Cystic Fibrosis part one

Question 1

CF is the most Most common life-limiting genetic disorder in which population?

Answer 1

caucasian

Question 2

expected life span of someone with CF

Answer 2

about 40 years

Question 3

CF Goals of treatment

Answer 3

Slow or stop the progression of the disease and Allow for normal growth and development

Question 4

Approaches to care for CF

Answer 4

good nutrition, Pancreatic enzymes and vitamin supplementation, Airway clearance and anti-inflammatory therapies, Antipseudomonal agents, Recognize altered pharmacokinetics

Question 5

examples of chest physiotherapy

Answer 5

postal drainage, chest percussion, vibration technique devices

Question 6

clearance therapy is for

Answer 6

for individuals ≥6 years and are administered concurrently with percussion therapy:

Question 7

what is the purpose of a bronchodilator?

Answer 7

Albuterol helps open up the airways and prevents bronchospasm

Question 8

what is the purpose of Hypertonic saline (HyperSal®)?

how does it work?

Answer 8

hydrates the airway mucus secretions and facilitates mucociliary function
draws water from the airway to establish aqueous surface layer (ASL)

Question 9

what is the purpose of Domase alfa (Pulmozyme®)?

Answer 9

this enzyme cleaves extracellular DNA which results in decreased viscosity of mucus therefore airflow in the lung is improved and risk of bacterial infection may be decreased

very $$$$$

Question 10

when are aerosolized antibiotics used? when are they indicated

Answer 10

administered after the CF patient completes percussion therapy
indicated based on severity of lung disease and sputum cultures

Question 11

Albuterol is a

Answer 11

Βeta-2 adrenergic agonists bronchodilator

Question 12

Theophylline is an

Answer 12

Anti-Obstructive-Bronchodilators that is infrequently used due to Lack of proven efficacy, narrow therapeutic margin, GI symptoms and Tachycardia

Question 13

what are examples of Anticholinergic anti obstructive bronchodilators?

Answer 13

Ipratropium – short acting

Tiotropium – long acting

Question 14

Tiotropium shows no

Answer 14

significant improvement

Question 15

In Cf there is a presence of abundant, purulent airway secretions composed primarily of what?

Answer 15

highly polymerized DNA which produces a viscous mucous

Question 16

principal source of highly polymerized DNA is from

Answer 16

the nuclei of degenerating neutrophils which is present in large concentrations in infected lung secretions

Question 17

in CF the decreased mucociliary transport and persistent infections is due to what?

Answer 17

large amounts of viscous mucous

Question 18

what are the main ADE of Dornase alpha?

Answer 18

chest pain, fever, rash, pharyngitis, rhinitis, dyspepsia, conjunctivitis, laryngitis

Question 19

is Chronic treatment with oral antibiotics to control infection encouraged?

Answer 19

no, Benefits have not be shown to outweighs the problems associated with antibiotic resistance

Question 20

exceptions to the discouragement of chronic antibiotic treatment

Answer 20

Oral Azithromycin
Nebulized Tobramycin
Nebulized Aztreonam
Inhaled colistin (2nd line)

Question 21

discuss the use of azithromycin in CF patients

Answer 21

Benefits may be due to its anti-inflammatory and/or antibacterial properties

Macrolides are unable to kill pseudomonas bacteria, but reduce the ability of pseudomonas to produce biofilms

this macrolide suppresses the excessive inflammatory response

Question 22

who is given azithromycin?

Answer 22

children > 6yrs, with clinical evidence of airway inflammation
this may present as a chronic cough or any reduction in FEV1, whether or not they are infected with P. aeruginosa

Question 23

discuss the use of Nebulized Tobramycin in CF patients

Answer 23

improves lung function and reduces acute pulmonary exacerbations in patients who are chronically infected with P. aeruginosa

Mechanism-Interferes with bacterial protein synthesis by binding to 30S and 50S ribosomal subunits, resulting in a defective bacterial cell membrane

Question 24

how do patients take Nebulized Tobramycin

Answer 24

administered by nebulizer twice daily for 28 days, alternating with 28 days off treatment

Question 25

what are the major Inhaled tobramycin ADEs

Answer 25

sputum discoloration, rales, wheezing, voice alteration, couch, abnormal taste, eosinophilia and tinnitus

Question 26

Vitamin A, D, E, K Supplementation is based on what?

Answer 26

age

Question 27

CF patients experience which the of vitamin deficiencies

Answer 27

Fat-soluble vitamin deficiencies due to pancreatic insufficiency and liver disease which leads to fat malabsorption

this predisposes to deficiencies vitamins A, D, E, and K

Question 28

why do CF patients experience bone loss

Answer 28

Decreased calcium absorption and intake can lead to bone loss
Compounded by infections and chronic inflammation

Question 29

when is a lung transplant indicated

Answer 29

FEV1 below 30 percent predicted or a rapid decline in FEV1 (particularly in young female patients)
Increasing frequency of exacerbations requiring antibiotic therapy
Refractory and/or recurrent pneumothorax
Recurrent hemoptysis not controlled by embolization

Question 30

when do we give oxygen

Answer 30

when CF is progressive and there is a worsening hypoxemia

Question 31

BiPAP

Answer 31

Noninvasive positive pressure ventilation used when CF is advanced and patient is experiencing hypercapnia

Question 32

vaccines for those with CF

Answer 32

Influenza vaccine (>6months old)
Pneumococcal vaccine

Question 33

what is Palivizumab

Answer 33

Monoclonal antibody against respiratory syncytial virus used in children younger than 24 months of age

*Not a firm recommendation

Question 34

Common adverse drug reactions to Tezacaftor/Ivacaftor

Answer 34

headache, nausea, sinus congestion, dizziness

Question 35

what is Inhaled Aztreonam? MOI?

Answer 35

Monobactam (beta-lactam) antibiotic with antipseudomonal activity
Inhibits bacterial cell wall synthesis

Question 36

cross-allergenicity with other beta-lactams with Inhaled Aztreonam

Answer 36

is unlikely

Question 37

Inhaled Aztreonam ADEs

Answer 37

fever, cough, rash

Question 38

Inhaled Aztreonam: Do not repeat for ___ days after completion

Answer 38

28

Question 39

which two medication are alternated to provide continuous coverage in CF

Answer 39

Tobramycin and Aztreonam