Inhaled Therapy - for further information, see LTHT Nebuliser Guidelines for Adults or Children

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1 Inhaled Therapy - for further information, see LTHT Nebuliser Guidelines for Adults or Children Link to: Adult Nebuliser Guidelines Link to: Paediatric Nebuliser Guidelines and Monographs ANTIBIOTICS/ ANTIFUNGALS Dose Notes Amikacin Amphoteracin (AmBisome ) Aztreonam lysine Colistimethate sodium(colistin) Colomycin Colistimethate sodium(colistin) Promixin Inhaled colistimethate sodium Colobreathe capsules for inhalation Meropenem <12years: 250mg twice daily >12years: 500mg twice daily 25mg twice daily for 7-10 days, although longer courses have been used in adults. Over 6years: 75mg three times a day Under 2 years: 1 million units twice daily Over 2 years: 2 million units twice daily 1million units twice daily Adults and children over 6 years: 1.66 million units twice daily 6-11years: 125mg twice daily 12years+ : 250mg twice daily Use injection (250mg/mL) and make up to 4 mls with sodium chloride 0.9%. Dissolve 50mg vial with 12mLs sterile water. Nebulise with a CR50, CR60 or Turboneb compressor. Monitor for bronchospasm. Supplied with diluent. Using conventional nebulisers: 1 million units: reconstitute with 4 mls sodium chloride 0.9%. 2 million units: reconstitute with 2 mls WFI and 2 mls sodium chloride 0.9% Seek advice for use with other nebulisers. Using the I-neb -The 1 million units (1,000,000) vial is reconstituted with 1mL water for injection and administered TWICE daily. Requires verbal Microbiology approval. Make to 5mLs with water for injection.

2 Tobramycin Tobi 300mg in 5mL Bramitob 300mg in 4mL Tobramycin powder for inhalation Tobi Podhaler Vancomycin MUCOLYTICS Dornase alfa (Pulmozyme ) Mannitol (Bronchitol ) Sodium chloride 7% ( Nebusal ), 3% Mucoclear 6months-6years (unlicensed): 300mg twice daily, alternate months. >6 years: 300mg twice daily, alternate months. Although only licensed in children over 6 years, this dose is used in children of all ages over 6 months Adult and child over 6 years: 112mg (4 x 28mg capsules) every 12 hours for 28 days, subsequent courses repeated after 28-day interval without tobramycin inhalation powder Child: 5mg/kg (max: 250mg) twice daily 250mg twice daily For five days usually. 2.5mg (2500units) daily Adult 18yrs and above; 400mg twice daily (Ten 40mg capsules twice a day). 4mLs up to twice a day, before physiotherapy Ready for use solutions. No further dilution necessary. NTM in Cystic Fibrosis Some patients may be on continuous tobramycin or alternate it with another inhaled antibiotic. Prescriptions should clearly state whether it is alternate months or continuous. NTM in Cystic Fibrosis Some patients may be on continuous tobramycin or alternate it with another inhaled antibiotic. Prescriptions should clearly state whether it is alternate months or continuous. Use water for injection for reconstitution and make up to 4mLs with water for injection. i.e. for 250mg dose reconstitute a 500mg vial with 8mLs water for infection and use 4mL. Administer a bronchodilator beforehand, to minimise bronchospasm. Licensed for children over 5 years of age. Limited patients may benefit from twice daily administration. Patient needs to complete test dose regimen prior to starting. 3% used when patient is unable to tolerate 7%. OTHER Taurolidine solution 2% (Taurolin ) Adults: 3-4mLs of 2% solution twice daily Unlicensed product, available as an intraperitoneal lavage (Taurolin ) on a named patient basis. May cause bronchospasm, cough or mild burning sensation in the throat.

3 Give a test dose initially. Gastrointestinal and Liver Disease DRUG DOSE NOTES N-acetylcysteine 200mg/mL injection, Parvolex, given orally Sachets of granules 200mg (Fluimicil N, unlicensed) Treatment of DIOS Child 1 month - 2 years Child 2-7 years Child 7 years- adult Prevention of DIOS Child 1 month - 2 years Child 2-12 years Child 12 years - adult 0.4g (2mL)-3g(15mLs) as a single dose 2g (10mL)-3g (15mLs) as a single dose 4g (20mLs)-6g (30mLs) as a single dose mg three times a day 200 mg three times a day mg three times a day 400mg three times a day Erythromycin (for gastric stasis only) All ages 3 mg/kg (20 minutes before feeds) four times a day. Max: 250 mg four times a day Dilute injection to a concentration of 50mg/mL; orange or blackcurrant juice or cola drink may be used as a diluent to mask the bitter taste. Single dose may be repeated up to three times a day. Acetylcysteine 20%, 30mLs, can also be added to a phosphate enema and given rectally for DIOS. Oral Gastrografin DAY 1: 1 month- 2 years kg ml diluted to mL with water or cordial, once daily 50 ml diluted to 200mL with water or cordial, once daily Hydration of patient is essential, monitor intravascular fluid volume carefully. Fluid intake should be encouraged for 3 hours after administration. Over 25 kg 100 ml with 400 ml of water or cordial once daily DAY 2 & 3 Half Day 1 dose Rectal Gastrografin 1 month- 2 years 2-12 yrs, <25 kg ml single dose 50 ml single dose Dilute enema 3-5 times with water. Administer under radiological

4 Over 25 kg 100 ml single dose guidance only. Klean Prep orally or via nasogastric tube Children: 10 ml/kg/hr for 30 mins then 20 ml/kg/hr for 30 mins then increase to 25 ml/kg/hr if well tolerated. Max volume over 4 hours: 100 mls/kg or 4L, whichever is smaller. Patients must be reviewed at 4 hours. If output is not yet clean, a further 4 hours treatment may be prescribed. Max total volume over 8 hours: 200 ml/kg or 8L, whichever is smaller. In practice we seldom administer more than 4L in any 24 hour period, and more usually prescribe 2L a day for 2 days. Adults: 1 sachet in one litre water, twice a day Hypoglycaemia has been reported. Lansoprazole Orodispersible tablets or capsules Infant 2.5kg-5kg 5-10kg 10-30kg >30kg 3.75mg (¼ a 15mg tablet) once daily 7.5mg (½ a 15mg tablet)once daily 15mg once daily 30mg once daily Movicol Child: 1-5 years 2 sachets on first day, then 4 sachets daily for 2 days, then 6 daily for 2 days, then 8 sachets daily for 2 days. Treat until impaction resolves or for max of 7 days. Total daily dose to be taken over 12 hour period. For impaction 5-12 years 4 sachets on first day then increased in steps of 2 sachets daily to max 12 sachets daily. After disimpaction, switch to maintenance laxative therapy. See BNF/BNFc years and adults: Movicol 2 sachets twice daily increasing to 8 sachets /day. Total daily dose to be drunk within six hour period (BNF). Omeprazole capsules or MUPS (dispersible tablets) 1 month 2 years 10-20kg 20kg+ and adults 0.7mg/kg 3mg/kg daily(max 20mg/day) 10-20mg daily 20-40mg daily Total daily dose may be divided into two daily doses if required Swallow capsules whole or open and mix contents with fruit juice or yoghurt. MUPS tablets can be dissolved in water. Consider lansoprazole orodispersible tablets as an alternative for children, and disperse in water. Ranitidine Under 6 months 1 mg/kg three times a day (Max 9 In fat malabsorption, give 1-2hours

5 6 months 18 years mg/kg/day) 2 4 mg/kg (Max. 300 mg) twice daily before food to enhance effects of enzyme replacements. Ursodeoxycholic acid 150mg tabs, 250mg caps, suspension 50mg/mL 1month-18years: 10-15mg/kg twice daily, or total daily dose divided into three doses. Usual max: 600mg three times a day. Max: 2750mg daily in divided doses. Take with or after food. SALT SUPPLEMENTATION Sodium chloride tablets. Slow Sodium 600 mg NaCl (10mmol Na+) Sodium chloride solution 1mmol/mL. In hot weather, fever or vigorous exercise. <2 years additional 2mmol/kg/day in divided doses 2-7years; 10-20mmol/day ( mg) in divided doses >7 years: 2-4g/day in divided doses 60-80mmol per day (3.6g-4.8g, 6 to 8 tablets) in divided doses The usual dose for adults with Cystic Fibrosis is 2 tablets three times a day of Slow Sodium (600mg) daily. These should be taken at regular intervals during the day, with plenty of water Slow Sodium cannot be crushed. Dioralyte sachets can be used in young children or babies. Encourage fluid intake.