Cystic Fibrosis

Question 1

Most common lethal, genetic disease in the Caucasian population
Autosomal recessive genetic disease

Answer 1

Cystic fibrosis

Question 2

what does cystic fibrosis mainly involve?

Answer 2

exocrine glands

Question 3

cystic fibrosis causes dysfunction of what regulator protein?

Answer 3

CFTS (cystic fibrosis transmembrane regulator protein)

Question 4

problems with the GI tract with CF

Answer 4

deficient secretion of digestive enzymes
fat-soluble vitamin deficiency
insulin deficiency
intestinal obstruction 
reflux

Question 5

presentation of GI issues w/ CF patient.

Answer 5

Steatorrhea

malnutrition (below average for growth curves)

Question 6

how do you diagnose pancreatic insufficiency

Answer 6

72 fecal fat collection

Question 7

what causes digestive enzyme deficiencies in CF patient?

Answer 7

mucus plugging and damage to pancreas

Question 8

what enzymes do you need to give someone with CF GI problems.

Answer 8

lipase, protease, amylase taken with each meal or snack

Question 9

what can occur at really high doses of pancreatic enzymes?

Answer 9

fibrosing colonopathy

Question 10

what should you recommend in CF patients with GI issues?

Answer 10

High calorie diet
Fat soluble vitamin replacement
may require extra salt (dumping so much salt out)

Question 11

therapy for GI obstruction with CF patients.

Answer 11

Electrolyte lavage solutions to reach clear endpoints

Question 12

Liver dz with CF patients

Answer 12

GGT may be elevated and reflect damage

biliary fibrosis, bile duct proliferation, chronic inflammatory infilatration

Question 13

therapy for liver dz with CF patient

Answer 13

Ursodeoxycholate

decrease liver inflammation and bile duct proliferation

Question 14

Respiratory problems with CF

Answer 14

chronic rhinitis, sinusitis, nasal polyps

Question 15

5 therapies for pulmonary issues with CF

Answer 15

Airway clearance techniques (ACT)
Anti-inflammatory agents
Chronic antibiotics
Mucolytics
Bronchodilators

Question 16

how often is ACT done for patients with CF?

Answer 16

twice a day

gets rid of secretions and improves ventilation

Question 17

what antiinflammatory therapy do you use for CF patients

Answer 17

NSAIDs

Ibuprofen has shown decreased rate of decline with FEV1

Question 18

what drug is given 3x a week to CF patients to help with inflammation

Answer 18

Azithromycin

Question 19

what are some mucolytics for CF

Answer 19

Pulmozyme (most often used)

Mucomyst (n-acetylcystine)

Question 20

What does Pulmozyme do?

Answer 20

Decreases viscosity of sputum

nebulized for administration

Question 21

ADRs of pulmozyme

Answer 21

hoarseness
voice alteration
paryngitis

Question 22

problem with mucomyst

Answer 22

irritating, can lead to additional bronchoconstriction

Question 23

what does nebulized hypertonic saline do?

Answer 23

Draws water into airways, increases ability to cough out mucus
can cause bronchospasm

Question 24

what tx for CF patients is also helpful if they have asthma?

Answer 24

Inhaled beta 2 agonists

inhaled corticosteroids

Question 25

what is the major pathogen in CF patients in first year of life?

Answer 25

Staphylococcus aureus

Question 26

what organism is found by age 3 in CF patients

Answer 26

Haemophilus influenzae

Question 27

what organisms is colonized in CF patients by age 5

Answer 27

pseudomonas aerguinosa

Question 28

what chronic antibiotics

Answer 28

``` nebulized tobramycin (TOBI) nebulized aztreonam (Cayston) ```

Question 29

what do chronic antibiotics help with for CF patients?

Answer 29

prolong time b/w acute exacerbations

Question 30

how do you do the dosing of the chronic antibiotics.

Answer 30

28 days on, 28 days off

Question 31

what immunizations should CF patients specifically receive?

Answer 31

``` Flu vaccine Streptococcus pneumoniae (don't have to wait until 65) ```

Question 32

what is the high concentration of in the sweat glands of a patient with CF

Answer 32

Abnormally high concentrations of sodium and chloride in sweat

Question 33

reproductive problems in males with CF

Answer 33

males have congenital bilateral absence of the vas deferens

Question 34

reproductive problems of patients with CF

Answer 34

delayed onset of puberty

Question 35

what supplementation is needed for CF patients for low bone mineral density

Answer 35

calcium and vitamin D supplementation

Question 36

2 bone and joint problems with CF

Answer 36

low bone mineral density | arthritis

Question 37

tx of arthritis for patients with CF

Answer 37

short courses of nonsetroidal and steroidal inflammatories

Question 38

is eradication of P. aerguinosa likely in patients with CF?

Answer 38

No

Question 39

how to you tx acute pulmonary exacerabtions with CF

Answer 39

aminoglycosides in combo with an antipseudomonal penicillin | dose at upper range

Question 40

when can you get a lung transplant with CF?

Answer 40

older than 10 years old