lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Powder and solvent for solution for injection
ELECTROLYTES:May contain Sodium
▶Berinert P(CSL Behring UK Ltd)
C1-esterase inhibitor 500 unitBerinert 500 unit powder and solvent
for solution for injection vials| 1 vialP£ 550. 00
C1-esterase inhibitor 1500 unitBerinert 1 , 500 unit powder and
solvent for solution for injection vials| 1 vialP£ 1 , 650. 00
▶Cinryze(Shire Pharmaceuticals Ltd)A
C1-esterase inhibitor 500 unitCinryze 500 unit powder and solvent
for solution for injection vials| 2 vialP£ 1 , 336. 003 Conditions affecting
sputum viscosity
Other drugs used for Conditions affecting sputum
viscosityAcetylcysteine, p. 671MUCOLYTICS
Carbocisteine
lINDICATIONS AND DOSE
Reduction of sputum viscosity
▶BY MOUTH
▶Child 2–4 years: 62. 5 – 125 mg 4 times a day
▶Child 5–11 years: 250 mg 3 times a day
▶Child 12–17 years:Initially 2. 25 g daily in divided doses,
then reduced to 1. 5 g daily in divided doses, as
condition improvesIMPORTANT SAFETY INFORMATION
Mucodyne Paediatric®syrup 250 mg/ 5 mL has replaced
the 125 mg/ 5 mL formulation—take care to ensure the
appropriate dose is administered.lCONTRA-INDICATIONSActive peptic ulceration
lCAUTIONSHistory of peptic ulceration (may disrupt the
gastric mucosal barrier)
lSIDE-EFFECTSGastrointestinal haemorrhage.skin
reactions.Stevens-Johnson syndrome.vomiting
lPREGNANCYManufacturer advises avoid infirst trimester.
lBREAST FEEDINGNo information available.
lPRESCRIBING AND DISPENSING INFORMATIONFlavours of
oral liquid formulations may include cherry, raspberry,
cinnamon, or rum.lMEDICINAL FORMS
There can be variation in the licensing of different medicines
containing the same drug.
Oral solution
▶Carbocisteine (Non-proprietary)
Carbocisteine 50 mg per 1 mlCarbocisteine 250 mg/ 5 ml oral
solution| 300 mlP£ 8. 55 DT = £ 8. 39
Carbocisteine 75 mg per 1 mlCarbocisteine 750 mg/ 10 ml oral
solution 10 ml sachets sugar free sugar-free| 15 sachetP£ 3. 85
DT = £ 3. 85
▶Mucodyne(Sanofi)
Carbocisteine 50 mg per 1 mlMucodyne Paediatric 250 mg/ 5 ml
syrup| 125 mlP£ 12. 60
Mucodyne 250 mg/ 5 ml syrup| 300 mlP£ 8. 39 DT = £ 8. 39
Capsule
▶Carbocisteine (Non-proprietary)
Carbocisteine 375 mgCarbocisteine 375 mg capsules|
120 capsuleP£ 18. 98 DT = £ 4. 84
▶Mucodyne(Sanofi)
Carbocisteine 375 mgMucodyne 375 mg capsules|
120 capsuleP£ 18. 98 DT = £ 4. 843.1 Cystic fibrosis
Cystic fibrosis 10-Nov-2017
Description of condition
Cysticfibrosis 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,
cysticfibrosis-related diabetes, and distal intestinal
obstruction syndrome.Aims of treatment
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 cysticfibrosis and optimising lung function is a
major aim of care.Non-drug treatment
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
function and overallfitness.Drug treatment
Treatment for cysticfibrosis lung disease is based on the
prevention of lung infection and the maintenance of lung
function.gIn patients with cysticfibrosis, who have
clinical evidence of lung disease, the frequency of routine
review should be based on their clinical condition, but
children should be reviewed at least every 8 weeks. More
frequent review is required immediately after diagnosis and
during early life.hMucolytics
gPatients with cysticfibrosis who have evidence of lung
disease should be offered a mucolytic. Dornase alfa p. 186 is
thefirst choice mucolytic [unlicensed in children under
5 years of age]. If there is an inadequate response, dornase
alfa p. 186 and hypertonic sodium chloride p. 170 ,or
hypertonic sodium chloride alone should be considered.
Mannitol dry powder for inhalation p. 141 should be
considered for children [unlicensed indication] who cannot
use dornase alfa and hypertonic sodium chloride because of
ineligibility, intolerance, or inadequate response.h
Lumacaftor with ivacaftor p. 187 is not recommended for
treating cysticfibrosis within its marketing authorisation
(see lumacaftor with ivacaftor National funding/access
decisions).Pulmonary infection
Staphylococcus aureus
gFlucloxacillin p. 345 [unlicensed indication] should be
offered for prophylaxis against respiratoryStaphylococcus
aureusinfection in children from the point of diagnosis up to
3 years of age. Continuing prophylaxis in children up to
6 years of age should also be considered. An alternative oral
antibacterial should be given in children allergic to
penicillin.
In children who are already taking prophylaxis and have a
respiratory sample culture that is positive forStaph. aureus,
prophylaxis adherence should be reviewed and antibacterial184 Conditions affecting sputum viscosity BNFC 2018 – 2019
Respiratory system3