Week 3 (cystic and drug induced) Flashcards

1
Q

most common mutation in CF

A

F508 Delta
- 1 copy= hetero
- 2 copies= homo

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2
Q

Who can get Ivacaftor
(Kalydeco)

A

1 month and older w/ 1 copy of F508del OR another mutation that is responsive

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3
Q

Who can get Lum/Iva (Orkambi)

A

1 year and older w/ 2 copies of F508del

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4
Q

Who can use Tez/Iva (Symdeko)

A

6 years and older w/ 1 or 2 copies of F508del OR another mutation that responds to

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5
Q

Who can use Elex/Teza/Iva (Trikafta)

A

2 years and older w/ 1 or 2 copies of F508del

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6
Q

Most to least effacious modulator

A

Elex/Tez/Iva > Iva > Tez/Iva > Lum/Iva

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7
Q

Efficacy parameter

A
  • FEV1 improved/inc
    -Dec in acute pulmonary exac rates, inc BMI, inc in QOL
    -Acute inc in lung function and sustained improved function longer than control
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8
Q

General SEs (7) of CF modulators

A
  • Abdominal pain
  • Diarrhea
  • Rash
  • Inc in ALT/AST
  • inc blood creatine phosphokinase
  • rhinorrhea
  • Headhache
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9
Q

Monitoring

A
  • Liver transaminase & bilirubin @baseline, q3 mon x 1 yr, then annually
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10
Q

When to dose adj CF (2)

A

-If AST/ALT is 3x upper limit of norm w/ bilirubin 2x upper limit norm
- mod- severe hepatic dysfunction

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11
Q

Counseling points

A
  • Take w/ fat containing meal
  • Avoid grapefruit (inhs CYP3A4)
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12
Q

Drug interactions : CYP3A4 mod inhs

A
  • inc Cmax and AUC
  • Erythromycin and fluconazole
  • Dec Iva to QD and Tez/Elex alternate QD with Iva doses
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13
Q

Drug interactions : CYP3A4 strong inhs

A
  • Inc AUC and Cmax
  • Clarithromycin and Itraconazole
  • Dec of Iva AND Tez/Elex dosing to BIW
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14
Q

Treatment for pulmonary aspects: asthma/ bronchospasms

A
  • SABA (albuterol) > LABA
  • brochospasms from hypertonic saline
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15
Q

Treatment for pulmonary aspects: Mucolytic/ hydrating agents (who get it, se, efficacy)

A
  • ALL PATIENTS WITH CF
  • Dornase alfa
    -se: voice harshness & rash
    -inc fev1 and dec ape
  • Hypertonic Saline
    - se: bronchospasm
    - dec ape
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16
Q

Treatment for pulmonary aspects: Inflammation (who, dosing, se)

A
  • CF pt 6yr+
  • Azithromycin
    • m-w-f dosing
    • se: n/d/v, AB resistance?
    • improves all aspects of CF
  • Alternative : high dose ibuprofen
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17
Q

Treatment for pulmonary aspects: Inhaled Abx (who, dose, se)

A
  • CF pts 6yr+ w/ hx of pseudomonas
  • Tobramycin 300 mg inh BID for 28 days on/off
    -SE: Voice alternation, tinnitus
  • Azetronam 75mg inhaled for 2-5 min TID 28 days on/off
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18
Q

What vaccinations should CF pts get

A
  • Flu, if 6 months+
19
Q

What happens to home regimen during acute exacerbation of CF

A
  • inc vest treatment and SABA to 4x daily
  • inc dornase alfa and hypertonic saline to BID
  • HOLD inhaled Abx
20
Q

Treatment for CF APE w/ hx of MSSA no PA

A
  • Anti-staphyococcal penicillin: Naficillin
    or
    -cephalosporin
21
Q

Treatment for CF APE w/ hx of MSSA and PA

A

-Double PA coverage: Cefepime & Aminoglycoside (extended interval dosing)

22
Q

Treatment for CF APE w/ hx of MRSA no PA

A

Vancomycin or Linezolid

23
Q

Treatment for CF APE w/ hx of MRSA and PA

A
  • Vancomycin or linezolid
    AND
  • Double PA coverage: Aminoglycoside + B-Lactam (Ceftazidime)
24
Q

CF APE ABx duration

A
  • 10-14 days (can be continued at home in picc line or dec to oral)
  • if no improv in 5-7 days reassess
25
Q

APE ABx monitoring

A
  • symptom persistance/ resolution
  • sputum culture & susceptibilities
  • pulmonary function test (FEV1, FVC) x weekly
  • BUN/SCr
  • ABx conct (draw conct q3-7 days)
26
Q

ABx concentration monitoring (Amino)

A
  • Aminoglycosides
    • Traditional P 10-12 mcg/ml , T <1.5 mcg/ml
    • Extended interval P 22.5-27.5 mcg/ml, T <0.1 mcg/ml, 18 hr <1 mcg/ml, AUC 80-100 mcg/ml
27
Q

ABx concentration monitoring (Vanco)

A

T 10-20 mcg/ml
AUC/MIC > 400

28
Q

Drug-related risk factors for DIP

A
  • dose
  • admin rate
  • treatment duration
  • O2 therapy
  • Radiation therapy
  • Cumulative dose
29
Q

Patient-related risk factors

A
  • Age (extremes)
  • respiratory acidosis)
  • pre-existing lung disease
  • impaired renal or hepatic function
30
Q

DI interstitial lung disease patho

A

Drug induced, fibrosis may lead to acute pneumonitis

31
Q

DI interstitial lung disease symptoms

A
  • Sudden onset dyspnea w/ non productive cough
  • fever, rash
  • crackles on expiration w/clubbing
    CT: dec lung volume, bilat ground glass
32
Q

DI interstitial lung disease causative agents

A
  • Antimicrobals: Nitrofurantoin
  • Anti- rheumatics: Leflunomide + Methotrexate
  • Cardio: Amiodarone
  • Anti-neoplastics : Bleomycin, Busulfan, cyclophosphamide, Gemcitabine
33
Q

Treatment For DIILD

A
  • Grade 2: hold med and give pred 1-2 mg/kg/day
    • continue until it becomes grade 1 and monitor
    • if no improv in 48-72 hrs inc grade
  • Grade 3/4: permanent d/c med, give methylpred 1-2 mg/kg/day
    - continue and taper over 4-6 weeks
    • if no improv give infliximab/IVIG/MMF
34
Q

Treatment For DIILD caused by mTORI’s

A
  • Grade 2 : Pred .75-1 mg/kg and dec dose or hold
  • Grade 3: Pred .75-1 mg/kg and hold med
  • Grade 4: Pred .75-1 mg/kg and permanently d/c
35
Q

Treatment For DIILD Caused by Bleomycin

A
  • Pred .75-1 mg/kg for 4-6 weeks with taper
36
Q

Treatment For DIILD Caused by Carmustine

A
  • pred taper 60 mg BID to 30 mg QD to 10 mg wkly to 5 mg weekly
37
Q

Treatment For DIILD Caused by Amiodarone

A

Pred .5-1 mg/kg/day
*relapse if therapy given for <6 mons
-monitor baseline status, q3-6month x 1 yr

38
Q

Pneumonia : BOOP symptoms and xray

A
  • Cough, dyspnea, BL crackles
  • chest x-ray: bilateral pathy, infiltrates
39
Q

Pneumonia : Meds that cause BOOP (4)

A

-Bleomycin, Amiodarone, Carbamazepine, Cocaine

40
Q

Pneumonia : Eosinophilic symptoms and xray

A
  • Dry cought, chest pain, fever
  • Xray: ground glass
41
Q

Pneumonia : Meds that cause Eosinophilic

A

-Daptomycin, Mesalamine Sulfasalazine, Nitrofurantoin, and Minocycline

42
Q

Pneumonia : Meds that cause Hypersensitivity pneumonia

A

NSAIDs, Methotrexate

43
Q

Pneumonia : Meds that cause Pulmonary edema

A

Narcotics, treat w/ naloxone

44
Q

Pneumonia : Meds that cause Lupus

A

Procainamide, Hydralazine, Isoniazid