inherited disease that causes thick, stick mucus to form in the lungs, pancreas and other organs.

In the lungs, this mucus blocks the airways, causing lung damage and making it hard to breathe. In the pancreas, it clogs the pathways leading to the digestive system, interfering with proper digestion

Antibiotics to prevent and treat lung and sinus infections

Inhaled medicines to help open the airways (bronchodilators)

DNAse enzyme replacement therapy to thin mucus and make it easier to cough up

Lung transplant, in some cases

Pancreatic enzymes to replace those that are missing

Vitamin supplements, especially the fat-soluble vitamins A, D, E, and K

What are the three most common organisms that cause lung infections in CF patients?

Pseudomonas aeruginosa

Staph aureus

Haemophilus influenzae

What two drugs are given should a patient have a P. aeruginosa infections

2 drugs given IV; these could be aminoglycosides, beta lactams, quinolones, and oters than cover pseudomonas

Indicated in CF patients ≥ 6 years who are colonized with Pseudomonas to reduce infection/hospitalization

TOBI should be taken using only the ____ resuable nebulizer and _______ air compressor

PARI LC PLUS;

DeVilbiss Pumo-Aide

TOBI comes in a plastic vial called an ampule. Each foil pounch contains 4 ampules (2 days worth). These should be stored in the refrigerator; however, they can be stored at room temp for up to 28 days. If it is not refrigerated, the product may darken with age. Color change does not indicate change in the quality of the product.

Take doses as close to 12 hours apart (and not less than 6 hours apart)

If you are taking several medications, the recommended order is as follows: bronchodilator first, chest physiotherapy next, then other inhaled medications, and finally, TOBI.

Sit or stand in an upright position during your treatment. Place mouthpiece between your teeth and on top of your tongue and breathe normally only through your mouth (use nose clips if you have difficulty breathing through your mouth)

Name a mucolytic agent used to treat CF that is also used for APAP toxicity?

Acetylcystenine (Mucomyst) is a mucolytic that is also used for APAP toxicity. As a mucolytic, it is given in a nebulizer. It has questionable benefit in CF as it may damage lung tissue and promote bronchitis, whereas Pulmozyme does not and is the preferred mucolytic agent.

Bronchodilators (albuterol or other short - acting beta 2 agonist) are given prior to chest percussion, which clears mucus. ___ ____ are always given last.

Dietary measures must ensure adequate nutrition and include high calorie, high protein, high-fat diets with liberal use of salt to encourage normal weight and growth. The needs are high due to the extra work involved with breathing and a hyper-metabolic state associated with bronchial infection. Due to poor fat absorption, patients use 1-2 multivitamins daily, and some require additional doses

They get to eat all the good stuff!!!!

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YES! Thick mucus obstructs pancreatic enzyme flow, resulting in a paucity of these enzymes reaching the gastrointestinal tract. Frequently, greasy, foul-smelling stools are manifestations of this pancreatic insufficiency. Most CF patients need to supplement their diet with appropriate amounts of pancreatic enzyme supplements

Enzymes are given at meals, with 1/2 doses given with snacks. Meals with high fat content require higher doses

Take the entire dose at the beginning of each meal

Do not substitute pacreatic enzyme products. This is an FDA recommendation. They do not require refrigeration

Creon

Pancreaze

Zenpep