Cystic Fibrosis is complicated to manage. Click on a topic to read articles on airway clearance techniques, nutrition, updates on devices, or advice about CF complications.
Nutrition and CF
Good nutrition for a child or adult with CF means more than just eating a balanced, normal diet. A high-calorie diet containing all the essential nutrients without restriction on fat intake is recommended, along with pancreatic enzymes to control digestive symptoms, and supplemental fat-soluble vitamins: A, D, E, and K. To maintain weight, frequent and high-calorie meals and snacks are recommended.
What is a " good diet " for someone with CF?
Nutritional requirements for each person with CF are unique; there is no universal recommendation that applies to everyone. Requirements and recommendations depend on many factors including age, gender, severity of lung disease, malabsorption and the type of food a person likes. But there are some general recommendations for anyone with CF:
More Calories
Most people with CF have a higher caloric requirement than other individuals of the same age and sex because:
Vitamins
Vitamins A,D, E, and K are known as the fat-soluble vitamins. These vitamins require an adequate amount of fat to be absorbed properly. Because people with CF can have problems with absorption of fat, it is important to replace these vitamins.
Pancreatic Enzymes
CF causes production of a thick mucus that plugs the duct leading from the pancreas to the small intestine. Consequently, the enzymes produced by the pancreas to help digest food cannot move into the small intestine. If left untreated, one result is malabsorption, an inability to properly absorb nutrients. To compensate for this enzyme shortage, many people with CF must take replacement pancreatic enzymes.
When Do I Take Enzymes?
Enzymes must be taken to help digest every meal and snack, except for snacks that are virtually free of protein, starch, and fat (such as apple juice). Studies show that enzyme preparations are equally effective when taken anywhere from a half-hour before to a half-hour after eating. That way, the enzymes are in the small intestine when needed.
What If I Can't Eat Enough Calories?
Some people with CF cannot take in enough calories by mouth to gain or maintain weight. For them, calories and all the essential nutrients can be given by tube feedings. Many achieve excellent nutrition through this method.
Is There Any Help With Nutritional Products?
The Scandipharm Comprehensive Care Program for CFTM provides free ADEKs® multivitamins, Scandishake® nutritional supplements, and Flutter® mucus-clearance devices to people taking Ultrase®. The program enhances Scandipharm's mission of improving the overall quality of care, while lowering its cost. For more information on this program, talk to one of the CF team members.
How Can I Manage CF and Nutrition?
The key to managing nutritional needs successfully is to match dietary needs to each individual:
Pulmonary Function Tests
What are Pulmonary Function Tests (PFTs)?
Pulmonary function tests are machine assessments of the performance of the lungs, measuring basic function and volume. Many of the tests are done in a special Pulmonary Function Laboratory. There are various computerized machines that answer very specific questions about lung function.
Some Common Tests:
Spirometry: tests done at every clinic visit to determine the vital capacity of the lungs. Spirometry measures the amount of air breathed and the speed at which it is blown out.
Lung Volume Measurements: these indicate the size of the lungs and the amount of air remaining inside after exhalation
Exercise testing: Problems may become more apparent during exercise, therefore, sometimes PFTs are measured during treadmill or other exercise while heart and respiratory rates are monitored.
Blood oxygenation measures(ABGs): These indicate how effective the lungs are in bringing oxygen into the bloodstream and removing carbon dioxide from it.
What do these tests show in my lungs?
How often should they be done?
How some of the spirometry tests are done:
A spirometer is a tube leading into a pneumotach which measures air flow and volume. The results are graphed and displayed on the computer monitor. In order to make accurate measurements, nose plugs are worn to close the nasal passages so that the air can only be blown out through the mouth.
Metered Dose Inhaler
What is a Metered Dose Inhaler (MDI)?
An MDI is device that sends a measured amount of medicine directly into the lungs. Sometimes it is used with a spacer to make it easier to use. The spacer can help to deliver the medicine to the lungs and lessen the bad taste of the medicine. |
How do I use an MDI?
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How to avoid common inhaler mistakes
How to clean your inhaler
It is very important to clean your inhaler and spacer regularly. Without cleaning, a build-up can occur which will stop the medicine from being delivered.
What else do I need to know?
It is important to monitor expiration dates and the amount of medication remaining.
How can I tell how much medicine is left?
InspirEase and E-Z Spacer
These spacers are especially helpful in delivering medications to children. After attaching the medication canister and pressing down, medicine is released into a bag which collapses when the child breathes in. Here are the steps to using the InspirEase. Be sure to read the directions that come with your spacer.
Nebulizers
What is a nebulizer?
A nebulizer is a device driven by a compressed air machine that changes a liquid medicine into a mist so it can be breathed into the lungs. It consists of a cup, a mouthpiece attached to a T-shaped part or a mask, and thin, plastic tubing to connect to the compressed air machine.
Important points to remember:
How to use a nebulizer
Caring for your nebulizer
After each use: rinse
Once every day: wash
Wash the mask or mouthpiece and T-shaped part with a mild dishwashing soap and hot water. If your manufacturer's instructions allow, you may use an automatic dishwasher. Rinse well.
Once or twice a week after washing and rinsing: disinfect
Measuring Peak Flow
What is a Peak Flow Meter?
A peak flow meter is a simple device that measures the amount of air you are able to exhale quickly. This can help show whether your airways are constricted (narrowed). If you can expel a lot of air quickly, your bronchial tubes are open. Regular peak flow monitoring can sometimes detect loss of lung function even before you notice the symptoms.
How to Take a Peak Flow Reading
How to Care for Your Peak Flow Meter
If Your Child Is Sick
All children occasionally get sick. It is usually OK to first try common remedies such as
Handwashing: Your Mother Was Right
" Hand washing is the single most effective way to prevent the spread of communicable diseases ", Center for Disease Control
Why is hand washing important?
The most important thing that you can do to keep from getting sick is to wash your hands.
By frequently washing your hands you wash away germs that you have picked up from other people or from contaminated surfaces.
What is good hand washing technique?
There is more to hand washing than you think! By rubbing your hands vigorously with soapy water, you pull the dirt and the oily soils free from your skin. The soapy lather suspends both the dirt and the germs trapped inside. And then the rinse water quickly washes it all away.
Cystic Fibrosis Related Diabetes
What Is It?
Diabetes is a disease that may occur in a person with Cystic Fibrosis because of
What Happens?
People may become glucose intolerant, that is, less able to clear glucose from the bloodstream. The body is then unable to use or store food effectively; so the blood sugar level rises and can cause damage to many parts of the body.
How Would I Know if I Had It?
There may be no noticeable symptoms. Random blood or urine glucose levels may be normal.The oral glucose tolerance test (OGTT) is the most reliable method of identification. This test involves drinking a glucose (sugar) solution and measuring blood glucose at baseline and two hours later. This test should be considered for CF patients over the age of 16 on an annual basis.
What Can Be Done if I Develop Diabetes?
Management usually includes dietary modification in order to regulate the types of food eaten and the timing of food intake. In addition, insulin or oral medications may be given to lower blood sugar. Blood glucose values can be monitored at home to determine if the glucose levels are acceptable.
Vascular Access Devices
Vascular access devices are tools designed for the administration of medications, I.V. fluids, parenteral nutrition solutions, and blood products. They can also be used to take a blood sample. Some are totally implanted, such as ports, and can remain in the body for 3-5 years. Others, such as PICC lines and tunneled catheters, can be seen and accessed from the surface of the body.
Why have a vascular access device?
After repeated courses of intravenous medications, veins may become scarred, and it may become more and more difficult to find veins suitable for blood tests and administration of medication. Several different devices and procedures have been developed to provide improved access to veins.
Who should consider vascular access?
Some common types of vascular access devices
Implanted port
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Implantable ports: This small device (about the diameter of a quarter and about a half inch thick) is completely under the skin, and out of the way. You will still feel a small bubble, however. It is connected to a catheter (a small flexible tube) that is placed in a large vein within the chest. You can also feel the catheter tunneled under the skin until it enters the vein. |
Tunneled Central Venous Catheters (TCVC):
The advantage of these catheters is their larger diameter, which is helpful for situations where good blood flow is required.
Tunneled catheter
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The TCVC is inserted into the vascular system, then a portion of the tubing is tunneled through the subcutaneous space and exited through the skin at a convenient location on the chest. A dacron cuff just above the exit site acts as a barrier against infection and prevents the catheter from slipping out. |
Other names of Tunneled Central Venous Catheters: Hickman, Broviac.
PICC Lines:
Peripherally Inserted Central Venous Catheters (PICC) consist of a thin plastic line that is placed into one of the large antecubital veins (inner elbow area, where blood is commonly drawn) and is threaded into the superior vena cava above the right atrium of the heart. What's nice about this catheter is that it doesn't require a surgeon to insert - an IV nurse can do it. It can also be removed by a nurse. The catheter can stay in for as long as a year. Disadvantages: The catheter needs to be flushed about once a week, if not used. With repeated use, veins can become scarred and unusable. An x-ray is needed to check the placement of a centrally placed line.
Landmark catheter:
A short catheter (3-6 inches long) that is placed into the vein with a needle. It is usually placed in the lower arm or inside the elbow. Landmark catheters usually last up to several weeks. Usually does not require an x-ray afterwards for placement.
Maintaining an Implanted Vascular Access Port
Vascular access ports are totally implantable devices that permit the infusion of medications, parenteral nutrition solutions, blood products and other fluids; ports are also useful for blood sampling. Implanted ports were developed to help provide reliable vascular access for patients who require long-term drug or fluid therapy. Ports are available in a variety of materials and designs; some maintenance is required to use these devices.
Flushing a port during intermittent infusions:
During intermittent infusions, use 5cc's of Heparin ( 10 U/cc) to flush a port.Flushing a port that is not being accessed:
When not accessed, ports are flushed once a month with 5 cc's of Heparin ( 100 U/cc). The exception to this is a Groshong Implanted Port which is flushed monthly with 10cc's of Heparin ( 100 U/cc).Site preparation prior to flushing:
Application of EMLA cream to numb the site 1 hour before access is highly recommended.
PORT ACCESS IS A STERILE PROCEDURE. Ports may be accessed only with a non-coring (Huber) needle. Care must be taken to follow accessing instructions carefully.
Equipment needed:Instructions:
- Non-coring (Huber) needle of the proper size. Use 22g x 1 inch if unsure.
- Normal saline
- Heparin flush solution 10u/cc
- 12cc syringes with needles
- Alcohol preps
- Sterile gloves
- Sterile field
- 3 Persist swabs
- Biopatch
- TegadermHP Transparent Dressing
- 3 packages 2x2's
- Tape
- Extra needles, 25 gauge 5/8"
- Injection cap
- Safety pin
Conditions to Watch For:
The site should remain clean and dry. If a port dressing becomes wet or soiled, change the dressing immediately. Watch the site carefully for any signs or symptoms of infection/phlebitis.
Possible complications:
VENOUS THROMBOSIS: a clot can form around the catheter
- Pain in the arm, neck, and/or shoulder which is noted on the same side of the body as where the port is inserted.
- Swelling may be noted above the clavicle, neck, and/or face.
CATHETER CLOTS: the catheter becomes blocked.
AIR EMBOLISM:
If the pump reads occlusion or high pressure and you have checked to make sure that there are no kinks in the line or the clamp is not on, try to flush the line with normal saline. If it flushes sluggishly, gently heparin lock the line and call the MD.CATHETER EMBOLUS: A piece of the catheter may tear off and enter the blood stream.
- Patient may feel chest pain or pain with deep inspiration.
- Shortness of breath or breathing rate increases.
- Skin may appear pale of blue colored around the lips and nailbeds.
- Coughing noted.
- Patient may feel dizzy or faint.
- Patient may have chest pain, especially with deep inspiration.
- Patient may have shortness of breath and breathing rate increases.
- Skin may appear pale, or blue color may be noted around the lips and nailbeds.