Suppurative lung disease

Question 1

Classes of CFTR mutation

Answer 1

Class I: defective protein synthesis e.g. G542X
Class II: defective protein maturation and trafficking e.g. F508del
Class III: Impaired chloride channel opening (gating) e.g. G551D
Class IV: Defective channel ion transport (Conductance) e.g. R117H
Class V: Defective splicing
Class VI: Accelerated turnover at cell surface

Question 2

Treatment of pseudomonas in CF

Answer 2

Colomycin nebs and PO ciprofloxacin 3 months
IV abx e.g. ceftazidine, tazocin, aztreonam, meropenem and tobramycin, amikacin, colomycin 2 weeks prior to PO switch

Maintenance:
colomycin or tobramycin nebs/DPI
aztreonam nebs
levofloxacin nebs

Question 3

Treatment of staph aureus in CF

Answer 3

IV fluclox 2 weeks
add anti-staph antibiotic e.g. sodium fusidate or rifampicin for 2 weeks

If MRSA
4 week PO rifampicin with sodium fusidate
linezolid or neb vancomycin
IV teiciplanin or vancomycin

Question 4

Treatment of haemophillus in CF

Answer 4

co-amox or doxy 2 weeks - even if asymptomatic
prolonged course - IV co-amox, ceftriaxone

Question 5

Treatment of burkholderia cepacia complex in CF

Answer 5

Sensitivities
Meropenem
ceftazidime
pip-taz
aminoglycosides
temocillin

Question 6

Treatment of stenotrophomonas maltophilia in CF

Answer 6

co-trimox
doxycycline
ticarcillin-clavulanic acid
tigecycline

Question 7

Treatment of achromabacter xylosoxidans in CF

Answer 7

minocycline
meropenem
pip-taz
chloramphenicol

Question 8

tCFTR modulators

Answer 8

Ivacaftor (Kalydeco) - CFTR potentiator, in G551D or other gating mutation
Tezaceftor-Ivacaftor (Symkevi/Symdeko) - homozygous for F508del or heterozygous for F508del (1st line)
Lumacaftor-Ivacaftor (Orkambi) - F508del homozygous
Triple therapy (Kaftrio) - one F508del mutation and one minimal function mutation and F508del homozygous patients - approved in NHS

Question 9

Bronchiectasis severity index

Answer 9

age
BMI
FEV1
Hospital admissions in 2 years
exacerbations ibn 12 months
MRC
Pseudomonas colonisation
radiological severity

Question 10

Bronchiectasis infection treatment - first isolate of pseudomonas aeruginosa

Answer 10

2 weeks PO ciprofloxacin
IV pseudomonal penicillin (at least 2 weeks) with nebulised colistin, gentamicin, tobramycin for 3 months
Add IV amnioglycoside if lack of response

Colonised patient
long term nebs colistin or gentamycin
+/- macrolide