Week 3 (cystic and drug induced) Flashcards
most common mutation in CF
F508 Delta
- 1 copy= hetero
- 2 copies= homo
Who can get Ivacaftor
(Kalydeco)
1 month and older w/ 1 copy of F508del OR another mutation that is responsive
Who can get Lum/Iva (Orkambi)
1 year and older w/ 2 copies of F508del
Who can use Tez/Iva (Symdeko)
6 years and older w/ 1 or 2 copies of F508del OR another mutation that responds to
Who can use Elex/Teza/Iva (Trikafta)
2 years and older w/ 1 or 2 copies of F508del
Most to least effacious modulator
Elex/Tez/Iva > Iva > Tez/Iva > Lum/Iva
Efficacy parameter
- 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
General SEs (7) of CF modulators
- Abdominal pain
- Diarrhea
- Rash
- Inc in ALT/AST
- inc blood creatine phosphokinase
- rhinorrhea
- Headhache
Monitoring
- Liver transaminase & bilirubin @baseline, q3 mon x 1 yr, then annually
When to dose adj CF (2)
-If AST/ALT is 3x upper limit of norm w/ bilirubin 2x upper limit norm
- mod- severe hepatic dysfunction
Counseling points
- Take w/ fat containing meal
- Avoid grapefruit (inhs CYP3A4)
Drug interactions : CYP3A4 mod inhs
- inc Cmax and AUC
- Erythromycin and fluconazole
- Dec Iva to QD and Tez/Elex alternate QD with Iva doses
Drug interactions : CYP3A4 strong inhs
- Inc AUC and Cmax
- Clarithromycin and Itraconazole
- Dec of Iva AND Tez/Elex dosing to BIW
Treatment for pulmonary aspects: asthma/ bronchospasms
- SABA (albuterol) > LABA
- brochospasms from hypertonic saline
Treatment for pulmonary aspects: Mucolytic/ hydrating agents (who get it, se, efficacy)
- ALL PATIENTS WITH CF
- Dornase alfa
-se: voice harshness & rash
-inc fev1 and dec ape - Hypertonic Saline
- se: bronchospasm
- dec ape
Treatment for pulmonary aspects: Inflammation (who, dosing, se)
- CF pt 6yr+
- Azithromycin
- m-w-f dosing
- se: n/d/v, AB resistance?
- improves all aspects of CF
- Alternative : high dose ibuprofen
Treatment for pulmonary aspects: Inhaled Abx (who, dose, se)
- 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