Return to flip book view

This is the issue n The condition is as a result of an inherited gene mutation n The fast anaerobic energy pathway is blocked n This results in a severe shortage of energy at times n Cramping pain occurs if the energy reservoir is emptied Aerobic energy Ideas on adjustments Line managers or supervisors should liaise with the employee and listen to any concerns The degree to which individuals are affected by McArdle s can vary greatly Also the impact will vary depending on the physical demands of the particular job and task Anaerobic energy Slow flow Fast flow Blocked in McArdle s Rises with rest or very low energy use ENERGY RESERVOIR Muscle damaged when empty Refills to 80 with 30 seconds rest Falls with higher energy use Energy out to power the muscle Strategies you will notice A person with McArdle s will use these key strategies to help them handle their physical activity and avoid pain n Recognise a different style of work n Avoid time pressures n Inform others and beware of peer pressure n Offer tools and equipment to suit n Swap some duties with a colleague n Adjust working positions n Consider the working day People with McArdle s may swap back and forth between physical tasks a few minutes of one task swap to another then return to finish the first With each swap the depleted muscles have time to recharge Rushing is a main enemy of McArdle s The energy demand can be too great with a risk of muscle cramps Slow and steady is the motto It is helpful to liaise with the employee to decide what and how to advise work colleagues There may be a need for support to resist peer pressure to keep up or to do something they should avoid Slowing or pausing to balance energy During activity such as walking people with McArdle s n Slow down to ease the energy demand n Pause for 30 seconds rest then continue more slowly n Do these frequently in the first minutes of activity n Find symptoms easing after about 10 minutes when a second wind occurs as other energy pathways open up n With each muscle group used this process is repeated n Second wind is lost about 30 minutes after activity ends Breaking intense tasks into sections During maximum intensity tasks such as carrying a heavy object or standing on tip toe people with McArdle s n Interrupt the task after 6 seconds n Rest for 30 seconds so the energy reservoir refills then try again n This avoids a painful contracture which may otherwise occur last for days and might need medical attention Resting before a risk Before a risk like climbing a ladder or lifting something heavy they often first rest for at least 30 seconds to ensure energy is available to get them safely through Liaise with the employee about equipment they will know what works for them A wheelbarrow with two central wheels under the load to avoid lifting weight with the arms A stool to enable sitting rather than squatting or crouching both damaging A step stool A sack truck A trolley Arrange that the colleague does the more anaerobic duties and the person with McArdle s does the more aerobic duties which can be good for them Holding arms up to work becomes very anaerobic especially if lifting something so better to work on a platform Awkward positions such as working in a confined space or crouching to read a meter also may cause a cramp If an unstressed position is impossible people with McArdle s will change position frequently If in a physical job people with McArdle s may prefer a short meal break to be back to work before second wind is lost Some may prefer to work part time or job share

At work In the event of overdoing it Very occasionally a person with McArdle s may get something wrong and hurt themselves n n n n with McArdle s Allow them to rest and have water to drink Ask if you should call a partner or relative In severe cases ask if they need to go to hospital They carry a card to remind of the decision points Information for employers For people with McArdle s who manage well it is a very rare occurrence to need to be hospitalized A plain language guide to McArdle Disease and how to assist an employee to carry out their duties safely and e ciently Further information The person with McArdle s can share these books with you or you can request copies from IamGSD 101 Tips for a good life with McArdle Disease 164 page paperback Easy to read guide with practical tips for patients Introduction 14 Disease What is McArdle Specialised clinic n Genetic confirmatio Inheritance ions Concomitant condit is Rhabdomyolys ctures Cramps and contra Pain medication REencies emerg al O MedicM N THA Lab results Creatine Kinase enzymes Blood Urate Liver Urine Exercise and diet activity Problems with se Beneficial exerci Physiotherapy Diet n They have a serious shortage of energy early in 1 ew One page overvi EUROMAC Through contract no 2012 12 stored in the muscle Information for care decisions support primary bolic myopathy Information to this very rare meta people living with Disease www euromacregistry eu n People with McArdle s cannot utilise the energy erview medical ovfor GPs iving with McArdle Disease is challenging in many ways A great introduction to living Running out of energy cramping with this condition I wish I and muscle pain are everyday had this book 50 years ago experiences Even simple Nancy McVey tasks like chewing or drying after a shower can Very important concepts be a challenge simply explained that oft en We pass on practical tips which get lost or never communi cated McArdle people have found with the diagnosis to be useful We also provide explanati Terri Chambers ons of four key subjects second wind the six second rule your ATP reservoir About the author see inside and guidance on emergenc the back cover ies This book will help McArdle people to reduce intense anaerobi activity and extend more c gentle aerobic activity Th ese changes can improve day to day life We also provide references to sources of further information The aim is to avoid pain and muscle cramps eliminate hospital visits and reduce or get more out of life Government information Employers may wish to search online for information about employing people with health conditions and for disability rights legislation which restricts fuelling of skeletal muscles ease McArdle Dis 101 TIPS for a good life with McArdle McArdle Disease L medical overview 20 page booklet A quick guide primarily for GPs and other medical professionals n McArdle s is an ultra rare inherited condition Alerts Statin therapy Drug side effects al anaesthetic Surgery Gener ysis Rhabdomyol Tourniquets s nation Exami any activity and throughout all intense activity 2 3 4 4 5 n They can develop cramping If activity continues AT the P Six Se re S co s e ec n 6 rv on d W oir d 6 Ru ind sim le 7 ply and ex you pla r in 8 ed 9 9 muscles become sti swollen and very painful n They have a second wind somewhat reducing 10 11 12 13 their symptoms after about 10 careful minutes 14 14 15 15 15 15 n They achieve a lot by utilizing techniques to protect themselves but for a desk based role few techniques will be needed ort Andrew Supp Wakelin 16 With a foreword by Dr Ros Quinliva 16n Information cards ce 17 Further referen McArdle books Back cover group Patient support IAMGSD n They tend to develop determination and Association for se International gen Storage Disea Muscle Glyco stickability making them good workers More questions n Major adjustments in their role are not normally required but some flexibility is helpful If you have any further queries the IamGSD will be happy to assist www iamgsd org International Association for Muscle Glycogen Storage Disease 746 4th Avenue San Francisco CA 94118 USA info iamgsd org www iamgsd org v1 10 2017 IAMGSD IAMGSD International Association for Muscle Glycogen Storage Disease