Edition 1 October 2018  The CATS team would like to say a huge thank you to Dr Elaine Murphy of the Charles Dent Metabolic...
What is Cori Disease  Cori disease is a rare disease of varying severity, affecting primarily the liver, heart and skeleta...
Who Wrote the Book   The CATS     Cori Action Team Support CATS is a group of patients and carers who have got together to...
Ailsa Arthur  Trustee AGSD UK   Ailsa has twenty years    experience of supporting her son with Cori   s Disease. She feel...
Sylvia Wilson  Type III co-ordinator   I have a granddaughter diagnosed with Cori   s disease. She lives abroad and it too...
Gary, Andrea and Lauren Thompson  Lauren is 18 years old and our only child. Lauren was diagnosed with GSD type 3 at the a...
The Tips in this Book The tips in this book were collated from many people with Cori disease from all over the world and a...
They are just tips  First, before reading this book, make sure you have the correct diagnosis. Then, remember we are all a...
Using this book Glycogen storage disease type III  GSD III, previously known as    Cori disease     Top Tips These tips ar...
Shopping      11
1  Shopping  When doing your shopping try to think about including some protein in every meal.     12
Shopping  2  Make your own information card for shopping, include foods you can and cannot eat and your likes, this will m...
3  Purchase an SOS Bracelet, or download a medical ID app onto your smartphone.      14
4  Shopping  Use shopping apps that support AGSD UK.      15
Emergencies        16 16
Emergencies  You can apply for a free message in a bottle in case of an emergency.  5  www.scas.nhs.uk news campaigns mess...
6  Emergencies  Smart phone  Keep medical ID in this section paramedics will look for this in an emergency situation.     ...
Emergencies  Smart phone  use the ICE     In  Case of       Emergency          number.     19      7
8  Emergencies  The British Inherited Metabolic Disease Group have published agreed guidelines on the emergency management...
These are freely available on their website  www.BIMDG.org.uk     21
Prescriptions        22 22
Prescriptions  Uncooked cornstarch  cornflour  can be obtained on prescription. Ask your dietitian or GP     23      9
10  Prescriptions  Protein powder can be obtained on prescription. Ask your dietitian or GP.      24
Prescriptions  You may be exempt from prescription costs. See eligibility here   11     www.nhsbsa.nhs.uk exemption-certif...
12  If you are not exempt from prescription charges then you may want to purchase a prepayment certificate to reduce the c...
Monitoring your GSD        27 27
13  Monitoring your GSD  At 18 although you are an adult, if you wish, you can tell your medical team that they CAN talk t...
Monitoring your GSD  14  Keep a running notebook of all your medical appointments so you can remember who said what and wh...
15  Monitoring your GSD  Keep a record in a notebook of how you are affected daily, this will help if and when you have to...
Monitoring your GSD  16  Keep any letters that consultants have copied you in on  these can also be used to help to suppor...
17  Monitoring your GSD  Take videos of your worst days - these can also be used to support PIP and ESA claims. Make sure ...
Monitoring your GSD  18  Very rarely, blood sugar levels are difficult to control in GSD III     if this is you, you might...
Gastrostomy   PEG Tubes        34 34
Gastrostomy   PEG Tubes  Keep it clean and follow the guidance for care of your tube.  19  A few people might need a gastr...
20  Gastrostomy   PEG Tubes  Speak to your local nutritional care teamcommunity nurses     they can help a lot. Your dieti...
Dentist        37 37
21  Dentist  Keeping your mouth open can be painful.  Ask for a mouth prop.      38
Dentist  22  If your diet includes a lot of carbohydrate or sugar then see a dental hygienist regularly.     39
Exercise        40 40
Exercise  23  In some parts of the UK you can self-refer yourself for physiotherapy, which may lead to a course of hydroth...
24  Exercise  Don   t exercise on an empty stomach.      42
Exercise  25  Be careful not to push to the maximum, take regular breaks and explain to others why you need this.     43  ...
26  Exercise  Swimming  Take regular breaks and use a clear dressing over stoma sites.     44
Exercise  27  It is important to keep exercising. If you don   t use it you will lose it.      45
28  Exercise  Get a dog or walk a friend   s dog. When walking - take rests and snack on route.     46
Exercise  29  Walking up hills take the gentler route even if longer and    zig-zag   ...    Take the gentler route throug...
30  Exercise  Avoid dehydration during exercise to reduce the risk of muscle cramps.     48
Exercise  Consider an e-bike, if you find standard cycling too strenuous.      49      31
Diet        50 50
Diet  32  If you do not have a dietitian, find one. Ask your GP to refer you to a dietitian who knows about GSD III.      ...
33  Diet  Don   t be afraid to ask your dietitian questions, or talk to them if you are finding it hard to manage your die...
Diet  34  Eggs, chicken, pork and  lean beef are good protein source.      53
35  Diet  Fish and seafood are good sources of protein and are typically low in fat.     54
Diet  36  Salmon and other oily fish have a slightly higher fat content compared to white fish, because they contain omega...
37  Diet  Use minimal fat when cooking protein-rich foods     use a spray oil to stir fry or pan fry.     56
Diet  38  Reduced-fat dairy products  such as lowfat or fat-free yoghurt, lower-fat cheeses and semi-skimmed or skimmed mi...
39  Diet  Unsalted nuts and seeds  are a practical protein choice if you   re on the move  but do contain fat .  50 pistac...
Diet  40  Use nut butters on toast instead of jam or honey.      59
41  Diet  Sprinkling seeds on salads will boost protein content.     60
Diet  42  Beans and pulses are cheap protein sources. Add them to stews, soups and casseroles.       61
43  Diet  Quorn is a good source of non-animal protein.      62
Diet  44  If you are eating out in an Indian restaurant, the tandoori option will be higher in protein and contain less fa...
45  Diet  Slow release or low glycaemic index  GI  carbohydrates are broken down more slowly, so can be helpful for mainta...
Diet  46  Oats are a good source of low GI carbohydrate     try porridge or overnight oats for breakfast.     65
47  Diet  Granary, wholemeal, sourdough and rye bread are all low GI, therefore good to try instead of white or brown brea...
Diet  48  Both wholewheat and white pasta are low GI carbohydrates, but wholewheat contains more fibre.     67
49  Diet  Berries are a better low GI fruit option     including frozen.      68
Diet  50  Many fruits are low GI     including apples, oranges and pears.      69
51  Diet  Milk contains both protein and also low GI carbohydrate, so can be helpful for managing blood sugar levels.     ...
Diet  52  Small portions of breakfast cereal can be a handy carbohydrate snack at school.      71
53  Diet  In general, try to limit sugary foods in your diet     except in situations to treat hypoglycaemia where quickly...
Diet  54  Natural sugar is still sugar     aim to limit portion sizes of foods that contain a lot of natural sugars such a...
55  Diet  Some reduced fat products can often have added sugar     check the label.     74
Diet  56  Always check food labels, and keep checking them because food manufacturers change their recipes.     75
57  Diet  Try sweeteners such as Xylitol or Stevia as alternatives to sugar.     76
Diet  58  Don   t forget savoury products often contain high levels of sugar     eg. tinned soups or ready-made sauces.   ...
59  Diet  Check all ingredients on tins and packaging to avoid added sugars.     78
Diet  60  Be cautious with alcohol as this can lower blood sugar levels     avoid this before exercise, and be sure to hav...
At Home         8080
At Home  61  Batch cooking  If you make complicated measured meals, make a lot and freeze portions.     81
62  At Home  Contact your local occupational health therapist. They will help with making sure you are equipped with any a...
Daily Activities        83 83
63  Daily Activities  Don   t wait until your blood sugar is low - eat small amounts regularly.      84
Daily Activities  Education  64  Consider whether your child needs extra time to complete exams or assignments. Ask for  e...
65  Daily Activities  Education  Consider an Education Health Care Plan, speak to AGSD-UK and your medical team for suppor...
Daily Activities  66  Wear a backpack, not a shoulder bag or handbag, this will be easier for you to manage if you have mu...
Support   Benefits        88 88
Support and Benefits  67  Consider PIP and ESA  get professional support letters as evidence. DWP might not understand you...
68  Support and Benefits  Benefits  Don   t give up, understand the system and make your case within the rules. REMEMBER  ...
Support and Benefits  69  Understand that complex feelings happen around inherited conditions, there is no shame or guilt ...
70  Support and Benefits  AGSD-UK will help you.      92
Support and Benefits  71  Don   t be afraid to reach out to as many support agencies as you can. GSD is now officially und...
72  Support and Benefits  Government scheme, Access to Work will help with any aids and support you in your fight to stay ...
Support and Benefits  73  Keep fighting for the best care, the best doctor, the best consultant. Not everyone is a GSD exp...
74  Support and Benefits  If the time comes that you need to use a wheelchair, try not to feel embarrassed  mobility aids ...
Support and Benefits  75  Reach out for support, try Facebook, there are several different groups you can join  Adults wit...
Spread the word        98 98
Spread the Word  76  We need to raise awareness of our rare condition. Tell everyone about GSD.  You never know who might ...
77  Spread the Word  Talk to schools and groups  as appropriate  about GSD. Most people are interested and will support yo...
Travel        101 101
78  Travel  Preparation is key.     102
Travel  79  Make sure you have enough cornstarch and   or protein powder for your entire trip, including possible delays. ...
80  Travel  Make sure you have appropriate travel insurance. See AGSD-UK website or phone for advice      104
Travel  81  Even if you are making a car journey make sure you always have a snack with you.     105
82  Travel  Research, research research  Make sure your holiday destination has wheelchair access if needed.     106      ...
Travel  83  If travelling abroad, your power chair charger may not be compatible with foreign voltage.     107
84  Travel  It may be easier to hire a wheelchair when you arrive instead of taking yours with you. Power chair joysticks ...
Travel  85  A really useful travel site for wheelchair users   www.curbfreewithcorylee.com     109
86  Travel  Ask your dietitian or doctor for a customs letter before flights     this will allow you to take medications, ...
Other Tips        111 111
87  Other Tips  Do not underestimate how much hypoglycaemia can affect intellectual and emotional states as well as physic...
Other Tips  88  Everyone is different  including everyone with  GSD III - pay heed to your own body and  reactions,   and ...
89  Other Tips  Be adaptable and attentive symptoms and tolerances change with time and age.      114
Useful Links        115 115
Access to Work  www.gov.uk access-to-work atwosu.london dwp.gsi.gov.uk  Association for Glycogen Storage Disease UK www.ag...
Disabled Railcard  www.disabledpersons-railcard.co.uk  Raise Funds for Charity with your everyday shopping www.giveasyouli...
Self referral Physiotherapy Hydrotherapy www.nhs.uk conditions pysiotherapy  Joining Groups  www.facebook.com  Travel Site...
National Database of Volunteering Opportunities www.do-it.org  Organised Walks Group for people with Mental  Physical Heal...
A-Z Glossary        120 120
Blood Glucose  The amount of glucose that is in your blood.  Protein  Nutrient   Essential nutrients for the human body. T...
condition effects you, not the condition itself. You   ll need an assessment to work out the level of help you get.  ESA  ...
Physiotherapy  A therapy to help restore movement and function when someone is effected by injury, illness or disability. ...
can   t function properly. You can have mild, moderate, or severedehydration  depending on how much fluid is missing from ...
Occupational Health Therapist  Occupational therapists provide practical support to help children and adults of all ages, ...
Notes