Fascio-scapulo-humeral muscular

dystrophy: A cross section of in-depth

patient interviews on their

perceptions of their healthcare and

living with the condition.

Marina Di Marco

Principal Neuromuscular Physiotherapist

Lead Clinician of the Scottish Muscle Network

January 2016

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Acknowledgement I would like to thank Bernard McDermott from Medical Illustration at the Glasgow

Royal Infirmary for his patience, willingness to travel and his help and support in

recording these interviews. Having a visual and audio record has been extremely

beneficial whilst transcribing these interviews and I am grateful for his insight.

Thank you to Karyn Robertson, previous manager of the Scottish Muscle Network for

her ideas and support in capturing the feedback from our service users. This

gratitude extends to NSD for continuing to support the network, enabling the

members of the steering group to provide a forum for ongoing patient engagement,

which informs the service we provide in a positive manner.

Thank you to Laura Craig, Network Co-ordinator for her help with proof reading,

editing and formatting this report and for her patience during the deadline slippage.

Thank you more recently to Carsten Mandt, our new Network Manager for his

helpful comments and support.

Lastly and most importantly, I would like to thank all those who agreed to participate

in these interviews as without you this report would not have been possible. Thank

you for giving your time so willingly especially those who travelled to the Queen

Elizabeth University Hospital to speak with me. To those who preferred to be

interviewed more locally, thank you for allowing me in to your home.

It has been a great privilege for me to listen to your story and I thank you for sharing

your unique experiences with me. I am aware that entrusting your thoughts and

feelings to someone, who for some of you was a complete stranger, can be daunting

but you did so in the knowledge it will help inform the way in which we work in

partnership with you in our shared healthcare experience and I hope this report

reflects that.

In a world where we are constantly bombarded with questionnaires and calls for

public feedback on all services from energy suppliers to supermarkets, I would like to

ensure the time taken to participate in feedback will benefit the current service

users as well as those service users in the generations to come. The willingness of

our participants to become involved in these interviews was very much appreciated

by the Scottish Muscle Network.

In the NHS, it is well known that it is our patients who are our teachers. To all of you

who participated in these interviews I would like to thank you for teaching me so

well.

Kindest regards

Marina

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Contents

Acknowledgements Page 2

Executive Summary Page 4

Introduction Page 6

The Scottish Muscle Network Page 6

The Healthcare Experience Page 7

The Patient Interview Page 8

Patient One Page 9

Patient Two Page 11

Patient Three Page 16

Patient Four Page 24

Patient Five Page 30

Discussion Page 39

Recommendations and Interview Conclusions Page 46

Conclusion Page 48

References Page 49

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Executive Summary

Fascio-scapulo-humeral muscular dystrophy (FSHD) is the third most common

muscular dystrophy after Duchenne Muscular Dystrophy and Myotonic Dystrophy. In

the United Kingdom, we believe it affects at least 1:50,000 people. In Scotland,

patients with FSHD are managed within their territorial Health Board areas. The

clinicians and healthcare professionals who work with patients with a neuromuscular

disorder are supported by the knowledge, expertise and clinical governance of the

Scottish Muscle Network (SMN), a National Managed Clinical Network (NMCN).

NMCN’s are described as virtual entities designed to drive upwards the standards of

patient care through a collaborative approach and service integration. These

managed networks link healthcare professionals and stakeholder organisations from

the primary, secondary and tertiary sectors to enable coordinated working

unconstrained by territorial Health Board boundaries. This model of healthcare

delivery is designed to ensure equitable service provision of high quality, clinically

effective services throughout Scotland. NMCN’s aspire to the multiple dimensions of

health care quality, which not only focuses on the quality of healthcare but also

addresses the healthcare experience perceived by those who access the NHS.

With this in mind, the SMN undertook a series of interviews to collate rich and

detailed information on the healthcare experience of a cross section of patients

residing in different Health Board areas in Scotland.

Themes emerging from the interviews:

• Self-awareness of the condition: Prior to diagnosis, patients knew there was something wrong however, they had often put it down to just the “way I am”

and accepted they were different in terms of strength and physique.

• Healthcare professionals: There was a feeling of frustration that many healthcare professionals did not know about the condition. Patients felt

weary of repeatedly explaining their symptoms and issues. Patients

expressed relief when experiencing a consultation with a healthcare

professional who specialised in neuromuscular disorders and understood

their condition. Specialist teams were felt to be essential in the healthcare

journey.

• Hospital appointments: On the whole, hospital appointments were viewed positively. Patients commented on the information exchange and felt that

the giving as well as receiving of information was a key attribute in a

successful hospital appointment. Whilst there were comments on the fact

that often they felt they had to be the ones who asked about research and

new technologies, there was an understanding that busy hospital clinics can

be a barrier to a full exchange of information on their specific condition.

• Housing: This was a source of concern for all the interviewees and was directly related to how their condition would progress. There was consensus

on the importance of suitable housing in terms of safety and energy

conservation. For those who had accessible housing, patients experienced a

great sense of relief as fears of falling were alleviated and future concerns

over access were dispelled.

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• Carers: There were mixed views on the experience of carers. On the one hand, they afforded the interviewee an improved quality of life, help with

personal care, support and a feeling of maintaining independence but on the

other hand, it could be an intrusive element within the patient’s life.

Compromise was a necessary element when engaging with carers.

• Transport: This was a key factor in maintaining quality of life and provided a direct link with the community. All patients expressed gratitude for their

powered wheelchair, mobility cars, voluntary transport services as well as

free bus passes and accessible public transport.

Recommendations for discussion:

• Could clinicians better engage with patients’ expectations of clinic appointments?

• Working in partnership is a two-way process. How can clinicians better support patients in their understanding of the appointment process?

• Specialist clinics were deemed to be of importance to the patient. Can the SMN better promote the expertise and experience to patients within the

clinic setting?

• For patients who do not attend clinics, is it possible to have a call back and information system that would respect the patients’ right to privacy whilst

ensuring the patient is making an informed choice of the reasons why they

are not attending?

Conclusion

The SMN is fortunate to have access to a variety of methods at their disposal for

capturing patient feedback. Patient feedback can present itself via the complaints

system, engagement with charities, clinical information forums managed by the NHS,

feedback questionnaire forms, one to one clinic appointments as well as in-depth

patient interviews to name but a few. All of these forums provide the SMN with a

landscape of how well the network is performing as well as initiating areas where the

network’s focus can provide beneficial and sustainable improvements in our Scottish

neuromuscular service. It may be argued that the most important measurement of

these improvements is in the perception of the stakeholders.

For further information, queries or comments, please contact Marina Di Marco,

Principal Neuromuscular Physiotherapist, Queen Elizabeth University Hospital, 1345

Govan Road, Glasgow, G51 4TF E-mail: marina.dimarco@nhs.net

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Introduction

Fascio-scapulo-humeral muscular dystrophy (FSHD) is the third most common

muscular dystrophy after Duchenne Muscular Dystrophy and Myotonic Dystrophy. In

the United Kingdom, we believe it affects at least 1:50,000 but this is an estimate

due to the fact collating information on patients is difficult as many patients are only

mildly affected and therefore remain undiagnosed. It is thought there could be up to

3000 cases in the UK alone (www.musculardystrophyuk.org). However, more recent

estimations believe the prevalence to be far greater and in fact in the region of 1 in

15,000 to 1 in 20,000 (Tawil et al, 2015).

FSHD is an autosomal dominant genetically inherited muscle wasting condition and

affects both males and females presenting in childhood, adolescence and adulthood.

30% of cases are sporadic, arising from de novo mutations. It is usually noticed

before the age of twenty although diagnosis may not be sought until the patient’s

symptoms begin to affect activities of daily living. There is a slightly higher incidence

of this condition affecting males than females and males tend to be more severely

affected.

Initial presentation is facial weakness, followed by weakness of the shoulder girdle

muscles (Santos et al, 2015). As the condition progresses, weakness is also noted in

the shoulder, upper arms and lower legs with abdominal muscles also affected.

Cardiac and respiratory muscle is usually preserved although some of the more

severely affected patients do have symptoms of expiratory muscle weakness and

respiratory function can be compromised (Santos et al, 2015) The degree of muscle

weakness varies widely with some patients remaining ambulant whilst others

become wheelchair dependent (www.mda.org/disease/fsh-muscular-muscular-

dystrophy). The rate of progression is also variable and even within families, siblings

can be affected differently in terms of muscle weakness distribution and severity.

Other symptoms can include retinal vascular disease, hearing loss and the possibility

of the patient developing cardiac arrhythmias is also of note (Tawil et al, 2015).

In Scotland, patients with FSHD are managed within local Health Board areas with

additional support through the Scottish Muscle Network.

The Scottish Muscle Network

The Scottish Muscle Network (SMN) is a national managed clinical network (NMCN)

which was established in 1998 in order to bring together patients with a

neuromuscular condition, their families and carers, professionals and charitable and

third sector organisations who have an interest in care for those living with a

neuromuscular condition (www.smn.scot.nhs.uk).

The Scottish Muscle Network is funded by the National Services Division (NSD),

which is a division within NHS National Services Scotland (NSS). NSS commission and

performance manage nationally designated specialist services and screening

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programmes to ensure the provision of high quality, effective specialist health and

screening services to meet the needs of the population of Scotland.

NSD describe NMCN’s as “virtual entities” designed to drive upwards the standards

of patient care through a collaborative approach and service integration. These

managed networks link healthcare professionals and stakeholder organisations from

the primary, secondary and tertiary sectors to enable coordinated working

unconstrained by territorial Health Board boundaries. This model of healthcare

delivery is designed to ensure equitable service provision of high quality clinically

effective services throughout Scotland (www.nsd.scot.nhs.uk).

NMCN’s aspire to the multiple dimensions of health care quality discussed in Better

Health, Better Care (2007) which focuses on services being person-centred, safe,

effective, efficient, equitable and timely (The Healthcare Quality Strategy for

Scotland, 2010). Better Health, Better Care (2007) does not only focus on the quality

of healthcare but also addresses the healthcare experience perceived by those who

access the NHS.

The Healthcare Experience

Improving the patient experience is not a new concept. According to Luxford et al

(2011) exploring the links between healthcare delivery and patients needs and

preferences began in the 1980’s and in 1993 the Picker Institute identified eight

domains that embody the person-centred model:

• Respect for patient preference and values

• Emotional support

• Physical comfort

• Information

• Communication and education

• Continuity and transition

• Coordination of care

• Involvement of family and friends and access to care (Luxford et al, 2011) Luxford et al (2011) report that there is evidence to suggest that focussing on patient

–centred care improves both clinical outcomes and the patient experience and other

studies have also described similar core concepts with respect for patient needs and

preferences being the most consistent. The way patients experience the health

service is an important factor in their perception of the quality of their care (de Silva,

2013)

Jenkinson et al (2002) are also of the opinion that in the continual improvement of

medical services, a balance between clinically effective services and evidence-based

medicine alongside patient’s perceptions that their care has been “acceptable and

beneficial” has to be explored. They believe the most important determinants in a

good healthcare experience are:

• Physical comfort

• Emotional support

• Respect for patient preferences (Jenkinson et al, 2002).

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The Patient Interview

It is well accepted that “enhancing people’s experience of health services may be a

key goal of improvement initiatives” (de Silva, 2013) and there are a variety of ways

in which this can be achieved. Individual measures to evaluate the patient

experience, such as in depth interviews, focus groups, surveys, complaints etc are

each components of a broader landscape which work well together to provide the

NHS with a more complete panoramic view of patient satisfaction. Using a variety of

methods diffuses the burden of providing, collecting and analysing data on both staff

and patients.

Rationale: It is well recognised that understanding patient experience is a unique

learning tool within healthcare. As a NMCN the SMN has a responsibility to seek

patients’ views and perceptions of their healthcare experience in order to highlight

areas that are working well and focus on areas where improvement could benefit

the patient’s healthcare experience.

Aim: This investigation aims to explore the healthcare experience of a small cohort

of patients with FSHD residing in different Health Board areas throughout Scotland.

The patients are from different social backgrounds and represent a cross section of

the FSHD population.

Objectives:

• To undertake a small number of in-depth interviews of patients living with FSHD.

• To analyse the patient interviews in order to extrapolate similar themes of the healthcare and social experience. This will help inform the SMN’s

understanding of the patient journey of this particular patient cohort.

• To document variations in the health experience to facilitate discussion within the SMN

• To make recommendations to facilitate positive health experiences and outcomes across healthcare service providers within the Scottish Muscle

Network.

Methods: The physiotherapy network covers all three Health Board regions; the

North of Scotland, South East of Scotland and West of Scotland. Physiotherapists

were invited to speak with patients in their area to ascertain whether they would be

keen to be interviewed in order to participate in service development within the

SMN. These patients were selected from the physiotherapy database of patients

who had recently undertaken a physiotherapy review.

Ten patients were sent a follow-up letter with the option of being interviewed within

their own home or within the hospital environment. Six patients replied. Two

patients preferred to be interviewed within the hospital environment and three

preferred to be interviewed at home. A suitable time and date could not be reached

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within the interview timescale for the sixth patient. Patients were given the option of

being recorded with written notes, audio recording or audio and visual recording.

Health Board Region Interview at

home

Interview at

clinic

Written

Notes

Audio

Notes

Audio and

visual notes

North of Scotland * *

North of Scotland * *

East of Scotland * *

West of Scotland * *

West of Scotland * *

Interviews were transcribed manually and divided into categories to aid analysis.

The interview process was undertaken by Marina Di Marco, Principal Neuromuscular

Physiotherapist for the West of Scotland. The interviews were carried out using

grounded theory where no set questions were asked. The interviewer explained to

patients that the purpose was to listen to their story of what was important to them

rather than being led by a set of questions perceived to be of importance by the

healthcare practitioner. There was no time limit on the interview to enable rich data

collection i.e. the interviewer can facilitate a more in depth exploration of patient

perceptions when not adhering to a specific script within a specific time frame.

As some patients were known to the interviewer, agreement to participate was via

an opt-in letter. Interviews were chaperoned to ensure the process was impartial

and equitable.

The Patient Interviews

Patient One: John (76 yrs old), Lothian. John continues to live in his family home

with his wife. He is ambulant and enjoys life to the full.

I feel my muscles are having to work very hard to do things I could

easily do a few years ago. I feel as though I am running when others

are strolling. It feels as though there is a weight pushing down on my

shoulders all the time.

When did you start to become aware of your condition? I did not start to become

consciously aware of my condition until later in life. I was aware of certain aspects of

the disability such as having difficulty raising my hands above my head when I was

decorating. I used to use the other hand to help me. I sometimes fell when I was out

and about. It was my wife who noticed I had foot drop.

As time went on I remember I couldn’t lift the jug of water when I was out for a meal

for example.

From an early age however, I was aware that I never saw any improvement in my

physique with exercise. I just couldn’t understand that. No matter how much

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exercise I did, I couldn’t make my muscles any stronger. In honesty, there were

subtle signs throughout my life that I put down to “just the way I am”.

Other people noticed things about me too: As a young man I was in the Forces (aged

20 years) and the medical examiner told me at the time that I had a dropped

shoulder. I had to have my suits made to measure in the national services as off the

peg clothes did not fit. I remember the tailor being amazed at my shoulders. He had

never seen anything like it.

When were you diagnosed? My first visit to the GP was with fatigue. I had joined a

walking club and noticed I had to lift my leg over a stile whilst my peers could do this

quite easily. I started to notice differences like that. By the end of the walk I would

be completely exhausted. I began visiting my GP intermittently with reports of my

ankle or foot not working properly.

When I was in my 50’s, I was referred to orthopaedics when I developed sciatica. The

Doctor noted I had muscular imbalance in my spine but further investigation was

never discussed.

I eventually found myself being referred to Genetics in Edinburgh where I met a

Neurologist who was visiting from Dundee. The Neurologist knew right away I had

FSHD and sent my test to Newcastle for confirmation. The results were positive as

suspected.

How has the condition progressed: In the last couple of years I feel my muscles get

very hot when I am exerting myself. I now feel the walk along hospital corridors for

example is troublesome and more things are becoming challenging in my day to day

life. I feel my muscles are having to work very hard to do things I could easily do a

few years ago. I feel as though I am running when others are strolling. It feels as

though there is a weight pushing down on my shoulders all the time. I now feel I

have stiffness in my neck and shoulders and it is more difficult to keep my neck

comfortable. If I’m going out, I have to choose the cinema I go to now as there is no

handrail in some cinemas and sitting for long periods without good neck support is

very difficult. I do get neck pain and stiffness if sitting too long.

I have also developed a tremor which is the part of the condition that frustrates me

most as it is interfering with my photography hobby and I am currently looking for a

way around this. There’s always a way round things; I won’t be stuck.

Healthcare Appointments: With regards to the healthcare professionals I have met

along my way, I believe everyone I have come into contact with has been well

intentioned. However, I often feel that not enough is being done in terms of finding a

cure. At least that’s my impression…maybe I just don’t know what’s being done.

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Health Appointments that have been particularly beneficial: I have particularly

enjoyed my time in the hydro pool. It’s the social aspect too as well as the exercise in

a nice atmosphere.

I think that during clinic consultations it’s a two–way process. Healthcare

professionals should be prepared not to just ask questions but to give information on

trials, research and more information in general on what’s going on in the world of

neurosciences and Genetics. I know that clinic time can be extremely busy and this

could be a barrier but I think it’s important that we don’t just answer the Doctor’s

questions that we have time to ask our own questions too.

Awareness of the Condition: I do become frustrated at the lack of knowledge when

undertaking day-to-day tasks as those around me have never heard of this condition.

This is one of the challenges with having a rare condition and it is not limited to

members of the public as I often come across healthcare professionals who also

have no knowledge of the condition. This can be really frustrating having to explain

to people.

Quality of Life: I have never let my condition hold me back. I still do all the things

that I want to but sometimes I have to adapt the way I do them. I feel very happy.

I’m a very happy man.

Transport: One of the things that has really added to my quality of life is my free bus

pass. We use it regularly…and not just for appointments. I visit my family and friends

and we sometimes go away for the day too. In fact if you ever get the opportunity to

tell MSP’s how important the bus pass is to people like us, please do tell them.

Patient Two: Margaret (67 yrs) Highland. Lives alone, is ambulant and is very

independent enjoying life to the full.

I feel very strongly that children shouldn’t be told they have

these conditions early on when they have no idea and there’s no

real signs because my mother then was very overprotective, very

anxious and it did affect me. It did make me feel as though

there’s something horrible to look forward to in the future.

When were you diagnosed? I was diagnosed when I was seven because my mother

had muscular dystrophy and so had my grandmother although they had different

experiences of it. My mother was much more disabled than my grandmother. You

wouldn’t actually know my grandmother had it. She was up and about until the day

she died.

My mother wanted to have me tested – or maybe it was the doctor – I don’t really

know who wanted to have me tested but I remember distinctly the consultant saying

to my mother, “Yes, she does have muscular dystrophy”, and what I remember most

vividly was my mother’s reaction. She was very distressed and I didn’t really

understand it but it has left me feeling very strongly that children shouldn’t be told

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they have these conditions early on when they have no idea and there’s no real signs

because my mother then was very overprotective, very anxious and it did affect me.

It did make me feel as though there’s something horrible to look forward to in the

future. This was in 1955 / 56.

Children are very aware of their parent’s reaction and if their parents are distressed

then you’re going to feel, gosh, something awful is going to happen to you. I had no

signs or symptoms at the age of seven. The doctor looked at my back and did some

physical examinations but I was very healthy; running about etc. I was diagnosed

somewhere in Glasgow, I’m not sure where it was.

It is a burden for children to know they have a condition when they are having a

carefree and healthy life.

Although there were no signs and symptoms, at the back of your mind you are

always looking to see if there was something wrong. I mean I was brought up in the

country and although I got a lot of freedom as children in the country did – it’s hard

to describe – but my mum was always over anxious. She worried about me a lot. I

kept very well until I was twelve and then I had encephalitis through rubella so I was

very ill then. I went to Ruchill Hospital and I almost died but I mean I’m here and I

came through it.

Awareness of the condition: I was last in the canteen at mealtimes and I didn’t think

much about it other than I was a slow eater. I think when I went to High School, the

one thing was that people used to take a short cut by jumping over this small wall

and I just knew instinctively that I just couldn’t jump up that wall and also in gym we

were supposed to jump over the box and do things like that but I just couldn’t do it. I

used to just make excuses. I didn’t tell anyone I had muscular dystrophy, I just made

excuses. My teachers didn’t know about it either. Well the gym guy wasn’t that

bothered cos lots of girls my age did the same – we would bunk off and go to the

café instead – we were quite rebellious (laughs). I wouldn’t say it was a big problem

for me.

Stairs were alright, I was a good runner strangely enough.

I got tested again at the age of twenty one and my GP said to me, “Now, you’ve got

it so my advice is to you to forget about it. It’s mild – just live a normal life”, and I

thought that’s just exactly what I’ll do so I did everything that everybody else did.

I did lots of travelling. I did a Greyhound bus tour around the United States and went

to all sorts of places. And then I trained as a social worker and of course I did have to

say, because you know they go into your medical history and so forth, but again the

consultant I spoke to said, “Your prognosis is very good so there’s nothing to stop

you taking on this course and becoming a social worker”, so that was fine.

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Having muscular dystrophy didn’t change my choices in life. As I grew I took the

doctor’s advice – lead a normal life.

Family History: The fact my grandmother wasn’t so disabled was a very good spur for

me because my grandmother was up and about ‘til the day she died of a heart

attack. In fact, she was actually standing up tapping her feet to music when she died.

I did have that as a model of what you can be with muscular dystrophy.

My cousin has got it and she’s almost eighty now but it’s only in the past five or six

years that she’s been very disabled. Before that she was up and about, very mobile

and taking the dog for a walk. It affects people differently.

Health: Of course I don’t just have FSH, I have type I diabetes which affects my feet.

Diabetes complicates things. My eyesight isn’t good and I don’t have feeling through

my feet. Seeing the edge of a step is difficult – things like that. My balance is

affected. A physiotherapist looked at me a few weeks ago and gave me a stick in

case I needed it. Losing my sight – when I look to the future, losing my sight is my

biggest health concern but that’s not to do with my muscular dystrophy. I’ve learned

a huge amount – how to manage my muscular dystrophy.

Falls: I’ve had a few falls. I fell in a lift when I went to Gartnavel. I fell on the path in

the house. I’ve had several falls but when I look at it I can see the reason. It’s always

dusk, dark or not seeing a pavement properly. I just seem to go over. It’s my eyesight

more than anything. I just fall straight forward. I can get back up but I need to hold

on. If there’s nothing to hold on to I would be in trouble. The fall I had on my path in

my old house – I had to get on my knees and crawl somewhere I could hold on to. It

affects me going out and choosing where I can go. I don’t go out at night but through

the day I go out on my own. Living in a smaller community, people know me and

understand. I lived in Glasgow for sixteen years. People don’t patronise you but help

you when you need it. I have great neighbours.

Housing: I had lived in my old house for a long time. It was daunting knowing I had to

move – in fact it was the specialist neuromuscular physiotherapist who suggested I

should move and I am really very grateful for that. I don’t know what I would’ve

done. I had so many falls in the back garden – all the different levels and all the

different areas. The path was terrible. It was a lovely family house for myself and my

son but I’m so relieved to be away from all that. Initially, I bought that house

deliberately because it was up a hill and I wanted to practise walking up and down

the hill to the shops. Stairs keep you a bit fitter and so partly I was thinking about my

future health but I did realise there would come a point where that house wouldn’t

be suitable for me……...it’s not really a house…it’s a house for a younger person. The

physiotherapist helped me to focus and think about the house. I looked around at

houses and decided that a level house, easy access to the car, shops just down the

road. It couldn’t be better.

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Transport to Hospital Appointments: I’m up and down to Glasgow twice a month. I

can’t complain….I’m obviously getting good care. I need to go to Gartnavel Hospital

or the Southern General Hospital and there’s a voluntary transport service and of

course you get help financially to go when you’re in the Highlands and Islands. So

you pay the voluntary transport person who is driving their own car and you give

them £50 and you claim back £40 retrospectively from the Health Board so it only

costs £10 for a door-to-door service. It’s a marvellous service – a local service. It can

take 2 1/2 hours to get to Glasgow and although something closer would be more

convenient – if there’s road works or something it can be a long day plus the fact we

get landslides and things in Argyll, so I would say it would be better if you could get

things done locally.

I never need an escort as it’s always somebody I know or at least I know who they

are. Its friends or ex-classmates, so this is where the community side of things kicks

in. I’ve been terribly lucky. I could take someone with me in the transport if I wanted

to – I just don’t need to.

My diabetes is managed at Gartnavel Hospital and that’s the way I want it. I’ve been

going there for 34 years and when I moved back I got the choice to either come to

Glasgow or stay in Argyll. I have relatives in Glasgow, there’s plenty to do. I make it a

day out with the driving and everything. I still go to Gartnavel and I’m still very happy

to continue going there. I would go to the Southern for respiratory. I don’t need

ventilation – very delighted with that – I don’t need to go back there for a year.

Work: I worked as a social worker and retired aged 60. My condition didn’t impact

on my work. I did retire early in 1996 for a time. Basically I was looking after my

Mother at the time because she was very disabled and my son was about ten…..and

being a single parent with family responsibilities. I was very exhausted and I got the

opportunity to retire early on health grounds, which I did. But after my Mother died

and after my son grew up a bit, I went back to work, because, well, I needed to get

back to work and I needed to boost my pension so I went back for six years but it

meant I had had that space….that time off.

Fatigue: Certainly exhaustion is one of the things I remember from work and from

other things as well. Sometimes I would feel very fatigued. Now I’m not working, I

don’t feel that way very much anymore. I can rest when I want to. That’s made a

difference. Also the advice from the specialist neuromuscular physiotherapist has

helped cos I really didn’t know much….I didn’t have much information at all until the

specialist physiotherapist visited. I felt I was in the dark before that.

Pain: One of the things that would help me enormously is to get painkillers that

actually work because it’s two years and ten months that I’ve had back pain and hip

pain and nothing really shifts it. Local physiotherapy gave me a sheet of exercises

which I do – nothing else other than exercises – you get a couple of weeks and a

sheet of exercises – nothing else. A more specialist physiotherapist who would do

manipulation would be good. I asked for an MRI scan of my hip and back – I asked

for that myself – again I pushed for that.

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Everything gets blamed on my MD. If you’ve got two things, then anything gets

blamed on those two things. It’s the most frustrating thing in the world. It would be

nice if I didn’t have to push although I’m lucky I can push for things. I feel sorry for

the people who can’t push – for those who don’t have a voice themselves, don’t have

the confidence or are too depressed.

Advocacy: I work now in advocacy as a volunteer. It’s a follow on from being a social

worker but it’s your own personal experience too. I didn’t think too much about that

for me…myself although I see a lot more now with the roads, access etc. I raise

things with the Council etc. I just mean the practicalities….but if you actually look

about at the pavements, the things they call access for disabled people…and it’s just

a wee thing….if you were in a wheelchair it would be impossible. Things like

automatic doors, you just don’t get. It might work if you have someone to push you

but it won’t work for everyone. It would be dreadful for people with no one behind

them – it would be dreadful.

Specialist Services and the NHS: I referred myself to the Southern. The doctors, the

GP hadn’t said “Are you going anywhere?” I was lost to the system for several years

and then I thought, I need to do something so I phoned up the Southern, spoke to a

lady on the switchboard who told me she hadn’t seen me for several years now and

I’ll need to be re-referred by my GP. So I asked my doctor if he’d refer me so that’s

how I got referred.

There’s been no proactive…..the NHS….not proactive enough. I think if you’ve got a

condition that they can’t cure or do something about, they’re not interested. You are

left to your own devices – maybe I’m wrong?

Prior to this I felt I was given my diagnosis and left to get on with it. I’m trying to

think when I went to the neurologist…I’ve always gone to Gartnavel to get my

diabetes checked. Being re-referred this time around felt different. You were left to

your own devices the first time around. I suppose if I went looking for help I might

have got it. I never spoke to my GP about my muscle condition; I just got on with it.

There is a difference now with more people working in this area like the nurse

specialist, the care adviser, the specialist physiotherapist. I know where I can get that

help now and it’s a very good feeling to know you’ve got that. It’s a big improvement.

If there’s anything you could change, what would it be? Information is one of the big

things. I didn’t know about fatigue for example, or how to manage it. It was the

specialist physiotherapist coming that taught me about it. That would have been

useful to have more of an understanding of that when I was younger. I always

thought my fatigue was my sugar levels cos sometimes my sugar level would be up

and occasionally people would say to me – you look absolutely exhausted but you

16

just carry on, don’t you? If you’ve got to work, then you just work. You tend to just

accept things until someone tells you otherwise.

Information is one of the key areas.

And also a recall system if you haven’t been seen for a long time. Even if there’s

nothing that can be done, you sometimes still have questions and things – nice to

have a choice – I think if you are offered an appointment and don’t take it, it would

be wise to find out why you don’t want it. You shouldn’t make assumptions they are

just busy – there may be a reason they don’t want to go.

Seeing someone face to face is also very important. I know you can invent things like

telephone consultations and things but I think interpersonal contact is much better. I

do think it’s very important and much more therapeutic for people. I can see the

value of teleconferencing – I’m not saying it’s not needed or anything and it’s better

than nothing but it’s not as good as seeing somebody.

I think we may need to be more open when asking for help and I suppose if you do go

to your GP, you do have a responsibility yourself to raise things….it’s not just them.

Patient Three: Andy (68 years) lives in a sheltered housing complex in Renfrewshire.

He is wheelchair dependent, drives his own vehicle and is very content with life.

I’m a great believer that if you dwell on things for too long then

that becomes a worse illness than what you’ve got – it only

exacerbates the thing.

Family History: It’s been noted within the past few years I have a cousin with the

same condition but it wasn’t known at the start when I was diagnosed.

My parents were checked out and there was nothing…..nothing was found. And at

that stage, that was another thing for me. I thought, that’s the last time I’m going to

do that. I’m not in the blame game here. You’re in this life and you’ve got what

you’ve got. You’re in the life; don’t blame it on somebody else because it could be so

many generations back that has caused this problem so…..

Both my parents were extremely supportive about the thing in fact I can remember

my dad saying to Douglas Wilcox who we saw at Yorkhill. “Is there any way, can I

give my muscles in some way, can I donate them?”

Health: I was diagnosed in February 1989 – I was coming up for forty three. How did

it all start? Well the first thing was I had a problem with the lower spine and was

taken in and I had keyhole surgery on one of the lower discs. It was after that when I

went back to the Western there was a doctor who came in, looked at me, checked

17

me over and said, “Have you always been like that?” And that was the first time

anyone said anything to me.

I was born without pectoral muscles – round shouldered type of thing. He (the

doctor) queried this and the next thing was I went to the Southern General Hospital

and there was a doctor there and he did a test on me – the needles into the muscles

– and then they told my parents it was some form of muscular dystrophy that they

suspected. They couldn’t pin point it at that stage but they thought it was some form

of muscular dystrophy. So I then went and had a core sample, a muscle biopsy which

I had done twice actually. They did it once and then sent it away to Newcastle I think

and then they came back and said it looks like some sort of scapulo-humeral type

dystrophy that you’ve got but we’re not sure to pinpoint it down…..and then I think

with the progression in the DNA testing, you know, perfecting that a bit, they did

another biopsy and then sent that down and they confirmed at that stage it was FSH

I had.

Was the diagnosis a long process? That would maybe be about a year and a half or

something like that. Like I say when they went in and said it was dystrophy that was

fine. I could walk. I really was fine. What I did, one of the things I did notice though

was when I was walking, I had this kind of drop foot. I kind of swung the foot out and

if it caught you know, I was tending to go forward.

None of this took me to the doctor. I just thought it was part and parcel of the way I

was and it was one of the times when I got the thing in the spine – they checked up

the spine and I thought it was because of something I had lifted cos we had been

shifting things about in the drawing office and I’m sure I heard a click, and I thought

– well I just forgot about it – but then that’s what it turned out to be…..a twisted disc

and part of the core of the disc had gone into the spinal column and was touching on

the nerves in the spinal column.

It was after that, that I mean I went in on the Monday, went through the operation

on the Wednesday and actually walked out on the Friday and my folks and I came

home on the bus because neither of them drove. It was marvellous and I thought-

well that’s it – everything is hunky dory and then it was when I went back to get the

dressing changed and things like that, that this guy – he must have had a keen eye-

he spotted something wasn’t right at the time and then….they made the diagnosis.

How did you feel about the diagnosis? I took it in my stride – I mean at the time I

didn’t fully understand what it was – I had heard of it.

I mean I had heard more about multiple sclerosis and things like that but dystrophy

seems like the second cousin to it I think, but it was only afterwards I started reading

in to it and asking questions about it. I thought – well – it’s a muscle wasting disease

etc etc and I tried to find out as much as I could about it.

18

I think people are becoming more aware of muscular dystrophy. There was a

programme on the television – that lady – the Scottish actress – she has relatives

that have got it – the condition – and I saw the programme of her going over to

Denmark which was of interest and I think that has probably raised awareness with

people about it and also now, all the colleagues I’ve got, they’re all now au fait with

what it is. They’ll say Andy has got this whereas before they didn’t know anything

about it sort of thing so it does help to promote it more, promote awareness of it.

Health: I tend to adopt a policy that when I’m doing something, and I find a difficulty

with doing it, then I try and adapt. If I feel I am having a difficulty doing a certain

thing then I try to adapt to get round about it. That’s a thing I still hold by you know.

If I get to a stage where I can’t do it anymore, I just draw a line under it and try

something else sort of thing. It’s one of these things that I think when you’re aware

of it at the time you sort of say to yourself…well…I adopt a policy…well, you’ve got it

and that’s it – there’s not much really you can do about it – you’ve just got to adjust

your lifestyle and your life accordingly to get on with it.

When I got into the fifties age sort of thing….and yet it wasn’t so marked a change…it

just mean’t I couldn’t really run ‘cos of this out of kilter type thing with my leg and

my strength wasn’t as good as it had been because I was really quite physically fit

because I was a climber / hill walker and you know, I took pride in keeping myself fit

sort of thing so I think that helped plus the fact with me being a hill walker, you

watch where you place your feet so I was very aware of where I was. I was always

having points of contact because the last thing I wanted to do was fall. At that stage I

could arrest my fall but now I can’t.

Work: I was fortunate with the job I was in you know, in a drawing office. I think the

biggest change for me was the fact you don’t have to stand at a big drawing board

any longer. The computer age sorted that out so you were sitting – so it was kind of

a sedentary job that you had – so there wasn’t much – and I still used to go up and

down to the factory floor and any projects I was working on, I would go down and

get involved in that so it didn’t have any impact on my working life. I can honestly

say I didn’t let it stop me. I put it to the back of my mind. I’m a great believer that if

you dwell on things for too long then that becomes a worse illness than what you’ve

got – it only exacerbates the thing.

I retired from work early because the company I worked for originally was an

American company and they were bought over and within two or three years there

was a wind down and they decided they were going to relocate to just outside

Leicester. At that stage my condition had deteriorated slightly. I was still working and

my dad’s condition, he had quite a few problems, and my Mum was starting to show

problems so I thought – well, that’s a bridge too far for me to go away down there

because a) I would be leaving them on their own and b) I would be away from the

sources of medical people I knew – so I thought well….that’s it.

19

I’d been with the company for about 24 ½ years, even the Trustees, the people who

were the Trustees of the pension were very good. We got a watch at ten years, a tie

pin with sapphires in it, we were well done too. The clock in the hallway, the

barometer you’ll see out in the hall, that was for twenty years and then I got my

watch. A £350 watch from the company for retiral…I did well (laughs) but as I say

when they decided they were going to move south, that was just a bridge too far for

me but the Trustees said, “Look, go and enjoy your retirement,” so that’s what I did.

I had some offers because of the nature of my job that I could possibly have sat for a

few days and did the draughting and so forth and the design that I was doing, but at

that stage, with the condition my folks were in, I thought you know, my first duty is

to them. I knew it was time to draw the line and just move on from there.

I have a good attitude. Someone said, “You use less muscles smiling than frowning,”

and I’m a lazy person so I just like to smile.

Hospital Appointments: I used to go on a regular basis to the hospital and I was

assigned to a neurologist and he kept a clinic sort of thing with me, just like a talk

shop more or less sort of thing. The people I did get very involved with were at

Westmarc, the Westmarc Centre. I went along and it was the Physio Jane Lough, and

Barry Meadows was there and there was a lad from….Roy Bowers from the

University and we developed a (laughs) – I called them the A-team, of which I was

part; we were very much part of the unit. There was another physio who got

partially involved and they used to go to the clinic and they were developing the

ankle foot orthoses. There was a couple of things – me being an engineer - about the

hingeing and stuff like that which we talked about and so it was something that I

could get involved in proactively and they took wire frame models which they used

in the seminars and things like that. I felt like the bionic man (laughs).

I can’t commend them highly enough because the work that they did and the way

that they did it – friendly way that they did it, will always be with me.

The experience of Westmarc was a good experience and I can’t commend the people

I was working with highly enough – their dedication to what they were doing. They

were obviously looking for solutions to get things done and I think so long as you’ve

got people like that, they’re obviously going to be there for the next generation

coming up.

I mean I got to the stage where it was practically dangerous for me to go because my

walking ability left me sort of thing so it was a gradual thing. I mean I tried different

things with it and I could walk on my own, and then with the stick and orthotics, and

we did a lot of different things with it and then gradually I had to graduate to using a

stick, just more or less like an outrigger, additional security, you know – mentally I’ve

got this so I can lean against it.

20

Another thing that I got at one time was at the hospital in Johnstone. It’s closed now.

They had a hydo pool type thing and the Physio came in and did things in the pool to

relax the muscles but again through cut backs as I say the hospital isn’t there

anymore, it just died a death.

The hydro was good in a sense. I’m not a great swimmer – it was very relaxing when

you went there but I feel you know, I know my cousin who has also recently been

diagnosed with FSH; he goes to Chester, he lives down in England and he goes there

(the neuromuscular centre) and he gets the hydro and he finds it very beneficial. I

think it’s an individual thing. As I say, I tend to do, if I can do them. I’ve got a carpet

sweeper thing and I do that every day; it’s just a regime I’ve got but that gives me

exercise and all the different things, I mean make my bed – it might take me three or

four times longer than it normally would but I do that for my exercise.

Housing: The house I came from, we were down there for nearly forty years more or

less, since it opened, the house that we had was upstairs and at that time we had a

chair lift, one of these stair lift sort of thing and eventually we got a converted walk

in shower which benefitted not only me but obviously my parents. So we thought

the house we had was excellent; it really was an excellent house and we were up

there for thirty odd years and then this opportunity came up. My mother was quite

ill at the time and the social workers and doctors and all that came in and said you

really need to think of the future, to move on, so my Dad was really quite astute that

way and when these facilities (there’s two in Erskine) were built he came down and

had a look at it and then eventually it was the social workers and all that came and

they said it’s the first time ever in their history that they had three people from one

family that needed care so it was a first for them; so at that stage we signed up for it

sort of thing.

Sadly as you know, my Mother died before coming down here but my Dad and I

came down here and my dad had about three and a half happy years down here.

My flat is very spacious. I class it as a Rolls Royce – that’s the standard I would put it

at and I feel if everything was done this way for everybody it would be a better world.

It really is good. There are so many facilities in the place; it’s very well kitted out. The

spaciousness of the flat itself is good. Everything is suitable – the only thing is the

sink - if I was doing a lot at the sink (I don’t think I really do much though), it’s just

the breakfast dishes and things like that which I do – but I can’t get under the sink

with the wheelchair because there are cupboards under the sink and that’s

restrictive so…..in the bathroom there’s a wash basin there that you can actually get

under. The toilet is festooned with armrests and handrails and things like that so –

and there’s a pull cord and everything in there in case you fall. It’s a wet room.

Carers: There’s staff available in here 24 / 7. At the moment, there’s four staff in the

morning, three in the afternoon and there’s one at night so there’s always someone.

We have the buzzers if you fall. There’s only once I’ve used it for myself and that was

21

when I found out I couldn’t get myself up on my feet. I fell in one of the bedrooms

and I’d just come back from shopping and normally I’ve got the buzzer round my

neck. I always put it on and here I’d taken it off to go shopping and I’d left it in the

hallway. So I came in in my power chair and then transferred into this manual chair

which I’ve done countless times. This time I ended up, within a split second, I was

looking at the ceiling. I had misjudged it for some reason – hurried up and I fell.

I was fine. I rolled over and usually did my thing that I could normally do but when

I’ve went to push myself up, my arms couldn’t do it because my upper arms have,

they’re only about that (shows his thumb and first finger making a circle), half the

circumference of normal. That’s the size of the muscle and the muscle weakness is

just…..

The forearms and the hands are strong enough to press down on but I couldn’t get

the thrust. I get round on my knees and I was trying to get myself up but because of

the deterioration in the upper arms and round the midriff sort of thing I just couldn’t

do it. So there was me lying there and I thought…how am I going to…..so I had

actually literally as the Americans say – bumped my way along into the corridor and

along and pressed the button and I must admit, the girls were here “winky”. And

they had one of these sling mechanisms – cos I had been trying for twenty minutes

and the sweat was lashing off me and the harder I was trying, I was becoming more

frustrated – it’s a definite frustration, but as I say, if I’d known that earlier, I would’ve

buzzed the girls earlier and I would’ve been back up in my wheelchair.

Personal Life: It went from using one stick to two sticks and then eventually when I

came in to my present home, I could walk in ‘cos the day we moved, my dad and I

moved in here, I could walk in with the aid of a stick so at that stage I would be sixty

three at the time when I moved in here so that was the stage I was at then – but

since then I graduated to using the zimmer frame to go along for meals and walk

with that and eventually I ended up in the chair.

It goes back to what I said earlier; it’s about accepting its part of the problem I’ve

got. To my mind like everything in life, you’ve got to be positive about it because if

you adopt a negative attitude then that’s you thrown in the towel. In my book, I

mean don’t get me wrong, there’s times when I really get frustrated and I think,

“This is stupid, I should be able to do this”, and you can’t and I’m fortunate that my

sister and brother-in-law live particularly close to where I am and I rely on them a lot

– particularly my brother-in-law – he’s excellent with me and if there’s anything to

be done that needs – I can’t stand up properly now – so if there’s anything that is out

of reach or something, he’ll get it for me’ or anything of that nature, he can do that

for me.

I try to continue and I get into a regime, more so the like of the flat here, I have a

regime. I still clean the flat myself, I still do my own laundry; I do my own ironing and

things like that – well it’s the old thing, if you don’t use it you lose it and the more

and more you do it, sometimes it becomes more and more difficult or it takes a

longer time but then I’m retired. I have plenty time to do it.

22

Aids and Appliances: Everything in the flat, I’ve got a hospital bed courtesy of the

specialist physiotherapist, and she got the wheelchair away back in November 2005

– I don’t forget these things (smiles). So I’ve got all these things, all these facilities

and the thing about being in here is the fact that you don’t have to cook meals. I

mean breakfast is the only meal I have to cook – well I don’t even cook it – I just

shake it out the cereal box. It’s one of these things. I mean you’ve got that and you

don’t need to attend to anything. That’s good because I can get up whenever I want.

Also they have set mealtimes in here and you just go along and everything is set up

for you. Excellent food I might add – they have an excellent cook here. We have two

choices here every day so again that is a big burden that is taken off you – as I say,

you don’t need to do any shopping or so on so that frees up some extra time for you.

In the evening, about 6.30pm or so, I go along and the girls make a cup of tea or cup

of coffee and you can get a scone or something like that.

Social Life: The social aspect…..well, when we came in here at first, we were

introduced to all the – there’s twenty flats in here at the moment, and we were

introduced to the people and it was an eclectic bunch of people (smiles)…you could

put it that way. We then proceeded to get quite heavily involved with the social

aspect and things – both my dad and I, because with my dad being in the community

association we were used to doing that, running dances, working at the bar

whatever, so really quite heavily involved. My Mother was too, so it just became

second nature to come here so. So if we can do this for the people then we’ll do it

‘cos it gives us something to do as well – to take up our time and get involved. So we

organised things like bingo – I did my bingo caller and things like that. I made up

quizzes for the people; we had quiz nights, we had a movie might so on and so forth

and we had to balance the things, particularly the quiz night because there were

some of the people who had learning difficulties; they couldn’t read or write so we

actually included them in with people that could sit with them and say, “What do

you think the answer is to this, what do you think it is?” so there was nobody

excluded. The balance and the difficulty of questions for things you know – I mean

one of the opening questions was always about a nursery rhyme or that – that a lot

of people would know – so that was good.

But as the years have gone on sadly, there has been the demise of quite a few of the

people in here and there’s always the problem if you have new people coming in

then are they capable of actually socialising. There is definitely a change in the fact

there are fewer with a physical disability and more with learning difficulties. There’s

people with greater demands from the staff – the staff level has increased to

accommodate that because when we came in here at first, there were only two staff

in the morning, sometimes only one in the afternoon and one at night and even at

that, it was down to this call system – there wasn’t someone available. If they were

off sick or on holiday then you had to rely on the call system so when that was the

case your response time was increased because depending on, I think they call them

responders – if they were an hour away from you, then it was going to take an hour

before you would get here whereas just like that incident I told you about with me

falling out the chair, they were here in an instant – that was it-they were at the door.

23

The staff are now doing more physical things than before. There is also a bigger

group of older people here now too. At the moment we have four nonagenarians,

ninety year olds in the place- I mean sixty eight years – I’m one of the bairns in the

place so there is that difference. There’s some of the originals, the Doyens, that have

been here since the very beginning. Sadly their conditions have got worse but I still

find time to sit with them and talk to them and spend time with them because I feel

we are all part of a family and I think – you don’t desert your family in times of need.

Being in a community where people are frail and elderly and watching them progress

can have a negative effect on you. One of the care social workers that actually got us

in here, one of her questions directly to me was, “How do you feel about going into a

facility that has got lots of older people in it and my response to that was, “Where

they go – I go”.

That was my choice but I get on well with all age groups and I feel there’s a lot I can

learn from them but you know, I feel it does have its negative effect on you in as

much as conversation can be stymied, you don’t have much conversation so again

looking back to where I was, you just adapt and do something else.

I do get the opportunity to go out and about. Fortunately I’ve got a motability car

that is adapted. That is a legacy me dad left me sort of thing – we got it adapted –

and I have a power chair and the power chair goes straight in to the drivers position

and I can still drive – I am still capable of driving and that in itself….every Sunday I

have a regime that I have that what we call the Big Breakfast is here. It’s a brunch we

have at half past eleven so if you want a big fry up, come here folks (laughs).

So after that I just get ready and I go out and I go over to the cemetery where my

folks are and sit for a while and then I take the car for a run and go different places-

charge the battery- recharge the battery and things like that so that gets me out and

has given me a high degree of independence – motability wise and also with the

power chair that I’ve got, I can actually take it off road at times. I can go over to the

local shops – again it’s weather dependant. If it’s heavy rain, it’s a no-no. Not with an

electric chair you know….what hair I’ve got would actually stand up (laughs).

Holidays are fairly restricted – I can’t recall- I mean I’ve had ….my brother-in-law and

sister are the ones who mainly help me with that regard. In fact about two months

ago I was down in London: they took me down by car to London to see my niece and

her daughter. It was her second birthday so it was good. I was down there for about

six nights, down in London, and again, as I mentioned earlier, my brother-in-law is

fantastic. He took me out and about and we did a bit of touring. I was also down last

year with my eldest nephew. He took me down to London again and we did a Marty

Feldman’s lightening trip of London which was quite good. When my Dad was alive

we went up to visit folks up in Forres, he lived up there but apart from that, holidays

is not a thing – that is a limiting factor for me you know but I still like to try and get

out and about. We used to try and organise trips here but at the moment, it’s well,

24

the amount of organisation it would need and the amount of carers that you’ve got

just precludes doing that.

One of the things here I’m very grateful for in this facility here is the garden – they

have a really good garden. Our sister complex have quite a small puny garden but we

are blessed with a large garden and my dad and I, we used to have an allotment so

we were used to things like that. I tend to go out when the weather is suitable.

I got a small hoe and a rake which I can use from the wheelchair and I go out and

tend to the garden and that so that gives me a good outlet – that gives me a lot of

pleasure.

I’m such an independent person; I mean anyone you meet will tell you, I’m such an

independent person.

Information: I was recently up seeing the nurse specialist and gave her my tales of

woe and she has responded to that and kept in touch with the local doctors so

there’s a network there which is saying well, this is the condition, this is where I’m

currently at – I’ve gone from there to there sort of thing so I think it’s invaluable –

moreso to doctors because local GP’s I find when you come to mention muscular

dystrophy, it’s one of the rarer sort of things they look at - all the other subjects are

fine, but when you come to that it is truly a specialised subject and some people

tend to shy away from it.

Like if I say something about my health, some people will just say, “It’s just your

condition”, which is fine to a degree, but it’s not always.

And there’s something I might say – like there’s something underlying that’s causing

that. I mean I had problems, renal problems, a kidney and I had a 5.2mm stone in my

kidney which, and again with the spinal surgery (the key hole surgery) - so I had that

but at the time I was sent away – it was just my condition – just go and take

paracetamol – the cure for everything – take paracetamol. But no I like to, if there’s

something I’m not sure about as you mentioned earlier, I’ll look into it, but I know

sometimes you can look into it and overdo that and it becomes a thing you become

obsessed with when you say well, “I’ve got this, that and the next thing,” and that’s

not me you know. I say, well I’ve looked into it, it could possibly be that so I then say,

I’ll go the people who I know and I say, “I’m experiencing this, this and this and my

own thoughts are, it might be this or that….but it’s not up to you to make the

decision you know. I don’t like to impose on anybody and feel I know better ‘cos

that’s their forte and they’re the specialists at it so let them make the decision.

Patient Four: Louise (21 year) Highland. Lives in student accommodation with 24

hour carers.

“Good accommodation makes such a big difference and it’s

shown me that independent living is something I can do.”

25

Diagnosis: I think I found out about having muscular dystrophy probably when I was

four years old. My parents had noticed a sort of decline in my walking and also from

a really young age about my facial expressions, like from being a baby – not smiling

at the usual time. They made an appointment and then discovered I had FSH

muscular dystrophy. It was probably about a year or two before they knew exactly

what it was but yeah, I was really young so I don’t remember much from that time. I

think it was in Inverness probably.

Did things change with the diagnosis? It was probably my parents who had to come

to the realisation.

It’s probably easier being diagnosed really young ‘cos you’re growing up with it

rather than living a normal life and then being told you’ve got a disability

Things didn’t change significantly for me when I was that age. It was quite hard

growing up with it at school but like I say it was from a young age so it was easier.

What was hard at school? Joining in with all my friends throughout primary it was- I

had quite a normal upbringing. Probably in Primary 7 I noticed my friends were

running around and I couldn’t always keep up so it was just like trying to join in as

much as I could. I was probably quite frustrated with my friends for not

understanding but at that age it’s not really, not really something you would

understand at that age so it was probably frustration that I felt. No one (healthcare

professionals) came in to the school to talk about it really. It was probably from

being in a really small area, they didn’t know much about the condition so maybe

that was something to do with it. I don’t remember having much support, like

anyone coming in to speak to me about it.

I had really good friends because they grew up with me and knew I had the

condition. I was treated like everyone else so I was quite normal – being around my

friends. The teachers again – like there was no sort of barrier – like they were quite

open to it. Secondary school was different. All our schools got merged together.

There were new people coming in. There probably was a little bit of a barrier, just

‘cos they didn’t know who I was from a young age but again like, it really didn’t take

long for them to get to know me and yeah, it was fine.. i think I make friends quite

easily, I like to think I do.

In secondary school, moving from class to class was quite challenging ‘cos I had to

leave 5 minutes early before each class ended so that I missed all the rush. I think I

was in a wheelchair maybe most of the time in 2nd

year so it made it a bit easier. But

1st

year I was still walking around so it was hard, like getting knocked over – I had to

be careful when I fell although i was still able to get up myself so it was fine.

The classes would always end 5 minutes early so I didn’t miss out that much on

learning, maybe socially i did but not to any great extent.

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Studying and Homework: I had an operation in 2008 which was the first year of my

standard grades so I had to do a lot of the work at home, like recovering from the

operation so it was probably a lot more effort for me to teach myself but then I also

had the 4th

year back at school where I could get taught again so the operation

probably came at a really good time but I feel I managed to keep up with the school

work fine and the teachers were always there to support if I needed. I had a maths

tutor but that’s just ‘cos I’m not very good at maths (laughs).

Healthcare professionals: I had an OT and a physio. The physio would come every

two or three weeks and she would go over all my physio regime; make sure that if

there were any changes that they were addressed and if I had any issues, that I

would raise them with her and she would speak to....... Physio was a bit of a chore

but I knew it needed to be done so I just got on with it. Do I feel differently now? Yes

– now- I don’t look forward to it obviously but I just sort of – I make a point of

making sure I do something every day ‘cos I know it’s going to benefit me in the long

term.

Transition: I was always told that adult services are going to be a lot different. There

wouldn’t be as much assistance – it was probably ‘cos I was going to university and

the Social Work changed from Children’s to Adults. For me it was like they were still

quite helpful. They would treat me the same, they didn’t really have any different

opinions. They were still just as helpful. It’s just physio is the only issue. I’m not

seeing a regular physio like they don’t come in every two or three weeks like they

used to so it’s a bit different. I think someone coming in regularly is really useful.

Even though my carers are trained, they’re – it’s not the same – like they don’t –

they’re not trained in physio so they don’t actually really understand my condition

properly.

I’ve got a GP. I’ve had a meeting with her and she went over – I told her all my issues

and she........I’ve not heard anything back from her though. I don’t think they’re as on

the ball as children’s services. The issues we discussed were physio. Wheelchair

services.......from not being in childrens (wheelchair services have been more

challenging). Maybe it was also moving from Inverness to Aberdeen – they’re not as

open to extra features on the wheelchair. I’ve got a recliner but they weren’t

wanting to give me a new wheelchair with a recliner on it but it might just be being

from Inverness and then moving to Aberdeen. The wheelchair service is only able to

provide one of those advanced functions

On a regular basis, my GP would look after my condition so I don’t actually have a

specialist doctor....where when I lived in Inverness I did but that was also Children’s

Services – so I did have someone that would always oversee things. I have a lot of

different Doctor’s now....for my heart, my breathing.

My GP didn’t know about my condition. I was going to them with my issues, but they

didn’t know anything about my condition and they weren’t necessarily that open to

like learning about it – they were just a generalist GP but they were told they had to

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oversee all my appointments but they didn’t have any idea about my condition so it

doesn’t necessarily work that well.

Transition has only happened recently. Luckily, I don’t have many issues, like regular

issues that need to be checked up so it’s fine because if I have one appointment, I

can overview everything all at once, but if I did have a lot of separate issues, it would

be a hassle. But obviously it would be a lot better if I had someone to oversee the

whole thing.

My paediatrician was so good – she oversaw everything, all my appointments and

she was like giving me suggestions about things that could be improved but here I

don’t have someone to do that. It would be useful to have someone like that in the

adult service. My paediatrician did all the referrals to adult service but it took a long

time for the appointments to come through.

In the adult services it’s a lot busier. I’ve had a few appointments I’ve been sent a

letter for and then actually turned up and they’ve said, “Oh, we’ve made a mistake,

you don’t have an appointment today so it’s a lot more time consuming – also

waiting for the appointments takes a lot longer. Sometimes I’ll be there missing Uni,

like I’ll be an hour waiting just to get to see the doctor. That is definitely different to

the children’s services. The children’s services were so much more personal – the

adult services, it’s just, I don’t feel like you’re, I don’t know how to say it – it’s just

like you’re there for the appointment and then you leave, whereas in children’s

services, I don’t know…just much more personal, a lot friendlier. I mean I understand

that they’ve got all of their deadlines to stick to, but just even if they were like more

friendly…How are you? How are you enjoying your day or anything like that would

just make - ‘cos hospital appointments aren’t nice to go to so it would make a big

difference if they were a bit more friendly.

Hospital Appointments: Getting to hospital appointments is okay. I have my own

Carers so they drive my car and the hospitals are quite helpful; they ask if you need a

pick up or dropped off or whatever. My appointments do sometimes clump together

but like I say, I don’t have many issues. It’s just regular, well check-ups every year.

When I’m at University it’s quite hard if they clash with my timetable so sometimes

I’ve got to ring them up and change them which is obviously not the best but it’s

fine. It’s easy to change the appointments. I’ve got the direct number so I just ring

them up to change the appointment and usually they are really helpful. It’s just like

occasionally I have to miss a class because they can’t work around it. The classes are

usually put online so you can just do the lecture at home which is really helpful.

Changes in Healthcare Delivery: When I was living in Inverness, they were a lot more

helpful like they were trying to find better ways to deal with my condition and now

that I’ve moved to Aberdeen I feel it’s a lot more about the cut backs and they don’t

really want to put extra effort in to finding alternative ways of doing things. This also

coincided with the move from children’s to adult services. I think it’s a mix of both to

be honest….moving area and then being a child and going into adult services.

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It would be good if the hospital could do more ‘cos I feel I have to be the one – if I

want an appointment I’m the one that’s got to be like giving them, obviously I give

them the information, but I feel they’re not sort of looking to me for anything that

can be improved. I’m asking them rather than them seeing if they can improve

anything. I feel like unless I start the conversation, that information is not coming to

me.

I think the hospital could be doing a little bit more looking into alternative

wheelchairs or having the physiotherapist coming in but I think it may be just

because I am in a new area or because I’m now in the adult services.

Social Life: I like to keep active so I play the piano. I’m on Grade V playing the piano.

Obviously socialising….I like going to the cinema, meeting up with friends. I like…I’m

doing management with HR so I quite like business so I started up a little mini

business selling jewellery online…hmmmmm…what else do I like doing? I mainly like

just meeting friends and socialising.

It’s a bit frustrating when you’re going out and then you find you can’t actually get in

(night clubs / bars etc). Sometimes I e-mail them in advance and then they say they

are accessible and then you turn up and….nightclubs….not many nightclubs are that

accessible but usually there is an alternative way to get in – obviously some night

clubs – you can’t adapt them so you just can’t go to them. It’s not the best.

My friends are really helpful; it depends who I am out with. Sometimes they’ll be just

like – well if- like we’ve walked all the way there then I don’t want to ruin their night

by saying like – let’s not bother going in but you know, they’re usually really helpful

and if they can, they’ll help me get in.

Carers: I’m really really lucky to have my 24 hour care team so I’ve always got

someone with me which it sort of benefits me independently in terms like I can get

out when I want to get out, I can do what ever I want but it also means someone’s

with me all the time so it’s sort of weighing up the balance; trying to be independent

but not…..it was really hard to get the 24 hour care. It was challenging – just to get

the finance. I was the first disabled individual from the Highlands to go to University

– I was the Trailblazer- setting the way for the new generation (laughs). I don’t really

know anyone else that’s had the same challenge.

To get the 24 hour care – it was making them realise how independence is important

to you ‘cos if you’ve just got someone coming in at night time say, to cook you dinner,

you’ve got to be in at that time – like you can’t do anything else while they’re there

so having someone with you all the time, just explaining how you can go out when

you want, you can do what you want – it means that the care works around you

rather than you having to work around the care. The care has just been brilliant

really.

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I think it would be – like even speak to younger people who wanted to go to

University to say that it is possible.

I’ve got a flat so I’ve got my room and then the carers room so they sleep; it’s a

sleepover which they do and then if I have any issues on the night then they’ll come

through and then they’ll just go back to bed and sleep.

I’m currently in University accommodation but I would always look to have

somewhere where my carer could stay over. I get on well with my carers. There’s

never been any personality difficulties with my carers ‘cos I interview the carers so I

get to know them really well individually and you can tell straight away whether you

are going to get on with someone and it’s really important. Like I know with agency

staff- it’s just that anyone can come in. You might not necessarily know them or get

on with them but with this care company that I went with, it meant I was involved

with the interview process so it meant I could choose people that would get on with

me. I’ve had the carers a long time. They are university students as well so it works

around their timetable and my timetable and they come in obviously when they are

available but it works really well cos it’s really flexible and……if someone isn’t well

and can’t cover a shift, we’ve got five members on the team, so if someone can’t

come in, then someone else can cover.

Funding: That was really hard – that nearly put me off going to university – just how

challenging it was. I was told “well why don’t you just go to College”, but I’d always

wanted to go to Uni but when the challenge of getting the actual finance came

around – it was just really challenging to get it. It took a long time. I had to start the

process early. I was lucky ‘cos the school were with me – like they were helping me

to obviously get the grades and then set UCAS up to go to Uni – to get my

unconditional offer, to then be able to start setting up the care so I had to start

maybe two years in advance before going away.

Training the carers: With the care company I’m with, the training is adapted to

exactly what I need so when they learn – like they don’t necessarily have any

experience in caring so they start with a blank sheet and then they’ll learn exactly

how I work and what I like. My team leader does the training but they’ve been

trained on me individually.

I only train one person and they cascade the training so I don’t have to continually do

the training.

With my Physio (programme) there’s probably a little bit of fear – like they’re too

scared to push too hard or maybe they’re afraid they’re stretching me in the wrong

way and that’s where I think having a Physio coming in every two weeks would be

really helpful just to even reassure them that they’re doing things right.

Transport: All my carers drive –they’ve all got their driving licences for my car so they

can all drive it and it works really well ‘cos obviously if it was only one person, I

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wouldn’t have that independence. The car is on the motability so it’s fully adapted

but the ramp to get my wheelchair in – I don’t drive so I just sit in the back – the

company we went through were really helpful and it happened pretty quickly.

The Future: When I had finished school – if someone had maybe suggested the

possibilities that were out there for a disabled individual leaving school,