shots (immunizations) up to date – including flu vaccines
Use and throw away tissues
Clean and disinfect nebulizers
Avoid sick people
Do not share eating utensils, dishes, cups/glasses, straws, etc.

Inhaled medication

Several different types of inhaled medications are used in treating Cystic Fibrosis. They are:

Bronchodilators – opens up the airways – albuterol, Xopenex®, brovana®
Mucolytics – helps to thin the mucous – Pulmozyme®, Hyper-Sal™
Antibiotics – reduces the amount of bacteria in the lungs – tobramycin, Cayston®, colymicin
Corticosteroids – reduces inflammation when asthma symptoms present

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.

Aerosolized Colymicin (colistimethate)

Colymicin is a medication that treats Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).

Why should I take this treatment?
Colymicin fights the bacteria Pseudomonas aeruginosa which is the leading cause of declining lung function in people with CF. When used as part of a regular treatment regimen, it can reduce the number of days in the hospital, reduce the need for IV antibiotics, and help improve lung function.
Is Colymicin right for me or my child?
Colymicin is recommended for patients that have Pseudomonas aeruginosa in their sputum or throat cultures. Colymicin should be taken twice a day and follow the 28 days on/28 days off treatment cycle. You should not take Colymicin if you have ever had an allergic reaction to it before. Your CF care team can help make this decision.
What are the side effects of Colymicin?
Side effects of Colymicin may include coughing or wheezing. Pretreatment with a bronchodilator can help prevent this. If your symptoms are significant, you should tell your doctor.

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 (tobramycin inhalation solution)

TOBI is an inhaled medication, by nebulizer or dry powdered inhaler, that treats Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).

Why should I take this treatment?
TOBI fights the bacteria Pseudomonas aeruginosa which is the leading cause of declining lung function in people with CF. When used as part of a regular treatment regimen, it can reduce the number of days in the hospital, reduce the need for IV antibiotics, and help improve lung function.
Is TOBI right for me or my child?
TOBI is recommended for patients that have Pseudomonas aeruginosa in their sputum or throat cultures. TOBI should be taken twice a day and follow the 28 days on/28 days off treatment cycle. If you're allergic to antibiotics in the same family as TOBI (i.e. aminoglycosides), you should not take TOBI. Your CF care team can help make this decision.
What are the side effects of TOBI?
Side effects of TOBI may include coughing or temporary difficulty in breathing. Some people experience ringing in the ears, hearing loss, or changes in voice (hoarseness). If you have these symptoms, you should tell your doctor.

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 (DNase)

Pulmozyme® is used to thin and loosen the mucus in your lungs.

Why should I take this treatment?
Daily administration of Pulmozyme along with other standard therapies has been shown to improve lung functions. It helps reduce the risk of respiratory tract infections that require antibiotics.
Is Pulmozyme right for me or my child?
Pulmozyme is recommended for patients 6 years of age and older. The decision to start Pulmozyme is made after considering the potential for a benefit in lung function or in decreasing the risk of respiratory tract exacerbations. Your CF care team can help make this decision.
What are the side effects of Pulmozyme?
Side effects can include changes or loss of their voice, throat discomfort, cough, rash, red watery eyes, chest pain, dizziness, fever, or runny nose when first taken. The side effects are usually mild and short-lived.

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 (HYPER-SAL)

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.

Why should I take this treatment?
Studies have shown that 7% hypertonic saline helped improve lung function and decreased lung infections.
What are the side effects?
Side effects include chest tightness, and sore throat. After the first week of treatment, most symptoms should subside. Notify your doctor if they continue. A bronchodilator (albuterol or Xopenex) is recommended before every dose of HTS.
Is hypertonic saline right for me or my child?
Hypertonic saline is recommended for CF patients 6 years of age and older. It isn't advised for post-transplant patients or people who haven't tolerated the treatment. Your CF care team can help make this decision.

IMPORTANT INFORMATION:

Hypertonic saline should not be mixed with other medications.
Hypertonic saline should be used with a PARI LC PLUS jet nebulizer.

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 (aztreonam for inhalation solution)

Cayston is an inhaled antibiotic for the treatment of Pseudomonas aeruginosa infections in people with cystic fibrosis (CF).

Why should I take this treatment?
Cayston is used to improve respiratory symptoms in people with CF who have pseudomonas. Pseudomonas aeruginosa is the leading cause of declining lung function in people with CF. When used as part of a regular treatment regimen, it can reduce the number of days in the hospital, reduce the need for IV antibiotics, and help improve lung function.
Are there medicines that should be taken before Cayston?
Before taking Cayston, you should do some of your inhaled therapies. These include you bronchodilator (e.g. albuterol), airway clearance and your mucolytics (e.g. dornase alfa, hypertonic saline).
How can I obtain Cayston?
Cayston is available at select specialty pharmacies, including the CF Services Pharmacy. You can't get your Cayston prescription filled at your local pharmacy.
Can I use the Cayston Altera Nebulizer System for other inhaled antibiotics?
Your Cayston will come with an Altera Nebulizer System. The Altera nebulizer is only approved for use with Cayston. You should use the right nebulizer with the right drug. Using a different nebulizer could cause you to get too much or too little of the medicine.
What was the CF Foundation's role in developing Cayston?
Cayston, developed by Gilead Sciences, Inc. is the first CF drug to advance from beginning to end through the Foundation's Therapeutics Development Program.

For more information, contact your Cook Children's CF care team or visit the Cystic Fibrosis Foundation at www.CFF.org.

Lung transplant

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.

What happens in an evaluation?

While each center has specific criteria they use to determine transplant candidates, there are some general things they will be looking at:

Social support system including your family, friends, and other support that will be needed during a transplant.
Substance use including tobacco, alcohol, and illicit drugs.
Insurance and medical coverage.
Other important aspects will be your participation in your care, other medical problems, and psychological history/exams.

Potential medical tests may include:

Blood work: kidney and liver function, complete blood count (CBC), arterial blood gas(ABG), obtain blood type
Pulmonary function test
Sputum cultures
Chest x-ray
CT scan of chest and sinus
Bone density
Ventilation perfusion scan
Heart evaluation: Echocardiogram, Catheterization, Angiogram
PT evaluation
Ultrasound of abdomen

The results from the full evaluation will go before a Medical Review Board to be reviewed. The Board can decide a few things:

You are a good candidate for a transplant and can be placed on the national transplant waiting list and will be transplanted as soon as suitable lungs become available.
You don't need a transplant at this time and won't be placed on the transplant waiting list. You will continue to be followed by your CF Center and referred back to the transplant team at a later time.
You are in need of more tests before being placed on the national transplant waiting list.
You are not a good candidate for a transplant due to medical reasons or due to not meeting the standards set forth by the transplant center.

What happens after you're approved?

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.

After your transplant:

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.

After my transplant, will I be cured?

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.

We're here to help.

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.