The Dietitians Group was formed in 1999 as a sub-group of the BIMDG. The objective was to unite and form a network of dietitians working in the field of inherited metabolic disease.

A committee was formed in October 2002.
 

The main aims of the group are to:

• review and develop current dietary treatments for inherited metabolic diseases
   
• to produce guidelines/ resources/ publications where appropriate
   
• be a resource for dietetic colleagues involved with the dietary management of children/ adults with inherited metabolic disease

Membership of the group is open to any practicing clinical dietitian working in inherited metabolic disease.

The dietitian must be a member of the BIMDG (see ‘Further information’ section for details). Currently the group has 33 members. To become a member contact the group secretary via the ‘contact page’ on this site.

• The group meets biannually. Meetings are usually in a workshop format to facilitate discussion.
   
• A group e-mail is used for liaison.

Dietary Information Booklet for the Treatment of Phenylketonuria 2009/10

Planned Launch date March 20th 2009

Click here to download text of this item

There are some important changes to this latest edition of the diet booklet. Historically there has been inconsistency in the way some of the vegetables are counted, specifically the following:

Phenylalanine content

• Broccoli 76mg/100g
• Brussels Sprouts 92mg/100g
• Cauliflower 89mg/100g

Currently Broccoli and Brussels Sprouts are counted as exchanges and cauliflower is classed as free. This is inconsistent and difficult to explain to patients. Dr Anita MacDonald wrote an award winning paper* in which she challenged the validity of using these vegetables is such different ways. This work and the subsequent relaxation from weighing of these vegetables over 10 years have not seen any deterioration in blood phenylalanine levels. The dietitians on the medical advisory panel of the NSPKU have made the decision to place the three vegetables in the ‘Restrict to one normal serving per day’ list – this means that you can only choose one item per day from this list but with no need to weigh.

*MacDonald A, Rylance G, Davies P, Asplin D, Hall SK, Booth IW (2003). Free use of fruits and vegetables in phenylketonuria. J Inherit Metab Dis. 26(4): 327-38.

During pregnancy control has to be very strict and in these cases it may be necessary for treatment to be individualised.

Other changes to the booklet:-

The cut-off level of protein for margarines which are freely allowed has been raised from 0.5g/100g to 1g/100g.

Vegetable stock powders and cubes with a made-up protein content of 0.3g per 100ml or less can be included without counting.

Our amino acid analysis last year enabled us to add some new foods to the diet either as free foods or exchange foods.

New Free Foods

• Dragon Fruit
• Potato Flour (do not confuse with instant potato flour like Smash)
• Okra
• Gellan – a gelling agent used in sweets and jellies, so useful to know it’s not like gelatine which is high in phenylalanine

Restrict to one Normal serve a day

• Whole Hearts of Palm
• Try on pizza, in a stir fry, add to salads or serve with mayonnaise

Exchanges

• Passion Fruit 40g = 1 exchange (slight increase in amount for exchange)
• Kale 35g = 1 exchange
• Vine leaves 30g = 1 exchange

Eleanor Weetch
Dietitian Advisor NSPKU

• MSUD, June 1999 – the group addressed how to incorporate manufactured foods into the MSUD diet. These are now included and have greatly expanded the food choice for MSUD patients. Led by Marjorie Dixon, Specialist Dietitian, Great Ormond Street Hospital for Children, London.
   
• Homocystinura, October 2001 - a round table review of personal experience with management of homocystinuria followed by detailed discussion of dietary management, biochemical monitoring and the evidence for cystine supplementation.Led by Fiona White, Chief Metabolic dietitian, Royal Manchester Children’s Hospital, Manchester.
   
• GSD III, October 2002 - high protein versus high CHO diet was debated. Marjorie Dixon presented this at the SHS Inborn Errors Meeting, Friday 7th March 2003, London (see abstract book). Led by Marjorie Dixon, Specialist Dietitian, Great Ormond Street Hospital for Children, London.
   
• Familial Hypercholesterolaemia, June 2003. Led by Tricia Rutherford, Chief Dietitian, Royal Liverpool Children’s NHS Trust, Liverpool.
   
• Soya and Phytoestrogens in Galactosaemia, October 2003. Conclusions – with the current state of knowledge soya infant formula remains the best choice of baby milk for infants with Galactosaemia. This needs to be continually reviewed as more scientific evidence becomes available.
   
• Dietary guidelines for the “UK Collaborative Study of Newborn Screening Medium chain acyl CoA dehydrogenase deficiency”, June 2004. These guidelines will become available in Spring 2005. Led by Marjorie Dixon, Specialist Dietitian, Great Ormond Street Hospital for Children, London.
   
• Navigating your way through ethics and dietetic research, January 2005. Led by Joanna Eardley, Specialist Dietitian, Guy’s Hospital, London.
   
• Tyrosine Supplementation in maternal PKU, January 2005. Led by Paula Hallam, Specialist Dietitian, National Hospital for Neurology & Neurosurgery, London.
   
• Emergency Regimens, May 2005. Led by Fiona White, Chief Metabolic Dietitian, Royal Manchester Children ’s, Hospital, Manchester.

To become a member of BIMDG contact the BIMDG membership secretary - Anny Brown email: ann.brown @ nbt.nhs.uk

For further information on BIMDG dietitians group, please contact the group secretary.

Secretary (contact for membership details)

Barbara Cochrane
Yorkhill Hospital

E-mail: barbara.cochrane @ ggc.scot.nhs.uk

Contact BIMDG