Paediatric Respiratory Flashcards
What is acute epiglottitis?
rare
serious infection caused by haemophilus influenzae type B.
treat quick or airway obstruction.
used to be childhood in uk but now more common in adults because of immunisation programme.
decreased since HiB vaccine
features of acute epiglottitis?
rapid onset
high temp, generally unwell
stridor
drooling of saliva
“tripod” position : pt finds it easier breathing if learning forward and extending neck in seated position
how would you diagnose acute epiglottitis?
direct visualisation by senior airway staff.
x-ray poss? esp if concerned about foreign body:
- lateral vein in acute epiglottitis will show swelling of epiglottis - the thumb sign
a posterior-anterior view in croup will show subglottic narrowing - steeple sign
how would you manage acute epiglottitis?
oxygen
iv abx
senior involvement - emergency airway support - anesthetics, ent
ENDOTRACHEAL INTUBATION might needed to protect airway.
if suspected dont examine throat due to risk of acute airway obstruction.
only done by senior staff that can intubate if needed.
what is the epiglottis?
The epiglottis is a flap of cartilage located in the throat that prevents food and liquid from entering the lungs while swallowing
What is croup?
URTI in infants+toddlers.
characteristed by stridor caused by combo of laryngeal oedema and secretions.
PARAINFLUENZA VIRUSES account for most cases.
epidemiology of croup
peak incidence at 6mnths-3yrs
autumn more common
features of croup
cough - barking seal like. worse at night
stridor - dont examine throat due to risk of airway obstruction
fever
coryzal sx
increased work of breathing eg retraction
when would you consider admitting a child with croup?
moderate or severe croup
under 3 months
known upper airway abnormalities like downs or laryngomalacia
uncertainty of diagnosis - differentials are: acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)
how would you investigate croup?
clinically mostly
if cxr:
posterior anterior view would show subglottic narrowing - steeple sign
in acute epiglottitis an anterior view would show the thumb sign - swelling of epiglottitis
how would you manage croup?
single dose of oral dexamethasone - 0.15mg/kg to all kids regardless of severity
prednisolone alternative if dex not available
grading the severity of croup
mild
moderate
severe
mild:
ocassional barking cough
no audible stridor at rest
no/mild suprasternal and/or intercostal recession
happy child - eat drink play
moderate:
frequent barking cough
easily audible stridor at rest (indication for admission)
-suprasternal and sternal wall retraction at rest
-no/little distress or agitation
- child can be placated and is interested in surroundings
severe:
frequent barking cough
prominent inspiratory/poss expiratory strioder @ rest
marked sternal wall retrctions
tachycardia with more seveere obstructive sx and hypoxeia
signficant distress and agitation, or lethargy or restlessness (sign of hypoxaemia)
how can wheezes be divided?
episodic viral: only when pt has URTI and is sx free inbetween episodes
multiple trigger wheeze: aswell as URTI other factors trigger it like exercise, allergens , cigarette smoke
is episodic viral wheeze associated with an increase risk of asthma in later life?
NO !!!
but a proportion that have multiple trigger wheeze will develop asthma
how would you managed episodic viral wheeze?
encourage parents to stop smoking
symptomatic tx
1st like: salbutamol - short acting beta 2 agonist . or anticholinergic via a spacer.
next step:
intermittent leukotreine receptor antagonist (montelukast) , intermittent inhaled corticosteroids (beclamethasone), or boht
little role for oral pred in kids that dont need hospitalising.
how would you treat multiple trigger wheeze?
tell parents stop smoking
trial either inhaled corticosteroids (beclamethasone) or leukotreine receptor antagonist - montelukast
for 4-8 weeks.
What is pneumonia?
any inflammatory condition affecting alveoli of the lungs, usually secondary to bacterial infection but can be viral/fungal too.
causes of pneumonia
bacterial - MC
viral
Fungal - eg pneumocystic jiroveci
what is the most common cause of pneumonia?
tell me a little about it
streptococcus pneumonia - pneumococcus - bacteria
80% pts
high fever
rapid onset
herpes labialis
vaccine for pneumococcus available
tell me all bacterial causes of pneumonia
streptococcus pneumoniae
haemophilus influenzae - copd pts
staphylococcus aureus - pt following influenze infection
mycoplasma pneumoniae - atypical. dry cough and atypical chest signs/xray : autoimmune haemolytic anemia, erythema multiforme could be seen
legionella pneumophilia - atypical pneumonia.
hyponatremia and lymphopenia common.
classic secondary to infected AC unit.
klebsiella pneumonia - alcholics
tell me the fungal cause of pneumonia
pneumocystis jiroveci -
hiv pts
dry cough
exercise-induced desaturations
absence of chest signs
what is idiopathic interstitial pneumonia
non-infective cause of pneumonia
eg’s:
cryptogenic organising pneumonia - form of bronchiolitis : comp of : RA or amiodarone therapy.
SYMPTOMS OF PNEUMONIA
COUGH
SPUTUM (purulent - rust coloured/bloodstained)
DYSPNOEA
CHEST PAIN : POSS PLEURITIC
FEVER
malaise
SIGNS OF PNEUMONIA
SIGNS OF SYSTEMIC INFLAMMATORY RESPONSE: fever tachycardia
reduced oxygen saturations - below 95% or below 88% in COPD pts
tachypnoea
auscultation:
reduced breath sounds
bronchial breathing
crepitations/crackles
dullness on percussion (fluid)
community vs hospital acquired pneumonia
difference
why is that even important?
most within community - CAP
within hospital 48hrs or more after admission: HAP.
causative organisms diff and therefore 1st line abx is different.
investigating pneumonia
cxr : consolidation (opacity on xray film in area of infection) - possibly effusion too
bloods:
fbc - neutrophilia in bacterial infection
u+e : check for dehydration - U for urea in curb 65
other changes in atypical pneumonia
CRP: raised in response to infection
blood cultures
sputum sample: causative organism after culture
ABG: if ox sats are low or if pt has pre-existing resp disease : COPD
check legionella antibodies - in intermediate/high risk pts
what is the scoring system to risk stratify pneumonia patients? (primary care)
CRB 65
IN PRIMARY CARE ITS CRB-65 because u cant get serum urea result
C - confusion - AMTS (abbreviated mental test score) - less than or equal to 8/10
U
R - RR 30 or more /min
B- bp - systolic less than or equal to 90 and/or diastolic less than or equal to 60 mmHg
65 - AGE EQUAL TO OR MORE THAN 65
interpreting crb 65 score (primary care)
home based care if crb65 is 0 - oral abx - amoxicillin 1st line
1-2: intermediate risk - 1-10% mortality
3/4 : high risk - 10% + mortaility
hospital assessment for all other pts - particularly if score 2 or more
if crp is less than 20 mg/L : no abx
if 20-100 - delayed abx prescription
if over 100 - abx
CURB 65 - hospital
c - confusion - amts - <=8/10
u - urea>7 mmol/L
r - rr >=30/min
b - bp <= 90/60
65 - age>=65
home based care if : 0/1 - low risk - <3% mortality
hospital-based care - 2+ - intermediate risk - 3-15% mortality
intensive care assessment if 3+ - high risk - 15%+ mortality risk
risk factors of pneumonia
under 5 or over 65
smoking
ivdu
recent viral RTI
chronic resp disease: CF, COPD
immunosuppression: cytotoxic drug therapy , HIV
pt risk of aspiration : parkinsons , oesophageal obstruction
non resp co-mordity: DM, CV disease
pathophysiology of pneumonia
pathogen enters lower resp tract.
starts inflammatory cascade.
neutrophils migrated to infected alveoli.
release cytokines - active immune response. induce fever.
accumulation of lfuid and pus in alveoli.
impairs gas exchange
= leads to hypoxic state = characteristic of pneuomonia.
management of pneumonia
low severity CAP:
- amoxicillin - 1st line
if allergy: macrolide (erythromcycin) /tetracycline
5 day course of abx.
moderate/high severity CAP:
- 7-10 day course
- beta-lactamase penicillin : coamoxiclav, ceftriaxone/piperacillin with tazobactam and a macrolide.
what is the discharge criteria for pneumonia pt ?
you cant if in the last 24 hrs they have 2 or more of:
- temp higher than 37.5
- rr 24 breaths per min or more
HR over 100 bpm
systolic bp: 90 or less
ox sats 90 % O/A
abnormal mental status
inability to eat without assistane
what do doctors tell pts about symptoms clearing up?
1 wk - fever resolved
4wk - chest pain/sputum reduced
6wk - cough and breathless reduced
3mths - most sx resolves - fatigue poss
6mth- normal
when should you repeat CXR in pneumonia pts?
why?
cxr at 6 weeks after clinical resolution.
make sure consolidation resolved.
no underlying secondary abnormality - eg lung tumour.
what is the most likely causative agent in bacterial pneumonia in children?
s.pneumoniae
treatment of pneumonia in kids
amox - 1st line
macrolides (erythromcyin) added if no response to above.
macrolides : if mycoplasma or chlamydia suspected
in pneumonia with influenza: co-amoxiclav
What is Mycoplasma Pneumoniae?
cause of atypical pneumonia affecting younger pts.
epidemics of it usually occur every 4 yrs.
associated with erythema multiforme + cold autoimmune haemolytic anaemia.
may not respond to penicillin’s or cephalosporins because it lacks peptidoglycan cell wall.
features of mycoplasma pneumoniae
disease typically prolonged and gradual onset
flu-like sx classically precede dry cough
bilateral consolidation on x-ray
complications might occur as below
investigations of mycoplasma pneumoniae
diagnosis generally by mycoplasma serology
positive cold agglutination test = peripheral blood smear may show rbc agglutination
Management of mycoplasma pneumoniae
doxycycline
or
macrolide (erythromcyin/clarithromycin)
difference between legionella pneumonia and mycoplasma pneumonia
legionella : lymphopenia
mycoplasma: haemolytic anaemia/ITP
legionella: hyponatremia
mycoplasma: erythema multiforme. encephalitis/GBS. peri/myocarditis.
legionella: diagnosis - urinary antigen
mycoplasma pneumonia: diagnosis - serology
similarities of legionella and mycoplasma pneumoniae
atypical pneumonia
flu-like sx
dry cough
deranged lfts
treat with macrolide - erythromycin
what is aspiration pneumonia?
pneumonia caused by foreign materials gaining entry into bronchial tree - usually oral/gastric contents : food and saliva.
depending on acidity of aspirate, a chemical pneumonitis can happen - and bacterial pathogens adding to the inflammation.
how can aspiration pneumonia happen?
incompetent swallowing mechanism like in stroke,MS, intoxication.
or iatrogen causes: intubation.
risk factors of aspiration pneumonia
poor dental hygiene
swallowing difficulties
prolonged hospitalisation or surgical procedures
impaired consciousness
impaired mucociliary clearance
most common sites affected for aspiration pneumonia
and why?
right middle and lower lung lobes
due to larger calibre and more vertical orientation of the right main bronchus.
bacteria implicated in aspiration pneumonia - aerobic
strept pneumonia
staph aureus
haem influenza
pseduomonas aeruginosa
klebsiella: seen in aspiration lobar pneumonia in alcholics
bacteria implicated in aspiration pneumonia - anaerobic
bacteroides
prevotella
fusobacterium
peptostreptococcus
what is pneumocystis jiroveci
also called carinnii pneumonia (PCP)
unicellular eukaryote - fungus
MC opportunitistic infection in AIDS
features of pneumocystis jiroveci pneumonia : hiv
dyspnoea
dry cough
fever
very few chest signs
name a common comp of pneumocystic jiroveci pneumonia
pneumothorax
name 3 extrapulmonary manifestations of pneumocystis jiroveci pneumonia
RARE 1-2%
hepatosplenomegaly
lymphadenopathy
choroid lesions
investigations of pneumocystis jiroveci pneumonia
CXR - bilateral interstitial pulmonary infiltrates. can present with lobar consolidation. could even be normal.
Exercise-induced desaturation : PCP causes this
sputum: fails to show PCP - so you do BAL - bronchoalveolar lavage - shows PCP - silver stain shows characteristic cysts!!
management of pneumocystis jiroveci pneumonia
co-trimoxazole
IV pentamidine in severe case
aerosolized pentamidine alternative tx for PCP but less effective and risk of pneumothorax too.
steroids: if hypoxic (po2<9.3) then steroids reduce rep failure risk by 50% and death by 1/3
at what cd4 count should patient recieve prophylaxis for pneumocystis jiroveci pneumonia
less than 200/mm3
complications of mycoplasma pneumoniae
pericarditis/myocarditis
renal: acute glomerulonephritis
gi: hepatitis, pancreatitis
cold agglutins (IgM): poss haemolytic anaemia, thrombocytopenia
erythema multiforme: erythema nodosum
meningoencephalitis, GBS, immune mediated neuro diseases
bullous myringitis: painful vesicles on tympanic membrane.
What is asthma?
chronic inflammatory disorder of airways secondary to t1 hypersensitivity.
variable sx
reversible bronchospasm - resulting in airway obstruction.
present at any age. - typical childhood.
risk factors of asthma
personal/fhx of atopy
antenatal factors: maternal smoking, viral infection during pregnancy (RSV)
low birth weight
not being breastfed
maternal smoking around child
air pollution
exposure to high conc of allergens (house dust mite)
hygiene hypothesis: reduced exposure to infectious agents in kids prevents normal development of immune system = th2 predominant response
patients with asthma suffer from which other IgE mediated atopic conditions
ATOPY
atopic dermatitis (eczema)
allergic rhinitis (hay fever)
fun fact about nasal polyps and asthma patients
pt most sensitive to asthma also suffer from nasal polyps
what drug are asthma pts sensitive to
aspirin
How would you figure our occupational asthma?
common occupational allergens
may present with concern of chemicals at work. (worsening their asthma)
observe reduced peak flows during working week with normal readings when not at work.
isocyanates MC - spray paint, foam moulding using adhesives
platinum salts, epoxy resins, proteolytic enzymes, soldering flux resin
flour, glutaraldehyde
SERIAL MEASUREMENTS OF PEAK EXPIRATORY FLOW - AT WORK AND AWAY.
symptoms of asthma
cough: often worse at night
dyspnoea
wheeze, chest tightness
signs of asthma
expiratory wheeze on auscultation
reduced peak expiratory flow rate (PEFR)
explain spirometry
tell me the key metrics
spirometry - measure amount (volume) and speed (flow) of air during exhalation and inhalation.
can help categorising resp disorder as obstructive (obstruction to airflow like bronchoconstriction in asthma) or restrictive where there is restriction to lungs like lung fibrosis)
fev1: forced expiratory volume - volume exhaled at the end of the 1st second of forced expiration
fvc: forced vital capacity - volume exhaled after maximal expiration following full inspiration
what is the investigations in asthma?
spirometry :
fev1 - significantly reduced
fvc: normal
fev1% (fev1/fvc) <70%
cxr: particularly in older pts with smoking hx
Fractional exhaled nitric oxide (FeNO): 40 ppb in adults or 35ppb (upto 16) considered positive
tell me a little bit about FeNO
fractional exhaled nitric oxide
nitric oxide produced by 3 types of nitric oxide synthases (NOS)
one of the types is iNOS - inducible - rises in inflammatory cells - particularly eosinophils
levels of NO therefore typically correlate with inflammation levels
features of acute asthma
worsening dyspnoea , wheeze and cough - not responding to salbutamol
might be trigged by RTI
how to split acute severe asthma into moderate, severe or life threatening
PEFR
moderate: PEFR 50-75% - BEST OR PREDICTED - speech normal - RR<25/MIN - Pulse <110 bpm
severe: PEFR : 33-50% or predicted. cant complete sentences. RR>25/min. Pulse >110 bpm
life- threatening : PEFR <33% AT BEST OR PREDICTED. ox sats <92%. - silent chest, cyanosis or feeble resp effort. bradycardia, dysrhythmia or hypotension. exhaustion, confusion or coma
in an acute asthma attack what would indicate exhausation and therfore lifethreatning?
normal pCO2
at what age should a patient with suspected asthma have objective tests?
5 or older.
diagnostic testing for asthma in 5-16 yr olds
spirometry with a bronchodilator reversibility test (BDR)
FeNO test request if normal spirometry or obstructive spirometry with negative BDR
how would you make a diagnosis for a pt under 5 for asthma?
clinical judgement
patients 17 or over how would you diagnosis with asthma?
ask pt if sx better on days from work/.during holidays. - if so refer poss occupational asthma.
spirometry with BDR
FeNO test
results quick summary for asthma
FENO : adults - 40ppb children 35ppb - that or more
Spirometry : fev1/fvc less than 70% - obstructive
BDR : adults : improvement in FEV1 of 12% or more and increase in volume of 200ml or more - positive
children : improvement in fev1 of 12% or more
how would you identify “near fatal asthma”
raised pCO2 and/or requiring mechanical ventilation with raised inflation pressures.
when would you recommend cxr in acute severe asthma?
if lifethreatening
suspected pneumothorax
failure to respond to tx.
when would you do an ABG for acute severe asthma
in pts with ox sats <92%
how would you manage acute severe asthma?
admission : lifethreatening, not responding to tx, pregnant,
ox: if hypoxaemic supplemental ox, if acutely unwell 15L of supplemental via non-rebreathe mask - then titre down to flow rate when maintaining 94-98% sats
bronchodilation with SABA : salbutamol, terbutaline. give by standard pressuring metered dose inhaler (pMDI) if not life threatening or near fatal. or oxygen driven nebuliser
if life threatening , NEVBULISED SAMA
corticosteroid: 40-50mg of pred orally daily, continue for 5 days until pt recovers from attack. - continue normal meds too. included inhaled corticosteroids.
ipratroopium bromide : if severe/life threatening asthma - or if pts havent responded to beta agonist/corticoteroid tx, nebulised ipratoprium bromide, a SAMA.
IV MAGNESIUM SULPHATE: can give for severe/life threatening asthma
consultation required to give iv aminophylline
what happens if patient is admitted into hospital and fails to respond to tx and are becoming acidotic?
ITU/HDU
intubation and ventilation
extracorporeal membrane oxygenation (ECMO)
what is the criteria for discharge for acute severe asthma attack?
stable on discharge meds - no nebulisers or oxygen for 12-24 hrs
inhaler technique checked and recordd
PEF >75% of best or predicted.
general management of asthma - adults
stepwise
1- new asthma diagnosis - SABA
2- not controlled prior or new diagnosis with symptoms 3 or more/week or night-time waking : SABA + low dose ICS
3 - SABA + low dose ICS + LTRA
4- saba + low dose ics+ LABA (continue ltra is respond well)
5 - SABA +/- ltra - SWITCH ICS/LABA FOR A maintenance and reliver therapy(MART) that includes a low dose ICS
6- SABA +/- LTRA + medium dose ICS MART
or consider changing back to fixed dose of moderate dose ics and a seperate LABA
7 - SABA +/LTRA + ONE OF :
INCREASE ICS TO HIGH DOSE
TRIAL ADDITIONAL DRUG - LAMA OR THEOPHYLINE
What is a mart?
mainentance and reliever therapy
ics+ laba in single inhaler - used for both daily maintenance and relief of sx as required.
only available for ics+ laba in which laba has a fast acting component eg formoterol
dosing of inhaled corticosteroid doses
low dose: <=400 micrograms budesonide or equivalent = low dose
400 micrograms-800 budesonide or equivalent = moderate
over 800 - high dose
assessing acute attack of asthma in children
moderate and severe
moderate: ox over 92 and no clinical features of asthma
spo2 under 92%
PEF 33-50% best or predicted
too breathless to talk or feed
hr : over 125 - if over 5. or over 140 if 1-5 yrs
rr :
over 30 - if over 5 or over 40 breaths per min if 1-5 yrs
use of accessory neck muscles
assessing acute asthma attack in children
life threatening
spo2 less than 92%
PEF <33% best or predicted
silent chest
poor resp effort
agitation
altered conciousness
cyanosis
asthma management for children 5-16
same stepwise as adults.
only difference is when you give ics : it is a paediatric low,moderate, high dose
also in step 4. stop ltra if it hasnt helped.
management of asthma in children under 5
stepwise
1 - SABA - newly diagnosed
2- SABA + 8wk trial of paediatric moderate dose of ICS
3. SABA + Paeds low ics + ltra
4 - stop ltra and refer to paeds asthma specialist.
tell me a little bit about stepping down tx in asthma pts
every 3 months.
not a strict move from step 3 to 2 - take into account duration of tx, side effects and pt preference.
when reducing dose of ics - 25-50% at a time.
in step 2 of stepwise management of asthma in children under 5, what to do if sx didnt resolve with the trial of ics
alternative diagnoses is likely
what would you do if in step 2 of stepwise management of asthma in children under 5, the sx resolved then reoccured within 4 weeks of stopping it?
longer than 4 weeks after stopping?
restart ics at paeds low dose as 1st line maintenance therapy
if they start after 4 weeks then repeat 8 wk trial of ics
paediatric ics dosing - asthma
<=200 micrograms budesonide or equivalent = paeds low dose
200-400 - moderate
over 400 - high dose
when stratifying a child for moderate,severe, and life threatening attack what is the diff?
severe for under 5 is hr over 140. for over 5 its over 125.
for under 5 is over 40/min for over 5 its over 30/min
and ofcourse you cant do PEF in under 5
so
over 5
pef>50% - mod
pef 33-50 - severe
pef <33% - lie threatening
for a kid with mild-moderate acute asthma what to do you do?
bronchodilator therapy :
give beta 2 agonist via a spacer : - for kid under 3 use close-fitting mask
1 puff every 3-60 seconds upto max of 10 puffs
if sx not controlled repeat beta 2 agonist and refer to hosp
steroids:
give to all kids with exacerbation
tx for 3-5 days
dosing of pred:
2-5 : 20mg OD / 1-2mg/kg OD - max 40mg
over 5 : 30-40 mg od / 1-2mg/kg OD - max 40mg
what is bronchiolitis?
acute bronchiolar inflammatory.
respiratory syncytial virus - RSV - pathogen in 75-80% cases.
other cases: mycoplasma, adenoviruses
could be secondary to bacterial infection.
more serious if bronchopulmonary dysplasia (eg premature), congenital heart disease or CF
epidemiology of bronchiolitis
MC of serious lrti in under 1 - 90% are 1-9 mths - peak incidence 3-6 mths.
material IgG gives protection to newborns against RSV.
higher incidence in winter
features of bronchiolitis
coryzal sx (including mild fever) precede:
- dry cough
- increasing breathlessness
- wheezing, fine inspiratory crackles (not always)
- feeding difficulties with increasing dyspnoea = often reasons for hosp admission
when should you do immediate referral by ambulance for bronchiolitis pt?
if they have any of :
apnoea - observed/reported
child looks unwell to professional
central cyanosis
persistent ox sats less tha 92 on air
severe resp distress: grunting, marked chest recession, RR over 70/min
when should you consider referral to hospital for bronchiolitis pt?
RR over 60/min
clinical dehydration
difficulty breastfeeding/inadequate oral fluid intake (50-75% of usual volume)
what investiagation would you do for bronchiolitis?
immunofluorescence of nasopharyngeal secretions might show RSV
how would you manage bronchiolitis?
mostly supportive - no abx
humidified ox via head box - if ox sats persistently under 92
nasogastric feeding - if kid cant take enough fluid/feed by mouth
suction - if excessive upper airway secretions
what is cystic fibrosis?
autosomal recessive.
organisms which may colonise CF patients
-Staphylococcus aureus
-pseudomonas aeruginosa
- burkholderia cepacia - previously known as - pseudomonas cepacia
- aspergillus
80% of CF cases are due to what genetics?
delta F508 on long arm of chromosome 7.
CF affects how many births?
carrier rate?
1 per 2500 births
carrier rate : 1 in 25
Presenting features of cystic fibrosis
neonatal period (20% approx.) - meconium ileus, less commonly prolonged jaundice
recurrent chest infections (40%)
malabsorption (30%) : steatorrhea (due to fat malabsorption) , failure to thrive
other features (10%) - liver disease
other non presenting features of cystic fibrosis
short stature
DM
delayed puberty
rectal prolapse (due to bulky stools)
nasal polyps
male infertility (absent vas deferens leading to infertility) : female subfertility (delay in conceiving naturally)
when is cf picked up?
newborn screening programmes or early childhood.
5% pts - after 18
cystic fibrosis : how to diagnose?
sweat test:
- patient with CF have abnormally high sweat chloride
- normal value <40 mEq/l, CF : >60 mEq/l
causes of false positive sweat test - cystic fibrosis
malnutrition
adrenal insufficiency
G6PD
ectodermal dysplasia
hypothyroidism, hypoparathyroidism
nephrogenic diabetes insipidus
glycogen storage diseases
causes of false negative sweat test - cystic fibrosis
skin oedema due to :
hypalbuminaemia/hypoproteinaemia secondary to pancreatic exocrine insufficiency
chronic management of cystic fibrosis
twice daily chest physio and postural drainage - parents taught how to do. - deep breathing poss?
high calorie high fat intake.
minimise contact with each other prevent cross infection with BURKHOLDERIA CEPACIA complex and PSEDUOMONAS AERUGINOSA.
Vit supplementation
pancreatic enzyme supplements - take with meals
lung transplantation - but if you have chronic infection with burkholderia cepacia it is a cf-specific CI to lung transplant.
what drug is used to treat cf patients homozygous for delta F508 mutation ?
lumacaftor/ ivacaftor (orkambi)
treats CF patients homozygous for delta F508 mutation
how does lumacaftor work?
increases number of CFTR proteins that are transported to the cell surface.
how does ivacaftor work?
potentiator of CFTR that is already at cell surface.
increases probability that defective channel will be open and allow chloride ions to pass through channel pore.
