Managing CF

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:

• More energy is used in breathing.
• Extra energy is used in fighting infections and during fevers.
• Fewer of the energy-producing nutrients in food are properly digested and absorbed into the body, even when treatment is optimal.

Research has shown that good nutrition is important for pulmonary function, and can contribute to a longer life expectancy.

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:

• Assess individual needs
• Adjust for clinical status
• Factor in age-related needs

A comprehensive nutritional management plan developed jointly by the CF Team, the person with CF, and the family is needed.

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.

• SVC: (slow vital capacity)
• FVC: (forced vital capacity) This measures the volume of air that is blown out in a single maximum exhaled breath. It is inversely related to the amount of air trapped by obstruction. The worse the airway obstruction, the less the FVC.
• FeV1: (forced expiratory volume) This measures how fast the lungs can push air out in one second. Your result is measured against a predicted result, a mean of values in healthy people of the same sex, height, and weight. The result is written as the percent of the predicted result. The less air expired in the first second, the higher the level of airway obstruction.

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 well my lungs are working
• If antibiotics are helping to clear up an infection
• If the current daily care program is adequate

How often should they be done?

• Spirometry is initiated at 5 or 6 years of age or whenever a child can understand the instructions and cooperate. After that, spirometry is done at every clinic visit so that the clinician can evaluate your progress. Research is being done on methods to measure infants.
• Full PFTs are done on an annual basis. They are extremely accurate monitors of change in lung function, even when no new symptoms or signs have emerged.

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.

• FVC: (forced vital capacity) After several slow deep breaths in, you breathe out as hard as you can for six full seconds. This measures the volume of air that is blown out in a single maximum exhaled breath.
• FeV1: (forced expiratory volume) After several slow deep breaths in, you blast all of the air out of your lungs as fast as possible in one long burst. This measures how fast the lungs can push air out in one second.
  

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?

1. Remove the cap and hold the inhaler upright.
2. Place the spacer, if used, on the inhaler.
   
3. Shake the inhaler.
   
4. Tilt your head back a bit. Breathe in and breathe out. Then place the spacer or mouthpiece in your mouth.
   
5. Press down on the inhaler to release the medicine. This is called one "puff."
   
6. Start breathing in SLOWLY.
   
7. Hold your breath for 10 seconds to allow the medicine to reach deeply into the lungs.
8. Repeat puffs as prescribed. If you can, try to wait a minute in between puffs.

How to avoid common inhaler mistakes