378FM.2 VITAMIN SUPPLEMENTATION FOR PAEDIATRIC CYSTIC FIBROSIS (CF) PATIENTS

Transcription

1 378FM.2 VITAMIN SUPPLEMENTATION FOR PAEDIATRIC CYSTIC FIBROSIS (CF) PATIENTS Oxford University Hospitals and the Royal Brompton are the tertiary CF centres serving Buckinghamshire. Many patients are under joint care with a tertiary centre and paediatrician at Buckinghamshire Healthcare Trust, where this is more convenient, for follow-up appointments. CF patients may become deficient in fat soluble vitamins (A, D, E and K) due to fat malabsorption. This results from a lack of pancreatic enzymes. The aim of vitamin supplementation is to maintain vitamin levels towards the upper limit of the normal range. Patients with CF will have annual blood tests to check vitamin A, D and E status, and clotting/lfts to assess the need to replace vitamin K. The secondary care specialist will make an individualised recommendation based on these results. Usual recommended daily amounts (RDA) in CF are shown in the table: (Referenced to Royal Brompton guidelines 1 and local preferences.) Age Vitamin A 1 microgram = 3.3 Max level age <10 = 1.7 micromols/l age >10 = 2.9 micromols/l <6 weeks 600 micrograms (2,000 ) 6 weeks to 1,200 micrograms 6 months (4,000 ) 6 months to 2,400 micrograms 1 year (8,000 ) 1 10 years 2,400 micrograms (8,000 ) 11+ and 2,400 micrograms adults (8,000 ) = usual dose Max. dose 3,000 micrograms (10,000 ) Vitamin D 1 microgram = 40 Target 25-OH vitamin D level = nmol/l (400 ) (400 ) (400 ) micrograms ( ) micrograms ( ) Vitamin E 1 mg = 1 unit (approximately) mg mg Individual doses will be adjusted by the specialist at the annual review according to vitamin levels. Vitamin D If vitamin D levels are lower than recommended for CF (25-hydroxyvitamin D levels <75 nmol/l), the hospital may give a short course of high dose oral colecalciferol (e.g. 20, doses a week for between 1 and 4 weeks depending on how low the levels were) before requesting that the GP continues the licensed supplement below. The vitamin A and D product has maximum doses imposed to ensure that the individual is not given too much vitamin. Guideline 378FM.2 1 of 5 Uncontrolled if printed

2 Vitamin K In the past the use of vitamin K has been restricted to children who either have abnormal liver function, abnormal clotting of have hepatic changes on abdominal ultrasound at annual review. More recently the use of vitamin K has been extended. The current Oxford guideline states the following: Vitamin K should be offered to all children aged 6 years (including pancreatic sufficient) and is mandatory for those with liver disease (with or without clotting abnormalities). The CF Trust nutrition guidelines suggest vitamin K1 (phytomenadione) is used; unfortunately this is only available as a hospital special and cannot be sourced by community pharmacies. In practice (like the Brompton) we use menadiol, a man-made water soluble version of vitamin K. The tablet can be swallowed or crushed and dissolved. The dose for 6 years and above is 10 mg once per day. Families that would like to use vitamin K supplements from 2 years of age can do so - the menadiol tablets can be broken, then crushed and dissolved - the dose for 2-6 year old is 5 mg once per day. Patients who take DEKAs are supplemented with vitamin K in the form of phytomenadione but only at a low dose, and also need to take menadiol. (Dose of vitamin K in DEKAs = 0.5 mg/ml of liquid.) Recommended Vitamin Supplementation Children who cannot swallow tablets or capsules: 1. DEKAs plus Dose: <1 year 1 ml daily >1 year 2 ml daily OR 2. Dalivit and Vitamin E Dalivit dose: <1 year 0.6 ml daily >1 year 1.2 ml daily Vitamin E dose: As per table on page 1 (100 mg/ml solution) Dalivit 1.2 ml provides 3,000 micrograms of vitamin A and 20 micrograms (800 ) of vitamin D. Children who can swallow tablets or capsules: ALL of the following: 1. Vitamins A and D BPC 1973 Each capsule contains 5,000 vitamin A and 400 vitamin D. Usual dose 2 daily (usual maximum dose 4 daily). (Note: If patient requires calcium be aware that preparations such as Adcal-D3 or Calceos can be given in older children/adults (1 tablet contains calcium with 400 vitamin D)) 2. Vitamin E suspension 100 mg/ml for dose see table on page Vitamin K - menadiol 10 mg tablets 1 daily if indicated. Note: Patients should be advised to take fat soluble vitamins with a fatty food or drink such as milk, as they will be better absorbed. If a patient is on pancreatic enzyme supplements these need to be taken at the same time as the vitamins to maximise absorption. ***Ketovite or Forceval are not recommended*** Guideline 378FM.2 2 of 5 Uncontrolled if printed

3 DEKAs plus Solution DEKAs plus solution is a useful, cost effective and recommended source of vitamins (including vitamin K) for CF patients who are unable to swallow tablets to achieve the required level of supplementation. It is on the Buckinghamshire Formulary. DEKAs plus solution (60 ml) is marketed in the USA, it contains vitamins A, D, E and K, and is easy to take. It is prescribable, but is an unlicensed product which can be ordered from Alium Medical (telephone ) or Alliance Healthcare ( for use in the UK. DEKAs plus solution contains: Vitamin A 5,751 /ml Vitamin D 750 /ml Vitamin E 50 /ml Vitamin K 500 micrograms/ml It also contains other vitamins and antioxidants. The usual daily dose is 1 ml daily if <1 year or 2 ml daily for older children. For patients able to swallow capsules it is recommended to switch them to a combination of vitamin A and D capsules, vitamin E suspension and, if needed, menadiol tablets, as these preparations are all licensed. The DEKAs plus softgel and chewable tablet are non-formulary. References 1. Clinical Guidelines: Care of Children with Cystic Fibrosis 2014, 6 th Edition, Royal Brompton Hospital Cystic Fibrosis Team. 2. Oxford Paediatric Cystic Fibrosis Guidelines. Accessed 21/10/13. *1 mg of vitamin E is approximately 1 (see table on page 1) 1: Values for Dalivit based on dalvit SPC: accessed Aug : Values for DEKAs plus liquid based on: Guideline 378FM.2 3 of 5 Uncontrolled if printed

4 Comparison Table Range recommendations for age Dalivit DEKAs Dose 0.6 ml 1.2 ml 1 ml 2 ml Vitamin A per ml ,000 5,751 11,502 <6 weeks 6 weeks - 6 months 6 months to 1 year 1 10 years 11+ and adults 2,000 4,000 8,000 8,000 Usual dose = 8,000 Max: 10,000 Vitamin D Vitamin E Nil * * * * * Vitamin K Nil 500 mcg 1 mg N/A N/A N/A 2-6 years old: 5 mg >6 years old: 10 mg Other vitamins B vitamins Vitamin C B vitamins Vitamin C Other minerals 10 mg Guideline 378FM.2 4 of 5 Uncontrolled if printed

5 Title of Guideline Guideline Number Version 2 Effective Date February 2018 Review Date February 2021 Original Version Published February 2015 Approvals: Paediatric Information and Guidelines Group Vitamin Supplementation for Paediatric Cystic Fibrosis (CF) Patients 378FM 5 th October 2017 Paediatric Governance 10 th October 2017 Medicines Management 14 th December 2017 Subcommittee (Primary Care) Clinical Guidelines Subgroup 25 th January 2018 Author/s Daud Abdulla, Pharmacist, BHNHST Sarah Crotty, Interface Pharmacist, Aylesbury Vale & Chiltern Clinical Commissioning Groups Dr Craig McDonald, Consultant Paediatrician, BHNHST Alexa Conrad and Nicola Greenburg, Paediatric Pharmacists, BHNHST SDU(s)/Department(s) responsible Paediatrics for updating the guideline Pharmacy Uploaded to Intranet 14 th February 2018 Buckinghamshire Healthcare NHS Trust/Aylesbury Vale and Chiltern Clinical Commissioning Groups Guideline 378FM.2 5 of 5 Uncontrolled if printed