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Given the issues it might seem best for people with McArdle s to avoid all physical activity However n Increased aerobic fitness can make it easier and quicker to enter second wind see inside n Both increased aerobic fitness and increased muscle mass and strength make activities of daily living easier and avoids serious disability n Exercise helps with weight loss there is a tendency to be overweight and at increased risk of insulin resistance n Regular exercise helps with mood a chronic condition is challenging Very occasionally a person with McArdle s may get something wrong and hurt themselves n Allow them to rest and have water to drink n Ask if you should call a relative or friend or in rare severe cases take them to hospital n They carry a card to remind of decision points More Emergency button on www iamgsd org How you can help 1 12 Minute Walk Test guidelines for this test to show second wind and monitor progress 2 Second wind a full explanation in the Living with McArdle Disease booklet 3 Rating scales to assess pain and exertion 4 Further guidelines video presentations and more detail on aerobic and strength training Partnership n Trainer and trainee need to form a partnership to bring together their respective training expertise and understanding of McArdle disease n Both parties listening to respecting and trusting each other n The McArdle person needs to agree to the points in the box inside with McArdle s Briefing for physical therapists and personal trainers Explaining the problems for people with McArdle disease why they need to be physically active and how you can help Training Support The following are available from www iamgsd org on the menu Medical Training Support Further information A range of publications available on our web site Browse on line or download free PDFs Medical overview A quick guide for medical professionals 20 pages 101 Tips for a good life Simple practical tips for people living with McArdle s 164 pages n McArdle s is an ultra rare genetic condition n Myophosphorylase is deficient so glucose cannot be released from muscle glycogen n A severe energy crisis arises early in all physical activity and exercise Energy pathways at increasing duration of submaximal activity Each pathway s approx share n Guide supervise use of gym equipment and facilities n Allocate equipment e g desk cycle treadmill static cycle cross trainer in a quiet area time whereby the McArdle person will not feel embarrassed by slow pace and rests in front of other gym users n Discuss log results and help with motivation n If required help them to recognize secondwind using the 12 Minute Walk Test by tracking their walking speed heart rate and pain level 1 n Guide strength training with low number repetitions to be carried out after achieving second wind n Check on their symptoms during and at the end of the session At the gym In event of overdoing it McArdle Dis ease medical overvi ew Information to support decision s in primary care specialisms and emergency care this very rare metabol for people living with ic myopathy One page overview 1 Introduction What is McArdle Disease McArdle s specialised service Genetic confirmat ion Inheritance Concomitant condition s 2 3 4 4 5 Rhabdomyolysi s Cramps and contractu res Pain medication Medical emergenc ies Creatine Kinase Urate Liver enzymes 6 6 7 Lab results 8 9 9 Exercise and diet Problems with activity Beneficial exercise Physiotherapy Diet Statin therapy Drug side effects 10 11 12 13 Alerts General anaestheti c Tourniquets Examinations Suppor t Information Cards Further reading publications Support groups 16 16 17 Back cover Questions Contact us on info iamgsd org DISCLAIMER IamGSD uses its best endeavors to provide accurate factual and up to date information on this ultra rare condition However each person must take into account their own circumstances diagnosis and any additional genetic and medical factors and is advised to consult with their doctor before making use of our generic information and guidance Please refer to the disclaimer on the Medical menu of our website www iamgsd org 5 seconds 1 minute Creatine Phosphate Anaerobic Glycolysis from glycogen Deficient in McArdle s Minutes Hours Aerobic Glycolysis Immediate ATP ANAEROBIC AEROBIC n In activities such as walking at a normal pace cramping may start within a minute or two n In maximal effort isometric and anaerobic activity duration should be limited to about six seconds otherwise cramping will start n Cramping may develop into a contracture with rhabdomyolysis myoglobinuria and a risk of acute kidney injury or compartment syndrome 14 14 15 15 15 v2 05 2022 Why they need to exercise

Second wind n Early in aerobic activity as the start of cramping is felt people with McArdle s slow their pace or pause for a rest n They rest for at least 30 seconds to recover approx 80 of immediate ATP and the creatine phosphate system n By managing pace and pauses after about 6 to 10 minutes a second wind is achieved largely specific to the muscle groups being used n Activity then becomes somewhat easier n Glucose is released from the liver glycogen store and fat metabolism and aerobic glycolysis start n Second wind is lost within about 30 minutes of inactivity of the affected muscles More In the Living with McArdle Disease booklet from IamGSD 2 Assess the individual McArdle s can manifest differently between individuals due to n Additional conditions or genetic factors n Loss of aerobic fitness and or muscle mass often due to under use n Damage caused by incorrect management often before diagnosis Assessment Assess the individual by using the 12 Minute Walk Test 1 and discussing their current fitness so as to agree their starting level Plan to start gently in case badly affected No pain Gain n No pain no gain does not apply in McArdle s as damaging cramps and pain develop much sooner n In the event of pain exceeding 3 on a scale of 0 to 10 the exercise should be slowed or paused n If pain rises rapidly immediately stop the exercise The contents of this leaflet and the Training Support web page are based on the Clinical Practice Guidelines published in Neuromuscular Disorders the journal of the World Muscle Society December 2021 Aerobic training n Light warm up during which the rating of perceived pain should not be allowed to exceed 3 moderate to 4 on the scale of 0 to 10 where 0 is no pain and 10 is maximum pain 3 n Once in second wind regular moderateintensity aerobic exercise should improve muscle metabolism n In training heart rate should be 50 to 75 of max HR calculated as 208 0 7 age n Training 2 to 4 x week 20 to 90 mins per session n After session light dynamic stretching and good hydration are recommended More Further guidelines on aerobic training 4 The McArdle person must n Seek personal medical guidance before commencing training n Establish their baseline CK through several blood tests it is typically 2 to 25 x top of the normal range or approx 400 to 5 000 IU L n Accept responsibility for compliance with the No pain Gain statement Creatine Kinase n Plasma CK also known as CPK is a marker of muscle damage n If concern arises regarding pain or cramping a CK test will help assess whether training has been too intense higher than baseline CK Strength training Warm up Walk or pedal to achieve second wind as for aerobic training continue for 12 minutes Then do 12 minutes upper body exercise to achieve secondwind in upper body You only get second wind in the muscles you are using Strength training Use equipment to exercise large muscle groups Sets of 6 reps Gentle dynamic stretching for 10 30 seconds after each set to reduce stiffness Circuit training structure rotating on multiple equipment recovery of 3 minutes between sets Example 3 to 4 turns to circuit Leg Press Chest Press Seated Rowing Lat Pull Down Abdominal Machine Load should be set to elicit a rating of perceived exertion RPE of 6 7 and a rating of perceived pain RPP of 0 1 both on scales of 0 10 3 Load can be increased when RPE is