Cystic Fibrosis

Christa QuintosElysia Becerra

Laura PerezShirley Mendence

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

CF Complications

1. Lungsa. Chronic respiratory infections

2. GIa. Pancreas - symptomatic DM1b. Liver - blocked bile

3. Reproductivea. Vas deferens agenesis

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

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)

Signs & Symptoms

3. Reproductive:

a. Both male and female have delayed puberty.b. Menstrual irregularities c. Male infertility, female difficulty conceiving.

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.

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

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.

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.

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.

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

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

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

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

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

Quick Knowledge Check!

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.

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

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.