Week 3 (cystic and drug induced)

Question 1

most common mutation in CF

Answer 1

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

Question 2

Who can get Ivacaftor
(Kalydeco)

Answer 2

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

Question 3

Who can get Lum/Iva (Orkambi)

Answer 3

1 year and older w/ 2 copies of F508del

Question 4

Who can use Tez/Iva (Symdeko)

Answer 4

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

Question 5

Who can use Elex/Teza/Iva (Trikafta)

Answer 5

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

Question 6

Most to least effacious modulator

Answer 6

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

Question 7

Efficacy parameter

Answer 7

• 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

Question 8

General SEs (7) of CF modulators

Answer 8

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

Question 9

Monitoring

Answer 9

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

Question 10

When to dose adj CF (2)

Answer 10

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

Question 11

Counseling points

Answer 11

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

Question 12

Drug interactions : CYP3A4 mod inhs

Answer 12

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

Question 13

Drug interactions : CYP3A4 strong inhs

Answer 13

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

Question 14

Treatment for pulmonary aspects: asthma/ bronchospasms

Answer 14

• SABA (albuterol) > LABA
• brochospasms from hypertonic saline

Question 15

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

Answer 15

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

Question 16

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

Answer 16

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

Question 17

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

Answer 17

• 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

Question 18

What vaccinations should CF pts get

Answer 18

• Flu, if 6 months+

Question 19

What happens to home regimen during acute exacerbation of CF

Answer 19

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

Question 20

Treatment for CF APE w/ hx of MSSA no PA

Answer 20

• Anti-staphyococcal penicillin: Naficillin
  or
  -cephalosporin

Question 21

Treatment for CF APE w/ hx of MSSA and PA

Answer 21

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

Question 22

Treatment for CF APE w/ hx of MRSA no PA

Answer 22

Vancomycin or Linezolid

Question 23

Treatment for CF APE w/ hx of MRSA and PA

Answer 23

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

Question 24

CF APE ABx duration

Answer 24

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

Question 25

APE ABx monitoring

Answer 25

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

Question 26

ABx concentration monitoring (Amino)

Answer 26

- 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

Question 27

ABx concentration monitoring (Vanco)

Answer 27

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

Question 28

Drug-related risk factors for DIP

Answer 28

- dose - admin rate - treatment duration - O2 therapy - Radiation therapy - Cumulative dose

Question 29

Patient-related risk factors

Answer 29

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

Question 30

DI interstitial lung disease patho

Answer 30

Drug induced, fibrosis may lead to acute pneumonitis

Question 31

DI interstitial lung disease symptoms

Answer 31

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

Question 32

DI interstitial lung disease causative agents

Answer 32

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

Question 33

Treatment For DIILD

Answer 33

- 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

Question 34

Treatment For DIILD caused by mTORI's

Answer 34

- 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

Question 35

Treatment For DIILD Caused by Bleomycin

Answer 35

- Pred .75-1 mg/kg for 4-6 weeks with taper

Question 36

Treatment For DIILD Caused by Carmustine

Answer 36

- pred taper 60 mg BID to 30 mg QD to 10 mg wkly to 5 mg weekly

Question 37

Treatment For DIILD Caused by Amiodarone

Answer 37

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

Question 38

Pneumonia : BOOP symptoms and xray

Answer 38

- Cough, dyspnea, BL crackles - chest x-ray: bilateral pathy, infiltrates

Question 39

Pneumonia : Meds that cause BOOP (4)

Answer 39

-Bleomycin, Amiodarone, Carbamazepine, Cocaine

Question 40

Pneumonia : Eosinophilic symptoms and xray

Answer 40

- Dry cought, chest pain, fever - Xray: ground glass

Question 41

Pneumonia : Meds that cause Eosinophilic

Answer 41

-Daptomycin, Mesalamine Sulfasalazine, Nitrofurantoin, and Minocycline

Question 42

Pneumonia : Meds that cause Hypersensitivity pneumonia

Answer 42

NSAIDs, Methotrexate

Question 43

Pneumonia : Meds that cause Pulmonary edema

Answer 43

Narcotics, treat w/ naloxone

Question 44

Pneumonia : Meds that cause Lupus

Answer 44

Procainamide, Hydralazine, Isoniazid