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Pharm II > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

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

A

Cystic fibrosis

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2
Q

what does cystic fibrosis mainly involve?

A

exocrine glands

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3
Q

cystic fibrosis causes dysfunction of what regulator protein?

A

CFTS (cystic fibrosis transmembrane regulator protein)

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4
Q

problems with the GI tract with CF

A 
deficient secretion of digestive enzymes
fat-soluble vitamin deficiency
insulin deficiency
intestinal obstruction 
reflux
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5
Q

presentation of GI issues w/ CF patient.

A

Steatorrhea

malnutrition (below average for growth curves)

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6
Q

how do you diagnose pancreatic insufficiency

A

72 fecal fat collection

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7
Q

what causes digestive enzyme deficiencies in CF patient?

A

mucus plugging and damage to pancreas

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8
Q

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

A

lipase, protease, amylase taken with each meal or snack

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9
Q

what can occur at really high doses of pancreatic enzymes?

A

fibrosing colonopathy

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10
Q

what should you recommend in CF patients with GI issues?

A

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

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11
Q

therapy for GI obstruction with CF patients.

A

Electrolyte lavage solutions to reach clear endpoints

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12
Q

Liver dz with CF patients

A

GGT may be elevated and reflect damage

biliary fibrosis, bile duct proliferation, chronic inflammatory infilatration

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13
Q

therapy for liver dz with CF patient

A

Ursodeoxycholate

decrease liver inflammation and bile duct proliferation

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14
Q

Respiratory problems with CF

A

chronic rhinitis, sinusitis, nasal polyps

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15
Q

5 therapies for pulmonary issues with CF

A 
Airway clearance techniques (ACT)
Anti-inflammatory agents
Chronic antibiotics
Mucolytics
Bronchodilators
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16
Q

how often is ACT done for patients with CF?

A

twice a day

gets rid of secretions and improves ventilation

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17
Q

what antiinflammatory therapy do you use for CF patients

A

NSAIDs

Ibuprofen has shown decreased rate of decline with FEV1

18
Q

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

A

Azithromycin

19
Q

what are some mucolytics for CF

A

Pulmozyme (most often used)

Mucomyst (n-acetylcystine)

20
Q

What does Pulmozyme do?

A

Decreases viscosity of sputum

nebulized for administration

21
Q

ADRs of pulmozyme

A

hoarseness
voice alteration
paryngitis

22
Q

problem with mucomyst

A

irritating, can lead to additional bronchoconstriction

23
Q

what does nebulized hypertonic saline do?

A

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

24
Q

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

A

Inhaled beta 2 agonists

inhaled corticosteroids

25
Q

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

A

Staphylococcus aureus

26
Q

what organism is found by age 3 in CF patients

A

Haemophilus influenzae

27
Q

what organisms is colonized in CF patients by age 5

A

pseudomonas aerguinosa

28
Q

what chronic antibiotics

A 
nebulized tobramycin (TOBI) 
nebulized aztreonam (Cayston)
29
Q

what do chronic antibiotics help with for CF patients?

A

prolong time b/w acute exacerbations

30
Q

how do you do the dosing of the chronic antibiotics.

A

28 days on, 28 days off

31
Q

what immunizations should CF patients specifically receive?

A 
Flu vaccine
Streptococcus pneumoniae (don't have to wait until 65)
32
Q

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

A

Abnormally high concentrations of sodium and chloride in sweat

33
Q

reproductive problems in males with CF

A

males have congenital bilateral absence of the vas deferens

34
Q

reproductive problems of patients with CF

A

delayed onset of puberty

35
Q

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

A

calcium and vitamin D supplementation

36
Q

2 bone and joint problems with CF

A

low bone mineral density

arthritis

37
Q

tx of arthritis for patients with CF

A

short courses of nonsetroidal and steroidal inflammatories

38
Q

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

A

No

39
Q

how to you tx acute pulmonary exacerabtions with CF

A

aminoglycosides in combo with an antipseudomonal penicillin

dose at upper range

40
Q

when can you get a lung transplant with CF?

A

older than 10 years old

Pharm II (16 decks)

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