Pancreatic Enzymes
- Get to know one preparation properly. This clinic uses Creon Micro (for infants) or Creon 10,000 for all children except under exceptional circumstances. See section 7.2 on PERT.
- Both creon preparations are porcine (pig) origin.
- Dose for a child established on pancreatic enzymes is approximately 1 capsule per 3-5 grams of fat.
- In babies, start with ½ scoop per feed (average fat content of 150ml standard infant milk is 5g) mixed with small amount of expressed breast milk, infant formula or apple puree*, just before feeds and increase in half scoop steps (quarters is too fiddly). Do not put Creon granules into the bottle.
- Enzymes may not be chewed or mixed into food, do not mix into hot foods
- Dose should not exceed 10,000 units/kg/day of lipase without considering why needed.
Creon Micro = 5,000 units of lipase per scoop
Pancrex V powder = 25,000 units of lipase per gram
Creon 10,000 = 10,000 units of lipase per capsule
Creon 25,000 = 25,000 units of lipase per capsule
*Note: At RBH we use apple puree to provide enzymes from birth as the puree keeps the enterically coated enzyme spheres in a suspension. This ensures that the child takes in the entire dose, and minimizes the chance of gum breakdown caused by trapped enterically coated spheres in the mouth. If apple is not available, other fruit purees may be used. If apple purees for enzyme administration are introduced from birth, they must be done so carefully as it contradicts the WHO and Department of Health recommendations on the age that solids should be introduced to infants.
See patient/parent/carer advice
Fat soluble vitamins
Empirically, the aim is to have plasma levels of vitamins A and E at upper limit of normal range. Daily recommendations from the CF Trust Nutrition Working Party are:
| Age |
Vitamin A 1 mcg = 3.3 IU |
Vitamin D 1 mcg = 40 IU |
Vitamin E 1mg = 1.5 IU |
| < 1 Year |
1200 mcg (4000 IU) |
10 mcg (400 IU) |
10 - 50 mg |
| > 1 Year | 1200 - 3000 mcg (4000 –10,000 IU) |
10 - 20 mcg (400 – 800 IU) |
50 – 100 mg |
| Adults | 1200 - 3000 mcg (4000 –10,000 IU) |
20 – 50 mcg (800 – 2000 IU) |
100-200 mg |
Preparations:
- DEKAs® Plus are a brand of all-in-one multivitamins designed for people with CF. 1ml of Paediatric Liquid contains 1,725 mcg of Vitamin A, 750 units of vitamin D, 33.6mg Vitamin E, and 0.5mg of Vitamin K. One Softgel or one chewable tablet contains 5505mcg of Vitamin A, 3000 units of vitamin D (2000 units in chewable tab), 101mg Vitamin E (67 mg in chewable tab), and 1mg of Vitamin K. All contain a number of other vitamins and trace elements, and have the advantage of containing both vitamin E and vitamin K.
- AquADEKs are a brand of all-in-one multivitamins designed for people with CF but currently unavailable. 1ml of Paediatric Liquid contains 1,743 mcg of Vitamin A, 10mcg of vitamin D, 48mg Vitamin E, and 0.4mg of Vitamin K. One Softgel or two chewable tablets contain 5505mcg of Vitamin A, 20mcg of vitamin D, 180mg (softgel)/97mg (chewable tablets) Vitamin E, and 0.7mg of Vitamin K. All contain a number of other vitamins and trace elements. Note if aquadeks is spilt it can stain clothes yellow.
We offer DEKAs Plus or Aquadeks to all newborn screened children (including those who are pancreatic sufficient). If children will not tolerate it, or if GPs are unable to continue supplies, then we will use standard dalivit and vitamin E. All patients will be supplied with a supply letter outlining information about the preparation for the GP and community pharmacist.
- Dalivit: 1.2 ml supplies 3000 mcg of vitamin A, 20 mcg of vitamin D, and no vitamin E.
- Abidec: not usually given due to low vitamin A content however may be a suitable alternative if Dalivit unavailable.
- One vitamin A+D capsule BPC contains – vitamin A 1200 mcg, vitamin D 10 mcg
- Vita-E gel capsules: 75 unit capsule ≈ 50 mg vitamin E, 400 unit capsule ≈ 268 mg vitamin E (Note that 200iu capsules no longeravailable from GPs)
Recommended dosing (empirical):
Birth to 12 months:
- Either DEKAs® Plus Liquid 1ml od
- Or AquADEKs Paediatric Liquid 1ml od
- Or Dalivit 0.6 ml + Vitamin E Liquid 50 mg (0.5ml) od
1 to 4 years:
- Either DEKAs® Plus Liquid 2ml od
- Or AquADEKs Paediatric Liquid 2ml od
- Or Dalivit1.2 ml + Vitamin E Liquid 100 mg (1ml) od
5 to 8 years:
- Either DEKAs® Plus Liquid 2ml od or DEKAs® Plus softgel or chewable tablet 1 od
- Or AquADEKs Paediatric Liquid 2ml od or 1 AquADEKsTM softgel or 2 chewable tabletsod
- Or Dalivit 1.8 ml + Vitamin E Liquid 100 mg (1ml) od
9 years and above:
- Either 2 - 3 Vitamin A&D capsules + Vitamin E (Vita-E Gel 75iu/400iu Caps) 150 - 400iu.
- Or 1 - 2 DEKAs® Plus softgels or 1-2 chewable tablets od
- Or 1 - 2 AquADEKs softgels or 2-4 chewable tablets od
Note: annual review blood levels may not reflect dosages prescribed as low levels may simply reflect poor adherence.
Vitamin D deficiency (see section 8.4)
Anyone with a vitamin D level below 50nmol/l should be treated.
Stoss therapy will be the default therapy. It involves a single oral administration of the total treatment dose of vitamin D. An alternative is the whole dose as a single intramuscular injection but there is no reason to use this. This may need to be repeated, if poor compliance persists with maintenance dosing. However the Sydney paper (Shepherd et al, JCF 2012) showed this regimen maintained vitamin D levels for a year.
Oral colecalciferol single dose:
- 1 - 12 months 150,000 units
- 1 - 12 years 300,000 units
- ≥12 years 500,000 units
The previous regimen can still be used if there are difficulties with prescribing high dose stoss therapy:
Oral colecalciferol for 3 months:
- Infant 1 - 6 months 3000 units daily
- 6 months - 12 years 6000 units daily
- ≥ 12 years 6000 - 10,000 units daily
- Alternative for older children – colecalciferol 20,000 units 3 times a week; or colecalciferol 50,000 units once a week.
This can be as:
- colecalciferol liquid 3000 units/ml.
- colecalciferol capsules or dispersible tablets 1000 units
- colecalciferol capsules 10,000 or 20,000 units
- colecalciferol liquid 50,000 unit/1ml
Vitamin K
Offered to all children aged 6 years (including pancreatic sufficient) and mandatory for those with liver disease (with or without clotting abnormalities).
Use water-soluble preparation: Menadiol phosphate tablet. Tablet can be swallowed or dissolved.
- 6 years & above: 10 mg od.
Newborn screened children will receive a small amount of vitamin K from diagnosis contained within DEKAs plus or Aquadeks.
‘Antacids’
If enzyme dose high and compliance and diet etc have been considered then consider:
- Ranitidine:
<1 month: 2 mg/kg tds (max 3 mg/kg tds)
1 – 6 months: 1 mg/kg tds (max 3 mg/kg tds)
6 months: 2-4 mg/kg bd (max 150 mg bd)
small risk of headache.
- Omeprazole:
| Once daily dose | Maximum daily dose | |
| <2.5kg | 0.7 - 1.4mg/kg | 3 mg/kg/day |
| 2.5 – 7kg | 5mg | 3mg/kg/day (max 10mg/day) |
| 7-15 kg | 10mg | 20 mg daily |
| >15kg | 20mg | 40 mg daily |
- Doses may divided and given twice daily if required.
- If using dispersible ‘MUPS’ tablets:
- Round to nearest 5mg (half of a tablet).
- Tablet can be cut in half but should not be crushed or chewed. Do not try to give a fraction of a tablet by dispersing it – it does not disperse evenly!
- Allow tablet (or portion of) to dissolve on the tongue or disperse in water/juice/yoghurt and give the whole amount.
- Alternatively, patients can open the capsule and swallow the contents with half a glass of water or after mixing the contents in a slightly acidic fluid e.g., fruit juice or applesauce, or in non-carbonated water. If using this method then doses should be rounded to nearest 10mg (whole capsule).
-
For administration through an enteral feeding tube, use Aclomep® oral liquid or the contents of a Losec® capsule dispersed in 10 mL Sodium Bicarbonate 8.4% (1 mmol Na+/mL).
-
If unable to tolerate omeprazole – lansoprazole can be tried as an alternative – see BNFc for doses.
Gastro-oesophageal reflux
Very common in CF.
- Omeprazole: see above (11.2c) for doses
OR
- Ranitidine see above (11.2c) for doses
Consider Infant gaviscon, <4.5kg: Half Dual sachet per feed; >4.5kg: one dual sachet per feed.
Erythromycin dose for gastric stasis is: 3 mg/kg tds
Distal Intestinal Obstruction Syndrome (DIOS)
Old name meconium ileus equivalent (MIE). See section 7.7. All therapies are osmotic in action therefore fluid support is CRUCIAL, if necessary, intravenously.
- Oral Gastrografin:
<15 kg, 25 ml BD with 75 ml flavoured juice / water
15-25 kg, 50 ml BD with 150 ml flavoured juice / water
>25 kg, 100 ml BD with 200 ml flavoured juice / water
Use for up to 3 days if no response in first 24 hours (but not if symptoms worsen).
Do NOT give in the presence of bile stained vomiting or bowel obstruction.
- Rectal Gastrografin: Use same doses as oral.
<5yrs: Dilute to 5 times its volume with water
>5yrs: Dilute to 4 times the volume with water
Requires IV line for IV fluids. - Oral N-acetylcysteine- tastes like rotten eggs – The 200mg/ml injection can be given orally and should be mixed with water, orange juice, blackcurrant juice or coke to a concentration of 50mg/ml. Alternatively 200mg sachets or 600mg tablets are available.
1month – 2 years 0.4 - 3g STAT
2 – 6 year 2 – 3g STAT
≥7 years 4 – 6g STAT - Polyethylene glycol (Klean-prep)
- Do NOT give in the presence of bile stained vomiting.
- Solid food should not be given for at least 2 hours before starting treatment.
- Add contents of 1 sachet to 1 litre water – can be flavoured with a clear fruit cordial. After reconstitution the solution can be kept in a refrigerator and discarded if unused after 24 hours.
- Can be given orally or via NG tube (usually latter) and a single dose of domperidone 30 minutes before starting can increase gastric emptying.
- Do not administer just before bedtime due to risk of aspiration.
- Start at 10ml/kg/hour for 30 mins then 20 ml/kg/hour for 30 mins.
- If well tolerated rate can go up to 25 ml/kg/hour.
- Maximum volume is 100 ml/kg or 4 litres (whichever is smaller) over 4 hours.
- Patients must be reviewed after 1st 4 hours.
- If not passing essentially clear fluid per rectum then a further 4 hours treatment can be given.
- Monitor for hypoglycaemia, which can occur with CF diabetics undergoing this regimen.
- N-acetylcysteine (oral) - Prevention of DIOS:
<2 years: 100 – 200mg tds
2 – 11 years: 200mg tds
<12 years: 200 – 400mg tds
Constipation
Ensure fluid intake is adequate.
Lactulose
<1 year: 2.5 ml bd
1-5 years: 5 ml bd
5-10 years: 10 ml bd
>10 years: 15-20 ml bd
then adjust dose according to response.
Movicol
Chronic constipation, prevention of faecal impaction:
1 - 5 years: 1 sachet of Movicol Paediatric OD.
Adjust dose accordingly - maximum 4 sachets daily.
6 - 11 years: 2 sachets of Movicol Paediatric OD.
Adjust dose accordingly - maximum 4 sachets daily.
12 - 17 years: Initially 1 - 3 sachets of Movicol per day in divided doses for up to 2 weeks. Maintenance dose 1-2 sachets daily.
Mix contents of each Movicol Paediatric sachet in 1/4 of a glass (60-65ml) water and each Movicol sachet in 1/2 of a glass (125ml) water
Liver disease
- Ursodeoxycholic acid: 10 – 15mg/kg bd
- Commonest side effect is diarrhoea (rare though), in which case, reduce dose. Last dose should be taken in late evening.
- Vitamin K - Menadiol phosphate 10 mg once daily.
Home delivery of medicines
NHS England Prescribed Specialised Services Commissioning Intentions (2014) has stated that responsibility for the ongoing prescription of high cost inhaled medicines (dornase, tobramycin, colistin, aztreonam, mannitol) for cystic fibrosis should defer to the Hospital Trust (GPs currently prescribed the majority of these medicines). A homecare delivery service to supply these medicines directly to patients is already in use, as responsibility for any new prescriptions of these medicines (aside from those already prescribed by GPs) transferred to the Trust in 2014. However, the repatriation of those medicines currently being prescribed by GPs, to the trust is expected to take place imminently.
This homecare service enables these medicines that are not able to be prescribed by the patient’s GP, to be prescribed by the CF team at RBH, and then delivered directly to the patient at home by the hospital’s chosen homecare provider, for as long as is required. The default for prescribing and supply of all other CF medicines except the ones listed above should be from the GP.
If homecare is required then please contact a member of the paediatric pharmacy team as soon as possible (Bleeps 7403/7410/7425/7428 or ext. 4375; paedpharmacy@rbht.nhs.uk; rbh-tr.paediatricpharmacy@nhs.net) who will then advise on the process to be followed. The paediatric pharmacy team should also be informed if there are ANY CHANGES to patient medicines that are supplied via homecare i.e. dose changes or discontinuations. Where possible copy the paediatric pharmacist into correspondence detailing such changes.