1 - Paeds - Resp - CF mgmt

Question 1

approach? at least __review with who?

Answer 1

MDT approach, at least annual review in specialist centre

Question 2

nutritional - what checked regularly? what to treat panc insuff?

Answer 2

dietary status

treat with oral enteric-coated panc replacement therapy, take with meals and snacks

Question 3

what is essential dietary wise? supplements given?

Answer 3

high calorie diet ~150% of normal is essential

fat sol vitamin supplements

Question 4

monitor for? look for what in younger kids? what about older kids?

Answer 4

for chest infections
younger - Sx
older - spirometry, FEV1 declines with disease progression

Question 5

what signs might you expect in treated children

from diagnosis what should happen? - how is this done for young and older kids? what is beneficial and encouraged?

Answer 5

NONE!
physio 2x a day to clear airway secretions - parents taught to do at home in younger kids, older pts do controlled deep breathing exercises and use devices to clear airway
exercise

Question 6

Abx given to kids?

what given daily

Answer 6

continuous prophylactic oral ABx (fluclox), with rescue oral ABx for decline
daily neb antipseudomonal ABx used to slow decline in lung function caused by PA

Question 7

persisting signs/Sx?

Answer 7

prompt and vigorous IV therapy for 14d to limit lung damage

Question 8

what else may be nebulised other than ABx

Answer 8

neb DNAse/hypertonic saline may decrease viscosity of sputum > increased clearance

Question 9

treated for end stage CF lung disease? how common in kids? prog?

Answer 9

bilateral sequential lung transplantation

fortunately this is rare during childhood - >50% survival at 10y