treatment started. A prophylactic antibacterial should be
restarted after treatment (consult local protocol).
Patients who are not taking prophylaxis and have a new
Staph. aureusinfection can be given an oral anti-Staph.
aureusantibacterial, if they are clinically well. If they are
clinically unwell and have pulmonary disease, oral or
intravenous (depending on infection severity) broad-
spectrum antibacterials with activity againstStaph. aureus
should be given (consult local protocol).
A long-term antibacterial should be considered to
suppresschronicStaph. aureusrespiratory infection in
patients whose pulmonary disease is stable. In patients with
chronicStaph. aureusrespiratory infection who become
clinically unwell with pulmonary disease, oral or intravenous
(depending on infection severity) broad-spectrum
antibacterials with activity againstStaph. aureusshould be
given. In those patients with new evidence ofmeticillin-
resistantStaphylococcus aureus(MRSA)respiratory
infection (with or without pulmonary exacerbation),
specialist microbiological advice should be sought.
Antibacterials should not be routinely used to suppress
chronic MRSA in patients with stable pulmonary disease.
If a patient with cysticfibrosis and chronic MRSA
respiratory infection becomes unwell with a pulmonary
exacerbation or shows a decline in pulmonary function,
specialist microbiological advice should be sought.h
Pseudomonas aeruginosa
gIf a patient with cysticfibrosis develops a new
Pseudomonas aeruginosainfection, eradication therapy with
a course of oral antibacterial should be started (by
intravenous injection, if they are clinically unwell), in
combination with an inhaled antibacterial. An extended
course of oral and inhaled antibacterial should follow
(consult local protocol).
If eradication therapy is not successful, sustained
treatment with an inhaled antibacterial should be offered.
Nebulised colistimethate sodium p. 346 should be
considered asfirst-line treatment (but see also
colistimethate sodium by dry powder inhalation National
funding/access decisions).
In patients withchronicPs. aeruginosainfection (when
treatment has not eradicated the infection) who become
clinically unwell with pulmonary exacerbations, an oral
antibacterial or a combination of two intravenous
antibacterial drugs of different classes (depending on
infection severity) should be used. Changing antibacterial
regimens should be considered to treat exacerbations
(consult local protocol).
Nebulised aztreonam p. 332 , nebulised tobramycin p. 313 ,
or tobramycin dry powder for inhalation p. 313 [unlicensed
indication in child under 6 years] (see tobramycin National
funding/access decisions) should be considered for those
who are deteriorating despite regular inhaled colistimethate
sodium p. 346 .h
Burkholderia cepaciacomplex
gPatients who develop a newBurkholderia cepacia
complex infection, should be given eradication therapy with
a combination of intravenous antibacterial drugs (specialist
microbiological advice should be sought on the choice of
antibacterials).hThere is no evidence to support using
antibacterials to suppresschronicBurkholderia cepacia
complex infection in patients with cysticfibrosis who have
stable pulmonary status.
gSpecialist microbiological advice should be sought
for patients with chronicBurkholderia cepaciacomplex
infection (when treatment has not eradicated the infection)
and who become clinically unwell with a pulmonary disease
exacerbation.
An inhaled antibacterial should be considered for those
who have chronicBurkholderia cepaciacomplex infection
and declining pulmonary status; treatment should be
stopped if there is no observed benefit.h
Haemophilus influenzae
gHaemophilus influenzaeinfection in the absence of
clinical evidence of pulmonary infection should be treated
with an appropriate oral antibacterial drug. In those who are
unwell with clinical evidence of pulmonary infection, an
appropriate antibacterial should be given by mouth or
intravenously depending on the severity of the illness
(consult local protocol).h
Non-tuberculous mycobacteria
gNon-tuberculous mycobacterial eradication therapy
should be considered for patients with cysticfibrosis who are
clinically unwell and whose pulmonary disease has not
responded to other recommended treatments. Specialist
microbiological advice should be sought on the choice of
antibacterial and on the duration of treatment.h
Aspergillus fumigatuscomplex
gTreatment with an antifungal drug should only be
considered to suppresschronicAspergillus fumigatus
complex respiratory infection in patients with declining
pulmonary status. Specialist microbiological advice should
be sought on the choice of antifungal drug.h
Unidentified infections
gAn oral or intravenous (depending on the exacerbation
severity) broad-spectrum antibacterial should be used for
patients who have a pulmonary disease exacerbation and no
clear cause. If a causative pathogen is identified, an
appropriate treatment should be selected (consult local
protocol).hImmunomodulatory drugs
gLong-term treatment with azithromycin p. 329
[unlicensed indication], at an immunomodulatory dose,
should be offered to patients with deteriorating lung
function or repeated pulmonary exacerbations. In those
patients with continued deterioration in lung function or
continuing pulmonary exacerbations, azithromycin should
be discontinued and the use of an oral corticosteroid
considered.h
Nutrition and exocrine pancreatic insufficiency
gA cysticfibrosis specialist dietitian should offer advice
on optimal nutrition.
Pancreatin p. 72 should be offered to patients with
exocrine pancreatic insufficiency. Dose should be adjusted as
needed to minimise any symptoms or signs of malabsorption
(see Exocrine pancreatic insufficiency p. 71 ). An acid-
suppressing drug, such as an H 2 receptor antagonist or a
proton pump inhibitor [unlicensed indications] can be
considered for patients who have persistent symptoms or
signs of malabsorption.h
Distal intestinal obstruction syndrome
gOral or intravenousfluids should be offered to ensure
adequate hydration for patients with distal intestinal
obstruction syndrome. Meglumine amidotrizoate with
sodium amidotrizoate solution p. 36 (orally or via an enteral
tube) should be considered asfirst-line treatment for distal
intestinal obstruction syndrome. An iso-osmotic
polyethylene glycol and electrolyte solution (macrogols)
(orally or via an enteral tube) can be considered as a second-
line treatment. Surgery is a last resort, if prolonged
treatment with a polyethylene glycol solution is not
effective. Suspected distal intestinal obstruction syndrome
should be managed in a specialist cysticfibrosis centre.h
Liver disease
gIf liver function blood tests are abnormal in patients
with cysticfibrosis, ursodeoxycholic acid p. 65 [unlicensed
indication] can be given until liver function is restored.h
Bone mineral density
gPatients should be monitored for cysticfibrosis-related
low bone mineral density.hBNFC 2018 – 2019 Cysticfibrosis 185
Respiratory system3