l CAUTIONS History of peptic ulceration
▶ Rare or very rare Haemorrhage
▶ Frequency not known Face oedema
l MEDICINAL FORMS There can be variation in the licensing of
different medicines containing the same drug. Forms available
from special-order manufacturers include: effervescent tablet,
CAUTIONARY AND ADVISORY LABELS 13
ELECTROLYTES: May contain Sodium
▶ NACSYS (Atlantic Pharma Ltd)
Acetylcysteine 600 mg NACSYS 600mg effervescent tablets sugarfree | 30 tablet P £5.50 DT = £89.50
▶ Adult: Initially 2.25 g daily in divided doses, then
reduced to 1.5 g daily in divided doses, as condition
l CONTRA-INDICATIONS Active peptic ulceration
l CAUTIONS History of peptic ulceration (may disrupt the
l SIDE-EFFECTS Gastrointestinal haemorrhage . skin
reactions . Stevens-Johnson syndrome . vomiting
l PREGNANCY Manufacturer advises avoid in first trimester.
l BREAST FEEDING No information available.
l PRESCRIBING AND DISPENSING INFORMATION Flavours of
oral liquid formulations may include cherry, raspberry,
l MEDICINAL FORMS There can be variation in the licensing of
different medicines containing the same drug.
▶ Carbocisteine (Non-proprietary)
Carbocisteine 50 mg per 1 ml Carbocisteine 250mg/5ml oral
solution | 300 ml P £8.55 DT = £8.55
Carbocisteine 75 mg per 1 ml Carbocisteine 750mg/10ml oral
solution 10ml sachets sugar free sugar-free | 15 sachet P £3.85
Carbocisteine 50 mg per 1 ml Mucodyne Paediatric 250mg/5ml
Mucodyne 250mg/5ml syrup | 300 ml P £8.39 DT = £8.55
▶ Carbocisteine (Non-proprietary)
Carbocisteine 375 mg Carbocisteine 375mg capsules | 120 capsule P £18.98 DT = £4.60
Carbocisteine 375 mg Mucodyne 375mg capsules | 120 capsule P £18.98 DT = £4.60
Symptomatic treatment of acute exacerbations of chronic
▶ Adult: 300 mg twice daily for up to 10 days
l CAUTIONS History of peptic ulceration (may disrupt the
▶ Common or very common Epigastric pain .taste altered
▶ Uncommon Allergic dermatitis . angioedema . common
cold . diarrhoea . dyspnoea . headache . nausea . vomiting
l PREGNANCY Manufacturer advises avoid—no information
l BREAST FEEDING Manufacturer advises avoid—no
l HEPATIC IMPAIRMENT Manufacturer advises caution in
mild to moderate hepatic failure; avoid in severe hepatic
Dose adjustments Manufacturer advises max. 300 mg daily
in mild to moderate hepatic failure.
l RENAL IMPAIRMENT Avoid if eGFR less than
l NATIONAL FUNDING/ACCESS DECISIONS
Scottish Medicines Consortium (SMC) decisions
The Scottish Medicines Consortium (November 2007) has
advised that erdosteine (Erdotin ®) is not recommended for
use within NHS Scotland for the symptomatic treatment of
acute exacerbations of chronic bronchitis as the economic
l MEDICINAL FORMS There can be variation in the licensing of
different medicines containing the same drug.
Erdosteine 300 mg Erdotin 300mg capsules | 20 capsule P £5.00 DT = £5.00
Cystic fibrosis is a genetic disorder affecting the lungs,
pancreas, liver, intestine, and reproductive organs. The main
clinical signs are pulmonary disease, with recurrent
infections and the production of copious viscous sputum,
and malabsorption due to pancreatic insufficiency. Other
complications include hepatobiliary disease, osteoporosis,
cystic fibrosis-related diabetes, and distal intestinal
The aim of treatment includes preventing and managing
lung infections, loosening and removing thick, sticky mucus
from the lungs, preventing or treating intestinal obstruction,
and providing sufficient nutrition and hydration.
Lung function is a key predictor of life expectancy in
people with cystic fibrosis and optimising lung function is a
Specialist physiotherapists should assess patients with cystic
fibrosis and provide advice on airway clearance, nebuliser
use, musculoskeletal disorders, physical activity, and urinary
incontinence. The importance of airway clearance
techniques should be discussed with patients and their
parents or carers and appropriate training provided. Patients
should be advised that regular exercise improves both lung
Treatment for cystic fibrosis lung disease is based on the
prevention of lung infection and the maintenance of lung
function.g In patients with cystic fibrosis, who have
clinical evidence of lung disease, the frequency of routine
review should be based on their clinical condition, but adults
should be reviewed at least every 3 months. More frequent
review is required immediately after diagnosis. h
g Patients with cystic fibrosis who have evidence of lung
disease should be offered a mucolytic. Dornase alfa p. 293 is
the first choice mucolytic. If there is an inadequate response,
dornase alfa p. 293 and hypertonic sodium chloride p. 275, or
hypertonic sodium chloride p. 275 alone should be
Mannitol dry powder for inhalation p. 295 is recommended
as an option when dornase alfa p. 293 is unsuitable (because
of ineligibility, intolerance, or inadequate response), when
lung function is rapidly declining, and if other osmotic drugs
are not considered appropriate (see mannitol p. 295 National
Lumacaftor with ivacaftor p. 294 is not recommended for
treating cystic fibrosis within its marketing authorisation
(see lumacaftor with ivacaftor p. 294 National
g Patients who are not taking prophylaxis and have a new
are clinically unwell and have pulmonary disease, oral or
should be given (consult local protocol).
A long-term antibacterial should be considered to
suppress chronic Staph. aureus respiratory infections in
patients whose pulmonary disease is stable. In patients with
chronic Staph. aureus respiratory infections who become
clinically unwell with pulmonary disease, oral or intravenous
(depending on infection severity) broad-spectrum
antibacterials with activity against Staph. aureus should be
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 cystic fibrosis 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
g If a patient with cystic fibrosis develops a new
Pseudomonas aeruginosa infection, 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
If eradication therapy is not successful, sustained
treatment with an inhaled antibacterial should be offered.
Nebulised colistimethate sodium p. 556 should be
considered as first-line treatment (but see also
colistimethate sodium by dry powder inhalation p. 556
National funding/access decisions).
In patients with chronic Ps. aeruginosa infection (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
Nebulised aztreonam p. 541, nebulised tobramycin, or
tobramycin dry powder for inhalation (see tobramycin p. 520
National funding/access decisions) should be considered for
those who are deteriorating despite regular inhaled
colistimethate sodium p. 556. h
g Patients who develop a new Burkholderia 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). There is no evidence to support using
antibacterials to suppress chronic Burkholderia cepacia
complex infection in patients with cystic fibrosis who have
Specialist microbiological advice should be sought for
patients with chronic Burkholderia cepacia complex infection
(when treatment has not eradicated the infection) and who
become clinically unwell with a pulmonary disease
An inhaled antibacterial should be considered for those
who have chronic Burkholderia cepacia complex infection
and declining pulmonary status; treatment should be
stopped if there is no observed benefit. h
gHaemophilus influenzae infection 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
g Non-tuberculous mycobacterial eradication therapy
should be considered for patients with cystic fibrosis 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
g Treatment with an antifungal drug should only be
considered to suppress chronic Aspergillus fumigatus
complex respiratory infection in patients with declining
pulmonary status. Specialist microbiological advice should
be sought on the choice of antifungal drug. h
g An 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
g Long-term treatment with azithromycin p. 536
[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, long-term
292 Conditions affecting sputum viscosity BNF 78
azithromycin p. 536 should be discontinued and the use of
an oral corticosteroid considered. h
Nutrition and exocrine pancreatic insufficiency
g The cystic fibrosis specialist dietitian should offer
Pancreatin p. 96 should be offered to patients with
exocrine pancreatic insufficiency. Dose should be adjusted as
needed to minimise any symptoms or signs of malabsorption
proton pump inhibitor [unlicensed indications] can be
considered for patients who have persistent symptoms or
A short-term trial of an appetite stimulant (for example
up to 3 months) [unlicensed indication] can be considered in
adult patients if attempts to increase calorie intake are not
Distal intestinal obstruction syndrome
g Oral or intravenous fluids should be offered to ensure
adequate hydration for patients with distal intestinal
obstruction syndrome. Meglumine amidotrizoate with
sodium amidotrizoate solution (orally or via an enteral tube)
should be considered as first-line treatment for distal
intestinal obstruction syndrome. An iso-osmotic
polyethylene glycol and electrolyte solution (macrogols)
treatment with a polyethylene glycol solution is not
effective. Suspected distal intestinal obstruction syndrome
should be managed in a specialist cystic fibrosis centre. h
g If liver function blood tests are abnormal in patients
with cystic fibrosis, ursodeoxycholic acid p. 89 [unlicensed
indication] can be given until liver function is restored. h
g Patients should be monitored for cystic fibrosis-related
Cystic fibrosis-related diabetes
g Patients should be monitored for cystic fibrosis-related
Cystic fibrosis: diagnosis and management. National
Institute for Health and Care Excellence. NICE guideline 78.
(Phosphorylated glycosylated recombinant human
deoxyribonuclease 1 (rhDNase))
l DRUG ACTION Dornase alfa is a genetically engineered
version of a naturally occurring human enzyme which
cleaves extracellular deoxyribonucleic acid (DNA).
Management of cystic fibrosis patients with a forced vital
capacity (FVC) of greater than 40% of predicted to
▶ BY INHALATION OF NEBULISED SOLUTION
▶ Adult: 2500 units once daily, administered by jet
nebuliser, patients over 21 years may benefit from
DOSE EQUIVALENCE AND CONVERSION
▶ Dornase alfa 1000 units is equivalent to 1 mg
l PREGNANCY No evidence of teratogenicity; manufacturer
advises use only if potential benefit outweighs risk.
l BREAST FEEDING Amount probably too small to be
harmful—manufacturer advises caution.
l DIRECTIONS FOR ADMINISTRATION Dornase alfa is
administered by inhalation using a jet nebuliser, usually
once daily at least 1 hour before physiotherapy; however,
alternate-day therapy may be as effective as daily
For use undiluted with jet nebulisers only; ultrasonic
l MEDICINAL FORMS There can be variation in the licensing of
different medicines containing the same drug.
▶ Pulmozyme (Roche Products Ltd)
Dornase alfa 1 mg per 1 ml Pulmozyme 2.5mg nebuliser liquid 2.5ml
ampoules | 30 ampoule P £496.43 DT = £496.43
l DRUG ACTION Ivacaftor is a cystic fibrosis transmembrane
conductance regulator (CFTR) protein potentiator that
increases chloride transport in the abnormal CFTR protein.
Cystic fibrosis (specialist use only)
▶ Adult: 150 mg every 12 hours
DOSE ADJUSTMENTS DUE TO INTERACTIONS
▶ Manufacturer advises reduce dose to 150 mg twice a
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