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Cystic Fibrosis
Christa QuintosElysia Becerra
Laura PerezShirley Mendence
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What is Cystic Fibrosis?
Genetic, autosomal recessive, multiorgan disease
Caused by:- mutations to CF gene (chromosome 7)
- Abnormal expression of “cystic fibrosis transmembrane conductance regulator” (CFTR) protein
- defective epithelial Cl- transport - thick mucus secretions
- Airways - GI tract- Liver ducts- Pancreas- Sweat glands
Prevalence: - Disease= 1/3500 whites- Carrier= 1/29 whites
Median Dx age: 6 monthsMedian life expectancy: 37 years
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CF Complications
1. Lungsa. Chronic respiratory infections
2. GIa. Pancreas - symptomatic DM1b. Liver - blocked bile
3. Reproductivea. Vas deferens agenesis
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Signs & Symptoms
1. Lungs/Respiratory:a. Cough is the most common symptomb. Viscous, purulent, yellow or green sputumc. Wheezing or shortness of breathd. Frequent lung infections; pneumonia, bronchitis, bronchiectasis, and
pneumothorax, which could lead to respiratory failure
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Signs & Symptoms
2. G.I:
a. Poor growth or weight gainb. Malabsorption of fat, protein and fat-soluble vitaminc. Frequent,greasy, foul-smelling stoold. Very salty-tasting skine. CF-related diabetes (CFRD),unique to CFf. Liver and Gallbladder damaged by mucus
g. Distal Intestinal Obstruction Syndrome (DIOS)
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Signs & Symptoms
3. Reproductive:
a. Both male and female have delayed puberty.b. Menstrual irregularities c. Male infertility, female difficulty conceiving.
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Diagnostic Procedures
1. Clinical presentation, family history,
laboratory testing, and genetic tests
2. The sweat chloride test; gold standard
-Genetic test is required if sweat test is unclear.
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Treatment / Therapy
● Inhaled medications○ Bronchodilators
■ Widens airways (bronchi) by relaxing muscles lining airway walls.
■ Taken before ACTs.○ Mucolytics (Mucus thinners)
■ Thins & moves mucus out of the airways so it can be coughed out.
■ Taken before ACTs.■ E.g Dornase alfa (Pulmozyme®)■ E.g.Hypertonic Saline
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Treatment / Therapy
● Airway Clearance Techniques (ACTs)○ Use percussion (clapping) & vibration to loosen and move the
mucus to the larger airways where it can be coughed or huffed out of the body.■ CPT (Chest Physical Therapy) ■ High Frequency Chest Wall Oscillation (the vest)
○ Huff Coughing■ Helps move mucus from the lungs.
■ Involves taking a breath in, holding it and actively exhaling.
Breathing in & holding it enables air to get behind the
mucus & separates it from the lung wall so it can be blown out.
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Treatment / Therapy
○ Antibiotics■ Taken after ACTs are finished & lungs are clear of mucus.■ ACTs allow antibiotics to reach deeply into the smaller airways to attack bacteria. ■ Come in 3 different forms
● Oral antibiotics○ Liquids, tablets or capsules that must be swallowed
● Intravenous (IV) antibiotics
○ Liquid medicine that goes directly into the blood through an IV catheter. An IV may require a hospital stay, but can also be done at home.
● Inhaled antibiotics○ An aerosol or mist that can reach the airways directly.
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Treatment / Therapy
● Fitness○ Better lung function
■ Regular exercise can improve your ability to get mucus out of your airways.
● Lung Transplant (long term intervention)○ Can extend & improve patient’s quality of life
○ Involves an extensive evaluation process & a
commitment to living the lifestyle required to keep your new lungs healthy.
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Nutrition & Teaching - Pancreatic Enzymes
● Do not take generic pancreatic enzymes. ● Supplemental enzymes must be included with
every meal or snack.● Take just before eating - avoid skipping ● Always take the right dose● Keep at room temperature
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Nutrition & Teaching - Vitamins & Minerals
Health & Strong Bones
● Vitamin A, D, E, & K○ Egg yolks, fatty fishes
● Calcium○ 1000-1300 mg daily○ whole milk, yogurt, & cheese
● Zinc & protein○ meats, eggs, & nuts
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Nutrition & Teaching - Vitamins & Minerals
● Iron: carry O2 from lungs to the body● Sodium: eat more salty foods when the weather is
hot○ soups, pickles, pretzels or chips as a way to get extra salt
● Fat-soluble, essential fatty acids, and calcium are the greatest concern.○ multivitamin & fat soluble vitamin supplements are
routinely recommended
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Nutrition & Teaching - Fats, Proteins, & Carbs
● Extra intake of 500 kcal/day to gain weight● Eat foods that are high-calorie, high-fat
○ 35%-40% kcal come from fat○ top ice cream, yogurt, or pudding with fresh fruit
○ add vegetables to pizza, lettuce and tomato to sandwiches, and shredded carrots to spaghetti sauce
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Nutrition & Teaching - Fats, Proteins, & Carbs
● Protein○ meat and meat products, milk and milk products,
fish, seafood, soy products such as tofu, beans, eggs and nuts
● Encourage frequent meals/snacks; supplement milk shakes or liquid dietary supplements
● Avoid: Alcohol and caffeine, carbonated drinks, tobacco
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Quick Knowledge Check!
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Quiz Me, Baby!1. Cystic Fibrosis is caused by defective
transport of?A. K+
B. Cl-
C. Mg2+
D. Ca2+
2. CF affects which body system?A. LungsB. GIC. ReproductiveD. All of the above
3. Which therapy/treatment should be done first?A. Antibiotics B. Airway clearance Techniques
(ACTs) C. Bronchodilators & MycolyticsD. It does not matter, they can be
done in any order.
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Quiz Me More, Baby!
4. What is the Gold Standard diagnostic test for CF?A. DNA testB. Sputum analysisC. Sweat Chloride testD. A1C test
5. What must a patient take before taking any meals?A. AntibioticsB. Pancreatic enzymesC. MultivitaminsD. Bronchodilators
6. What type of diet must a person with CF follow?A. Vegetarian dietB. Low-fat dietC. High-calorie dietD. Renal-diet
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ReferencesCFChef. (2016). Important facts for adults this CF. Retrieved from https://www.chef4cf.com/nutrition-guide/healthy-eating/adult.html.
CFF. (n.d.). About Cystic Fibrosis. Retrieved from https://www.cff.org/What-is-CF/About-Cystic-Fibrosis.
CFF. (N.d.). Enzymes. Retrieved from https://www.cff.org/Living-with-CF/Treatments-and-Therapies/Nutrition/Enzymes.
Huether, S. & McCance, K. (2012). Understanding pathophysiology. St. Louis, Mo: Elsevier. p. 718-720.
Lewis S.L., Dirksen S.R., Heitkemper M.M., Bucher L. & Camera, I.M. (2013). Medical Surgical Nursing. Assessment and Management of Clinical Problems (9th ed.). St. Louis, MO: Mosby Elsevier.
Rolfes, S. R., Pinna, K., & Whitney, E. (2012). Understanding Normal and Clinical Nutrition (9th ed.). Belmont, CA: Wadsworth Cengage Learning. p. 738-739.