Cystic Fibrosis and Solid Organ Transplant

Question 1

What is the order of administration for cystic fibrosis treatment?

Answer 1

1. bronchodilator inhaler
2. wait 15 minutes for airway to open
3. hypertonic saline to improve hydration and thin secretions
4. airway clearance device
5. pulmozyme (to break up mucus)
6. inhaled steroids
7. inhaled antibiotics (always do inhaled antibiotics last)

Question 2

What are the 3 inhaled antibiotics for CF, and their brands?

Answer 2

1. Tobramycin for psuedomonas >/= 6yo, nephrotoxic, ototoxic
   
   • Bethkis^®
   • Kitabis^®
   • TOBI podhaler^®
   • TOBI solution^®, refrigerate
2. Aztreonam for gram negative >/= 7yo
   
   • Cayston^®
3. Colismethate (off label in CF)

Question 3

How are nutritional malabsorptions treated in CF?

Answer 3

1. Pancreatic Enzyme Replacement Therapy (PERT)
   
   • with meals and snacks
   • Creon®, Zenpep®, Ultresa®, Viokcase®, Pertzye®, or Pancreaze®
2. Vitamins high in A, D, E and K such as AquADEK® and MVW Complete Formulation vitamins

Question 4

At what age is each of the CFTR modulators approved for use?

Answer 4

1. Ivacaftor (Kalydeco®): > 6 months
   
   • not for F508del
2. Lumacaftor/ivacaftor (Orkambi®): > 2 years
   
   • need 2 copies of F508del mutation
3. Tezacaftor/ivacaftor (Symdeko®): > 6 years
   
   • at least one copy of F508del and one other listed mutation
4. Elexacaftor/tezacaftor/ivacaftor (Trikafta®): >12 years
   
   • 2 copies of F508del-CFTR mutation
   • F508del + any other mutation

Question 5

What is the REMS for mycophenylate mofentil (Cellcept^®​, MMF)?

Answer 5

REMS program due to potential for birth defects or 1st semester pregnancy loss. Mycophenylate Sodium (MPA, Myfortic^® has the same REMS.

Question 6

What are some important counseling and monitoring points for the CFTR modulators?

Answer 6

• take with a high-fat meal
• Ophthalmic examinations – Baseline and follow-up examinations are recommended in pediatric patients
• Hepatic monitoring
  
  • baseline
  • q 3 months for 1st year
  • yearly thereafter

Question 7

What are the target trough levels for cyclosporine?

Answer 7

• <3 months post transplant: 200-300 ng/mL
• >3 months post transplant: 50-150 ng/mL

Question 8

What are the average 12-hour trough trough levels for tacrolimus?

Answer 8

In practice, patients are started on 5mg BID and the dose is adjusted based on lab draws

• <6 months post transplant: 8-10 ng/ml
• 6-12 months post transplant: 7-8 ng/mL
• >1 year post transplant: ~5 ng/mL

Question 9

What is the conversion factor from Prograf® to Astagraf® XL and Envarsus® XR?

Answer 9

• the total daily dose of Prograf, taken in one dose for Astagraf XL
• the total daily dose of Prograf, times 0.8 in one dose for Envarsus XR

Question 10

What is the conversion factor between Cellcept^® and Myfortic^®?

Answer 10

500mg Cellcept^®=360mg Myfortic^®