Cystic Fibrosis

WHAT YOU SHOULD KNOW:

• Cystic fibrosis, also called CF, is a condition where your exocrine glands do not work normally. Exocrine glands make fluids such as sweat, mucus, tears, and saliva (spit). With CF, the fluids made are too thick and sticky, and can clog-up ducts inside body organs. It is caused by problems with the gene that affects normal functions of your body. Your parents may have passed on genes that cause CF. Signs and symptoms include frequent cough, wheezing, and shortness of breath that gets worse through time. You may have frequent respiratory system infections or repeated illness with pancreatitis. You may also have problems breaking down and absorbing food, losing weight, and feeling very weak. You may have a hard time getting your female partner pregnant.
  
  
• To diagnose CF, you may need blood tests, genetic testing, chest x-ray, and a sweat chloride test. There is no cure for CF and the treatments given are to prevent you from getting worse. You may be given medicines to help you breathe easier and kill germs causing any infection. You may need breathing exercises and special devices to help remove mucus and let you breathe easier. You may need medicines to help break down and absorb nutrients in food properly. Your caregiver may put you on a special diet and give you extra vitamins. These diet changes should help you gain weight and have more energy. You may need surgery to repair, or completely replace damaged organs, such as you lungs or liver. Diagnosing and treating your CF as soon as possible may help relieve problems with breathing and digestion.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

RISKS:

• Treatment of your CF may cause side effects. You may get an allergy with your medicines and have itching, swelling, and redness of your skin. You may have an infection or bleeding with surgery. Even after successful treatment, your symptoms may not disappear at once, may still continue, or may come back later on.
  
  
• If left untreated, your organs may continue to get damaged and lose their function over time. You may not get enough nutrients and you may lose weight, and get very weak. Your bones may get very weak and you may have an increased risk for fractures (broken bones). You may get pancreatitis or have diabetes if your pancreas gets damaged very badly. Your liver can have cirrhosis (scarring) and not work normally. You may get ongoing damage to your lungs. You may have trouble breathing, or get bleeding, collapse of a lung, or widening of your airways. Infections in your lungs can happen often and cause more trouble breathing or even death. Call your caregiver if you have questions about your condition, medicines, or care.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that you may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Diet:

You may need a special diet to help you gain weight and have good nutrition. A caregiver called a dietician will help you choose the right foods to help you get back your energy and strength. You may be given extra nutrients such as vitamins A, D, E, and K, or calcium.

Oxygen:

You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your caregiver before you take off the mask or oxygen tubing.

Medicines:

You may be given the following medicines:

• Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.
  
  
• Bronchodilators: You may need bronchodilators to help open the air passages in your lungs, and help you breathe more easily.
  
  
• Mucus thinning medicine: This is a medicine you inhale (breathe in) to help thin the mucus in your respiratory system.
  
  
• Pancreatic enzymes: These chemicals help your digestive system break down food and absorb nutrients properly.
  
  
• Steroids: Steroid medicine may be given for a short time to decrease inflammation (swelling), and help you breathe better.

Tests:

• Blood tests: A sample of your blood is sent to a lab for tests. It can be used to check for problems such as infections or damage to your organs. Your blood may also be tested to check your health condition and to examine the gene that causes CF.
  
  
• Chest CT scan: This is also called a CAT scan. A special x-ray machine uses a computer to take pictures of your lungs and airway structures. Caregivers will look to see if you have bronchiectasis (widening and damage in large airways) caused by mucus blockage.
  
  
• Fecal fat test; Caregivers may ask for a sample of your stool to test its fat content. With CF, your body may not be able to absorb fat from the foods you eat.
  
  
• Pulmonary function tests: Pulmonary function tests (PFTs) help caregivers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. PFTs help your caregivers decide the best treatment for you.
  
  
• Sputum sample: Sputum (mucus from your lungs) is collected in a cup when you cough. The sample is sent to a lab to be tested for the germ that is causing your illness. It can also help your caregiver choose the best medicine to treat the infection.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.

Treatment options:

Your treatment may change if your health problem is not being controlled. This is often decided after you have tests. You may have some of the following treatments alone or together.

• Airway clearance techniques: The thick mucus in your lungs caused by CF can lead to infections and lung damage. Your caregiver may teach you special exercises to help remove mucus and let you breathe easier. These exercises may be used along with machines or special devices to help decrease your symptoms. These therapies may take some time and effort on your part. You and your caregivers will need to find the techniques and schedule that works best for you. Doing these on a set schedule can help decrease your symptoms quicker and help you heal faster. You may have one or more of the following:
  
  
  • Active cycle breathing techniques: These involve controlled breathing, forced breathing, and chest exercises. Controlled breathing involves minding each breath and breathing gently, slowly, and deeply. Sometimes, you may need to breathe out air as fast as you can, to force out mucus in your airways. Chest exercises help you fill your lungs with air completely by letting your abdomen swell as you breathe. Minding how your abdomen moves increases the amount of air you can breathe in.
    
    
  • Autogenic drainage: This is a breathing technique used to bring up mucus from lower parts of your airways or lungs. In this exercise you will sit up straight and slowly breathe in through your nose, to fill your lungs. You will hold your breath for 3 to 4 seconds, and breathe out as fast as you can through your nose. You may need to repeat this exercise several times to push out the mucus.
    
    
  • High frequency chest wall oscillation: You will need to wear a special vest that vibrates to loosen up mucus in your airways.
    
    
  • Oscillating positive end pressure therapy: This uses a pocket sized device that controls your breath as you breathe out through it. You will need to breathe in and fill your lungs, and hold your breath for 2 to 3 seconds. You will put the device tightly to your mouth and breathe out normally through it. This device may seem to vibrate, or flutter as the air goes out in a controlled way. This helps remove mucus and help you breathe easier.
    
    
  • Percussion: This involves a caregiver forcefully pat on your back with a cupped hand or soft plastic cup. This is used to loosen mucus stuck in your airways. This may look to be uncomfortable, but really is not. Percussion may also be done with an electric percussor.
    
    
  • Positive expiratory pressure therapy: This is used to decrease the amount of air you breathe out. This leaves some air in your small airways, so that they do not close up when you breathe out. A mask attached to a machine is put over your face to help control your breathing. Your caregiver sets the machine and watches you during this procedure.
    
    
  • Postural drainage (PD): This treatment uses body position and gravity to help bring up sputum (mucus) from your lungs. Your caregiver will place you in different positions to help the sputum drain to larger air passages. Then you can cough it out more easily. During postural drainage, your caregiver may also lightly clap on your back and chest with their hands, or use a small machine that vibrates on your skin. This breaks up the sputum in your lungs, making it easier to cough up. Postural drainage may make it easier for you to breathe, decrease the chance of infection, and help you get better faster.
  
  
• Genetic counseling: Your caregiver will help you or a family member learn why CF happened. If you plan to have a baby, you may find out about the risk of your child having CF.
  
  
• Surgery: Organs damaged by CF can be repaired through surgery. If your lungs or liver get badly damaged by CF, you may need a lung or liver transplant.
  
  
• Ventilator: This is a machine that gives you oxygen and breathes for you when you cannot breathe well on your own. An endotracheal (ET) tube is put into your airway through your mouth or nose. You may need a trach if an ET tube cannot be placed. A trach is an airway tube put into an incision (cut) in the front of your neck. The ET tube or trach is attached to the ventilator.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

Learn more about Cystic Fibrosis (Inpatient Care)