What level is diagnostic for CF?
>/= 60 mol/L
Unique Non-classic symptoms of CF
1. Chronic bacterial infection of LATER onset
2. Adequate pancreatic function/pancreatitis
3. Sweat Cl 60-90 mol/liter; sometimes normal
Unique Classic symptoms of CF
1. Severe chronic bacterial infection of airways
2. Severe hepatobiliary disease
3. Pancreatic exocrine insufficiency
4. Meconium lieus at birth
5. Sweat Cl 90-110
Kalydeco
Ivacaftor; Do not use as mono therapy for homozygous F508delta
Orkambi
Lumacaftor/ivacaftor
Symdeka
Tezacaftor/ivacaftor
Trikafta
Elexacaftor/tezacaftor/ivacaftor
Which agent is used in Delta F508 heterozygous greater than 4 months of age
Kalydeco (Ivacaftor)
Which two agents can be used in Delta F508 homozygotes between 2 and 5
Trikafta
Orkambi
Which agent is used in Delta F508 heterozygous greater than 2 years of age
Trikafta
Which agent is used in Delta F508 heterozygous greater than 6 years of age
Trikafta
Symdeka