MDF Magazine Newsletter Issue 57 December 2018

Summer Issue 57 

December 2018 

R25.00 incl. VAT 

Music therapy 

for MD children 

Beating Duchenne 

one step at a time... 

Dissecting 

Wobbles 

GET INTO THE GREEN SCENE

COMPETITION ENDS 31 DECEMBER 2018

DF 

Magazine 

05 MDF notice board 

06 National news 

10 MD information 

MD INFORMATION 

06 Muscular dystrophy awareness 

07 Facts about Duchenne & Becker muscular dystrophy 

10 The use of antioxidants in FSHD 

Events 

16 Music therapy for MD children 

18 Telkom 947 Kiddies Ride 

People 

20 Natarshja Meyer – an inspiration for others 

21 Hloni’s dream came true – visiting Disneyland 

22 Living with congenital myopathy – Lavinia Petersen 

24 Beating Duchenne one step at a time … 

25 Dissecting Wobbles 

27 By talking and knowing about CMT, people can 

understand Ella better 

28 While it’s not a cure, it’s buying us valuable time 

29 Family fund cure for George on living with Becker 

Regular Features 

36 The View from Down Here 

37 Doctor’s corner 

38 Sandra’s thoughts on … 

Research 

30 New insights into muscle fibre loss in Duchenne 

muscular dystrophy 

31 CMT gene therapy trial to start in 2019 

31 Update on DMD gene therapy trial 

31 Pain-free and more effective: improving how we monitor 

SMA 

C O N T E N T S 

Published by: 

Muscular Dystrophy Foundation of SA 

Tel: 011 472-9703 

Fax: 086 646 9117 

E-mail: national@mdsa.org.za 

Website: www.mdsa.org.za 

Publishing Team: 

Managing Editor: Pieter Joubert 

Copy Editor: Keith Richmond 

Publishing Manager: Gerda Brown 

Design and Layout: Divan Joubert 

Cover photo of Lemique Consulting CC courtesy of Sarie 

Truter 

Future Issues: 

April 2019 

(Deadline: 8 March 2019) 

The Muscular Dystrophy Foundation 

of South Africa 

We are a non-profit organisation that supports 

people affected by muscular dystrophy and 

neuromuscular disorders and that endeavours to 

improve the quality of life of its members.

From The 

Summer is finally in full swing! Whether it’s long sunny days or showers and 

thunderstorms that you prefer, a typical South African summer has it covered. 

For most of us there’s the expectation that summer will be filled with long, hot, 

lazy days and sunshine from dawn to dusk. We anticipate getting the braai 

out and spending time outdoors with loved ones. Overall, summer is just a 

wonderful season with so much to offer. 

In this summer issue you can read about how the Foundation got involved 

with raising awareness for International Muscular Dystrophy Awareness month 

during September. This campaign was launched this year and we hope that 

it will grow in years to come. Hopefully it will be extended to the African 

continent next year. 

You can also read how small children care for those less fortunate than themselves 

by raising money and riding the 947 Kiddies Ride. Well done to our little Muscle Riders! 

You are absolute STARS! 

As always there are also various inspiring stories of some our members and their journeys with muscular 

dystrophy. As well as interesting news on events, MD information and research articles. 

Our thoughts and prayers go out to Pieter Joubert and his family. Pieter suddenly fell ill and had to be 

hospitalised. We eagerly await his return to the office in 2019. 

Thank you to everyone who supported us this year. Without you the Foundation will not be able to provide 

services to our very special members. 

Wishing you a magical and blissful holiday. Have a merry Christmas and a prosperous new year! 

Until next year! 

Kind regards, 

Gerda Brown 

We would like to Thank You 

Thank you to everyone who supported us during the 

past year and helped to make a difference in the lives 

of people who have muscular dystrophy. 

From all of us at Muscular Dystrophy Foundation of South Africa 

Wishing you a wonderful festive season, 

and may the New Year bring you joy, 

peace and happiness. 

4

Subscription and contributions to 

the magazine 

We publish three issues of MDF Magazine 

a year and you can subscribe online 

to the magazine or by calling your nearest 

branch. 

If you have any feedback on our publications, 

please contact the National Office 

by e-mail at national@mdsa.org.za 

or call 011 472-9703. 

Get all the latest news on the fight 

against muscle-wasting conditions and 

the latest research updates. It is our editorial 

policy to report on developments 

regarding the different types of dystrophy 

but we do not thereby endorse any 

of the drugs, procedures or treatments 

discussed. Please consult with your own 

physician about any medical interventions. 

If you are interested in sharing your inspirational 

stories, please let us know 

and we'll be in touch to discuss this 

with you.The Foundation would love 

to hear from affected members, friends, 

family, doctors, researchers or anyone 

interested in contributing to the magazine. 

Articles may be edited for space 

and clarity. 

MDF SA database 

If you know people affected by muscular 

dystrophy or neuromuscular disorders 

who are not members, please 

ask them to contact us so that we can 

register them on our database. If we do 

not have your current e-mail and postal 

address, please contact your branch so 

that we can update your details on our 

database. 

How can you help? 

Branches are responsible for doing their 

own fundraising to assist members with 

specialised equipment. Contact your 

nearest branch of the Muscular Dystrophy 

Foundation of South Africa to find 

out how you can help with fundraising 

events for those affected with muscular 

dystrophy. 

Fundraising 

Crossbow Marketing Consultants (Pty) 

Ltd are doing invaluable work through 

the selling of annual forward planners. 

These products can be ordered from 

Crossbow on 021 700-6500. For enquiries 

contact the National Office by 

e-mail at national@mdsa.org.za or call 

011 472-9703. 

MDF :: 

MDF support information 

For more information about the Muscular Dystrophy Foundation, the benefits of 

being a member and details on how to become a member, call your nearest branch. 

CAPE BRANCH (Western Cape, 

Northern Cape & part of Eastern 

Cape) 

E-mail: cape@mdsa.org.za 

Tel: 021 592-7306 

Fax: 086 535 1387 

Address: 3 Wiener Street, Goodwood, 

7460 

Banking details: Nedbank, current 

account no. 2011007631, 

branch code 101109 

GAUTENG BRANCH (Gauteng, 

Free State, Mpumalanga, Limpopo 

& North West) 

E-mail: gauteng@mdsa.org.za 

Website: www.mdfgauteng.org 

Website: www.muscleriders.co.za 

Tel: 011 472-9824 

Fax: 086 646 9118 

Address: 12 Botes Street, Florida Park, 

1709 


account no. 1958323284 


Pretoria Office 

E-mail: swpta@mdsa.org.za 

Tel: 012 323-4462 

Address: 8 Dr Savage Road, Prinshof, 

Pretoria 

KZN BRANCH (KZN & part of 

Eastern Cape) 

E-mail: kzn@mdsa.org.za 

Tel: 031 332-0211 

Address: Office 7, 24 Somtseu Road, 

Durban, 4000 


account no. 1069431362 


General MD Information 

Cape Town 

Lee Leith 

Tel: 021 794-5737 

E-mail: leeleith@mweb.co.za 

Gauteng 

Pieter Joubert 

Tel: 011 472-9824 

E-mail: gauteng@mdsa.org.za 

Duchenne MD 

Cape 

Win van der Berg (Support Group) 

Tel: 021 557-1423 

KZN 

Maxine Strydom (Support Group) 

Tel: 031 762-1592 

Cell: 083 290 6695 

Gauteng 

Jan Ferreira (Support Group – Pretoria) 

Cell: 084 702 5290 

Estelle Fichardt 

Tel: 012 667-6806 

Christine Winslow 

Cell: 082 608 4820 

Charcot Marie Tooth (CMT) 

Hettie Woehler 

Cell: 079 885 2512 

E-mail: hettie.woehler@gmail.com 

Facioscapulohumeral (FSHD) 

Francois Honiball 

Tel: 012 664-3651 

Barry Snow 

Cell: 083 66 66 270 

E-mail: barry.snow@worleyparsons. 

com 

Friedreich Ataxia (FA) 

Linda Pryke 

Cell no: 084 405 1169 

Nemaline Myopathy 

Adri Haxton 

Tel: 011 802-7985 

Spinal Muscular Atrophy (SMA) 

Zeta Starograd 

Tel: 011 640-1531 

Lucie Swanepoel 

Tel: 017 683-0287 

5

National 

September was 

Muscular Dystrophy 

Awareness Month 

Because public knowledge of muscular dystrophy is 

limited, attitudes towards people with muscular 

dystrophy are sometimes based on ignorance and can cause 

humiliation. Some might even believe that people with 

muscular dystrophy could do more if they just tried harder, or 

that they pretend to be unable to do something because they 

just don’t want to, and that if they were just to exercise more 

they would become stronger. When people are properly 

informed about muscular dystrophy, their attitudes and 

behaviour towards affected people become more reasonable 

and sensitive; thus, discussing disability increases 

understanding and tolerance. This helps to minimise the 

psychological stress that muscular dystrophy sufferers 

might otherwise experience when interacting with others. 

September was International Muscular Dystrophy Awareness 

Month, which is an important time for all persons affected 

by muscular dystrophy. In order to celebrate this special 

month, the National Office launched a new online awareness 

programme called “Get into the green scene” – green being 

the colour of the muscular dystrophy ribbon. 

The objectives of the programme were two-fold. Firstly, all 

the MDSA Facebook followers were requested to change 

their Facebook and WhatsApp profile pictures to the 

“Get into the Green scene” logo. Secondly, our Facebook 

followers and members were invited to share photos on our 

Facebook wall where they have “gone green”. From the 

photos it was clear that everyone had a lot of fun. 

Thank you very much to Lemique Consulting, Newcastle 

Post Office, West Rand School, the MDF branches and our 

members for making this campaign a huge success. 

6 

Muscles Green 

By Emile Smith 

My muscles became green 

It's quite a colourful scene 

I'm planted in the ground 

My wheelchair need sound 

Winter left me bleak 

I'm no more weak 

Can you see a blossom? 

It's green and I'm awesome 

I try my best 

Just chilax with the rest 

These muscles is bit slow 

My wheels on the go 

My muscles became green 

It's the colour of the winning scene.

MD 

Some facts about Duchenne and Becker 

muscular dystrophy 

What is Duchenne/Becker muscular 

dystrophy? 

Duchenne muscular dystrophy (DMD) is the most common 

and serious neuromuscular genetic disorder diagnosed in 

childhood. It is a progressive disorder that causes muscles 

to become weaker over time until it affects the whole body. 

DMD mostly affects boys and reaches across all races and 

cultures. Although genetic disorders are usually passed down 

from a parent to a child, Duchenne and Becker muscular 

dystrophy (BMD) can spontaneously occur even if no one in 

the family has had it before. 

Becker muscular dystrophy occurs when dystrophin is 

manufactured, although not in the normal form or amount. 

Because of this, people with Becker are more mildly affected 

and have a slower progression than people with Duchenne. 

As with Duchenne, Becker can be inherited from a parent 

or can be caused by a new or spontaneous mutation in the 

dystrophin gene. The symptoms of Becker can begin in 

childhood, the teenage years, or even later. PPMD’s 

mission and work extends to both Duchenne and Becker (both 

considered ‘dystrophinopathies’), but for simplicity we 

primarily refer to Duchenne. 

DMD is a “gender-linked or “X-linked” disorder. Males are 

born with one X-chromosome (from mom) and one Y-chromosome 

(from dad). Because males only have one X chromosome, 

if the X-chromosome they receive has a gene mutation 

that causes Duchenne, the male will be born with Duchenne. 

Females are born with two X-chromosomes (one from mom 

and one from dad). If females have one X-chromosome that 

has a gene mutation for Duchenne, the body will generally 

choose to “activate” the other normal X chromosome. If 

this happens, the female will be a carrier of Duchenne. This 

means that she can pass Duchenne on to her sons (her daughters 

can also be carriers) but she herself will have few, if any, 

symptoms of Duchenne. If the female’s body, for whatever 

reason, chooses to activate the X chromosome with the mutation 

for Duchenne, the female will still be a Duchenne carrier, 

but will also “manifest” symptoms of Duchenne. She 

will then be a “manifesting carrier” of Duchenne. 

What causes the symptoms? 

Parent Project Muscular Dystrophy writes as follows 

(©2018): 

Duchenne is caused by mutations in the gene that encodes 

for a muscle protein called dystrophin. Dystrophin is in 

every single muscle fibre in our bodies. Dystrophin acts as 

the glue that holds muscles together and the “shock absorber” 

that allows muscles to contract and relax without being 

damaged. Without dystrophin, muscles are not able to function 

or repair themselves properly. As muscles are used for normal 

day-to-day activity, tiny tears are created in the muscle. 

Because there is no dystrophin, the muscles can’t repair 

themselves by making new muscle, so the damaged muscle 

is replaced by fat and scar tissue. As muscle is replaced, the 

person with Duchenne loses muscle function and strength. 

There are many muscles in the body (skeletal muscles, heart 

muscles, breathing muscles, etc.). Because there are so many 

muscles in the body, many parts of the body can be affected 

by Duchenne. For that reason, people living with Duchenne 

need care for many areas of the body. 

What are the symptoms of DMD and which 

muscles are affected? 

The DMD fact sheet of the Muscular Dystrophy Foundation 

of South Africa (2000b) provides the following information: 

Most affected boys develop the first sign, which is difficulty 

in walking, at the age of 1 to 3 years. By approximately 8 to 

11 years they become unable to walk. By their late teens to 

early twenties the weakness is usually serious enough to put 

their lives at risk. 

Boys affected by DMD often walk on their toes with their 

abdomen pushed forwards and with a waddling gait. This 

is due to weakness of pelvic muscles, which normally 

extend to the hips in order to retain the upright position when 

standing. When these muscles are weak there is a tendency 

for the pelvis to tilt forward, and to compensate for this the 

7

MD 

affected boy pushes his abdomen forward (called lordosis) 

and his shoulders backward. Rising from the floor unassisted 

also becomes progressively difficult, which is also due to 

weakness of the muscles around the hips. At the same time 

as weakness of the hip muscles becomes evident, there is also 

weakness of the shoulder muscles so that the affected boy has 

increasing difficulty raising his arms. 

The confinement to a wheelchair increases the likelihood of 

contractures developing as a result of immobility. The sole 

of the foot often turns inward. The prolonged sitting in one 

position may result in the gradual curvature of the spine to 

one side and so compresses the lung on that side. This is referred 

to as scoliosis and can result in serious problems with 

breathing and chest infection. 

The heart is also affected in DMD and this may aggravate 

any respiratory problems. 

and thighs, while others that are less weak are often enlarged. 

The muscles of facial expression, speech and swallowing and 

the involuntary muscles (for example those of the bowel and 

bladder) are not affected in BMD. 

Things to remember when visiting an 

emergency room 

The DMD emergency card of the Muscular Dystrophy 

Foundation of South Africa (2017b) provides the following 

information: 

Duchenne muscular dystrophy 

Leg fracture 

• If ambulatory: Ask if internal fixation/surgery rather than 

casting may be possible. Surgery may help preserve 

walking. 

• If your child has had a fall or a leg injury and has rapid 

onset shortness of breath/difficulty breathing and changes 

in alertness (confusion, agitation, disorientation), this is an 

emergency; go immediately to the ER and alert staff that 

symptoms could be due to fat embolism syndrome (FES). 

Respiratory care 

• Risk: Respiratory failure. Please only give oxygen with 

close monitoring of CO2 levels; breathing may need to be 

supported (with BiPAP, for example). 

• If oxygen levels are low, assisted coughing (with cough 

assist machine or Ambu bag) may help. 

• Take your equipment (cough assist, BiPAP, etc) with you to 

the hospital/emergency room (ER); alert your neuromuscular 

team that you are going to ER/hospital. 

What are the symptoms of BMD and which 

muscles are affected? 

The BMD fact sheet of the Muscular Dystrophy Foundation 

of South Africa (2000a) provides the following information: 

BMD has a mild onset during childhood. Muscle cramps 

during exercise are often the only problem at first but a few 

affected individuals may also be late in learning to walk. 

From early childhood affected boys are unable to run 

very fast. During the teens or twenties, muscle weakness 

becomes evident causing difficulty in rapid walking, running 

and climbing stairs. Later it may be difficult to lift heavy 

objects above waist level. Individuals with typical BMD may 

become unable to walk in their 40s or 50s, or even later, but 

there are also more rapidly progressive variants of BMD in 

which the loss of mobility may happen in the 20s or 30s. 

Over a period of years some muscles become weak and wasted, 

especially certain muscles of the shoulders, upper arms 

General recommendations and precautions 

• Keep immunisations up to date and get influenza vaccine 

annually. 

• People taking daily, long-term steroids should avoid live 

vaccines when possible. 

• Always wear seat belts – in the car AND on the wheelchair/ 

scooter. 

Anaesthetic precautions 

• Avoid inhaled anaesthesia. 

• IV anaesthesia is considered to be safe (with close 

monitoring). 

• People with Duchenne should NOT receive 

succinylcholine. 

• Local anaesthetics and nitrous oxide are safe for minor 

dental procedures. 

If vomiting and/or unable to take daily corticosteroids for 24 

hours 

• Go to a hospital emergency room. 

• Request substitute IV corticosteroid until oral medications 

are tolerated (6 mg of deflazacort equals 5 mg of prednisone). 

• Remind clinicals that high liver enzymes (AST/ALT) are 

normal for people with Duchenne MD. 

8

MD 

The BMD emergency card of the Muscular Dystrophy 

Foundation of South Africa (2017a) provides the following 

information: 

Becker muscular dystrophy 

Cardiac care 

• Patients with Becker muscular dystrophy can develop 

cardiomyopathy. Those with less severe muscle symptoms 

are at risk of severe heart involvement. All need regular 

heart checks. 

• Early ACE-inhibitor and beta-blocker usage slow the 

progress of cardiomyopathy. 

• Cardiac arrhythmias must be considered for patients with 

palpitations and/or dizziness/pre-syncope and investigated 

with ECG, 24-hour tapes or similar. 

Respiratory care 

• Chronic respiratory failure in Becker muscular dystrophy 

may present without the usual signs of respiratory distress. 

Subtle signs include early morning headaches, poor sleep 

continuity, fatigue, daytime sleepiness, reduced appetite 

and weight loss. Consider underlying respiratory failure in 

case of a chest infection. 

• If supplemental oxygen is required during a respiratory 

crisis, this must be carefully controlled. Healthcare professionals 

must be alert to the possibility of acute respiratory 

failure with an arterial blood gas assessment of oxygen, 

carbon dioxide and bicarbonate concentration. Non-invasive 

ventilation, with oxygen entrained, may be required. 

• Assisted coughing with chest physiotherapy and breathstacking 

techniques with an Ambu bag help to clear lower 

airways secretions. This can also be facilitated by a cough 

assist device. These interventions should be performed 

only by trained and experienced persons. 

Anaesthetic precautions 

• Use intravenous general anaesthetics only (avoid suxamethonium). 

• Inhaled anaesthetics should not be used. 

• Local anaesthetics and nitrous oxide are safe, e.g. for minor 

dental procedures. 

Recommendations and precautions 

• Immunisations should be kept up-to-date. Do not use live 

vaccines if using corticosteroids. 

• Wear a medic alert bracelet. 

NOTE: Liver enzymes (AST/ALT) will be high on blood 

tests: this is normal in Becker muscular dystrophy and is 

attributed to muscle breakdown. This should not prompt liver 

investigations unless otherwise indicated. 

Sources 

Muscular Dystrophy Foundation of South Africa. 2017a. 

Becker muscular dystrophy. Emergency card. 

Muscular Dystrophy Foundation of South Africa. 2017b. 

Duchenne muscular dystrophy. Emergency card. 

Muscular Dystrophy Foundation of South Africa. 2000a. 

Becker muscular dystrophy. Fact sheet, 30 May 2000. 

Muscular Dystrophy Foundation of South Africa. 2000b. 

Duchenne muscular dystrophy. Fact sheet, 30 May 2000. 

Parent Project Muscular Dystrophy. ©2018. What is Duchenne/Becker 

muscular dystrophy? www.parentprojectmd. 

org/care/for-families/for-newly-diagnosed/learn-about-duchenne-and-becker/. 

Suppliers of Medical 

Equipment 

CE Mobility 

Phone: 0860 236624 

Johannesburg, Cape Town, Durban, 

Port Elizabeth, Pretoria & Rivonia 

Website: www.cemobility.co.za 

Impact Medical Supplies 

Phone: 011 469-1750 

E-mail: impactmed@worldonline. 

co.za 

Website: www.impactmedical.co.za 

Clinical Emergencies 

Phone: 011 443-9093 

E-mail: clinical@icon.co.za 

Website: www.clinicalemergencies. 

co.za 

Solutions Medical 

Phone: 021 592-3370 

Website: www.wheelchairs.co.za 

Medmedical 

Phone: 011 640-5262 

Website: www.medmedical.co.za 

Medop cc 

Phone: 011 827-5893/4/5 

Website: www.medop.co.za 

Wheelchairs on the Run (Pty) Ltd 

Phone: 011 955-7007 

Website: www.wheelchairs-ontherun. 

co.za 

Radical Mobility 

Phone: 011 664-6069 

E-mail: www.radicalmobility.com 

Hands on Lifts (Pty) Ltd 

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E-mail: lynn@handsonlifts.co.za 

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Chairman Industries 

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Website: www.chairmanind.co.za 

Flybrother SA 

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Jessen Dakile (Pty) Ltd 

Phone: 011 793-6260 

Website: www.jessendakile.co.za 

Sheer Mobility 

Phone: 021 552-5563 

Cell phone: 082 926 5414 

Website: www.sheermobility.co.za 

Shonaquip 

Phone: Cape Town 021 797-8239 

Phone: Pretoria 012 665-1211 

Phone: Port Elizabeth 079 524-4350 

E-mail: nina@shonaquip.co.za 

Website: www.shonaquip.co.za 

Botlhale Ke Katlego Trading (Pty) Ltd 

Phone: 083 424 7231 

Website: www.bkktrading.co.za 

9

MD 

The use of antioxidants in FSHD: 

What is the scientific rationale and what does 

the evidence show? 

By Adam Denny, PhD 

(Reprinted with the permission of June Kinoshita – FSH Society) 

In recent years, scientists around the world have begun to investigate the role of oxidative stress in FSHD. A 2018 study 

reported that muscle biopsies from people with FSHD had greater levels of oxidative stress markers when compared to healthy 

control muscle (Dias Wilson et al., 2018). In another study, a stem cell-based model indicated that high oxidative stress itself 

can lead to increased DUX4 expression (Sasaki-Honda et al., 2018). 

These findings indicate that DUX4 can increase oxidative stress, and that oxidative stress itself can increase DUX4 

expression, proposing a damaging feedback loop, although this hypothesis needs further investigation. 

What is clear in FSHD is the imbalance in reactive species and antioxidant defenses, the two key aspects underpinning 

oxidative stress. 

What is oxidative stress? 

Oxygen is essential to life, but it’s a double-edged sword. Certain oxygen-containing molecules are chemically very reactive 

and, if allowed to run amok in our bodies, can damage DNA, proteins, fats, and other components. To combat these “reactive 

species,” biology has evolved molecules called antioxidants that neutralize or repair the damage. 

Oxidative stress results when the production of reactive species outpaces the body’s antioxidant defenses. This imbalance can 

result in longterm damage. Increases in oxidative stress are beneficial in some circumstances, for example, to destroy cancer 

cells, but in disease, oxidative stress can be harmful. 

10

The role of oxidative stress in FSHD 

MD 

Most studies of FSHD and oxidative stress are conducted in laboratory models, so we have to be cautious about extrapolating 

these findings to humans. 

As depicted in the figure (above), lab studies have shown that DUX4 expression can induce a multitude of problems within 

skeletal muscle. These include impaired formation of muscle cells and increased inflammation, oxidative stress, and cell 

death. 

Given these lab findings, individuals with FSHD may well wonder if they should take steps to reduce the potential harm from 

oxidative stress. 

About antioxidants 

To combat oxidative stress, we have antioxidants—substances 

that can delay, prevent, or remove oxidative damage. 

There are two categories of natural antioxidants: 

enzymatic and nonenzymatic antioxidants. Enzymatic 

antioxidants are produced by our bodies and work 

through breaking down and protecting against the 

reactive species. Non-enzymatic antioxidants are a much 

larger class of antioxidants and work through disrupting 

the chemical reactions caused by reactive species. 

Some natural non-enzymatic antioxidants are made 

within our bodies, such as vitamin A and coenzyme Q10. 

When our bodies don’t make sufficient amounts, we can 

take them in pill form. Others stem from external sources, 

such as vitamins C, E, and K, along with zinc and selenium. 

Antioxidants from external sources are important to factor into a person’s diet. While these antioxidants are also available as 

pill supplements, many people prefer to obtain these antioxidants from a balanced diet. 

Antioxidant trials 

While antioxidants have shown promise throughout the 

years in laboratory studies, these results have been hard 

to replicate in clinical trials. 

One clinical trial investigated the effect of combined 

dietary supplementation of vitamins C and E, and minerals 

zinc and selenium in individuals with FSHD (Passerieux 

et al., 2015). The researchers found that supplementation 

did not improve the two-minute walk test, but it did 

improve muscle function: individuals could contract 

their thigh muscles (quadriceps) harder and for longer 

than they could before the supplementation. 

The participants who received supplementation also saw 

decreases in some of their oxidative stress markers and 

increases in certain markers of their antioxidant defense 

system. 

In another small clinical trial, a Dutch group (Van der Kooi et al., 2016) investigated the supplementation of folic acid and 

methionine in people with FSHD. Both supplements have antioxidant properties, and folic acid has previously been shown 

to boost DNA methylation, but this study found neither folic acid nor methionine had an effect on DNA methylation levels. 

11

MD 

Other ways to protect against oxidative stress 

Exercise is a very effective way to increase our antioxidant defenses. However, in order to obtain this benefit, one 

has to first use exercise to increase levels of oxidative stress. The body then responds by boosting its antioxidant 

defenses. 

While the oxidative stress produced in exercise is 

vital, the exact effect this may have on FSHD is not fully 

understood. The type, intensity, and duration of the 

exercise all may influence the outcome. 

Bankolé and colleagues (Bankolé et al., 2016) have 

shown that a combined strength and interval cycling 

exercise-training program significantly improved fitness 

and skeletal muscle function without negatively impacting 

muscle damage. While this study did not investigate 

the antioxidant levels of participants, it does highlight 

that certain types of exercise can be beneficial for people 

with FSHD regardless of the increases in oxidative stress 

induced by exercise. 

In conclusion… 

Research to date has shown some positive results, but important questions remain. First, oxidative stress has a strong link with 

FSHD cells in the test tube, but the relevance of oxidative stress in FSHD patients is unknown. 

Also, while antioxidant therapies have shown modest benefits, these results came from relatively small clinical trials. We need 

to conduct larger trials to understand if antioxidant therapies can protect muscles in people with FSHD. 

Editor ’s note: Adam Denny earned his PhD in the Department of Physiology at the University of Otago in New Zealand. 

Article available at: https://www.fshsociety.org/2018/10/the-use-of-antioxidants-in-fshd/ 

References 

Bankolé, L.C., Millet, G.Y., Temesi, J., Bachasson, D., Ravelojaona, M., Wuyam, B., Verges, S., Ponsot, E., Antoine, J.C., Kadi, 

F. & Féasson, L. 2016. “Safety and efficacy of a 6-month home-based exercise program in patients with facioscapulohumeral 

muscular dystrophy: A randomized controlled trial”, Medicine, 95(31): e4497. doi: 10.1097/MD.0000000000004497. 

Dias Wilson, V., Thomas, C., Passerieux, E., Hugon, G., Pillard, F., Andrade, A.G., Bommart, S., Pincemail, J., Mercier, J., 

Arbogast, S. & Laoudj-Chenivesse, D. 2018. “Impaired oxygen demand during exercise is related to oxidative stress and 

muscle function in FSHD”, JCSM (Journal of Cachexia, Sarcopenia and Muscle) Rapid Communications, 1(1). https://jcsmrapid-communications.info/index.php/jcsm-rc/article/view/29. 

Passerieux, E., Hayot, M., Jaussent, A., Carnac, G., Gouzi, F., Pillard, F., Picot, M-C., Böcker, K., Hugon, G., Pincemail, J., 

Defraigne, J.O., Verrips, T., Mercier, J. & Laoudj-Chenivesse, D. 2015. “Effects of vitamin C, vitamin E, zinc gluconate, and 

selenomethionine supplementation on muscle function and oxidative stress biomarkers in patients with facioscapulohumeral 

dystrophy: A double-blind randomized controlled clinical trial”, Free Radical Biology and Medicine, 81: 158-169. 

Sasaki-Honda, M., Jonouchi, T., Arai, M., Hotta, A., Mitsuhashi, S., Nishino, I., Matsuda, R. & Sakurai, H. 2018. “A patientderived 

iPSC model revealed oxidative stress increases facioscapulohumeral muscular dystrophy-causative DUX4”, Human 

Molecular Genetics, September 12. DOI: 10.1093/hmg/ddy293. 

Van der Kooi, E.L., De Greef, No effect of folic acid and methionine supplementation on D4Z4 methylation in patients with 

facioscapulohumeral muscular dystrophy”, Neuromuscular Disorders, 16(11): 766-769. DOI: 10.1016/j.nmd.2006.08.005. 

12

13

Where the Big Five meet Sylvester the lion 

The Karoo National Park is famous for many 

wonderful reasons; however, in 2015 the most 

famous reason was the grand escape of Sylvester 

the lion. 

The story began during the night of 4 June 2015 

when a three-year-old lion escaped from the Karoo 

National Park, triggering a long and arduous chase 

lasting 24 days. This event had the nation watching 

and waiting with bated breath and saw the start of a 

wild goose chase involving many devoted individuals, 

sniffer dogs, helicopters and even a microlight. 

By the first week the media had run with the story 

and Sylvester was a nationwide sensation, with a 

select team of SANParks' trackers and honorary 

rangers in hot pursuit of the wanderer. Sylvester 

was finally darted on the 24th day of the search, 

having travelled approximately 371 km and been 

spotted a mere four times. Along the way he had 

killed 27 sheep, a kudu and an Nguni cow. Although 

Sylvester took his toll on local livestock during his 

three-week adventure. it is said that during this time 

stock theft in the region dropped to an all-time low! 

Before Sylvester’s escape the other male lions in the 

Karoo National Park had been making life very difficult 

for him, provoking his intention to roam. When 

Sylvester, fearing for his life, managed to escape 

again from the Park within a matter of months after 

his return, SANParks made the decision to move 

him to a new home. 

And so we leave Karoo National Park and turn our 

attention 350 km south east, where lies the Kuzuko 

contractual area of Addo Elephant National Park. It 

is home to the Big Five and now to one of Africa's 

most famous sons, Sylvester the lion. 

During the day you can look out over the Karoo 

Plains and Zuurberg Mountains, and at night you, 

and Sylvester, can look up at a canopy of a million 

stars. Perched on top of a hill, Kuzuko is located 

in a malaria-free region and provides luxurious 

accommodation, fine dining, cheetah encounters, 

nature walks, bushman painting exploration and 

guided game drives. 

Kuzuko has a fully wheelchair accessible 

chalet available for disabled visitors, providing 

plenty of space, all the required grab rails and a large 

roll-in shower. The unit is accessed by a well-lit 

paved path from the main lodge building. The lodge 

itself has an accessible viewing deck, dining room 

and lounge areas with shallow gradient ramps 

linking most points. 

Although visitors are expected to leave their 

vehicles at the park entrance, those using wheelchairs 

will be given the opportunity to drive their 

personal vehicle to the lodge entrance when 

needing to disembark or embark. 

The game drive vehicles are not specifically 

wheelchair accessible, but the rangers and 

staff will do whatever it takes to allow disabled 

visitors to enjoy their game drives! We found them 

to be extremely willing and helpful and very 

considerate when negotiating with the rugged 

terrain of the park.

These days Sylvester has settled down and found 

the company of a male buddy, Fielies, and together 

they have connected with two younger lionesses 

who have their own remarkable story to tell of 

survival against overwhelming odds – but that is for 

another day. Sylvester has found a territory he can 

call his own and, together with Fielies, they reign as 

the kings of Kuzuko. 

Kuzuko Lodge 

Contact number: + 27 (0)42 203 1700 

Email address: kuzuko@legacyhotels.com 

Web: https://www.kuzuko.com 

ACCESSIBLE HOLIDAY ACCOMMODATION 

The following establishments offer more wheelchair accessible accommodation which has been used and 

evaluated by a member of the MDF. If you have stayed at a venue and found the accommodation to be wheelchair 

accessible, please let us know so that we can add it to our database. 

Addo Elephant National Park 

www.sanparks.co.za/parks/addo/ 

Cornerway House, Plettenberg Bay 

info@cornerwayhouse.co.za 

Excelsior Manor Guesthouse, Robertson 

www.excelsior.co.za 

FunkyTown, Jeffreys Bay 

www.funkytown.co.za 

Gubas de Hoek, Robertson 

www.gubas-dehoek.com 

Mountain Zebra National Park 

www.sanparks.co.za/parks/mountain_zebra/ 

Rocherpan Nature Reserve 

www.capenature.co.za/reserves/ 

Storms River Mouth 

www.sanparks.co.za/parks/garden_route/camps/ 

storms_river/default.php 

West Coast National Park 

www.duinepos.co.za/ 

Makaranga Garden Lodge, Kloof, Durban 

www.booking.com/hotel/za/makaranga-gardenlodge.en-gb.html 

15

Events 

Music therapy for MD children 

Music therapy 

Music therapy is the use of music and musical elements 

by a registered music therapist to promote, maintain, 

and restore mental, physical, emotional, and spiritual 

health. Music has non-verbal, creative, structural, and 

emotional qualities. 

Characteristics and need areas: 

• Need of independence 

• Feeling of accomplishment 

• Opportunities to participate in meaningful activities 

• Enjoyable and recreational experiences 

• Opportunities for expression 

How can music therapy address the need areas for an individual with 

muscular dystrophy? 

• Motor skills: Strengthening of muscles through movement and instrumental play. Music also 

provides motivation for movement and for improving the range of motion. Instrumental play and 

movement can improve coordination. 

• Communication skills are enhanced through the relationship with the therapist or group members. 

Turn taking and listening during sessions helps to enhance communication. 

• Cognitive skills are activated through composition and song writing. 

• Emotional support is given through the opportunity for frustration to be vented verbally and 

non-verbally and through verbal processing, song writing and instrumental expression. 

• Musical skills are exercised. 

• Social interaction takes place in music therapy and meaningful social relationships are built. This 

decreases isolation and boosts self-confidence. Music therapy provides space for unconditional 

positive regard and can build or restore self-esteem. 

16

Events 

The music therapist 

After designing realistic goals and target 

objectives to address identified needs, 

music therapists plan and implement individualised 

music therapy treatment programs with 

strategies, procedures, and interventions to 

develop skills necessary to achieve an 

optimum level of success or quality of life for 

individual clients, such as those with muscular 

dystrophy. Three clinical goals will be set for 

each individual or group. 

Music therapists document client responses, 

conduct ongoing evaluations of progress and 

performance, and make recommendations for 

future consideration. Music therapists work with team members and families, providing ways to include 

successful music therapy techniques that help to enhance the quality of life. 

For more information about music therapy in your area, please look at the website of the South African 

Music Therapy Association at www.samta.co.za. 

Music therapy session at West Rand School 

On 31 October, MD learners at West Rand School in Krugersdorp were treated to an amazing music therapy 

session lead by Mrs Estine Brown. 

At first they seemed apprehensive, unsure of what this lady with a guitar had in store for them. Soon all 

was revealed and they were all joined together in laughter, happiness and the pure joy of making music 

together. 

Afterwards they were treated further to gift bags from MDF Gauteng which contained various tasty treats. 

The day was an immense success, and each and every child had a fun-filled experience they would not 

soon forget. 

17

Events 

Telkom 947 Kiddies Ride 2018 

By Dr Sandeepa Rajbaran Singh 

Cayden Fourie, Dante Fourie, Ava Rudman, Juanru 

Roodt, Heinrich Muller, Beth van Til, Maheya Singh, 

Leyha Naicker, Kian Otto, Riley Vermaak, Henrico Verster, 

Dyllen du Plooy, Daniël Erasmus and Liam Dykstra 

may not have all the answers, but the one thing 

they know for sure is that “Improvement begins with I”. 

Motivated only by the desire to improve the lives of others, 

each of the junior Muscle Riders registered for the 

Telkom 94.7 Kiddies Cycle Challenge believing “I can 

make a difference”. 

Beneath bright blue skies and glorious sunshine, riders 

exchanged high-fives and encouraging hugs to slow 

the butterflies that fluttered in their tummies. The roar 

of excited cyclists, kitted and ready to go, were punctuated 

only by repeated safety checks. At the start line 

each of the children was already a champion, but that 

didn’t stop the zeal with which they furiously sped towards 

the finish line. With little beads of perspiration 

rolling down flushed rosy cheeks, the kids arrived at the 

finish knowing they had made a difference in the lives 

of their friends with muscular dystrophy. 

The reasons for choosing to ride are not only simple 

and true but also motivational enough to challenge each 

one of us into action! Riley Vermaak (aged 4), whose 

grandpa lost two sons affected by Duchenne muscular 

dystrophy many years ago, rode in support of anyone 

with this disease. Brothers Dante (3) and Cayden Fourie 

(6) rode to help their grandpa raise lots of money 

to buy wheelchairs for those who can’t walk. Cousins 

Maheya Singh (5) and Leyha Naicker (11) have grown 

up appreciating how blessed they are and realise that 

they need to help others whenever they can, especially 

when helping others feels so good! Grateful for the legs 

that Jesus gave him to be able to ride his bike, Juanru 

Roodt (5) took part in the race for friends who can’t 

walk, and he added that he was raising money to buy 

wheelchairs for them. 

On the day of the cycle challenge, these brave cyclists 

showed their parents and the community that they not 

only knew how to count but that they also knew what 

counts the most … kindness and generosity! 

18

19

People 

Natarshja Meyer: 

An inspiration 

for others 

By Mariska Meyer 

Natarshja is far from stopping her efforts to 

excel, achieve her goals and dreams, and exceed 

expectations. She would like to study tourism, and 

her dream is to visit holiday destinations and resorts 

to make sure they are wheelchair/disability friendly. 

Natarshja is my family’s biggest inspiration, and I 

know her future is bright and she will continue to 

achieve and excel in whatever she does. 

I believe I am the proudest mother as I write this. 

Our daughter, Natarshja Meyer, who is writing 

matric this year at the best school this side of the 

equator, Muriel Brand in Brakpan, is the biggest 

inspiration of them all. Our family has been 

through some tough, trying times, starting with my 

mother and my children’s grandmother passing 

away at a young age of 41, my three brothers being 

diagnosed with myotonic dystrophy, Natarshja being 

diagnosed with Friedrich’s ataxia, and losing my dad to 

murder in 2015, just after my husband left his 

company to go into business with my father and being 

unemployed since. Natarshja never lost hope, 

never gave up and always worked hard to achieve 

the goals she set for herself. 

We are blessed to call this young lady with a 

fighting spirit our daughter, who will never give up 

on her hopes and dreams and has the brightest 

outlook on her future. 

Note: Natarshja Meyer and Gert (a friend) 

attended Muriel Brand’s matric farewell at 

“Four One and Only”, Petit, Benoni. 

Natarshja’s hair and make-up were done by 

Jaco de Meyer (076 159 7356). 

The car was sponsored by GM Panelbeaters 

(Wynand van der Walt, 076 499 1584) 

I cannot ever remember Natarshja not being 

the biggest inspiration and motivator to always 

work to reach your goals and to never give up on 

your hopes and dreams. In 2016 at age 16/17 

Natarshja broke the u/18 javelin record for girls with 

disabilities, making me the proudest mother and the 

teachers jump for joy. In 2017 Natarshja found grade 

11 a bit difficult and challenging, with her health 

taking a dip to such an extent that teachers were 

concerned that the stress levels of grade 12 might 

be just too much for her fragile body and gentle 

personality. We decided to give Natarshja the 

option to leave school with a honourable grade 11 

certificate, which is still a very good achievement 

for a young lady with her challenges. Natarshja’s 

kind nature turned into the fighting spirit of a lion. 

She proclaimed she had not worked so hard over 

the years to give up at the finish line! This year 

she exceeded everyone’s expectations throughout 

the year and attended her matric farewell this past 

weekend. 

20

Hloni’s dream came true visiting 

Disneyland in Miami 

By Lerato Chiloane 

It all started when Lehlohonolo Chiloane was 

nominated by an MD social worker as a suitable 

candidate to have a wish fulfilled by Reach For A 

Dream. 

She was interviewed and had three dream 

choices: one was to visit Disneyland, which 

she chose. Our exciting journey started on 18 

September 2018 at OR Tambo airport, where we 

met with Melissa and Darren of Reach For A Dream 

for some drinks and itinerary explanation. We were 

given a lovely send-off. At 20:00 we boarded the 

plane heading for Atlanta. Hloni was very excited 

as she was doing an international flight for the first 

time. The staff of Delta airline were so friendly and 

helpful in catering for Hloni’s needs. The whole 

experience was just amazing for Hloni, from the 

movies on the plane to the food. On the 19th we 

arrived in Atlanta and took a connecting flight to 

Florida, where we were greeted by the sight of its 

many beautiful beach houses. 

last park we visited was Sea World, where we saw 

all the wonders of the sea. 

How we wished to have stayed for longer to enjoy 

the place, the people, and the amazing rides. When 

people heard we were from South Africa, they 

responded very warmly and asked us lots of 

questions about the country. We held our SA flag 

high with pride. 

The most amazing thing was sharing information 

about our Muscular Dystrophy Foundation of South 

Africa and hearing about the Muscular Dystrophy 

Association that is established over there. 

It was a truly amazing experience we were 

given, and we are grateful to the MDF social 

worker Rudzani and to Reach For A Dream for 

making Lehlohonolo Chiloane’s dream come true. 

We were welcomed by staff from Give Kids the 

World Village and taken by a shuttle vehicle to our 

destination, about 45 minutes away. The village 

is phenomenal, and once there you never want to 

leave. After the Disney itinerary was given to us 

and all procedures explained, we rested for the 

remainder of the day as we were still jet-lagged. 

Next morning, we had breakfast and left for the 

Disney World Magic Kingdom. The place was so 

beautiful. Hloni enjoyed all the rides as most of 

them were wheelchair friendly. We stayed until 

closing time, stopping only for lunch, and even then 

Hloni didn’t want to leave. On the second day of our 

adventure we went to Universal Studios where we 

saw many Hollywood characters; the most exciting 

part was The Wizarding World of Harry Potter. The 

21

People 

Living with 

Congenital Myopathy 

By Lavinia Petersen 

someone to help me as my legs were not strong 

enough for me to move around on my own. I loved 

going to school, learning and meeting new friends. 

At the age of 12 years (April 1998) I started walking 

on my toes as my ligaments were short and I had to 

undergo surgery on my legs. I was out of school for 

approximately six weeks. During that period I felt so 

helpless. In June of the same year I went to see an 

orthopaedic surgeon for my scoliosis. Consequently 

a spinal brace was constructed to control my spinal 

deformity. I hated every minute of wearing it, but 

I had no choice but to do so. I had to wear it to 

school, where the children teased me, and I had to 

sleep with it on. The only time I could take it off was 

when I washed myself. I was in between the Red 

Cross Hospital and Medi-Clinic, where I was treated 

by three specialists. 

I am Lavinia Petersen from Mossel Bay, a 31-yearold 

woman who was diagnosed with congenital 

myopathy, which is a muscular dystrophy (MD) that 

affects the muscles from birth. At the age of 1 year 

I started showing signs of MD when I frequently 

fell on my forehead. My parents, Ivan and Belinda 

Petersen, took me to a paediatrician in George. 

The doctor suspected that I had some sort of MD 

and he referred me to The Red Cross Children’s 

Hospital, where a muscle biopsy confirmed that I had 

congenital myopathy. The doctor told my parents 

that I would only live until the age of 9 years; what a 

shock that must have been for my parents. I had to 

visit Red Cross Hospital every year for check-ups. 

As time went by I had to go to school. My parents 

were told by the teachers to put me in a school 

for children with special needs, but my mother 

refused and I was allowed to remain at the school 

I was at. I had my challenges at school, especially 

when climbing steps to attend classes. I always had 

When I started attending high school in 1999, my 

mother went to the principal to arrange that I have 

classes in the same block because I could not walk 

far and climb so many steps. One time at school 

during break a friend asked me: “Are you not angry 

at God for making you go through all these things, 

being disabled?” I answered: “No, I’m not because 

God has a reason for making me this way and he 

has a purpose for me being here.” She said that 

she would have been angry at God. There was a 

time when I wanted to leave school because I could 

not cope with the workload, but God gave me the 

strength to go to school each day. 

In June 2001, I had another operation on the back 

of my neck muscles. The doctor at Medi-Clinic 

explained that it would be a long procedure and that 

my parents should remain calm when they saw me. 

When the nurses pushed me out of the operating 

room and into ICU, my mother collapsed when she 

saw me because I had a brace on with four screws 

in my head. I still have the scars of this operation. 

I had a lot of support from my family, teachers and 

friends. I could not attend school for six months. 

I could not walk alone or wash myself properly or 

wear proper clothes. During the night I had to call 

my parents to help turn me over on my sides. I got 

so tired of being helpless that I cried at times. My 

mom had to leave her work during this time to take 

care of me. 

22

People 

In 2003 I finished my matric, and between 2003 and 

2005 I completed a management assistant course. I 

started my first job straight after the exams. 

Today I am 31 years old, thanks to God’s grace. I 

believe God really has a purpose for me being here 

and everything that I went through. He was with 

me every step of the way. God blessed me with the 

most amazing parents. I can always count on them 

to help me. Going through all this since my childhood 

was not an easy road to walk, but every hurdle 

I crossed made me the person I am today. 

I would like to say thank you to my family and 

everyone who has played a role in my life. 

People should be reminded that even if we are 

disabled, we should be treated with the utmost 

respect, just as anyone would want to be treated. I 

want to inspire others and tell them not to give up 

but to believe in themselves. 

Convaid Rodeo 12 Inch special needs 

stroller for sale 

Includes: 

Tilt in space 

Adjustable hardware for left & right lateral support 

Firm left & right lateral supports 

Planer seat cushion - anti-thrust, 

H-harness 

3 Point positioning belt 

Fully adjustable foot plate to suit child's length 

Removeable & fully adjustable sun canopy 

Removable rear wheels 

Folds up, can fit in a car boot 

Excellent condition. This is really a fantastic stroller for special needs children. 

Rated for up to 35kg body weight. Reason for sale: my son has outgrown it. Price: 

R30000.00 

New would be +- R60000.00 

Contact: Francois on 0828893595 

Thank you, DStv for airing our advertisements about 

muscular dystrophy. 

We are most grateful for your support. 

23

People 

Beating 

Duchenne 

one step at 

a time... 

By Albi Viljoen 

We would like to introduce you to Jaco George 

Viljoen. Jaco is an 8-year-old boy who was born on 

the 12th of April 2010 at the Sunward Park Hospital 

in Boksburg. 

At the age of 7, with the world at his feet, Jaco dreamt 

of becoming a policeman, but his world and dreams 

were shattered on the 6th of November 2017 when, 

after a lot of testing, Jaco was diagnosed with 

Duchenne muscular dystrophy. 

Being the determined child that he is, Jaco decided 

that this was merely an obstacle in his life and not 

the end of it. His parents have done intensive 

research and Jaco is receiving various alternative 

treatments, which include biokinetics twice a week 

and hydrotherapy once a week. Jaco’s diet has also 

been changed to include only healthy eating with the 

exclusion of sugar and starch. 

Jaco is a firm believer in the story of David and 

Goliath and will tell you that he is David and DMD 

is Goliath and that with God on his side he will also 

defeat the enemy. With that thought, the NPO “Walk 

with Jaco” was registered to do various awareness 

and fundraising activities to support research in the 

treatment of DMD and to fund Jaco’s treatment. The 

Facebook page “Walk with Jaco” has his day-to-day 

stories and what is currently happening in this little 

warrior’s life. Please like his page and attend the 

various fundraisers were possible. 

Contact details for 

“Walk with Jaco” are albi@walkwithjaco. 

co.za and Albi at 060 540 1437. 

24

People 

In 2010 I was a complete and utter mess, from a psychological point of 

view. Yes, a muscle disease is a physical thing, but it’s also very much 

a battle of the mind. It presents psychological, philosophical and often 

spiritual obstacles that must be negotiated up here between our ears. 

For many years people encouraged me to write my story down, 

simply to share with others and maybe to encourage people in a similar 

position to me. But the truth is I was too young and too full of 

rubbish, too emotionally ‘crippled’ to put down anything of substance. 

But, eventually, after more than fifteen years of dealing with FA 

(Friedreich's ataxia) I started, little by little. 

That was eight years ago. 

By Andrew Marshall 

Once I started I found that writing my thoughts down without 

judgement – simply vomiting my raw emotions onto my keyboard – was 

incredibly and unexpectedly cathartic. Now, I am nowhere close to 

considering myself a ‘real’ writer, thanks to a combination of some pretty 

delicious dyslexia, instinctive procrastination/laziness operating at Olympic levels and super dodgy keyboard 

skills (which I suspect applies to a lot of you guys, too). But, after meeting my editor, Andrew Miller, and having 

him help me reshape the mess I had already created, guiding me in putting down more things he thought the 

reader would want to read, I finally had something. (Not only is Andrew an award-winning novelist, the author 

of multiple other books and someone who works as a full-time writer in both the business and literary worlds, 

he is also the ‘lucky’ beneficiary of spinal muscular atrophy type 2, a type of muscular dystrophy. He combined 

his writing expertise and his experience with his own disobedient body to add to my perspective, and he has 

helped me beyond comprehension.) 

The result, after eight long years of effort, is Dissecting Wobbles. A book that takes you through my childhood 

and that feeling of always being ‘different’, scrawny, clumsy. Then my Mom’s search for answers (and the 

eternal question: why isn’t Andrew like other children?). And, of course, the big bad diagnosis, followed by my 

weird, wild, rebellious teens. 

To be honest, some of it was really difficult to write down. The struggle with not being like everybody else 

is a lifelong battle, while girls and relationships (lack thereof!) have also been massive obstacles, as were 

occasional tangles with depression, alcohol and weed. It’s not easy to talk to the world about any of these 

things, or about moving into ‘adulthood’ and trying to find a place in society. But then the position I dreaded all 

though my life, close to total incapacitation, hasn’t been a breeze either. So, as hard as it’s been to be honest, 

there’s also been freedom involved in the process. Telling my story has given me a new way to relate to, and 

share with, other people. 

Of course, Dissecting Wobbles will also tell you about the wonderful and incredibly important people in my life, 

and some of the awesome things I’ve been lucky enough to experience, including swimming with dolphins, 

treetop ziplining, river rafting and skydiving. The skydive was organised by the MDF as a fundraising event, 

and I got a bunch of people to sponsor my plummet to earth at terminal velocity. Raising cash to help people 

in the same boat as I am felt phenomenal! 

Because I live an exceptionally privileged life and have had opportunities many of my boatmates only dream 

about, once overheads for the book have been covered 50% of sales will go to the MDF. The rest will be 

invested in an idea that’s been incubating for a while, so watch this space! 

Please have a look at my website (www.dissectingwobbles.co.za), and read the synopsis and samples. If you 

like what you see you can get it at any decent book shop. The tech savvy can also use loot.com or takealot. 

com, and a digital version is available on Amazon. 

Or if you want, email me directly on marshandyjames@gmail.com and I’ll sort you out… 

25

People 

Tony Caruso is taking on 

RideLondon for the second time 

Tony Caruso, 33 from Royston, Hertfordshire, will 

be taking on the Prudential RideLondon-Surrey 100 

for the second time this July in aid of Muscular 

Dystrophy UK. 

“I absolutely loved RideLondon last time, there is 

such a buzz about the event, and after a year out I’m 

ready to take on the challenge again for Sophie.” 

Tony’s niece and goddaughter, Sophie, was born 

with the very rare muscle-wasting condition, 

Ullrich muscular dystrophy. There are less than 300 

children who have been diagnosed. 

“To date, Sophie has undergone 8 different 

operations with more in the future. There is 

currently no cure for Sophie’s condition but there is 

hope. There are plans for a clinical trial of a new 

drug and lots of effort is being put in to understand 

more about the type of MD that Sophie has. That is 

why I’m taking part in RideLondon.” 

Tony was one of MDUK’s top fundraisers in 2016, 

raising an incredible £1140, more than double the 

target. 

“I achieved this through the persistence of 

chasing people. On Facebook, personal WhatsApp 

messages, sending round work emails, getting 

Sophie’s story out there. This helped to not only get 

donations from people who know Sophie, but also 

others who didn’t and wanted to help.” 

Tony is about to start a new job and the time off 

between has given him time to get some extra 

training in and follow people up for sponsorship. 

“I think Sophie is actually more excited about me 

starting my new job than me! My new job is for 

GoHenry, a child’s pocket money card that she has 

and loves!” 

Tony’s tips for the rest of #TeamOrange taking on 

this year’s challenge is to: 

“Look out for little towns and villages whilst cycling 

through Surrey. The cheers really give you a boost 

for another few miles. 

“Travel light! I took a backpack full of gels and food, 

but didn’t really end up using any of it. There are 

plenty of food and water stations along the way to 

top up if you need to. Do, however, try and carry as 

much water as you can and keep hydrated.” 

Article online at: https://www.musculardystrophyuk. 

org/your-stories/tony-caruso-is-taking-on-ridelondon-for-the-second-time/ 

26

People 

“By talking and knowing about CMT, 

people can understand Ella better” 

Lucy Brady, whose daughter Ella, six, has Charcot- 

Marie-Tooth disease, says that raising awareness of 

the condition makes life easier for herself and her 

family. 

She said: “Ella calls her condition her ‘cheeky feet’, 

which gives people a starting point to ask questions. 

Because Charcot-Marie-Tooth doesn’t sound like it’s 

related to limbs, people sometimes get confused. 

They think, ‘What’s wrong with her teeth?’ 

afraid to ask, ‘Why does your daughter have things 

on her legs?’ 

“Children are the best at addressing it, because they 

do ask questions and then you can explain. 

“We live in a small town in Cambridgeshire, and 

quite a lot of people know Ella. Once I’ve explained 

muscular dystrophy to them, quite a few people 

have said, ‘Oh, I think that someone in my family 

must have had that.’ 

“The best way to describe it is to say that her nerves 

don’t work the way that they should, so the signals 

her brain is trying to send to her feet don’t get there. 

Because these signals aren’t getting through, she’s 

getting muscle loss because they aren’t being used 

properly. 

“The diagnosis was a massive shock for us initially 

and we felt a lot of disbelief, but you just have to get 

on with it. We’ve always tried to tell her that if there’s 

something she wants to do, she should do it. We 

encourage her to cycle and use a scooter, and we 

go on day trips to lots of different places. 

“You get drawn into lots of appointments and treatment 

plans. You adapt, but life has changed a lot 

for us. We’ve had about eight different appointments 

in the last month. Luckily I’m self-employed, which 

makes it easier, but it does have quite an impact on 

your life. 

“A lot of people are interested in Ella, but because 

she wears splints you tend to get people staring and 

not asking questions. She’s oblivious, but as her 

mum I notice it. It would be nice if people weren’t 

“By talking and knowing about CMT, people can 

understand Ella better, and that helps us as well. 

“She had surgery in February on both of her feet to 

bring them into a better position. She’s a really busy 

girl so she doesn’t like being stuck in a wheelchair. 

We’re six months on so she’s done really well. She’s 

wearing ankle foot orthosis all day and she’s even 

got a little bit of control in the muscle of her left foot 

which she hasn’t had at all for about three years. 

We’re expecting an 18-month time frame to see if it 

has corrected more than just the foot position. 

“We seem to have got the feet straight, but now 

she’s suffering in her hands. We had hoped that 

we’d have a lot more time before that happened, 

but her fingers are curving and she’s losing a lot 

of strength and wearing hand splints at night. But 

she doesn’t let it get to her – she just gets on with 

things.” 


org/your-stories/by-talking-and-knowing-about-cmtpeople-can-understand-ella-better/ 

27

‘While it’s not a cure, it’s buying us valuable time’ 

The fight for access to Spinraza 

Amy Cameron’s son Zac, three, was diagnosed with 

SMA Type 1 in November 2016. He began receiving 

Spinraza – the first and only treatment for the 

condition – last year, and Amy is now campaigning 

for all families affected by the condition to have 

access to the drug. 

Amy, from Alloa, Clackmannanshire, said: “When 

Zac was diagnosed with SMA, my world began to 

fall apart. SMA is a cruel condition and the future 

for our family looked increasingly bleak. But all this 

changed when we heard about Spinraza. Our spirits 

were instantly lifted. 

While it’s not a cure, it is buying us valuable time 

with Zac and enabling him to reach milestones we 

never thought would be possible, such as picking up 

his cup for a drink and moving his legs. Zac has so 

much to offer the world and this is the only treatment 

that is giving him the chance at life. 

“As parents, my husband and I are determined to 

secure the happiest life possible for Zac and his 

brother. Life is for living and making memories and 

we will face whatever challenges lie ahead together 

as a family.” 

Article online at: https://www.musculardystrophyuk.org/your-stories/162422/ 

The Muscular Dystrophy Foundation of SA 

would like to thank the National Lotteries 

Commission for their support. 

28

People 

‘It’s so important to raise money 

for research’ Family fund cure for 

George on living with Becker 

George Booth, six, from Weston, Stafford, began 

getting muscle spasms in his calves aged two. As 

time went on, these became more frequent and 

painful, spreading to his thighs. When the spasms 

started to last for up to an hour, preventing George 

from being able to stand or walk, his parents Adam 

and Cerys took him to the GP. 

The doctor noticed that his calves were large and 

his muscles and tendons were tight. After more 

tests and consultations, he was referred to a muscle 

team at a local hospital. George was diagnosed with 

Becker muscular dystrophy following a DNA test, in 

January 2017. 

His parents and older brother Freddie set up the 

family fund, A Cure for George, to raise awareness 

and fund research into the condition. Since then, 

Adam has cycled a stage of the Tour de France this 

summer with five friends. Cerys and Adam’s brother 

Freddie, nine, made and sold bookmarks as part of a 

summer fete stall with other fundraising games and 

Adam’s employers also donated a Champagne bottle 

signed by six nations rugby players to the cause. 

In total the family fund has raised over £16,000 in 

less than 6 months. 

Adam, a project manager, said: 

“The hard thing about Becker is the uncertainty, 

because it affects people in different ways. We don’t 

know how quickly it’s going to progress, and how 

much it’s going to impact on George. 

“At the moment, he’s doing well. He uses a 

wheelchair to get to school and if we’re doing 

something involving a lot of walking, but he can still 

play rugby on a Sunday, just as long as he takes 

some breaks when he gets tired.” 

“It’s so important to raise money for research. It still 

surprises people when we tell them that there’s no 

cure for what George has. But between now and 

when he gets older, there’s lot of time for things to 

change. The scientists with the brains to do it are 

there, we just need to make sure they have enough 

money to fund the research.” 


org/your-stories/its-so-important-to-raise-moneyfor-research-family-fund-cure-for-george-on-livingwith-becker/ 

29

Research 

The following four articles are from the website of Muscular Dystrophy UK. 

(Published date: 07/09/2018) 

Professor Jenny Morgan and her team 

at University College London (UCL) 

have shown that a type of cell death 

called necroptosis has an important 

role in Duchenne muscular dystrophy. 

The study – published today in Nature 

Communications – helps improve our 

understanding of muscle fibre loss in 

Duchenne. 

Understanding muscle fibre death 

in Duchenne muscular dystrophy 

In this project, Professor Jenny 

Morgan and her team at University 

College, London used a mouse model 

to investigate a biological process that 

leads to muscle fibre death in Duchenne 

muscular dystrophy. 

This project was co-funded by three 

members of the Duchenne Forum – 

Muscular Dystrophy UK, Duchenne 

Children’s Trust and The Duchenne 

Research Fund. The Duchenne Forum 

is a group of charities working together 

to accelerate progress in the search for 

treatments and eventually cures for 

Duchenne muscular dystrophy. 

What did the research show? 

Muscle cells in people with Duchenne 

muscular dystrophy undergo an 

abnormal type of cell death, which is the 

basis of the muscle wasting. 

30 

New insights into muscle fibre loss in Duchenne muscular 

dystrophy 

There are two types of cell death 

mechanism: apoptosis and necrosis. 

An apoptotic cell digests itself and a 

necrotic cell explodes. In Duchenne 

muscular dystrophy, muscle fibres die 

by necrosis. Necrosis was believed to 

be a disorganized process, but it has 

been recently discovered that necrotic 

cells can be finely organized. This 

organized necrosis is called 

necroptosis. 

Professor Morgan, Dr Bencze and 

colleagues have investigated the role of 

necroptosis in Duchenne. When one of 

the proteins of the necroptosis mechanism 

was blocked in muscle cells 

grown in the lab, the cells were more 

resistant to some toxic treatments, 

which normally trigger cell death. 

The researchers also generated a 

dystrophic mouse model that is missing 

this protein; this protected cells 

from necroptosis. In these mice, muscle 

cell death and scarring (fibrosis) 

were decreased, and muscle function 

was improved. These results demonstrate 

that necroptosis is an important 

cell mechanism that is involved in 

dystrophic muscle damage. Targeting 

necroptotic cell death could therefore 

be a therapeutic strategy to counteract 

muscle loss in people with Duchenne. 

The team presented this work at 

conferences including the UK 

Neuromuscular Translational Research 

Conference and the 2017 World 

Muscle Society meeting, where they 

won a prize. They also published 

By Jenny Sharpe 

their findings in the scientific journal, 

Nature Communications. 

Why is this research important 

and what are the next steps? 

This work identifies for the first time 

necroptosis as a cell death mechanism 

in muscle. Although basic research 

such as this is a long way from a 

treatment, it is important to gain a 

greater understanding of how muscles 

are damaged in Duchenne muscular 

dystrophy. These results show that 

the proteins involved in this cell death 

pathway do have a role in the symptoms 

of Duchenne, but further work is 

needed to understand the mechanisms 

behind this. If a suitable molecule was 

identified to block the cell death mechanism, 

it could help to reduce the cell 

death of muscle cells and slow down 

the development of fibrosis. Such an 

approach could be used in combination 

with other treatments aiming to restore 

dystrophin to the muscle. 

How might this research impact 

on other neuromuscular conditions? 

It is likely that muscle fibres die by this 

type of cell death mechanism in other 

neuromuscular conditions. This would 

need to be investigated in cell and animal 

models of these conditions. 

Article online at: https://www.musculardystrophyuk.org/grants/understanding-muscle-fibre-death-in-duchennemuscular-dystrophy/

Research 

CMT gene therapy trial to start in 2019 

By Sofia Nnorom 


Sarepta Therapeutics has announced that 

it will be working with Dr Zarife Sahenk 

from Nationwide Children’s Hospital in 

the United States to develop a gene therapy 

for Charcot-Marie-Tooth disease (CMT). 

The therapy – called NT-3 gene therapy – 

uses a harmless adeno-associated virus to 

carry a gene called neurotrophin-3 (NT-3) 

into the body. Neurotrophin-3 is important 

for the functioning of Schwann cells, 

which are vital for maintaining the health 

and survival of our nerves. CMT causes 

nerves to become damaged and eventually 

die, which leads to muscle weakness and 

loss of feeling. Therefore finding potential 

treatments to improve nerve growth and 

health is vital. 

Dr Sahenk has spent many years developing 

and testing NT-3 gene therapy. Her 

research has shown it can improve nerve 

health and function in an animal model of 

CMT. In Sarepta’s press release, Dr Sahenk 

said: 

Gene therapy represents a potential 

new pathway for the treatment of CMT 

Neuropathy. We look forward to collaborating 

with Sarepta, whose dedication to 

those impacted by neuromuscular disorders 

and to rigorous scientific exploration, 

echoes our own at Nationwide Children’s. 

A clinical trial to test the NT-3 gene therapy 

is planned to start in 2019. Although the trial 

will only be recruiting people with CMT 

type 1A, the NT-3 gene therapy could potentially 

treat other CMT sub-types and 

muscle-wasting conditions. Further research 

is under way to explore its potential. 

https://www.musculardystrophyuk.org/ 

news/news/cmt-gene-therapy-trial-to-startin-2019/ 

Update on DMD gene therapy trial 

By Sofia Nnorom 


Sarepta Therapeutics has released an 

update on the four boys dosed in its 

ongoing gene therapy trial. The data 

was presented by Dr Jerry Mendell, 

the trial’s principal investigator, at 

the World Muscle Society conference 

yesterday. 

Building on Sarepta’s previous data, 

Dr Mendell shared results on the fourth 

boy treated with the gene therapy 

(AAVrh74.MHCK7.microdystrophin). 

Like the other participants, he had a 

significant increase in the dystrophin 

protein and a reduction in creatine kinase 

levels – a marker for muscle damage. 

So far, there have been no serious 

safety concerns reported for any of the 

participants. 

Dr Mendell also reported that all 

four boys have shown improvements 

in functional assessments (e.g. stair 

climbing, standing up and walking 

ability) compared to the start of the 

study. Whilst these preliminary results 

are very exciting and promising, it 

should be cautioned that this study is 

very small and has no controls. 

Sarepta is now planning to conduct a 

larger trial in the US – data from this 

trial will be used to seek marketing 

approval for the therapy. The company 

is mapping ways to expand its clinical 

program and will announce an update 

once it finalises its plans. 

In a press release, Doug Ingram, 

Sarepta’s president and chief executive 

officer said: 

“The encouraging results that we 

previously saw and reinforced in the 

fourth patient strengthen our resolve 

to rapidly move to a confirming trial 

and, assuming successful, to bring 

this therapy to the Duchenne community 

around the world with a sense of 

urgency.” 

Article online at: https://www.musculardystrophyuk.org/news/news/updateon-dmd-gene-therapy-trial/ 

Pain-free and more effective: improving how we monitor SMA 

By Andrew Mickel 

Published 01/05/2018 

Monitoring the progression of 

muscle-wasting conditions is essential 

to ensure they can be managed well. 

If you have spinal muscular atrophy 

(SMA), you may have experienced 

a test called electromyography on a 

clinical trial or during diagnosis. It 

tracks the loss of motor units, the links 

between the muscle and the nervous 

system which are gradually lost for 

people with SMA. But electromyography 

can be both painful and it is limited 

in how well it can monitor change. 

And with treatments like Spinraza now 

being developed, the need for effective 

testing to ensure patients get the right 

support at the right time has never been 

clearer. 

A new technique – discovered by 

accident 

A new research grant from MDUK will 

help develop a technique using MRI 

scanners to monitor motor units in a 

pain-free and more effective way. 

And like so many of science’s best 

discoveries, it was found by accident. 

Professor Andrew Blamire at 

Newcastle University has spent much 

of his research career trying to get 

scanners to take new measurements. 

But even he was surprised at the results 

of a recent test. 

31

Research 

He said: “A few years ago we were 

running tests using a technique that’s 

generally used to measure the structure 

of the brain. We were looking at 

the muscles of those with Duchenne 

muscular dystrophy, but when we got 

back the scans, we found we had some 

bad images. 

“They were described by others we 

were working with as ‘annoying artefacts’, 

a problem with the data. But we 

showed them to some colleagues who 

specialise in neurophysiology, and that 

was the Eureka moment – they told us 

they were motor units.” 

The MDUK research grant will fund 

Prof Blamire to fully examine how 

motor units could be monitored with 

MRI, and how it can be translated into a 

technology that can be easily deployed 

for people with muscle-wasting conditions. 

Crucially, the new technique could 

also be used as a way to track loss in 

clinical trials, helping to see how 

treatments work and finding the best 

ways to deploy them. 

Questions that still need 

answering 

The new technique – dubbed by the 

team as MUMRI, for motor unit 

magnetic resonance imaging – holds 

a lot of promise. Prof Blamire will 

now look at taking the initial idea and 

seeing if his team can turn it into something 

workable by answering some key 

questions. 

To start with, what precisely did Prof 

Blamire see in the scans? Were they 

single motor units, or was it several 

motor units firing at once? 

And how exactly will the team be able 

to isolate motor units to test them in a 

systematic way that will be helpful for 

clinicians? That second question could 

involve some new kit being developed 

so that individuals being scanned make 

precise movements that can be examined 

in a concise, systematic way. 

Using existing technology to 

tackle new problems 

While we think of MRI scanners as 

really large machines, there are some 

scanners designed just for limbs. Prof 

Blamire’s team will be looking to see if 

a new type of scanner could be set up to 

work in a clinic. 

Says Prof Blamire: “The sense from 

clinicians is that it would be great to 

have it as a device in their clinic. It 

could be a game changer for the way 

we work – they say there’s been no 

development in electrophysiological 

techniques in the last 50 years.” 

If a scanner can be built, it could have 

use for any condition involving the loss 

of motor nerves, or even the decline of 

muscle function in ageing. 

Dr Kate Adcock, Director of Research 

and Innovation at Muscular Dystrophy 

UK, said: 

“We are in such an exciting time for 

research into SMA. But to really get 

the most from emerging treatments, we 

need to understand how the condition 

progresses and how effectively we can 

manage it. This project is so exciting 

because it makes use of existing technology 

to do just that; it could mean the 

end of painful existing techniques and a 

new era in effective scanning. We look 

forward to seeing the results.” 

Article online at: https://www.musculardystrophyuk.org/news/news/painfree-and-more-effective-improvinghow-we-monitor-sma/ 

Thank you, ITV Networks for raising awareness about muscular dystrophy. 

We are most grateful for your support. 

32

Take a deep breath and cough … yes, you can! 

“Breathing is the greatest pleasure in life” – Giovanni Papini 

By Brenda Morrow & Anri Human 

Taking a deep breath, sighing, yawning and coughing are 

things healthy people don’t usually have to think about. 

However, for people with neuromuscular diseases (NMDs) 

it may become more and more difficult to breathe as the disease 

progresses. They may also get frequent chest infections, 

which could be severe, needing antibiotic therapy and sometimes 

even hospital admission and ventilator support. 

These breathing difficulties result from weakness of the 

breathing muscles and a poor cough, as well as complications 

like chest wall deformities which restrict breathing. 

People with a weak, ineffective cough cannot properly clear 

secretions from the lungs, which puts them at risk of more 

recurrent infections and may eventually lead to chronic, permanent 

lung disease. 

The good news for people with NMD and breathing muscle 

weakness is that a number of techniques can be used to help 

them take a deep breath and clear secretions from the lungs. 

These techniques may be performed at home by oneself or by 

a physiotherapist and should be started as soon as the cough 

becomes weaker. 

A peak flow meter with a mask attached can be used to measure 

the strength of the cough (Figure 1). The person is asked 

to take a deep breath (as deep as possible!) and then cough 

as hard as possible into the mask. The resulting air flow (the 

peak cough flow) is then measured. If the peak cough flow 

is under 160l/min it means the cough is not strong enough to 

clear secretions from the lungs, and techniques to help cough 

effectiveness should be done daily. When the peak cough 

flow falls under 270l/min, it is time to start learning techniques 

to improve cough effectiveness, because we know 

that cough flow drops even further when you have infections 

of the upper airways (like a cold). 

Figure 1: Peak flow meter with mask attached for measurement 

of peak cough flow (strength of cough). 

There are different types of airway clearance techniques. 

Some aim to move secretions from deep in your lungs to 

the larger, central airways so they can be cleared easily from 

there. Examples are percussions (when the physiotherapist 

or caregiver claps the person’s chest with cupped hands); vibrations 

(manually or using mechanical devices); and positioning 

to drain secretions. These might be effective in moving 

secretions, but without an effective cough none of these 

techniques will lead to secretion clearance. Some breathing 

exercise have also been developed to help clear secretions 

(like deep breathing exercises, active cycle of breathing 

technique, positive expiratory pressure (PEP) therapy, etc), 

but these are not likely to work where there is severe breathing 

muscle weakness, as people with NMD cannot take a 

deep breath without assistance. These breathing exercises/ 

techniques can however be adapted to be done with ventilation 

support (e.g. BiPAP), and your physiotherapist should 

be able to advise you about this. 

Other airway clearance techniques aim to clear secretions 

from the large, central airways, and these are essential in 

people with NMD and weak cough, usually after mobilising 

secretions into the main airways. These are sometimes called 

“cough augmentation” or “cough assistance” techniques. 

There are different parts to a cough, and all are needed for the 

cough to be effective. Firstly, you must be able to take a very 

deep breath in, then close the vocal cords (glottis) at the back 

of the throat to increase pressure inside the lungs, and then 

rapidly force the breath out with an explosive opening of the 

vocal cords with a cough. Depending on the type of NMD, 

any or all of these components of the cough might be missing 

and there are different techniques that can help. 

1. Helping the person to take a deep breath (inspiratory support) 

can be used to improve their ability to cough and expand 

the lungs, similar to when healthy people yawn or 

sigh. Techniques include the following: 

a. Manual insufflation – Air is actively pushed into the lungs 

using a self-inflating bag with either a mask or a mouthpiece; 

or insufflation can be done by adjusting the BiPAP 

or ventilator settings. The bag should be the right size so 

you don’t push too much air into the lungs, which can 

cause damage. Your physiotherapist or doctor should be 

able to advise on what size bag you should use. These bag/ 

mask kits are readily available and fairly inexpensive in 

33

South Africa. 

b. Breath stacking 

i Spontaneous breath stacking: The person takes a breath in 

and traps the air (holds their breath), and then takes another 

breath, traps the air and so it continues until the lungs are 

“full”, after which a cough can be performed independently 

or with assistance. This technique is useful because it can 

be done independently, without the need for a second person. 

An example of this technique (without a cough) is given 

at https://www.youtube.com/watch?v=JlgeRoI5vCw. 

ii Manual breath stacking: Breath stacking can also be done 

using a self-inflating bag and mask/mouthpiece. The 

mouthpiece is placed in the mouth or the mask is placed 

over the mouth and nose. Multiple breaths are applied with 

the bag and “stacked” onto each other with glottic closure 

between each breath or (if glottic closure is a problem), a 

one-way valve can prevent air flowing out back into the 

bag. Once maximum expansion is reached, the person can 

either cough themselves or have a supported cough (with 

the assistance of a parent/caregiver). An example of this 

technique used by a gentleman with ALS is available at 

https://www.youtube.com/watch?v=I3YGh6bVsQM. 

c. Glossopharyngeal or “frog” breathing: Air is forced or 

gulped into the lungs using the mouth and tongue muscles. 

This technique has the advantage of being able to 

be performed independently. A video of glossopharyngeal 

breathing is available at: https://www.youtube.com/ 

watch?v=9OswSaTG71I. 

2. Helping to breathe out (improving cough flow): 

a. Manually assisted cough (MAC): Cough flow is accelerated 

by applying an external pressure on the person’s abdomen 

and/or chest wall (Figure 2) during the expiratory 

phase of the cough. A second person is needed to perform 

a MAC. 

pressure to actively and quickly suck air out of the lungs, 

thereby simulating a cough. There have been reports that 

MIE is effective and safe, but care must be taken to start 

with low pressures so as not to damage the lungs, especially 

for young children. A demonstration is given at https:// 

www.youtube.com/watch?v=M--uuDpGxMM. 

All these cough clearance techniques have some precautions 

or contraindications, and some are more widely available in 

SA than others, so it is important that an experienced physiotherapist 

first assess every person and together decide which 

technique would be the most appropriate. Some techniques 

can be done independently and others need a second person, 

and this must also be taken into account when making a decision 

about which technique to use, according to individual 

circumstances. 

What is happening in South Africa? 

(Local is lekker!) 

Cough augmentation 

A recent survey revealed that most South African physiotherapists 

prefer using MAC for cough assistance in people 

with NMD. They still prefer traditional airway clearance 

techniques like percussion and vibrations, and very few use 

breath stacking, glossopharyngeal breathing or MIE. We are 

currently working to improve physiotherapists’ knowledge 

and skills in airway clearance for people with NMD through 

workshops, educational newsletters and publications. 

Inspiratory muscle training (IMT) 

Previously (December 2017) we reported that there was an 

on-going study in Gauteng and Cape Town to determine the 

effect of IMT on lung function, cough effectiveness, lower 

limb and upper limb function/coordination as well as quality 

of life. In total 23 children between the ages of 8 and 18 

years participated, and this study was completed at the end 

of October 2018. The preliminary analysis shows that the 

participants significantly improved their inspiratory muscle 

strength and their ability to cough! 

Watch this space for news of the final results of this study! 

For further information or any queries, please contact us at 

anrihuman@gmail.com (Gauteng), 012 521-4047, or brenda. 

morrow@uct.ac.za (Cape Town). 

Figure 2: Manually assisted cough demonstration on a 

dummy. 

3. Helping with both inspiration and expiration (breathing in 

and out) 

a. Combining one of the inspiratory support techniques with 

an MAC is very effective; an example is presented at 

https://www.youtube.com/watch?v=B_zJbMRuZT4. 

b. Mechanical insufflation/exsufflation (MIE) or “CoughAssist”: 

This is a fairly expensive machine that pushes air 

into the lungs (through a mouthpiece/mask/tracheostomy 

tube) and then rapidly switches to a negative (vacuum) 

Selected resources and recommended reading: 

Chatwin, M., Toussaint, M., Goncalvez, M.R. et al. (2018). 

Airway clearance techniques in neuromuscular disorders: A 

state of the art review. Respiratory Medicine, 136: 98–110. 

https://doi.org/10.1016/j.rmed.2018.01.012 

Morrow, B.M., Zampoli, M., Van Aswegen, H. & Argent, 

A.C. (2013). Mechanical insufflation–exsufflation in people 

with neuromuscular disease. The Cochrane Library, Dec 30, 

12: CD010044. 

Toussaint, M., Chatwin, M., Gonzalez, J. et al. (2018). 228th 

ENMC international workshop: Airway clearance techniques 

in neuromuscular disorders, 3–5 March, 2017, Naarden, the 

Netherlands. Neuromuscular Disorders, 28: 289–298. https:// 

doi.org/10.1016/j.nmd.2017.10.008 

34

Our Voices – Our Life 

Experiencing Myotonic Dystrophy around the World 

Hello 

Like you, I live with Myotonic Dystrophy (DM1). I am also the facilitator 

of the largest support group for individuals living with the DM in 

Switzerland and an active member of several DM Community 

Organizations throughout Europe. 

As a support group facilitator, I have heard many very personal stories. I have heard people 

speak from their hearts about their experiences with this disease and their happiness to 

finally meet someone to whom they do not need to explain what our frequent tiredness feels 

like. I also feel the need and, often, the joy of people living with DM as they share what they 

have learned on their journey with Myotonic Dystrophy. 

Each one of these stories is important. Each one of them can help others to find 

comfort, understanding, motivation, and advice. 

As part of my life’s work, I am trying to connect the international DM community. Yet, in our 

multilingual world, this exchange is often difficult because of language barriers. Together 

today, you and I can break through the language barrier and help build a truly worldwide DM 

community. 

Here is how. With the assistance from contributors from the international DM-Community, I 

have designed a website at 

www.dm-voices.com 

On this website, we invite you to share your personal story of living with Myotonic Dystrophy. 

Your story will be translated into more than ten languages and DM-affected individuals 

around the globe can find new inspiration in your experience. 

From the submitted stories, an illustrated book will be developed, copies of which we will 

provide in three languages to interested DM Community Organizations around the world. The 

distribution of this book via the information channels of these organizations will be 

coordinated in time with a Project Celebration in Basel, Switzerland. This Project 

Celebration will involve all DM Community Organizations and all DM-affected individuals 

participating in this project. 

Share your story with us! Share it with the whole world and help us to send a wave of 

awareness, support, and community to those living with Myotonic Dystrophy. 

Best wishes, 

Erich 

Erich Maurer & Andreas Herzog, kontakt@myotonie.ch 

in collaboration with 

sponsored by

THE THREE PHASES OF ME 

By Hilton Purvis 

We often chat with friends about how, over time, one's views 

on matters change. Things which used to upset us in our 

youth just wash off one’s back today, or vice versa! It got 

me thinking about matters which used to "push my buttons" 

in years gone by, and those which do so today, and how my 

attitudes have evolved over time. I found that I could see 

my life in three distinct phases, the "freedom fighting", the 

statistics, and the acceptance. 

I use the term freedom fighter carefully, because although we 

were never actively militant, we were fighting for freedom 

in our society in the 1970's and 1980's. Back then there were 

no disabled parking bays, no PAUs at airports, no ramps, no 

disabled toilets. In short, nothing which would have warranted 

sticking the little blue international wheelie man symbol 

onto. The mindset of the time was very much that we should 

be staying at home or living in institutions, being looked after. 

My middle years, the 1990's, became the "statistical" years. 

The years of adding up the new facilities which were slowly 

becoming available. Adding up how many new disabled 

people I saw out and about in town, attending a sports game, 

or working in a company. These were the times of the access 

committees, the independent living centres, the amakrokokroko, 

and the beginnings of employment equity. Doors were 

opening, albeit on the periphery, and increasingly disabled 

people were visibly living independent lives. 

Of late, I have to admit to entering a phase of "acceptance", 

but I am not a person who naturally accepts things. I believe 

we are well short of where we should be as a disabled community. 

My acceptance is therefore that we have not achieved 

the level of integration into society which we should have. It 

also means that where I should be entering a phase of calm 

and tranquillity, the whole rocking chair on the stoep thing, 

I am still having my buttons pushed. It also takes something 

away from the previous two phases. I'm left wondering 

where we went wrong in the freedom fighting, and just what 

we were really doing during the statistical period. 

I think one of the mistakes we made in the 1990s was in 

adopting the belief that if we could show society that 

disabled people could live independently it would gain 

momentum and build steadily and progressively on the initial 

breakthroughs that were being made. We also believed, falsely, 

that civil society would do the right thing, see the light, 

and increasingly break down the physical barriers which we 

face every day (transport, parking, access, etc). Along with 

that we thought that there would be greater inclusion for s 

disabled individuals in schooling and education, employment 

opportunity, etc but this has also fallen far short. We 

never really made any inroads into the architectural industry, 

which is so important to our infrastructural independence. 

Coupled with this is a representational shortcoming in the 

development and construction industries and, saddest of all, 

in the field of education (schooling, colleges and universities). 

Perhaps during the "statistical" period of the 1990s we also 

became so preoccupied with notching up little victories that 

we lost sight of the greater battle plan. We also allowed ourselves 

to become fragmented, with no coordination and with 

the individual disability groupings looking after their own 

interests. Whilst there is nothing intrinsically wrong with this 

concept it does open the door for a loss of momentum and 

focus, which is what has happened. 

However, I do believe that during this "statistical" period 

we fell right into the statistical trap and allowed ourselves to 

accept a quota system. We are now expected to accept that 

only 10% of hotel rooms might have an accessible bathroom, 

or 5% of employees might have a disability. This has come 

back to hurt us. If we are to make any further development, 

quotas such as these need to be eradicated as soon as possible. 

Everything should be accessible. Every single school 

should be able to accept disabled learners. Every job should 

be available to disabled individuals. 

The hope that people would join us in our quest for freedom 

voluntarily has not materialised. In the 1990s I firmly believed 

that we needed willing participants rather than ones 

who had been coerced into meeting our needs. I was mistaken. 

These days, in my acceptance phase, I believe that legislation 

is the only way we are likely to make any progress. 

Unfortunately, in that regard we do not seem to have made 

any real headway either, and existing legislation is really just 

words on paper, without any real teeth. I have yet to hear 

of a building construction being stopped because it did not 

provide disabled access. 

It seems that society has been distracted from the initial 

goals. From an enlightened and promising start in the 1970s 

and some strong development in the 1990s, our quest has lost 

momentum with the result that the disabled community has 

fallen behind in terms of gaining their educational, employment 

and financial freedom. At this stage in my life I have to 

accept that we have fallen short of our aspirations; the real 

problem, however, is that acceptance doesn't sit well with 

me. 

36

Prof Amanda Krause, MBBCh, PhD MB BCh, 

Medical Geneticist/Associate. Professor. 

Head: Division of Human Genetics. 

National Health Laboratory Service (NHLS) 

& The University of the Witwatersrand. 

Please e-mail your questions about genetic counselling to national@mdsa.org.za. 

What are muscular dystrophies? 

Muscular dystrophies are a large group of muscle diseases that cause progressive loss of muscle strength and 

also loss of muscle bulk. The different conditions vary widely in their age of onset, from infancy to adult life. They vary in their rate of progression, their 

severity, which muscles are primarily involved and their genetic inheritance patterns. Both males and females can be affected, although some muscular 

dystrophies predominantly affect males. Although muscular dystrophies affect family members in a broadly similar way, there can still be significant 

differences between individuals. It is thus important to try to reach an exact diagnosis as it influences prognosis, management and genetic risk. 

How does muscular dystrophy present? 

People with a muscular dystrophy often report progressive weakness and difficulty with movements or tasks they were previously able to perform. 

Fatigue is a common feature. Initially the weakness may appear more severe on one side of the body, although typically both sides are involved. The 

weakness may begin in different ways in different individuals. Individuals may report muscle pain as a presenting or accompanying symptom. As some 

muscular dystrophies involve the heart muscle, a diagnosis of a cardiomyopathy may be the first indication of a muscular dystrophy. 

How are muscular dystrophies diagnosed? 

Diagnosis relies primarily on clinical assessment by an experienced doctor, typically a neurologist. The diagnosis may require additional blood tests, 

muscle investigations – including electromyography (EMG), or muscle biopsy to narrow down the exact diagnosis. Genetic testing has advanced in 

recent years and a specific genetic test or a broad test looking for a genetic fault in a few 100 genes simultaneously may assist in identifying the exact 

causative genetic fault and thus the precise diagnosis. 

Are all neuro-muscular disorders genetic? 

Not all neuro muscular disorders are genetic. It is important to distinguish non-genetic causes from genetic ones, as the management and treatment 

may differ dramatically. Any neuro-muscular disease should be fully investigated by a neurologist to ensure that the individual receives the most 

appropriate management. 

Can muscular dystrophy be prevented? 

If a muscular dystrophy is known to occur in a family, there are genetic tests available which can predict with high accuracy which other family 

members are likely to be affected. This is important as individuals at risk can be identified and managed from as early an age as possible. Although 

early management does not prevent disease, it may improve the period of time during which the individual is able to maintain good function. In 

addition, if parents know that they are at risk of having a child with a muscular dystrophy, either because of their family history or because they have 

had a previous affected child, it is important that they discuss their options with a genetic counsellor. Prenatal testing can identify an affected fetus as 

early as 12 weeks of pregnancy. Preimplantation genetic diagnosis is an option whereby parents can test embryos after in vitro fertilisation and only 

implant unaffected embryos in the uterus in order to achieve a healthy pregnancy. 

Doctor’s 

What treatment options are currently available? 

No single treatment is available for all muscular dystrophies. No cures are currently available either and people with muscular dystrophy should be 

aware of unrealistic claims made by companies or individuals. Broad principles do apply in management. It is important that people with muscular 

dystrophies try to maintain their strength and mobility as much as possible. It is helpful to work with a biokineticist who can advise on appropriate 

exercises. Aggressive exercise is discouraged but aerobic gentle exercise is encouraged within the individual’s capability. Increasingly specific treatments 

are becoming available which allow for extended periods of improved functionality. These treatments are specific to the genetic condition and 

often to the exact genetic fault or type of genetic fault. 

What can I do to help a friend or family member diagnosed with a muscular dystrophy? 

People with muscular dystrophies need to be supported through their disorder. It can be very frustrating for someone to lose previous abilities. Friends 

and family should try to ensure that they are referred to the appropriate support services to ensure that they maintain optimal function and get the best 

possible management. Referrals include medical professionals, allied health professionals, including psychologists and genetic counsellors. Support 

groups such as the Muscular Dystrophy Association can assist in directing individuals to appropriate services. They also provide contact with similarly 

affected individuals. 

37

Sandra’s thoughts on… 

Time – the best gift you can give someone 

By Sandra Bredell (MSW) 

“The greatest gift you can give someone is your time. 

Because when you you’re your time, you are giving a 

portion of your life that you will never get back” (author 

unknown). 

According to Frank Weil (2013), all of us have our own 

sense and understanding of “time”. It may reflect a very 

good experience we had or the frustration experienced 

when waiting on an answer or outcome of a particular 

situation. Although we can all associate with these kinds 

of experience, no one really knows where the concept 

actually comes from. We understand that there is a yesterday, 

today and tomorrow and that if time is lost you 

can never get it back and therefore time needs to be 

spent wisely. This can be seen as one of the principles 

of life. But as Mr Miyagi stated in the movie, Karate Kid, 

“I think you know the principles. Everyone does. But 

99% of all people don’t do what they know” (Foroux, 

2016). 

We also know that time is very important to human beings 

and plays a significant role in our lives. We need 

to really value and respect time, for as the saying goes, 

“time and tide waits for none”. Mother Teresa agreed 

by saying, “Yesterday is gone. Tomorrow has not yet 

come. We only have today. Let us begin” (Bhalla, 2017). 

Volunteering your time to an organisation that renders 

a service to the community, to a neighbour, friend, colleague 

or family member indicates that these people 

are important to you and therefore you want to give 

them the gift of time. This gift is a part of your life which 

you are giving away. We should give more than we 

take. Life is not about taking but all about what we are 

giving. “We make a living by what we get; we make a 

life by what we give” – Winston Churchill (Santi, 2017). 

You will be surprised by the fact that the more you give 

to others, the more you will receive. And here I am referring 

to time. We should rather think about what we have 

to offer than what we want from the world. “The effect 

you have on others is the most valuable currency there 

is” – Jim Carrey (Foroux, 2016). 

But when you give your time to others, you benefit a 

great deal yourself. Research has proved that helping 

others is a fundamental part of humanity. People are 

helping others at soup kitchens, welfare organisations, 

children’s homes; and then there are others who assist 

the community in case of a fire, natural disasters 

and crime. According to a study done by Professor 

Michael Norton (Harvard Business School), giving of 

your time to others, makes you feel good; and a professor 

of psychology at the University of California, Sonja 

Lyubomirsky, saw similar results when she asked people 

to perform five acts of kindness each week for six 

weeks (Marsh & Suttie, 2010). To see a smile and eyes 

filled with joy definitely makes it all worth it. 

So next time you are wondering about what to give to a 

friend or family member, consider the gift of your time. 

Offering your time to do something for them or to spend 

time with them is the best gift you can give. 

References 

Bhalla, S. 2017. “Importance of time…”. My Edu Corner. 

http://www.myeducorner.com/importance-time-essayspeech-article/. 

Foroux, D. 2016. “7 principles of life that everyone 

knows, but only a few follow”. Updated 2017. Huffpost. 

https://www.huffpost.com/entry/7-life-principles-everyone-knows-only-few-follow_b_11318702. 

Marsh, J. & Suttie, J. 2010. “5 ways giving is good for 

you”. Greater Good Magazine. https://greatergood. 

berkeley.edu/article/item/5_ways_giving_is_good_for_ 

you. 

Santi, J. 2017. “The secret to happiness is helping others”. 

Time. http://time.com/4070299/secret-to-happiness/. 

Weil, F.A. 2013. “The meaning of time”. Huffpost. Updated 

2014. https://www.huffingtonpost.com/frank-aweil/the-meaning-of-time_b_4351464.html.

Family picnic at the Urban Park 

Cape Branch 

On 1st September we hosted our 

annual family picnic for affected 

members and their loved ones. 

We spiced things up this year by 

hosting an outdoor treasure hunt. 

What fun it was for both children 

and adults to explore the park to 

hunt down clues and fun goodies. 

Thereafter, we all celebrated 

awareness month together and 

shared a lovely picnic. Many 

thanks to everyone who came out 

to spend this special day with us, 

and to Mrs Gilda van der Merwe 

for planning and facilitating the 

event. 

IN MEMORIAM 

It is with great sadness that we bade farewell to Mr Basil Lerer on 10 July 2018. Our sincere 

condolences to his family. You are in our thoughts. It’s always too soon to say goodbye. 

It is with very heavy hearts that we said goodbye to Vuyolwethu Stuurman on 12 June 2018. Our 

sincere condolences to his family. We loved to see his gentle smile at our visits to Tembaletu School. 

Our deepest condolences to the Grobbelaar family on the passing of their daughter, Chante, on 26 July 

2018. Your family is in our thoughts. 

Condolences to family and friends. Ed. 

39

Cape Branch 

Grand West children’s outing 

On Wednesday 5 September 2018 our MDF children 

from Astra School enjoyed a fun day at our annual Grand 

West outing. We kicked off the day with a lovely brunch at 

Wimpy. Afterwards, the group got to go bowling and play 

arcade games at the Magic Company. What a joy it was to 

watch the faces of our MD children enjoying the thrill and 

excitement of all the activities on the day. This was once 

again a wonderful fun-filled day to remember, and each 

child returned home with a lovely goodie bag. 

Special thanks to Reach for a Dream for funding the lunch 

and for joining us on this special day, and to Mandy and 

Lawrence Green for funding the tokens. Also, thank you 

to Astra School for arranging for the children to be safely 

transported to the venue with staff to assist the children 

on the day. 

40

Cape Branch 

Awareness events 

The Cape Branch’s social service team conducted six different awareness 

campaigns during the last quarter. Many thanks to the schools, community 

centres, NPOs and clinics that hosted us. We greatly appreciate the 

opportunities to share information about muscular 

dystrophy within our surrounding communities. 

Pictures from APD Tygerberg 

Awareness Event and van Riebeeck 

Strand Primary School. 

Annual Golf Day 

A sincere thanks to the Goodwood Rotarians and Judy Bird for 

assisting in making a huge success of this event, held on 

12 October. A word of special thanks to Sanjay Narshi and 

Anne-Marie Stoman for their great support at the event. 

The National Lottery Commission 

We are very grateful for the funding received from the National 

Lottery Commission. We were able to purchase three motorised 

wheelchairs, two Bi-Pap machines and several cushions and 

mattresses, and to refurbish another B-Pap machine . 

A picture of 2 of the recipients of the wheelchairs purchased. 

41

Gauteng Branch 

Muscular Dystrophy Campaign 

SMS and stand a chance to win 1 of 3 Voyageur Schwinn bicycles 

The Blue Bottle Group has a team of cyclists that cycled with our logos 

on their jerseys at the Telkom 947 Cycle Challenge on 18 November 

2018. Additionally, the Blue Bottle Group bought three bicycles and is 

running an SMS campaign whereby, at a cost of R30 per SMS, people 

can stand a chance to win one of the bicycles. The campaign will run until the end of December 2018. 

Please support people affected with muscle-wasting conditions by SMSing your name and email address to 

42351 and stand a chance to win 1 of 3 Voyageur Schwinn bicycles valued at R6 500 each. 

Muscular Dystrophy Foundation Gauteng wishes to thank the Blue Bottle Group and everyone who supports 

our cause! 

New wheelchair for sale 

Please contact Lindsay Walsh on cellphone number 0834692877 

if you would like to buy this brand new wheelchair for R60 000. 

42


Donation of a vehicle 

We would like to thank the Talia family for donating 

a Chrysler Voyager with motorised lift to us. The 

vehicle is a great help, especially in transporting the 

General Manager on a daily basis. 

Nazier and Fatima Talia have been very good to 

us over many years and we wish to thank them 

sincerely for the great donation. We are most grateful 

for their support and willingness to help when needed. 

Pictured: Robert Scott, Fatima Talia and 

Pieter Joubert 

Ezibeleni School outing 

On 6 September, MD learners from Ezibeleni School 

went for an outing to East Rand Mall. They were 

treated to lunch at Mugg & Bean, where they had 

a choice of folded fillers, toasted sandwiches and 

something to drink. They enjoyed the outing very 

much. 

Ithembelihle School outing 

On 26 September, MD learners from Ithembelihle School were taken on an outing to East Rand Mall, where they 

were treated to lunch at Panarotti’s. They had monster pizzas and something to drink for 21 people. 

43


Special delivery at 

Pretoria School 

Learners living with muscular dystrophy were 

treated with gifts on Wednesday 31 October 

2018. 

The learners are all part of the muscular 

dystrophy group which is run by the school 

physiotherapist and the school Social worker 

at Pretoria School. I, the muscular dystrophy 

social worker does group sessions with them 

once a month. The learners were very happy 

to receive gifts from the Foundation. 

Lionel Smook surprised with 

CE Power Chair 

We could not find parts for my old wheelchair, and 

our social worker, Mulanga Kharidzha, told me that 

there was a waiting list but they would try their best 

to help as soon as funds became available. 

On 31 October, staff from the office surprised me and 

handed over the wheelchair. I would like to thank the 

Foundation and Muscle Riders for assisting me with 

a power wheelchair. 

Thank you for helping all our members with 

specialised equipment. 

(Pictured: Mulanga Kharidzha, Pieter Joubert, Lionel and Rina Smook) 

Photography/Videography 

• Annual reports • Golf days 

• Awards 

• Entertainment 

• Conferences • Team building 

• Product launches 

info@strikeaposephotography.co.za www.strikeaposephotography.co.za 

Call: Dee-Ann 082 412 9650 

44

Our son Matthew Wilson 

By Cindy Frantzeskos 

We were very privileged to have had a son like a Matthew. I want 

to tell you about the remarkable young man he was. 

Matthew was born with Duchenne muscular dystrophy, but he 

never used his disability as an excuse for anything. He never 

even allowed me to park in the disabled parking – gotta love that 

one.Everyone who knew Matthew had some special moments 

with him. For me there were many along the way. There is a song 

that Matthew and I used to sing in the car on the way home when 

he was a toddler. “You are my sunshine, my only sunshine. You 

make me happy when skies are grey … 

Matthew was and always will be my sunshine. He was one of 

those rare human beings whom everyone loved. His smile could 

light up a room and he had beautiful sparkly eyes. He had a dry 

sense of humour, and he loved to joke with his group of close 

friends and of course his family. He nicknamed my brother “Victor 

DJ skip skip” – for obvious reasons. 

I must say he was also fortunate to have cousins whom he was close to and who spent time with him. He was also adventurous 

and embraced life despite his illness. He could put able-bodied people to shame. If I look back over the years, to 

when he was still able to walk, he would run races and be the last one running but with a huge smile on his face, waving 

at us. At the age of 11 years he went into a wheelchair, still living life as only he knew how. 

Matthew once won a four-wheeled motorbike in a competition (I was not even aware that he had entered – his Gran had 

posted the letter). We spent many weekends at the race track. He would be lifted on to the motorbike, and although he fell 

off many times and broke some bones, he was right back on again the next week. He then sold his motorbike and bought 

a boat! This was while holidaying in Plettenberg Bay, where he made many friends. 

One day Matthew went paintball shooting, and when he came back he said it was one of his best days ever – never mind 

all the bruises! 

He also went white water rafting in Parys with Kyle, one of his closest friends, and the organisers were super impressed 

at his determination. They would sometimes camp in our back yard in a tent, but this was 5-star all the way – with TV, 

PlayStation, everything in that tent. 

Matthew loved to play 30 Seconds and of course his PlayStation. He also loved music, all types of genre – from Robbie 

Williams and Queen to Eminem and Ed Sheeran – and he would sing along. 

Matthew loved to watch movies and he always chose ones that had a life lesson – except for Harold and Kumar. He loved 

Superman and obviously Wonder Woman, but Batman not so much (a rich man with gadgets). 

Matthew also loved the Manchester United soccer team. Where this comes from I have no idea. He was fortunate to go to 

Old Trafford and watch them play. When they came to South Africa he met the entire team, shaking hands with everyone, 

from Sir Alex Ferguson to Wayne Rooney and John O Shea. In fact he made us wait from 9 in the morning until 10 that 

night at the Palazzo Hotel to get all their signatures. 

He also loved fast cars and was given opportunities to drive in many – Maserati, Lambhorghini, Ferrari, McLaren, Audi R8. 

He also raced around Kyalami and met Kerry McGregor and Baby Jake, among others. 

He loved going out and would “organise” tickets to Prime Circle and Black Eyed Peas. I remember a long road trip to Sun 

City with a bus full of family and friends. His last outing would have been to Comic Con – he had booked those tickets 

months in advance, but life had other plans for him when, on 15 September, he left us to go on his next journey …. 

In his last few days Matthew decided to stay home in bed. - I don’t know how he found the courage to say to the doctor, “I 

don’t want to be on anything mechanical”. It broke our hearts, but as he said, he had had a good life. 

Your wings were ready but our Hearts were not. You are our hero and we will never ever forget you, Matthew! 

It is always too soon to say goodbye! Our sincere condolences. Ed. 

45

KZN Branch 

A huge thank you 

Muscular Dystrophy Foundation KZN Branch are most grateful 

for the support and wish to express our sincere gratitude to all 

our donors for their sponsorship and donations, as well as to 

all our members who have been a tremendous support to KZN 

Branch. 

Your generosity is greatly appreciated by the executive committee, 

staff and volunteers of the MDF KZN Branch. 

Casual Day 2018 

The Muscular Dystrophy Foundation KZN Branch sold 2 793 stickers 

and would like to sincerely thank the following volunteers, schools and 

companies that assisted us in our Casual Day sticker sales: 

Mercedes-Benz, Riverhorse Valley; Reutech Communication management 

and staff; Cathy Khoon Khoon and family; Nedbank Kingsmead 

Phase 2; Mrs Bhanjee and family; SA Homeloans, La Lucia; Effingham 

Primary School staff and pupils; Effingham Secondary School staff and 

pupils; U & G Fabrics, Overport, Durban; Parmalat; St Raphaels School 

staff and pupils, Montclair; Mason Lincoln Special School staff and pupils; 

Mobility Solutions; Briardene, Durban North; New Frontier Tours, 

Westville; Kandice Govender and family; Netcare, Kingsway; Weziwe 

Magoso; 45th Engen, Sherwood; Namitha Chabilal from Inkosi Albert 

Luthuli Central Hospital and her team; Neil and Duane Goldstone. 

Thank you once again for your support and assistance in the sale of stickers. We look forward to your assistance, 

support and contribution again next year! 

Annalie Harris is selling this Motorised Shopping Scooter with trailer, battery charger and user manual. Price is R25 000, however 

Annalie is open for negotiation. For more information, contact her at 084 370 5477. 

FOR SALE 

46

MDF Magazine Newsletter Issue 57 December 2018

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