CysticFibrosis

Question 1

T/F: CF mainly involves the exocrine glands

Answer 1

TRUE

Question 2

What is the typical presentation of CF?

Answer 2

Failure to thrive, recurrent pneumonia, meconium ileus, nasal polyps, uncontrolled asthma, chronic sinusitis, salty taste to the skin

Question 3

How is CF diagnosed?

Answer 3

One or more characteristic signs/symptoms, hx of CF in a sibling, positive newborn screening test and evidence of CFTR abnormality (sweat test or a known mutation)

Question 4

What does newborn screening measure?

Answer 4

Cationic trypsinogen

Question 5

CF pts need to have quarterly visits. What should be done each visit?

Answer 5

Vitals, hx, PE, review of therapies, detailed dietary hx, spirometry, lab work

Question 6

What are the GI manifestations of CF?

Answer 6

Deficient secretion of digestive enzymes, fat soluble vitamin deficiency, insulin deficiency, intestinal obstruction, reflux

Question 7

What is the classic symptom of maldigestion of nutrients in CF?

Answer 7

Steatorrhea

Question 8

What are symptoms of pancreatic insufficiency and how is this diagnosed?

Answer 8

Steatorrhea, frequent loose stools, flatulence, cramping, bloating, and voracious appetite. 72 hour fecal fat collection

Question 9

What is the treatment for enzyme deficiency?

Answer 9

Pancreatic enzyme replacements that contain lipase, protease, and amylase

Question 10

How often do pt with CF take enzyme replacements?

Answer 10

1000 lipase units /kg/meal. 500 lipase units/kg/snack

EVERYTIME THEY EAT

Question 11

What are the clinical issues related to enzyme replacement?

Answer 11

Inadequate enteric coating and poor acid resistance that leads to denaturing enzymes. Poor outcomes when switching between brand and generic name

Question 12

What are some long term GI- therapies

Answer 12

High calorie diet with added salt and fat soluble vitamin replacement

Question 13

What is DIOS? What are the symptoms and treatment?

Answer 13

Distal intestinal obstruction syndrome. Vomit, abdominal distention, and pain
Electrolyte lavage solutions

Question 14

GERD often accompanies CF. what are treatment options?

Answer 14

Antacids, H2 blockers, proton pump inhibitors

Question 15

Can proton pump inhibitors be taken with other GERD treatments? Why or why not?

Answer 15

NO, need an acidic environment

Question 16

What drug can decrease liver inflammation and bile duct proliferation?

Answer 16

Ursodeoxycholate

Question 17

What is the goal for the pulmonary components of CF

Answer 17

Decrease the long term rate of lung function decline

Question 18

What are treatments for the pulmonary involvement in CF?

Answer 18

Airway clearance techniques, anti-inflammatory agents, chronic antibiotics, mucolytics, and bronchodilators

Question 19

What does ACT involve and how often should it be done?

Answer 19

Chest/back percussion w/ vigorous coughing, flutter valve, exercise, and mechanical vest. BID

Question 20

What is a non-steroidal anti-inflammatory agent that decreases the rate of FEV1 decline?

Answer 20

Ibuprofen

Question 21

What is an antimicrobial and an anti-inflammatory agent that can be used in CF for pulmonary involvement?

Answer 21

Azithromycin

Question 22

How often is azithromycin given and what does is do?

Answer 22

3x weekly, slows decline in FEV1. in CF pt with pseudomonas colonization it can improve FEV1 by 6% and decrease pulmonary exacerbations, well tolerated drug

Question 23

What are the mucolytics?

Answer 23

Pulmozyme and mucomyst

Question 24

What does pulmozyme do and what are its ADR?

Answer 24

Decreases viscosity of sputum, modest, FEV1 improvement , ADR: hoarseness, voice alterations, and pharyngitis

Question 25

What are some problems with mucomyst?

Answer 25

Not typically used chronically, Irritating and causes bronchoconstriction, Smells real ratchet

Question 26

How does the nebulized hypertonic saline increase the ability to cough out mucus?

Answer 26

Draw H2O into the airways

Question 27

What is the major pulmonary pathogen in CF pts in the first year of life?

Answer 27

Staph aureus

Question 28

What is the major pulmonary pathogen in CF pts by age 3?

Answer 28

H. influenzae

Question 29

What is the major pulmonary pathogen in CF pts by age 5?

Answer 29

Colonized pseudomonas aeruginosa

Question 30

How are chronic antibiotics useful in CF?

Answer 30

They prolong the time between acute exacerbations

Question 31

What are the chronic antibiotics used for colonized P. aeruginosa and how are they given?

Answer 31

Tobramycin: BID 28 days on, 28 days off, Cayston: TID 28 days on and 28 days off, Treated regardless of infection status

Question 32

What are the signs and symptoms of acute pulmonary exacerbations in CF pts?

Answer 32

Increased: cough frequency and duration, chest congestion, and sputum production, Decreased: exercise tolerance, PFTS, weight, O2 sat, Use of accessory muscle use and +/- fever

Question 33

What is the goal of therapy for acute pulmonary exacerbation?

Answer 33

Decrease pulmonary signs and symptoms, Eradicate P. aeuroginosa

Question 34

When do you dose at the upper end of the range in CF pts?

Answer 34

When they have increased volume distribution and increased clearance of the drug

Question 35

What is the treatment for acute pulmonary exacerbations?

Answer 35

Typically aminoglycosides (systemic) and antipseudomonal penicillin