IMD watch: cheese in galactosaemia

CheeSe iN GAlACtoSAemiA: A PrACtiCAl GUide

anita macdonald Consultant dietitian in imd, Birmingham Children’s hospital

Pat Portnoi, dietitian, Register Coordinator, galactosaemia support group (gsg)

One of the UK’s top paediatric dietitians, Anita’s specialism lies with inherited metabolic disorders. she spends 50 percent of her professional time in clinical work with children and 50 percent researching and teaching.

Pat worked as a dietitian in the NHs before joining sHs in 1981, becoming a director of the company in 1990. she is now retired, but continues to work with the galactosaemia support group and with PKU, attending NsPKU conferences.

it is now 15 years since some types of mature hard cheese were first allowed for patients with galactosaemia in the uk. fifteen years ago we found it hard to accept that some types of cheese could be sufficiently low in galactose to be suitable for galactosaemia, but we have made great progress since those early days.

In fact, since 2000, the UK Galactosaemia Support Group (GSG) has sponsored the testing of over 173 samples of cheese on 12 different occasions (1, 2). Even the USA dietitians are following the UK lead (3).

Which criteria are used to decide if a cheese is suitable in galactosaemia?

To ensure a cheese is suitable, at least five samples of a UK or European cheese (with knowledge of origin and processing) are analysed to check its lactose and galactose content. If they have a lactose and galactose content that is consistently below 10mg/100g when analysed, they are permitted in the diet. This is less than the amount of galactose in 0.5ml cows’ milk which is minimal. It is difficult to detect lactose/galactose below this amount.

how is laCtosE and galaCtosE REmoVEd in ChEEsE PRoduCtion? The lactose and galactose in cheese is removed through different processes. 1 Cheese is made by coagulating milk. This transfers milk into a semi-solid mass and separates milk into solid curds (casein) and liquid whey. Most cheese types contain high levels of casein (containing no more than 1.0% lactose), but low levels of whey (which contains 70 percent lactose). Therefore, removing whey by drainage is important in reducing the lactose content of cheese, and during this stage, most of the lactose will be removed (approximately 98 percent). 2 The temperature of coagulum, starter culture, coagulating enzyme, and the acid produced, influences the properties of the curd and degree of whey expulsion and hence the final lactose content. 3 As the cheese ages or matures it dries out and as it dries out, it loses lactose and galactose in the whey.

ChEEsE allowEd in galaCtosaEmia Cheddar cheese Unfortunately, we have had to change the advice we give on cheddar cheese, primarily due to the poor availability of the cheddar cheese that we first analysed. The GSG recommended mature or very mature cheddar cheese from the West Country Farmhouse Cheese makers group, which was consistently very low in lactose/galactose. The number of farms producing this cheese has fallen; it is no longer available to purchase online and very few supermarkets now stock it. To add to the difficulty, it became clear through the work of the GSG that some caregivers/parents were mistakenly using the wrong type of cheddar cheese. Therefore, we have reanalysed new types of mature cheddar cheese. We now only recommend cheddar cheese by brand name to avoid confusion for both caregivers and dietitians. Only five types of cheddar cheese are now allowed and are all suitably low in lactose and galactose (Figure 1).

Other hard cheese allowed in galactosaemia are Emmental, Gruyere, Jarlsberg, French Comté, Italian Parmesan and Grana Padano (Figure 2).

Figure 2: suitable mature cheeses tested and permitted in a low galactose diet in galactosaemia

lye Cross Farm - west Country Farmhouse mature Cheddar

tesco - west Country Farmhouse Extra mature Cheddar

sainsbury’s -taste the difference west Country Farmhouse Extra mature Cheddar lidl - Valley spire west Country Farmhouse mature Cheddar lye Cross Farm - west Country Farmhouse Vintage Cheddar

these cheeses are made to very specific recipes in certain areas of a country following old traditions and are suitable in galactosaemia.

Emmental is a swiss cheese characterised by holes in the cheese that are made by one of the starter bacteria (propionic bacteria). grated and sliced Emmental has also been analysed and is safe to use. Processed forms, i.e. Babybel Emmental, are not suitable.

gruyere is another mountain cheese made by adding rennet to the milk. the cheese is washed in a salt-bath and bacteria is added at a later stage. jarslberg is a norwegian cheese made in a similar way to Emmental. swiss farmers immigrated to norway and took the recipe for Emmental with them, and presumably the starter culture too.

Comté is a French mountain cheese made by adding rennet to milk and adding salt later. it has a slightly sweet mild taste and is popular in France. it is one of the oldest cheeses made in France, Records suggest it was made by shepherds in the 12th century

italian Parmesan is matured for a long period of time - at least one year and often two or more and as a result the lactose is leached out of the cheese. Parmesan is the common term for Parmigiano Reggiano doP and it usually carries a doP yellow and red seal to certify that it is made in northern italy. this type of parmesan is suitable. Ready grated parmesan which has the doP seal is also suitable. doP is the italian abbreviation for Pdo.american Parmesan is not suitable as it is often younger. grana Padano is a slightly different type of parmesan made in another area of northern italy. Both block and grated forms of this cheese with the doP seal are allowed. this is one of the world`s first hard cheese, as it was being produced over 900 years ago by the Cistercian monks of Chiarvalle near milan.

Emmi swiss Fondue is a mix of Emmental cheese potato starch and wine for making a cheese fondue.

• Milk is heated ( pasteurised) and starter culture of bacteria is added specific for the farm and cheese • Rennet is added - it separates milk into curds and whey • Curds are turned and cut - whey runs off • Curds are salted • Whey continues to drain away • Cheese is formed into truckles and pressed • Cheese matures in cool place for many months

other suitable non-animal milk cheeses allowed in a low galactose diet Various soya and milk-free cheese options are suitable: • Cheezly (various flavours) - made by Redwood

Foods • Pure Thick Cheese Slices • Pure Soft & Creamy Spread - a cream cheese style spread • Tofutti - soy cheese slices (mozzarella and

American) • Tofutti “Better than cream cheese” - various flavours • Bute Island Sheese - 100 percent dairy free. All types of soy cheese (hard, cream, slices) in various flavours • No muh - vegan cheese squares, melty cheese, herb cheese and cheese with walnuts • Free & Easy Dairy Free - cheese flavour sauce mix • Parmazano - dairy-free grated replacement parmesan • Mozzarisella Creamy Risella - a creamy Italian vegan mozzarella cheese made from rice • Violife Creamy Original - classic cream cheese spread in a creamy consistency • Violife - Cheddar cheese block/slices • Vegourmet - Montanaro smoked vegan cheese slices • Jeezini - celtic cheddar-style vegan cheese block • Jeezy - natural vegan cream cheese • Bianco - vegan cheese - similar to mozzarella

These cheese substitutes can be found in supermarkets, healthfood stores such as Holland and Barrett and online via sites such as Goodness Direct and Vegan stores.

ChEEsE not allowEd in galaCtosaEmia 1 Any other types of cheddar cheese not described in Figure 1. 2 All cheese added to manufactured foods, e.g. lasagne, cauliflower cheese, pizza, cheese pie, cheese sandwich 3 All soft cheese, e.g. Brie, Camembert, Roulade 4 All blue cheese, e.g. Stilton 5 All cheese spread, e.g. Dairylea, Philadelphia 6 Lactofree Products: Lactofree milk and cheese are not suitable because, although half of the lactose is removed, the rest is changed by enzymes into galactose and glucose. There is only a little lactose present, but there is plenty of galactose rendering them unsuitable. 7 Babybel is unsuitable as it is only fermented for a short time. The Emmental variety was tested by the GSG and contained some galactose (4).

ConClusions This is a quick guide to suitable cheese in a low galactose diet. In practice, five types of branded cheddar and six types of mature hard cheeses that are very low in lactose and galactose and suitable for a low galactose diet. There is also a number of non-dairy cheeses that are appropriate to use. Although it is fabulous that so many cheeses are now permitted, caregivers are confused about which types they can use. As dietitians it is important that we are fully aware of the suitable cheese types so that we can accurately advise and support our families.

references 1 Portnoi PA, MacDonald A. Determination of the lactose and galactose content of cheese for use in the galactosaemia diet. J Hum Nutr Diet. 2009 22: 400-8 2 Portnoi PA, MacDonald A. Lactose and galactose content of cheese. In Preedy Vc, watson rr, Patel VB (editors). Handbook of cheese in health. wageningen Academic Publishers, wageningen. 2013, p 495-516 3 Van calcar sc, Bernstein Le, rohr FJ, scaman cH, Yannicelli s, Berry Gt. A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosaemia. Mol Genet Metab. 2014 112: 191-7 4 Portnoi PA, MacDonald A. the lactose content of Mini Babybel and suitability for galactosaemia. J Hum Nutr Diet. 2011 24: 620-1