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Nayla Pharm > 3- Cystic Fibrosis > Flashcards

3- Cystic Fibrosis Flashcards

1
Q

What is CF

A

Autosomal recessive disorder on chromosome 7

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2
Q

Where do gene mutations occur

A

MC: delta F508
MC in caucasians: G551D
Also common, R117H

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3
Q

What are the classes of mutations

A

I: No protein production
II: Defect in protein processing (d F508)
III: “gating” mutation, defective regulation (G551D)
IV: Defective conduction
V: less stable/active CFTR proteins (d F508)

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4
Q

Goals of CF treatment are to

A

slow or stop progression of disease

allow for normal growth and development

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5
Q

Summary of CF available treatments

A 
Chest Physiotherapy 
Antibiotics
Pancreatic Enzyme Replacement Therapy 
Multivitamins (w/ ADEK)
Anti-obstructive 
Anti-inflammatories 
CFTR modulators
Vaccines and Pavilizumab
(supplemental O2, BiPAP, lung transplant)
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6
Q

What is involved in chest physiotherapy

A

postural drainage
chest percussion (cupping of hand)
vibration devices

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7
Q

What is Dornase-Alpha

A

anti-obstructive that cleaves DNA in mucus secretions to reduce viscosity (thickness)

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8
Q

What are toxicities of Dornase-Alpha

A 
fever
pharyngitis
rhinitis
rash
dyspepsia
conjunctivitis
laryngitis
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9
Q

What is my story for Dornase-Alpha

A

I travel to Dorn (GoT) to CLEAVE the dna and watch the thin mucus spill.
On my journey I developed a fever and a rash. When I cleaved the soldier’s bodies, the thin mucus got in my eyes, nose, and mouth (conjunctivitis, rhinitis, laryngitis/pharyngitis)
I was so sickened by this that I developed indigestion (dyspepsia)

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10
Q

What is a hallmark of CF

A

abundant and purulent airway secretions made of polymerized DNA from dead PMN (viscous)

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11
Q

How does hypertonic saline work

A

draws water from airway to re-establish aqueous surface layer (deficient in CF) AKA hydrates mucus

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12
Q

What abx can be used chronically in CF patients

A

Tobramycin (aminoglycoside) or Aztreonam (monobactam)

  • They are both aerosolized and have anti-PSEUDO properties
  • Both need to be used on 28 day cycles. Can alternate them if needed
  • *only use if benefit>risk
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13
Q

Do macrocodes kill pseudomonas

A

NO- but Azithromycin reduces ability to form biofilm so it is more susceptible to destruction
-Azithromycin also anti-inflammatory!

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14
Q

What are S/E of Aztreonam

A

fever, cough, rash

B-Lactam

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15
Q

What are S/E of Tobramycin

A

discolored sputum, abnormal taste
rales, wheezing, altered voice, cough
eosinophilia
tinnitus

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16
Q

What are other benefits of Tobramycin

A

improves lung function nx reduces acute exacerbations

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17
Q

What anti-obstructive bronchodilators can be used to treat CF

A

SABA/SAMA (albuterol, ipratropium)

Theophylline is not used as much because of it’s side effects

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18
Q

What is inhaled N-acetylcysteine

A

an anti-obstructive that can be used

19
Q

Recommended treatment sequence for CF is

A

bronchodilators
hypertonic saline
dornase alpha
aerosolized antibiotics

20
Q

What are the anti-inflammatories that can be used in CF

A

Azithromycin (macrolide)

Ibuprofen

21
Q

What are the MC pathogens in respiratory secretions of CF patients

A

<25: Staph Aureus

25+: Pseudomonas Aureginosa

22
Q

How does Ibuprofen benefit CF patients

A

reversibly inhibits COX 1/2 (AKA decreased prostaglandins)

-also an anti-pyretic and analgesic

23
Q

What are Ibuprofen toxicities

A 
Edema/fluid retention
HE
epigastric pain, GI bleed
Tinnitus 
**can use high dose if <18 y/o with FEV1 >60%
24
Q

What is PERT

A

admin of micro encapsulates pancreatic enzymes (lipase, amylase, protease) due to 85% of pts having pancreatic insufficiency
*Dose calculated based on lipase

25
Q

What are pancreatic enzyme ADE

A 
ulcers of oral mucosa (take with food and rinse after admin) 
Fibrosing colonopathy (inflammation and strictures) with excessive dose
26
Q

What does a multivitamin with A, D, E, and K need to be administered

A

Pancreatic insufficiency and liver disease can cause fat malabsorption

27
Q

What is Ivacaftor

A

CFTR modulator that improves gating of surface proteins (mainly G551D), potentiating chloride ion transport (improved Na/H2O absorption/secretion in tissues- lung&GI)

28
Q

S/E of Ivacaftor are

A 
*Hyperglycemia, increased transaminases
HA
Abd pain
naspharyngitis
arthralgia
29
Q

What is Tezacaftor/Ivacaftor (Symdeko)

A

Teza moves defective CFTR protein to the correct place on cell surface (Ivacaftor still improved gating)–
Take with fat containing food!
**Approved for those 12+ y/o with 2 delta F508 mutations

30
Q

Side effects of Symdeko include

A

same as Ivacaftor, + sinus congestion, dizziness, cataracts

31
Q

What must you monitor with Symdeko

A

ALT and AST; prior to starting Tx, every 3 mo. of first year, and yearly after

32
Q

When should you discontinue Tezacaftor/Ivacaftor

A

If ALT or AST >5x UL, or, >3x UL with bilirubin >2x UL

33
Q

What is Ivacaftor/Lumacaftor

A

Luma partially corrects CFTR misfiling of delta F508 mutation (Iva still improves gating)

  • *approved in those with 2 debts F508 mutations
  • Improves lung function and reduces exacerbations
  • Improves BMI
34
Q

What CF mutation represents the largest group

A

2 copies of the delta F508 gene mutation

35
Q

What vaccines do CF patients need

A

Flu (6+ mo)
Pneumococcal
Palivizumab

36
Q

What is Palivizumab

A

monoclonal antibody to RSV (resp. syncitical virus)

-given in <24 mo.

37
Q

What are CF GI treatments

A

ppi (omeprazole) which blocks the H/K/ATP-ase pump in gastric parietal cells so H+ is not secreted

38
Q

Why give a ppi?

A

GERD is 8x more common in CF because tummies have less mucus to protect against acid

39
Q

PPI toxicities include

A

Diarrhea, acid regurg, constipation

Back pain, weakness, cough

40
Q

Who gets oxygen therapy

A

progressive CF with worsening hypoxia

41
Q

Who gets BiPAP (non-inavsive positive pressure vent)

A

advanced CF with hypercapnia

42
Q

Who gets a lung transplant

A

FEV1 <30%
Increasing exacerbations needing ABX
Recurrent PTX
Recurrent hemoptysis

43
Q

What is Ursodeoxycholic acid (UDCA)

A

gallstone dissolution agent that reduces cholesterol secretion from liver and reabsorption by intestines= less cholesterol in bile and bile stones
*In CF bile can back up and obstruct gallbladder

44
Q

Side effects of UDCA are

A

Alopecia
Leukopenia
Thrombocytopenia
Increased SrCr

Nayla Pharm (12 decks)

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