Editorial - mdi.ie · Spinal Muscular Atrophy Spinal muscular atrophy (SMA) is a condition in which the muscle-controlling nerve cells (motor neurones) degenerate. This is because

Two monthly newsletter - Issue 12, July 2003

EditorialHi Folks,

Welcome to the July issue of our newsletter.With summer well under way, this meansholidays, holidays holidays! And here atMDI, it's no exception. Already we have hadthree holidays/camps for our members andone currently happening in Galway for our11-13 year olds (19th-26th July). Details onsome of the camps on page 8.

This issues "Research Update" columnfocuses on Spinal muscular atrophy (SMA),(see page 2). Also on the topic of research,there is a very encouraging article aboutgene therapy on page 5. And, on acautionary note, there is an article on page 3advising against myoblast transfer treatment,which has not proven to have anytherapeutic effect for any form of musculardystrophy.

On pages 6 and 7, you will find details on anAssistive Technology conference which takesplace in September and if you would like tolearn more about computers, there are alsosome courses that may be of interest to you.

"Dates for your Diary" also on page 7 givesdetails of our AGM in September and an MDIFamily Day planned for October (moredetailed information on these events will beavailable soon).

"Power Soccer" - Will this be the new crazefor our young members? I'm certainlyimpressed! See page 9 for more.

This is just a flavour of what's inside. Hopeyou enjoy the read. Have a nice summerand take care.

Hubert McCormackEditor

Muscular Dystrophy Ireland Coleraine House, Coleraine St, Dublin 7.Ph: 01 8721501 / 872 3826 Email: [email protected]. Website: www.mdi.ie

Pictured at the Turlough Musem in Castlebarwhile on camp with MDI from 14th-21st June is

Seamus Fagan from Rush, Co. Dublin.

MDI News Up

"Person of the Tournament"Serena Brennan (aged 6), from Mullingar; proudly

displaying her certificate after being awarded"Person of the Tournament" at the MDI Power

Soccer event on 1st July 2003

date Page - 1

mailto:[email protected]

http://www.mdi.ie/


MDI News Update Page - 2

Research Update 6Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) is a conditionin which the muscle-controlling nerve cells(motor neurones) degenerate. This isbecause of a deletion or mutation on theSMN1 gene, which makes a protein calledsurvival motor neurone (SMN). Everybodyhas a "backup" gene called SMN2, but itmostly produces a shortened, inactive form ofthe SMN protein. Although motor neuronesare affected, SMA is not the same as MotorNeurone Disease.

There are different strategies being tested totry to treat SMA. One of these works on thebasis that having more copies of the SMN2gene, lessens the severity of the condition, soif there was a drug that boosted SMN2 itmight be an effective therapy. Someresearchers, e.g. at Aurora Biosciences (aSan-Diego, USA-based biotechnology group)are testing large amounts of chemicals oncells from people with SMA, and in mice,hoping that some will stimulate SMN2 toproduce more of the full SMN protein. Groupsin the USA and Taiwan have identifiedseveral drugs that appear to increase theamount of SMN2, including the mineralsodium vanadate, the antibiotic and anti-cancer drug aclarubicin and sodium butyrate,a drug that has been used to treat certaintypes of anaemia. However, despitepromising results, it is doubtful if any of thechemicals can be used to treat SMA. Sodiumvanadate is toxic, and the other drugs couldturn out to be toxic at the doses required.One of the researchers, Arthur Burghes ofOhio State University, USA, said at this point"I would say absolutely do not takeaclarubicin. In my view, butyrate has notbeen tested rigorously enough in animals andthere is no evidence it works in humans (withSMA)". Even so, these results areencouraging as researchers now know thatthere are chemicals that can affect SMN2and Aurora Biosciences should be able toidentify new, safer compounds.

Gene therapy is also being attempted forSMA. In January 2003, Christine DiDonato

published a piece of research showing thatgene therapy replaced the defective SMNgene. They used a virus to ferry the SMNgene into skin cells derived from people withSMA. Although motor neurones are the targetfor gene therapy, skin cells are good to testthe technique as they are easy to obtain andgrow in the lab, and they also exhibit one ofthe hallmarks of SMA. Normal human cellscontain "gems", areas rich in SMN andrelated proteins, but in people with SMA thereare a reduced number of genes or none atall. Viral delivery of the SMN gene restoredgems to the cells.

The next step in gene therapy, saysDiDonato, "is to go into animal models ofSMA and see if we can correct thepathological defects that result from lowintracellular levels of SMN".

Stem cell therapy is another possibility.Jeffery Rothstein of the ALS Centre at JohnHopkins University, USA, has had somesuccess with stem cell transplants in rats.The cells did not become new motorneurones, but they did release proteins thatenhance neuronal survival and growth. It islikely to be a number of years before humantrials of stem cell therapy for SMA or ALSbegin.

Research is also taking place regardingpharmacological treatments for SMA.Francesco Muntoni and his colleagues fromthe Imperial College School of Medicine inLondon, published a paper in August 2002about albuterol. This drug was given orally tofive children with SMA type 2 and eight withSMA type 3. After six months on albuterol,the group improved in muscle contractionstrength, forced vital capacity (ameasurement of respiration) and lean bodymass, on average. Although there were nosignificant problems, nine children showeddecreased mobility in their joints after sixmonths, which might have been due to thedrug selectively strengthening some musclesover others. This needs to be looked at infuture, more rigorously controlled studies.John Kissel, a neurologist from the USA,believes the possibility of testing albuterol inSMA can be explored, but there have been


MDI News Update

serious obstacles to obtaining the oral form ofthe drug in the USA.

A study has also begun this year in Dallas,Texas looking at creatine. Eighty children andadolescents with SMA are taking part, andthe study will test whether or not the dietarysupplement creatine may benefit childrenwith SMA. Creatine is reported to increaseenergy production in cells, particularly thoseof the muscles and nervous system. It willtake a few years for results to becomeavailable, but I'll let you know of any updates.

On a separate issue, there have beensuggestions that children with SMA haveenhanced intelligence, and this has beentested in two universities in Germany. Theresearch concluded that the generalintelligence of children with SMA is in theaverage range, but their use of generalintelligence was superior by age 12. By laterchildhood and adolescence, skills required forverbal parts of certain intelligence tests weremore developed in those with SMA. Theresearchers suggested that children withSMA "compensate" for their physicaldisability by gaining cognitive skills andknowledge, so although their intelligencemight not be enhanced as such, they becomebetter at applying it.

Research is continuing worldwide to find atreatment for SMA. Families for SpinalMuscular Atrophy in the USA has itselfcommitted $8 million over the next threeyears for SMA research and there is also anInternational Spinal Muscular Atrophy PatientRegistry. This is to provide a resource bywhich individuals with SMA, their families andresearchers can come into contact with oneanother (see the FSMA website for moreinformation, details below).____________________________________

If anyone would like more information on SMA,there are several useful websites:Muscular Dystrophy Association (USA): www.mdausa.orgThe Jennifer Trust for Spinal Muscular Atrophy(UK): www.jtsma.org.ukFamilies for Spinal Muscular Atrophy (USA): www.fsma.org

Karen JameisonInformation Officer, MDI

Myoblast Transfer TreatmentA Cautionary Tale

MDI received an emailon 24th June 2003 fromthe World Alliance ofNeuromuscularDisorder Associations(WANDA) regardingmyoblast transfer treatm

MTT was heralded a nua potential cure for DMDwas that myoblasts, or imfrom healthy boys, woulmuscles of boys withtreatment was testedunfortunately found to be

It has come to the ahowever, that in an incountries, clinics were application of MTT for muscular dystrophy. between $125,000 atreatment, and make clatransplanted have eimprovement and/or coin muscle degenwww.celltherapy.com)

However, WANDA hfamilies in which a medystrophy has undergreports are very negstate that the diagnosischecked and that there wprior to acceptance forthat at least 40 or more this treatment for which of therapeutic effect, anby selling their houses, draining money from regional TV campaigns e

The Mediterranean S(MSM) state that MTTshown to be ineffectiveand potential risks cstrain to the patients'takes a strong positio

Page - 3

ent (MTT).

mber of years ago as. The idea behind itmature muscle cells

d be injected into the DMD. When the however, it was ineffective.

ttention of WANDAcreasing number ofestablished for thepeople who have a

The clinics chargend $150,000 perims that "all patientsither seen greatnsiderable decreaseeration" (see

as reports frommber with muscularone MTT, and the

ative. Some reports of patients was notere no examinations

treatment. It seemspeople have receivedthere is no evidenced several have paidcashing in pensions,family and friends,tc.

ociety of Myology "has been clearly while its high costause unnecessary

families. The MSMn in criticising or

http://www.mdausa.org/

http://www.jtsma.org.uk/

http://www.fsma.org/

http://www.celltherapy.com/



condemning unproven therapies whichare not only ineffective but also have thepotential to cause harm while exploitingdesperate patients and their families".

MTT has recently been prohibited in Brazil,and a lawsuit has been commenced by theMedical Council of Sao Paulo State againstthe Brazilian doctors involved. The ethicalviolations include recommendation of anunproven treatment method, giving falsehope to patients and their parents, and thepayment for what is still an experimental trial.

The man behind MTT in Brazil is Peter Law,a Chinese physiologist living in the USA. In1996 he was providing MTT at the CellTherapy Research Foundation in Memphis,Tennessee. However, in 2000 after the Foodand Drug Administration investigated hisresearches, they disqualified Law due toserious flaws and violations. As he wasforbidden to perform MTT in the USA, hestrengthened the Brazilian connection, with aclinic in Sao Paulo called the GenesysResearch Institute, which is affiliated to theCell Therapy Research Foundation. Lawhowever, remained in the USA. Theproduction of myoblasts, apparently obtainedfrom the muscles of healthy American boys,also takes place in the USA.

In order to unmask the fraud, a Brazilianjournalist posed as the godmother of afictitious 7 year old boy called Bruno who shesaid had Duchenne MD. Bruno wasscheduled for MTT without an examination bydoctors either in Sao Paulo or Memphis tocheck his diagnosis. Nobody ever asked howhis condition was progressing. He was notasked to come to Memphis, which shouldhave been required, to determine if he waseligible for MTT or not. Peter Law set up thedate of MTT even before receiving anymedical report or results of blood tests. Theonly report ever sent to Law was written byBruno's godmother!

Thankfully, Bruno's case was invented to seehow far Peter Law and the Brazilian clinicwould go. However, at least 20 people fromdifferent parts of the world, including the USAand Spain, and a number of Brazilian children(it is impossible to say how many) were given

MTT. Out of the known Brazilian children whohad the treatment, none showed anyimprovement or change in the natural courseof the condition, and in one case, it ispossible that MTT may have accelerated theprogression. One father said "I would rathergo to a witch than repeat this treatment".

Scientists from all over the world,including well known researchers likeTerence Partidge and Victor Dubowitzfrom the UK and George Karpati inCanada agree that myoblast transfer hasnot proven to have any therapeutic effectfor any form of muscular dystrophy.

However this does not mean that nothing canbe done for people with DMD. It is known thatregular physiotherapy, use of corticosteroidsin many cases, and assisted ventilation, hasimproved both life expectancy and quality oflife. There is a great range of quality researchtaking place over the world, and as soon as abreakthrough in treatment comes around,MDI and other muscular dystrophyassociations around the world will alert alltheir members. Until then, here are a fewpointers:

• Remember that as well as usefulinformation, there is a lot ofmisleading information on the internet.

• Be extremely cautious of anytreatment offered at the minute, whichclaims to be a cure for musculardystrophy, especially those asking forlarge amounts of money.

• If you find information about apotential treatment, ask your doctor,MDI Family Support Worker orInformation Officer to see if they canprovide more information.

• DO NOT BEGIN ANY TREATMENTWITHOUT CONSULTING YOURDOCTOR. Even herbal medicines caninteract with other medications.

____________________________________

If you would like more information about thisarticle, please contact Karen in the Dublinoffice, or email [email protected]

Karen JameisonInformation Officer, MDI


Gene Transfer Study YieldsPromising Results

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Participants were divided into three groups:Group 1: Received a single injection of 200micrograms of the plasmid-dystrophinconstruct.Group 2: Received a single injection of 600micrograms.


n 5th June 2003, it was announced that theirst stage in a clinical trial for gene therapysing a nonviral approach for Duchenne andecker muscular dystrophy (DMD and BMD)as been successful. The therapy appearsafe, and holds promise for futureffectiveness against these conditions.

he trials are taking place in France by thessociation Francaise Contre les Myopathies

AFM or French Association Against Muscleiseases) in conjunction with Transgene, aiotechnology company based in Strasbourg.

on Wolff, of the Department of Paediatrics athe University of Wisconsin, USA, was part ofhe French project. His team developed muchf the strategy to deliver the therapeuticene, which carries instructions for makingystrophin, the muscle protein that is missingr flawed in people with DMD and BMD.olff is now working on delivering genes

nder pressure through the body's arteries,o that specific muscles can be targeted withust a few injections.

n the trial, the results of which werennounced at a meeting of the Americanociety of Gene Therapy in Washington,

nvestigators used a "naked DNA" deliverytrategy, that splices the desired gene into aiece of genetic material called a plasmid.he plasmid-dystrophin combination was

njected into an arm muscle in 15 boys withMD or BMD.

Group 3: Received two injections of 600micrograms, two weeks apart.

The investigators studied muscle samples 21days after the first or single injections. Asmall amount of dystrophin that wasapparently made from the plasmid-dystrophinconstruct appeared in 1%-10% of musclefibres in half of the boys in groups 1 and 2 (3out of 6 in each group), and in all 3 boys ingroup 3.

It is generally believed that dystrophinproduction in about 20% of muscle fibreswould make a significant difference in musclefunction.

There were no signs of immune systemreaction against either the plasmid or the newdystrophin, which is good news in terms ofsafety.

The AFM and Transgene are continuing todevelop the nonviral DNA delivery system. Ina statement made on 5th June, they said"These results demonstrate for the first timethat it is possible to obtain local expression ofdystrophin in patients with Duchenne/Beckermuscular dystrophies following theadministration of a plasmid containing thehuman dystrophin gene."____________________________________

These results are very encouraging, but it isworth noting that this trial was designed toidentify if this gene therapy approach hadpotential, and although it does, there is stillwork to do. The boys in this study would havehad no therapeutic benefit so the aim now isto enhance the technique and make it moresuccessful, in order to make a real differenceto people with muscular dystrophy.I will keep you updated with any newdevelopments in this area and you can alsocheck the "What's New" pages on theAmerican Muscular Dystrophy Associationwebsite (www.mdausa.org).

http://www.mdausa.org/



Assistive Technology“Shaping the Future”

Dublin 31st August to 3rd September 20037th European Conference for the

Advancement of Assistive Technology inEurope (AAATE)

University College Dublin

The Association for the Advancement of AssistiveTechnology in Europe (AAATE) are holding their7th European Conference in Dublin in September2003. The conference is timely, coinciding withthe European Year of People with Disabilities.Consequently, it is appropriate that the focus isunambiguously placed on the users of assistivetechnology.

Under the theme “New Technologies”, theconference will explore the significant advances intechnology research and development and howthese can be harnessed to benefit people withdisabilities. This will involve an exhibition onevolving technologies, affording interestinginsights into the future. This exhibition will be thelargest exhibition of technology and technicaldevices to date in Ireland, and should be ofinterest to people with disabilities, and all whowork in the field of disability. The exhibition willfeature a wide range of technology including;computer hardware and software, seatingsystems, products for mobility, independent livingtechnology, communication aids, technology foremployment and education and ‘Safety in theHome’ technology. Access to the exhibition willbe free throughout the conference.

The conference will providea valuable opportunity foreveryone involved in thefield of assistivetechnologies- includingusers, researchers, serviceproviders and equipmentmanufacturers- to exchangeinformation and update theirknowledge in a rapidlydeveloping field.

The conference is being organised on acollaborative basis by the Central Remedial Clinic(CRC), University College Dublin and Media LabEurope.

The Central Remedial Clinic is a major nationalprovider of assessment, therapies and othermedical and non-medical services to people withdisabilities throughout Ireland. Its Client TechnicalServices (CTS) department offers user-centred

evaluation, information and education in assistivetechnology and has played a leading role in thedevelopment and provision of AT services inIreland. UCD and CTS are long term collaboratorson an number of research projects. New, state ofthe art accessible accommodation will beavailable on-campus for the event. Media LabEurope is engaged in research into new areas ofopportunity being opened up by new technologies.It therefore brings a fresh insight into futurepossibilities for people with disabilities.

With over twenty topics to cover, the scientificprogramme will offer great opportunity fordiscussion and debate.

Details on conference registration and to obtainfree entry tickets to the Trade Exhibition areavailable on the web at www.atireland.ie/aaate, orfrom: AAATE 03 Registration Office, 8 MainStreet, Finglas, Dublin11, tel: 01-8361611, fax:01-8361724

Information Technology TrainingCourse

Finglas, Dublin

• Are you in receipt of disability allowance or arelated benefit?

• Would you be interested in returning toeducation if you could keep your disabilityallowance?

• Are you interested in pursuing a career in theinformation technology and/or customerservice industry?

• Are you aged between 21-65 years?

If the answer to these questions is "yes", thiscourse might be for you. It is run by Colaiste IdeCollege of Further Education in Finglas and isdesigned to lead to meaningful qualifications in ITand customer service. Students will retain allDisability Allowances / Benefits.

The first class of students on this coursegraduated in June 2002 and the second grouphas just successfully passed all examinations. Ifyou would be interested in finding out more aboutthe course, contact:Eileen Daly, Disability Support Officer @ 01885 1381 or email [email protected]

http://www.atireland.ie/aaate

mailto:[email protected]



Computer Trainingat home

We do I.T. differently…!

Learn about information technology at home

The National Training & DevelopmentInstitute (NTDI) in Tallaght plans tocommence a home supported InformationTechnology programme in October 2003,designed specifically for people withdisabilities, who are unable to access existingcentre-based training opportunities.The NTDI will provide all necessary computerhardware and software to enable peopleaccess training without leaving their homes.The programme will provide EuropeanComputer Driving Licence (ECDL)certification and a variety of extra supportsincluding a study skill module. No previousIT skills are required. The programme willnot interfere with a person's currentallowances or benefits and is not meanstested.NTDI are now inviting applicants living inDublin West, Dublin South West & CentralDublin areas to contact them in order to beconsidered for a place on the course.

For an application form, contact:NTDI, 77 Broomhill Road, Tallaght

Industrial Estate, Dublin 24Tel: (01) 525777 Email: [email protected]

Dates foryour diary

MDI AGM – Sat 27th Sept. 2003The Annual General Meeting of MuscularDystrophy Ireland will take place on Saturday27th September 2003 in Carmichael House,North Brunswick Street, Dublin.Prof. Kay Ohlendieck has been invited tocome along before the AGM make apresentation. MDI are currently funding aresearch project co-ordinated by Prof.Ohlendieck at NUI Maynooth. This project isentitled "Identification of Novel TherapeuticTargets in Dystrophic Muscle Fibres" and isrunning for three years from November 2002.Prof. Ohlendieck's presentation willcommence at 12.00 noon (to be confirmed).

Family Day - Sun 5th Oct. 20032003 is the European Year for People withDisabilities and MDI have decided to markthis occasion by holding a family day formembers on Sunday 5th October 2003. Thiswill be an informal afternoon event, with lunchand entertainment, and will give membersand their families the opportunity to meet upwith each other, socialise and celebrate theyear.Further details on both of these eventswill be available in due course.

Pictured at a recentpresentation of a cheque toMuscular Dystrophy Ireland inBaltinglass which was raisedat a benefit night in theRathvilly Inn were Left-Right:Stephanie Giblin, SiobhanWindle (MDI Respite Co-ordinator), Margaret Giblin &Joan Doyle.MDI would like to thankeveryone who supported thisevent. We would also like tothank MDI member BelindaLacey for her contribution.Picture: Nigel Gillis.


MDI Youth Camp, Galway14th-21st June 2003

The first youth camp of the summer tookplace from 14th-21st June in Kilcuan,Clarenbridge, Galway for 14-18 year olds.The group spent 7 fantastic fun filled sunnydays in Clarenbridge. Everyone reallyenjoyed their time there. Visits to theAquarium in Salthill, Turlough Musem inCastlebar, and shopping in Eyre Square wereorganised. There were also trips to thecinema to see ‘Anger Management’ and‘2Fast & 2Furious’. A small group even wentto the circus during their visit.

Each evening there were different activitiesplanned such as Playstation Tournaments,Soccer Competitions, a Table Quiz andwatching videos. The winning Table Quizteam consisted of Keth Devoy, Ciaran Purcelland their helper Mary-Rose. The winner ofthe playstation tournament was Ben Styles.There were many celebrations going onduring the week especially for one of ourhelpers Grainne Brennan who passed all hercollege exams, Congratulations to Grainne.

At the end of the week the youth workers

came up with awards for each individual thatattended the camp. Awards included ‘Brainof the Week’, ‘Helper of the Week’ and‘Sidekick of the Week’. The week concludedwith pizza and a disco organised by our ownDJ Pinky!!!!

Noeleen FaganYouth Worker, Dublin

"Anyone for Crab Claws?!"Mark Anthony McHale (Galway) gets his hands

on some crabs, while Keith Devoy (Dublin) lookson, at the Atlantaquarum in Salthill while on

camp in Galway.

Lined up for action on the Galway Camp are MDI members (L-R): Mark Anthony McHale, Ciaran Purcell,Joe Styles, Des Mooney, Robert Doyle, Keith Devoy, Ben Styles, Paul Breen & Seamus Fagan




National Power Soccer Day

On the 1st July, MDI held its first nationalpower-soccer day in the Irish WheelchairAssociation sports hall in Dublin. The eventwas a huge success. A total of 16 youngmembers, their friends and family turned upfor the day. There were members from thesouth, west, east and midland branches.The group met at 1.00pm, the players hadprotection guards fitted to their chairs andthey were ready for action!

Players were put through some skills trainingby our volunteer trainer, Marc. They had tolearn how to block, manoeuvre, score, controlthe ball and so on. Some of the memberstook to it very well and others needed a littlemore practice. Once everyone had got thehang of the rules and techniques etc., it wastime for a short break for something to eat,before the tournament began!

After lunch, everyone was divided into threeteams to compete in the tournament. Theteams chose their names and the battlebegan. The three teams were called "TheBulls", "Annihilators" and "Delta Force". Thewinning team "Annihilators" which consistedof; Ed. O’Connell (Cork), Paul Breen (Dublin),Seamus Fagan (Dublin), Simon Jameson(Dublin) & Karl Butterly (Dublin) received goldmedals.

There was also a ‘Person of the Tournament’award, who was chosen by the leaders. Thisaward went to Serena Brennan fromMullingar, for her pure guts anddetermination. See photograph (front cover).

As the day wassuch a successit is hoped tohold anothertournament lateron in the year,so if you like theidea of powersoccer andfancy giving it ago, contact yourregional youthworker and letthem know.

A special ‘thank you’ to the parents andvolunteers who came along to help out on theday. Thanks also to Robert Fitzsimons forhis refereeing skills for the tournament.

Noeleen FaganYouth Worker, Dublin

"On The Ball"Serena Brennan, Mullingar and Kevin Fitzsimons,

Dublin at the Power Soccer Tournament inClontarf, Dublin on 1st July 2003

"Before Kick-Off!!" at the Power Soccer Tournament, the teams pose for a photo-shoot


News from Cork

From South to North West!

As part of my diploma (Youth & CommunityWork) in University College Cork, I travelledto the North West on 24th March for five days,where I was given the opportunity to workwith Fintan Flannery, Family Support Workerfor this region.

I visit families in Leitrim, Longford, Sligo andDonegal. I had a great time and met somevery nice people. I would like to take thisopportunity to thank all the families whoopened their homes and made me feel sowelcome. These include Barry Mulvey andfamily, David Cunningham and family, RobertQuinn and family, Darren Nealy and family,Darren McDonagh and family & JoshuaRoulston and family.

I would also like to thank Fintan for all thehelp and support he gave me throughout theweek.

Mary Rose HowellYouth Worker, Cork/Kerry

Wedding Bells

Atomic Kitten

I went to the Atomic Kitten concert on Friday4th July with my sister and mother. Wearrived at 6.30pm, just in time for the doors toopen. We were shown to our seats, whichwere up high so we could see everything.The first group to arrive on stage was "SkinDeep" who were four girls who wore greatclothes and were excellent. The next act upwas "Mankind" which was five boys who alsolooked great and the lead singer had a reallygreat voice. Next to come on stage waseverybody's favourite "Mickey Joe Harte".The first song he sang was "Something Right,Something Wrong" and the he sang "We'veGot The World Tonight", which is myfavourite. Then Atomic Kitten came on.They sang "Dancing In The Street" and manymore. They were excellent and when theywere finished the crowd kept saying "Wewant more", so they came out again andsang "The Tide Is High". It was then time togo home, after a really great concert.

Rachel Clarke, aged 11.Cork

Darren Nealy from Letterkenny at The BrunswickEntertainment Centre, pictured lining up a shot

while bowling, on a day out with Mary Rose, YouthWorker, Cork.

MDI News Upd

Congratulations to Elaine & Paul Moore fromBlarney, Co. Cork who were married recently. .

Elaine is daughter of June Scannell, Treasurer ofCork Branch, MDI.

ate Page - 10


New South East Branch

Thanks to all our members in the south eastwho attended our "Branch set-up" meeting on2nd June 2003 in the Springhill Court Hotel,Kilkenny. A very large attendance saw thatour goal (i.e. to have our own South EastBranch of MDI) was achieved and all had anenjoyable day.

We were fortunate to elect a Committee ofcommitted people, a good blend of youth andexperience, who bring expertise, enthusiasmand lots of ideas to their respective positions.

The Committee is as follows:

Chairperson: Linda McDonaldVice-Chairperson: Mary StevensonSecretary: Sandra RichterTreasurers: Packie & Heather LarkinPRO: Stephanie Richter

Sinead O'Brien, Dublin and Tony Farrell, Dublin,pictured on an MDI adult holiday, in Kilcuan,Clarenbridge, Galway - 12th – 17th May 2003


Congratulations to the members on theestablishment of their new Branch and wewish them and the new Committee everysuccess in their new respective roles.

Willie EganFSW South East

New CommitteeL-R: Sandra Richter, Secretary; Stephanie Richter, PRO;

Linda McDonald, Chairperson; Mary Stevenson, Vice-Chairperson; Packie & Heather Larkin, Treasurers.

Slane Competition Winners

Congratulations to the following, who werethe lucky winners of or Slane competition inour last newsletter.

Paul Breen (Dublin),John Dullaghan (Louth),Simon Jameson (Dublin)Mark McCreanor (Louth)Patricia Peoples (Donegal)Mary Sheridan (Meath),

Each winner gets two tickets to Slane2003.The answer was "Red Hot Chilly Peppers".As there were more than six correct entries,the winners were picked from a hat.



MDI Respite ServiceIn recent weeks, we have had more inquiriesabout the Respite Service which is availableto MDI members. The following article hasbeen included once again to clarify theservices members can avail of in relation torespite:____________________________________

MDI‘s respite service provides practicalassistance to families and members in needof such support. This is done in various ways,depending on the needs of the memberconcerned. Help can be provided in the homefor a weekend, a few hours during the day, ora night turning service. We will also try toprovide assistance in an emergency situationwhere for example a parent of a child who hasmuscular dystrophy becomes ill.

MDI provides short-term respite. However wewill continue to provide respite where there isno immediate alternative. We will help youlink in with the care attendant and personalassistants schemes run by the variousbranches of the Irish Wheelchair Association,the Centre for Independent Living,Rehabcare, and the Health Boards aroundthe country.

MDI runs youth camps for children of 11yearsand older, teenagers and a short holiday foradult members. If you are interested in any ofthese activities please contact your familysupport worker in your area. Unfortunatelythe places on the holidays are restricted. Ifyou do not get an opportunity this year we willtry to facilitate you the following year.

MDI also provides financial assistancetowards family and individual member’sholidays where one of the members has MDand cannot take part in the youth camps oradult holidays organised by MDI during thesummer. MDI can only pay towards one ofthe following, the MDI adult holiday / youthcamp or the family holiday. If parents orguardians take a holiday and neither haveMD, MDI can not provide financial assistancetowards their holiday, however MDI can paytoward the cost of a care worker or personal

assistant to replace them while they areaway.

At present MDI does not employ any full timecare workers or personal assistants. Howeverwe can help you to find the appropriate careworker or personal assistant.

The aim of the respite service is to be asflexible as possible and to work with what themember requires. We will also try to link inwith the respite service providers in your localarea.

If MDI employs a care worker or personalassistant chosen by the member or family theperson will have to register at their local taxoffice and get their “tax credit certificate withstandard rate cut off point” The tax officeusually sends out these forms to MDI veryquickly. The person may already have a PPSnumber which they can give to MDI instead.

This respite service relies on funding fromvarious sources. Some Health Boards areaware of the need for funding for this vitalservice and are increasing their grants.However other Health Boards supply a verysmall amount of funding. There are still areasof the country which are not funded. MDI hasto raise funds through various fund raisingevents to supplement this under funding sothat all the members of MDI will be able tobenefit from the respite services.

If you think you may need a break or anongoing respite service you should contactthe MDI Family Support Worker or YouthWorker in your area giving them as muchnotice as possible. For planned holidays andin home respite you will have to complete anapplication form which they will give you andsubmit invoices and receipts with themember's name on the receipt to the MDIoffice in Dublin. These will then have to beapproved before any payment is made. Ifyou are unable to contact them in the caseof an emergency, you can contact me in theMDI office in Dublin.

Siobhan WindleRespite Co-ordinator MDI


Thank you to everyone who participated in this years Ladies Mini Marathon on behalf of MDI. Wehope you had an enjoyable day. To those who have yet to return their sponsorship card(s) andmoney, we would appreciate it if you could do so as soon as possible. Once again, thanks to all.The photo collage below hopefully captures a flavour of the days atmosphere.




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Over 500 copies of the MDI News Update are printed every twomonths and distributed nationwide. In addition, our newsletter is also

emailed to over 200 people and is posted onto our website.On average the MDI website receives over 250 hits a week.

To help defray the cost of this newsletter, we offer advertisement spaceto corporate companies. With this in mind do you know of any

businesses that may be interested in purchasing advertising space.

For further details contact:

Hubert McCormackMuscular Dystrophy Ireland

Coleraine House,Coleraine Street,

Dublin 7

Tel: (01) 8721501Fax: (01) 8724482

Email: [email protected]

Documents

Editorial - mdi.ie · Spinal Muscular Atrophy Spinal muscular atrophy (SMA) is a condition in which the muscle-controlling nerve cells (motor neurones) degenerate. This is because