Emergency Care: My Experience

Muscular Dystrophy UK | #AmbulanceAction campaign

Throughout my 28 years, I have on many occasions had to call on the Emergency Care services.

I live with the progressive condition, Ullrich congenital muscular dystrophy. Consequently, I have contractures of the joints, a severe ‘S’ shaped scoliosis, and respiratory decline. I lost the ability to weight-bear at the age of 10, and now use an electric wheelchair to get around. I live with my parents and employ a part-time carer as I require support with daily activities including personal care.

My primary medical concern is respiratory related. Ambulances, A&E and hospital wards are all too familiar to me, having endured several bouts of acute pneumonia, a collapsed lung and pleurisy.

Although general knowledge of my disability is limited within all areas of Emergency Care, on the whole my treatment has always been thorough and adequate, if a little clueless at times!

I have found that whenever muscular dystrophy is mentioned, medics immediately assume it is the Duchenne form. This can be incredibly frustrating as it clearly indicates a lack of education and awareness.

There are many different variations of MD, the effects of which are wide ranging. I do feel that comprehension of these various forms needs to be increased throughout the Emergency Care services.

Each time I have called for an ambulance or been admitted to hospital, I need to relay every detail of my disability and how it affects me. This becomes unnecessarily repetitive and extremely tiresome.

Worryingly, there does seem to be a large gap in the most basic knowledge of muscular dystrophy.

I cannot complain about the care and conscientiousness shown towards me by paramedics, nurses and doctors. However, I am concerned about being in a position where I’m unable to answer their questions regarding my condition.

For instance, it can be dangerous to give those with Ullrich congenital muscular dystrophy supplementary oxygen as we retain carbon dioxide. It is therefore preferable to support breathing with non-invasive ventilation such as a Bi-pap machine. Failure to communicate this vital information can be literally life threatening.

Furthermore, the fact that I require the presence of a carer whilst an inpatient can be problematic. This again, has to be explained again and again, thus demonstrating a complete lack of awareness.


Find out more and join the #AmbulanceAction campaign.

Please share ~ Thank you!

Breathe | Film Review

Admittedly I was unaware of the pioneering disability advocate Robin Cavendish prior to the release of Breathe, a much anticipated biopic starring Andrew Garfield. Thankfully, this important figure’s story is sympathetically portrayed with charm, decency and humour in Andy Serkis’s directorial debut. 


The film brings to attention the life and achievements of one of Britain’s longest-lived responauts – Robin Cavendish (Garfield) who, at the age of 28, became paralysed from the neck down after contracting polio. Unable to breathe for himself, he was kept alive for almost forty years by a mechanical respirator.

The real Robin Cavendish with son Jonathan

We are first introduced to the handsome, sporty and awfully posh tea-broker in late 1950s England, where he meets and falls in love with the equally posh Diana, affectingly played by Claire Foy. The blissfully happy couple marry and relocate to Nairobi where Diana announces she is pregnant. Life was good and seemingly limitless.

Struck down only a year into their marriage, Robin and Diana are told curtly by doctors that he will survive no more than a few months. Confined to his hospital bed, Robin wished for death, mouthing to brothers-in-law the words ‘let me die’. Depressed and resentful, he spits in the face of a hospital chaplain who suggests his suffering is part of God’s plan.

A helplessly devoted Diana asks what she can do, to which her husband responds, “Get me out of here”. Choosing to courageously risk death rather than submit to merely exist, hidden away as a patient for the remainder of his days, Robin was the first to pave the way for all other incarcerated disabled individuals. He determinedly pursued life, freedom, social integration and acceptance.

Opposing contemporary medical convention, the Cavendishes defiantly leave the hospital constraints in a blaze of glory, ignoring a disgruntled doctor who calls after them, “You’ll be dead in two weeks!”

Upon their exit they pass by two women who comment that it’s “not right” and “cruel” even, for “them” to be out and seen in public. This brief dialogue epitomizes the narrow-minded medical and social prejudice towards the severely disabled population, at the time.

The remainder of the film chronicles Robin’s fight to challenge such perceptions, whilst also pushing the boundaries of possibility. Not only does he succeed in changing attitudes, he was instrumental in making revolutionary practical advancements, thus effectively changing the lives of thousands of disabled people worldwide.

With the assistance of Oxford professor and inventor Teddy Hall (Hugh Bonneville), Robin develops a wheelchair that incorporates a ventilator, allowing him the freedom to venture beyond four walls, which he does with gusto.

A particularly funny episode involves a family holiday to Spain, during which Robin’s electrical respirator blows up, leaving him, Diana, their son and Diana’s brother stranded on a dusty layby. Not so funny, you might think. But the interaction that follows adds much needed light-relief.

While wife and son keep a jovial Robin breathing with the aid of a bag respirator, Tom Hollander’s character goes in search of a phone to call Teddy Hall back in England. The group then set up camp and attract a local crowd who party with them until Teddy makes a comical arrival on the scene.

As in life, there are moments of heart-breaking despair adding shade to the sunny optimism and whimsical jollity throughout. In one such scene, Diana presents photos of their former life in Nairobi to young son Jonathan who asks, “Can we go to Africa, Daddy?”

Unable to explain his plight to the youngster, he stifles tears of anguish as a watery-eyed Diana can say only, “I’m so sorry”. “So am I”, Robin softly replies.

Some years later, Robin teams up with Doctor Clement Aitken and together they tour Europe, demonstrating the custom made wheelchair. Particularly shocking is their visit to a German hospital where disabled patients are maintained in what looks like a futuristic, white-washed morgue. If you didn’t know this is a true story, you wouldn’t believe it.

The film, which on the whole is a little too rose-tinted, benefits from stark, impactful reminders of the ways disabled people were viewed, treated and constricted. However, it lacks detail and grit, failing to depict the daily grind of real life, the mental strain and tensions within relationships.
While the central performances are commendable, they fail to achieve the same conviction and reaction as those of Eddie Redmayne and Felicity Jones in the comparable The Theory of Everything (2014).

Nevertheless, Breathe no doubt remains an inspiring tribute to the highly influential innovator Robin Cavendish and his triumph over adversity. His story has been realised with love, affection and sincerity, quite literally since the producer, Jonathan Cavendish, is his son.

*This article can also be found on the Muscular Dystrophy Trailblazers website.*

Scoliosis | Why I chose not to have a spinal fusion

In my latest piece for Muscular Dystrophy Trailblazers I explain how and why I chose not to have a spinal fusion as a child.


My experience

I was offered corrective scoliosis surgery at the age of nine. Back then I was able to walk short distances wearing leg splints and had a general diagnosis of congenital muscular dystrophy. Many years later this was specified as Ullrich congenital muscular dystrophy.

I had no idea why I was going to see a consultant orthopaedic spinal surgeon. I was nine! To me it was just another aimless, inconsequential appointment.

There was no faffing around; this doctor was straight to the point. I was told I needed imminent corrective surgery to prevent further decline. I was horrified to hear of the graphic details, the lengthy recovery and how it could even prove fatal.

Of course I now appreciate that with any surgical procedure, doctors are obliged to inform the patient of every potential risk and outcome, including death. But this was unexpected news falling on my young shoulders.

How I made my decision

As a child I attended Birmingham Heartlands Hospital annually where I saw a paediatric neuromuscular consultant.

My parents and I were never given a prognosis nor any indication regarding if or how my condition might progress. Life expectancy was never discussed and no doctor could tell if I would, in time, come to rely on a wheelchair. We were very much in the dark, living day-to-day.

Frankly these appointments frustrated and bored me to tears! We would wait hours – literally, hours – to see the doctor, and would leave knowing nothing more than we did before. I have in all honesty learnt infinitely more as an adult, through my own research, social media and from others with muscular dystrophy.

My point here is that my parents and I had nothing to base our decision on. It’s almost twenty years since I was told I needed a spinal fusion. This was pre-Google and pre-social media. We weren’t put in contact with anyone who had experienced the operation. So, other than a verbal overview from the consultant, we had no other information or point of reference.

After leaving the appointment in a state of shock, my parents told me that ultimately the choice was mine. I decided I didn’t want to put myself through such an ordeal. I was, at that age, considerably more able than I am now, and none of us had any reason to think I would deteriorate as severely as I have.

Do I regret my decision?

At the time, it was, or at least seemed the right decision for me personally. I was able to weight-bear, finding clothes wasn’t an issue, I was pain-free and did not require any inhalers, medication or respiratory support.

The procedure then was very different compared to today, and I was very young. My condition was stable, I was happy and relatively able. Under those circumstances, the disadvantages outweighed the potential advantages.

However, I do often wonder how my life could have been improved if I’d undergone surgery, two decades ago.

No one predicted that just a year after the offer was made, aged 10, I would become completely non-ambulant within a very short space of time. Had I any indication that this might occur, my decision may have been different.

Though my scoliosis was considerable, the ‘S’ shaped curve is now much greater. Consequently, my respiratory function is significantly affected and basic comfort is a distant memory.

On bad days when I’m in pain and struggling for breath or when I’m ill for months (yes, months) with respiratory infection; I do regret forgoing my one opportunity to correct my skeletal deformity.

But, what’s done is done, and cannot be undone. I’m stuck with me! I can’t change past decisions. I simply have to make the best of what I have and keep moving forward.


I’d love to hear about your experiences with scoliosis and spinal surgery.

– Can you relate to my story?

– Have you too turned down corrective surgical intervention?

– Have you had a spinal fusion? If so, how has your life changed as a result?

Thanks so much for reading, and please leave a comment.

Hoists in Hotels | MDUK Trailblazers

Last month Muscular Dystrophy Trailblazers launched their report following an investigation into the need for hoists in UK hotels.

Over 100 Trailblazers responded to the survey, sharing both positive and negative experiences.

This is an important issue that affects the lives of so many disabled people, myself included. Without the essential facility of a ceiling hoist, we are denied the opportunity to travel, whether for work or leisure purposes.

With only 18 UK hotels having installed ceiling hoists for disabled guests, this is clearly an overlooked and ill-considered feature. Who is designing these ‘accessible’ hotel rooms, anyway?!


I was one of the respondents to the Trailblazers survey. Here is my view:

“I am an infrequent traveller, not because I lack the desire but because it is so difficult to find appropriately adapted and affordable hotels. Even getting away for a single night is an almost impossible challenge, since hotel rooms are, disappointingly, not equipped with ceiling track hoists as standard.

Although some people get around this problem by hiring (at an extra cost) or taking with them a portable hoist, this is not practical for all. Portable hoists are cumbersome, difficult to store, transport and manoeuvre. Furthermore, many people simply don’t have access to a vehicle large enough to carry such large-scale equipment.   

I have Ullrich congenital muscular dystrophy and am completely non-ambulant. I can’t safely transfer and so I either have to be hoisted or manually lifted. Understandably most people, excluding family, are reluctant to do the latter. So, if I want or need to get away from home, my only current option is to ask family members if they are willing to lend their time and support (far from ideal).

With less than 20 hotels in the UK equipped with ceiling track hoists, our options are severely limited. For those of us who need this facility, a premium cost is incurred, and then we are restricted to specific locations. Sadly we are not free as others are, to occupy any hotel room in a hotel of our choice, anywhere in the country.”


Click here to find out more information and from other contributors.

Changing Places Awareness

Imagine being denied the basic human need to go to the loo; being unable to access a toilet whenever you need to. Imagine having to hold it in all day, every day. Having no choice but to strictly limit your fluid intake to the point where you cannot risk drinking from morning until evening.  

My experience

This was my life until 2011, when I underwent medically unnecessary surgery to insert a suprapubic catheter. Of course I didn’t want an operation or an indwelling catheter. By no means was this an easy fix, believe me! But I just couldn’t do it anymore; I was making myself ill and relied on assistance from others in order to carry out the seemingly simple task of toileting. No longer could I inflict undue stress on my body and mind.

So, I resigned myself to the only option available to me, that being the suprapubic catheter. I no longer depend on other people, nor do I have to struggle and suffer the indignity of using small and frankly ill-adapted disabled loos. But, 250,000 disabled people in the UK still do.

The truth about disabled toilets

Often there is not enough room to fit a wheelchair in disabled toilets, let alone space to transfer, adjust clothing and accommodate a carer too. Baby changing facilities get in the way, grab rails are too few and carelessly installed, the toilets themselves are too low, and hoists… what hoists?!

Changing Places

The 19th July 2017 marked the second Changing Places Awareness Day and eleven years since the campaign began. I’ll admit it’s only relatively recently, through social media groups, that I first heard of Changing Places toilets. Though there are now 1000 registered Changing Places toilets across the UK, I have yet to see one.

What is a Changing Places toilet?

Each registered Changes Places toilet includes:

  • a height adjustable adult-sized changing bench
  • a tracking hoist system, or mobile hoist where not possible
  • adequate space for the disabled person and up to two carers
  • a centrally placed toilet with room either side
  • a screen or curtain for privacy
  • wide tear off paper roll to cover the bench
  • a large waste bin for disposable pads
  • a non-slip floor

To support and raise awareness of the need for Changing Places toilets, Muscular Dystrophy UK established the #FitToBurst campaign (Keep up to date on Twitter!)

As a Trailblazer myself, I offered my thoughts in response to the question (posed on Facebook): What does it mean to you to have more Changing Places toilets?

 

Here is my full response:

To be honest I’ve never seen or used one. I don’t know if it’s because I live semi-rurally (are they located predominantly in cities/larger towns?)

If so, I think it’s important that there are Changing Places loos in smaller towns, villages and more rurally as there are people in need in these locations too.

The lack of such a facility locally makes me feel restricted, excluded from society and considered less important. I don’t know if I’m correct in assuming that Changing Places toilets are mostly in cities, but if so, it makes me wonder why. Is it a funding issue? Is it ignorance, i.e. the belief that disabled people don’t live rurally?

The majority of disabled toilets I have used throughout the years have been vastly inadequate, filthy, often neglected or used for storage!

As I say I’ve never seen or used a Changing Places loo – unfortunately. But I can think of so many people locally, young and old, who would greatly benefit from having access to one.


To read what other Trailblazers have to say click here.

Find out much more about Changing Places by visiting their website.

We can and we do: Highlighting the Ability in DisAbility

Hi folks, hope you’re all healthy and happy!

Here is my latest piece for Muscular Dystrophy Trailblazers. I really hope you like it!


I’m presently in the process of recruiting new carers. Many applicants have expressed their disbelief at how much I do for myself. One even congratulated me; “well done you!”, to which my current carer responded with a subtle mocking applause.

Why are people so surprised at my level of ability? Do they see me in a wheelchair and assume that all wheelchair users are similarly afflicted and completely dependent on others? Is the concept of disability really that black and white to the general public?

This made me think about how disabled people are perceived by society. Not for what we are able to do, but rather for all the things they assume we cannot.

Each of us is an individual and we therefore experience different limitations and variable degrees of severity.

I have Ullrich congenital muscular dystrophy and as a consequence there are certain things I am physically unable to do. However, not everyone with the same condition will be affected in exactly the same way. A persons disability is arguably as unique as their personality.

Raising awareness of the fact that there is also ability within disability is, I feel, essential in addressing the societal misjudgement that ‘disability’ equals ‘cannot’.  Disability is diverse. Yes it restricts us. But disability is not just about what we can’t do, it’s also about what we CAN do.

We can and we do…

1. Have sex – Yes, believe it or not sex is not exclusive to the young and beautiful. Guess what, old people do it too – shock horror!

2. Have romantic relationships, get married and have children.

3. Leave the house! Sometimes unaccompanied – While independently wheeling around town, I often encounter puzzled onlookers questioning, “where is your helper?”. Sometimes I tell them I’m trying to escape or that my “helper” fell in the river. Sometimes!

4. Drink alcohol – Having managed to leave the house and evade the confused locals (well done me!) I may join some friends for a drink. Yes, an alcoholic drink. Now of course not all disabled people can or do drink. Then again, not all able-bodied people drink, do they. So the next time you see someone with a disability enjoying a pint, don’t be so surprised.

5. Travel – Although many of us require support from friends, family or a carer in order to get out and about; travel, both nationally and internationally is becoming increasingly accessible to all.

6. Drive a car – You’d be amazed how vehicles can be adapted to accommodate disabled drivers. We too want to get out and explore the open road. And for those of us who are unable to drive, travelling as a passenger is another option.

7. Attend university, get a job and have a career – It still baffles me why people are so astounded by the idea of disabled people who are both intelligent and able to work. Two words: Stephen Hawking!

8. Have our own homes – It would appear the general consensus amongst society is that this is something disabled people cannot ever achieve. Of course, many of us do reside with family members, myself included. But there are also those who can and do, rent or buy their own property. Some are able to live independently, while others require assistance from carers.


This is just a brief overview of the ways in which society misjudges the potential and capabilities of those of us with disabilities.

I’d love to hear more examples from you – comment below.

If you liked this blog post, please share – thanks!

 

My view on dating for MDUK Trailblazers

My latest blog piece for Muscular Dystrophy UK Trailblazers:

“I’ve learnt that life is not defined by your relationship status and you don’t need a partner to be happy. It doesn’t hurt to keep an eye out though, does it!”

On Dating Diaries Day 2, we hear from Carrie Aimes who talks about why it’s okay to be single.

Read more of her great blog here.

This blog forms part of Trailblazers Dating Diaries, which looks to lift the lid on dating and relationships when having a disability.

I’m not at present in a relationship and that’s fine, that’s okay. I’ve never actively searched for a partner – dating sites in particular just aren’t for me. In fact, dating isn’t for me if I’m honest. Pretty socially awkward at the best of times, the whole dating thing feels far too daunting a prospect. It just seems so forced and unnatural. Of course it serves its purpose and is a means to an end. But knowing myself as I do I think I would just fail miserably!

A fairly solitary character, I like my own space, I like being able to do as I please, when I please. And I most definitely couldn’t bear to share my bed, it’s my haven!

As is the case for many with muscular dystrophy, much of my time is lost to frequent and prolonged bouts of respiratory illness and fatigue, which doesn’t exactly lend itself to dating or a cohabitative relationship.

I live with my parents in their home which is not exactly the dream for a 28 year-old woman. So privacy and finding time for myself is enough of an issue without adding another person to the mix.

Yes it’s the norm for those my age to be settled in relationships or even married, as most of my friends are. Perhaps my choice to remain single for the time being (and it is a choice) is selfish. But why not be selfish. This is my life and right now I’m content with things as they are. Relationships are hard work, they require you to compromise and invest your time and energy. At present, I’m just not willing to share myself with anyone.

This isn’t to say I’m not open to the possibility of meeting someone spontaneously, as I have found tends to be the best way. But if it doesn’t, I’m totally cool with that. I’ve learnt that life is not defined by your relationship status and you don’t need a partner to be happy. It doesn’t hurt to keep an eye out though, does it!

[Un]dateables

I recently wrote a piece for Muscular Dystrophy UK about the Channel 4 show The Undateables which you can find here.

However, here is my unedited version…

Last week Channel 4 aired the final episode of its reality series The Undateables, a dating show for disabled people. For those who are unfamiliar, individuals with any disability are invited to appear on the show, now in its sixth season. With the help of dating agencies and personal introduction services, they take part in blind dates, speed dating and match-making in the hope of finding love.

Now I’ve seen almost every episode since it premiered in 2012 and I have to say I am a fan. I appreciate the mounting controversy surrounding the show, particularly within the disabled community, although I disagree with much of the negative criticism. For this reason, as someone with a physical disability myself, I though it time to put forward my point of view.

Firstly I’d like to point out that all participants have applied of their own free will. Following their appearances, all have reported a positive experience, even those who did not find love as a direct result of the show. Tammy from series 5 says, “I put myself forward for The Undateables. At no point during filming did I feel like I was being used for entertainment. It’s an entertaining show [but] we all just want to find someone who loves us for us.”

The program has been invaluable and life changing for many, leading to long term relationships, marriage and babies. Furthermore, despite the claims of some, disabled individuals have not been coupled exclusively with other disabled people. For example, Brent with tourettes married his able-bodied date Challis, and Steve with Crouzon syndrome married able-bodied Vicky whom he met on Twitter after the show gave him much needed confidence – he remains friends with his able-bodied date from the show. Then there’s Carolyne from the first series whose childhood sweetheart left her when she became paralysed following a spinal cord lesion. She later met Dean who is also able-bodied. The couple had their first child together in 2014. These are just a few of the many success storie

Some critics have called into question the editing, which it can be argued is an issue with any reality show. However, taking into consideration the accounts offered by the participants themselves, it would seem to me that great care has been taken to ensure fair and accurate representation. Again, personally I have no issue with the tone or editing and have never found it to be exploitative, patronising, sensationalist or insincere. Quite the opposite in fact, I feel The Undateables realistically and positively depicts a range of disabilities thereby raising awareness and breaking down social barriers.

James, who has Asperger’s took part in the show last year. He told ITV’s This Morning, “It provides a lot of education on a wide range of things, not just conditions… The fact that people will tune in knowing they will learn a bit more, maybe take away the stigma, is a very positive thing. It paints a very positive picture of British audiences.”

The format itself is understandably a contentious issue: why is it not the norm for disabled people to participate in mainstream dating shows such as First Dates, also a product of Channel 4, and ITV’s Take Me Out? Why must the disabled community be confined to a show exclusively for them? There is no definitive answer, though I would argue that it comes down to choice and demand.

As previously stated, those that partake make the choice to do so. Many have learning disabilities and are supported by family, friends and caregivers, as viewers will know. Therefore to suggest they are being taken advantage of by producers, which some critics have, I feel implies that these people are not able to form rational decisions and make up their own minds. This is inaccurate and unjustified. Secondly, the show is now in its sixth year which proves there is continuing demand from both the viewing public and applicants eager to find love, friendship and companionship as others have on the program.

I have found that questions such as the aforementioned are regularly posed, more often than not by those with disabilities rather than those without. This indicates to me that in fact it is not predominantly the able-bodied community who have issues with the show. Yes you may hear the occasional ‘bless them’, ‘aw how sweet’ and ‘good for them’ from able-bodied viewers – how very dare they indeed! But to conclude that this is a form of ‘inspiration porn’is in my opinion, vastly overstretching the mark. I take issue with the term ‘inspiration porn’, particularly in relation to The Undateables. Frankly even if viewers are in some way inspired by the determination and go-getting attitude of those they see on the show, why is this so awful? Paralympians are equally as inspiring as Olympians. Yet there are some, particularly in the disabled community who deem this to be ‘inspiration porn’. That is to say people draw inspiration from disabled athletes solely due to their disability rather than their sporting achievement, as well as to feel better about their own lives. I think this is nonsense and insulting to both the able-bodied and disabled.

I cannot speak for the entire viewing public obviously, but I have watched with friends, family and associates over the years and the feedback has always been one of support and genuine happiness for the love seekers. Not one person I have spoken to has ever indulged in this so called ‘inspiration porn’ to, as critics say, make them feel better about themselves. Frankly this is the one accusation that frustrates me the most. I could go on and on with this point but I fear I may lose you (assuming I haven’t already that is).

Okay the title. Are Channel 4 saying that we, the disabled are undateable? Put simply, no. Producers have themselves stated that the title is to challenge this common misconception within society. Furthermore, as viewers will know, during the opening sequence of each episode, the prefix clearly falls from the word dateables, thus indicating the contrary.

The show itself is proof that no one is undateable, an eye opener to many viewers who might have previously thought otherwise or have just never considered the fact that like them, we too want love. For one reason or another, there remains a section of society that has never encountered anyone with a disability. Through no fault of their own, they as a consequence may be ignorant to the needs, desires and feelings of disabled people. I think The Undateables is great way to introduce this concept to such individuals. As James with Asperger’s says, the show is successfully removing stigma and raising awareness.

I have an older brother with complex learning disabilities and so I’m able to draw from his perspective in addition to my own. He has expressed a keen interest in appearing on The Undateables and my family and I would be more than happy for him to do so. Neither of us feel alienated, uncomfortable, ridiculed or patronised by the show. I do appreciate the criticism but for those who bother to watch it with an open mind, I believe you will find it to be well meaning, sincere and sympathetic. Those involved have benefitted, it has given others in similar circumstances the confidence to look for love, and it has made society realise that we all have basic human needs and desires, and the right to pursue them.

It’s easy for viewers to criticise on social media, having watched only one or none of the episodes. But I implore you, ask the participants. Their response says it all, for me anyway. It seems to me the majority of negative critics haven’t actually seen the show and are therefore judging it superficially. It is certainly not a freak show and is not treated as such.

The dating agencies, often run by the parents or relatives of those with disabilities, aim to match clients based on common interests. Disabled people are not merely bundled with others with similar disabilities. To assume so says more about those who think this than anyone involved with The Undateables.

So finally, I urge the harsher critics out there to actually WATCH (preferably more than once!) before judging so narrow-mindedly. Maybe even remove that chip off your shoulder (sorry, couldn’t help it 😉).

Who knows if Channel 4 will commission another series of the popular show. Based on viewing figures I’m guessing it’s more than likely they will. And, if so I’ll be watching.

My life with Ullrich congenital muscular dystrophy

Back in October I promised that at a later date I would get to the ins and outs of how my condition affects me. I think it’s about time I do just that in order for you to learn more about me and the impact UCMD has on me personally.

Well, I have a rare, degenerative, genetically-inherited condition called Ullrich congenital muscular dystrophy. The congenital part means that it’s present from birth. UCMD is just one form of muscular dystrophy, of which there are many. In fact, Ullrich CMD is in itself only one subtype of congenital muscular dystrophy.

I must emphasise that what follows is my personal experience.

There are, according to the muscular dystrophy UK website, over 60 forms of MD and its severity is wide-ranging.

In recent years I have learnt that the come number of sufferers, worldwide, is much greater than I previously thought. I’m aware that my use of the word ‘suffer’will cause controversy as many do live happy, fulfilled, adventurous lives despite their disability. Nevertheless, it is a debilitating disability that I have most certainly found sufferable. Life without UCMD would doubtless be immeasurably less stressful, physically and mentally.

Having read accounts from others with MD I’ve realised that we are all individuals and therefore our experiences differ significantly. Despite what some medical professionals believe, there is no definable check list. For instance: “everyone with UCMD will be affected by ‘X’ at ‘X’ age and they will not live beyond 40.” No, we’re not robots and we do not all operate, function and malfunction in the same way.


A brief introduction to muscular dystrophy:

  • 70,000 people with MD in the UK
  • Very rare, affects 1-1000 people
  • Genetically inherited muscle wasting condition
  • Progressive
  • There is currently no cure for MD
  • There are many forms of MD – over 60
  • The most commonly recognised is Duchenne MD
  • UCMD is a type of CMD. It affects c.50% of the 400-500 people with CMD
  • UCMD is caused by the lack of a vital protein needed to support muscle cells.

 My experience:

  • Born with dislocated hips and ‘floppiness’. At 2 weeks of age I was put in plaster for 12 weeks to realign my hips. 20161130_211543
  • I didn’t crawl, climb or walk at the normal rate. As a baby and even into infancy I ‘bum/belly shuffled’ from around 7 months old. I only started to walk at 19 months old. My parents were very much aware that something was wrong and so they pushed for a specialist referral. My mother was told by her GP that she was an “over-reactive mother”. What an asshole!
  • 4 years old – muscle biopsy performed by Dr Helen Roper at Birmingham Heartlands Hospital. I was diagnosed with congenital muscular dystrophy. 20161130_213022
  • My parents were told very little; just that I would experience overall muscle weakness but mental ability would not be impaired. Doctors didn’t know if or how my condition would progress, nor if it would stabilise following puberty. They didn’t know if I would ever need to use wheelchair.
  • I wore specially made leg splints as a child enabling me to walk short distances, around the house and school.
  • For longer distances I used a ‘buggy’ or manual wheelchair.
  • I have never been able to walk up or down steps.

  • At age 7, I had my tendons released in both feet and ankles as they were turned inwards. Following the operation my feet were held in position in plaster casts for 6 weeks.
  • I stopped walking quite abruptly at age 10. At the time this was unexpected, shocking and incredibly distressing for both me and my family.
  • I became non-ambulant and started to use a powered wheelchair full time. We raised the money for my Jazzy Pride electric wheelchair through sponsored events, charitable donations, fundraisers and public appeals; my story was put in newspapers and school newsletters (this was pre-social media folks!). It’s amazing how generous people – strangers – can be.
  • I then stopped wearing the leg splints as I was no longer walking. I could finally wear nice shoes, yeah! A silver lining after all.
  • From around 11 years of age I began to wake every morning with debilitating headaches and nausea. This was due to a nocturnal build up of carbon dioxide in my body, having failed to effectively expire the waste gas during sleep.
  • From the age of 4, I had 30-60 minutes of physiotherapy once a week at school. This mostly involved stretches to maintain what flexibility I had, but it came to an end when I turned 14. Literally one week my physiotherapist was there, and without a word of warning, the next week she wasn’t. I was then told that if I wanted to continue with my physio, it would have to be carried out by my parents or a carer.
  • Following a second muscle biopsy at around the age of 18, my diagnosis was narrowed down to Ullrich congenital muscular dystrophy. At the time this really didn’t mean anything to me. I didn’t learn anything new and nothing changed. It wasn’t a case of; we’ve defined it as UCMD which means we can give you this treatment. There is no cure for muscular dystrophy. And so I just carried on with my life as I had been.20161130_212939
  • I attended mainstream school before moving on to a Sixth Form College where I completed A-Levels in Fine Art, English Language and History. I then attended a local university, commuting everyday via taxi. After three years I achieved a BA (Hons) degree in Art and English Literature.
  • I’ve always had scoliosis (curvature of the spine), although the severity progressed significantly after I became non-ambulant.
  • At around the age of 9 my parents and I were told I would need a spinal fusion to correct the scoliosis and prevent any further curvature. Again we were given little information, no case study to refer to and little time to make a decision. I do remember vividly how the seriousness of the operation was emphasised. In particular, “you could die” stuck in my infant mind. In the end, we decided not to go ahead with the spinal fusion.
  • Scoliosis affects posture, balance, respiratory function and causes pain, discomfort, pressure sores and asymmetry of the torso.
  • It is very difficult to find clothes that fit because of my lumbar deformity.
  • Because of the scoliosis my torso is squashed and so too are my organs. This means that I become full, bloated and breathless after small quantities of food.
  • I have contractures of the joints & tightening of the tendons in my ankles, knees, hips, elbows and wrists.
  • Due to the weakness in my neck I’m unable to lift my head from a pillow when lying flat.
  • I cannot sit myself up from a lying down position or support my own weight at all.
  • I’m unable to transfer independently, and at only 5.5 stone (77lbs) I’m usually lifted manually or otherwise hoisted.
  • I can’t turn myself in bed. However, I bought a satin bed sheet and wear silky pyjamas which allow me to slide myself from side to side. This means I don’t have to rely on someone to reposition me throughout the night.
  • Because my movement is limited and I cannot exercise, I have poor circulation and very cold hands and feet. Corpse feet as I refer to them.
  • It is difficult for me to regulate my body temperature.
  • October 2011 – I opted to have a suprapubic catheter for practical reasons. I have no functional continence issues. Purely due to the severity of my contractures, muscle weakness and inability to transfer, there’s no other way for me to empty my bladder independently. My SPC (suprapubic catheter) means that I no longer need the assistance of anyone else to carry out this personal task. Boys, don’t get me started – you don’t know how lucky you are!
  • I currently live with my parents in their house. I have a ground floor bedroom and ensuite bathroom extension which was built when I was 12 years old. My parents received a grant towards the cost but were means-assessed and so they had to extend their mortgage in order to fund the excess. Prior to that I had a bedroom upstairs which I accessed via a stairlift. Having stopped walking at age 10, struggling up and down stairs and on and off the stairlift became impractical, hence the need for a ground floor extension.
  • My parents are my primary carers, although I employ someone a minimum of 5 mornings per week to get me out of bed, washed, dressed and ready for the day. I also employ another personal assistant who transports me in my Motability wheelchair accessible vehicle (WAV) to social activities and appointments. Occasionally my parents need a break from home, and from me I’m sure. When they’re away, my carer attends in the mornings as usual and returns in the evenings to cook dinner. She will then sleepover in the spare bedroom upstairs in case I need anything during the night and for safety reasons. After all I’d be pretty screwed if the house set alight while I was alone in bed. I’m afraid I would have to just lie there and fry. Good job I like the heat eh!
  • As I have aged, my declining respiratory function has become the main cause for concern. Chest infections have become worse and more serious as a result.
  • A frequent inpatient, I have had pneumonia more than five times, pleurisy twice and a spontaneous pneumothorax (collapsed lung) which required a chest drain.
  • Following a particularly bad bout of pneumonia in March 2012, I commenced nocturnal non-invasive ventilation. I use a Resmed Stellar 150 biPAP machine with the Resmed Swift FX Nano nasal mask.
  • NIV (non-invasive ventilation) ensures that oxygen and carbon dioxide levels are normalised. I no longer suffer from associated headaches or nausea.
  • Medication and treatments: BiPAP machine (NIV), Salbutamol inhaler, I have antibiotics on repeat prescription to treat a chest infection, and I have a Nebuliser with saline and Salbutamol nebules which I use when I am ill.
  • I now find at 28 years old, I am physically exhausted all day every day. Fatigue and overall weakness is the norm, and it is getting worse.
  • Although my condition is progressive and life limiting, there’s no way to determine exactly when my time is up. No doctor in the land can predict what age I can expect to live to. As such, I prefer to put this to the back of my mind and try to live as ‘normal’ a life as possible. Whatever normal is!carrie-aimes-recent_with-permission




If you or someone you know is affected by muscular dystrophy, in particular UCMD, perhaps reading my account may be of some use. I firmly believe that sharing our experiences, and offering advice and support is invaluable. If, as a child, I had known others living with the same condition, maybe my life could have been enhanced with the benefit of shared knowledge and wisdom.

Doctors, though essential, cannot tell you how it feels or what day to day life is like. This is why I really appreciate MD-associated Facebook groups and the MDUK forum. People can talk candidly with others who understand exactly what they’re going through.


If you have any questions at all, feel free to ask.

And, if you liked this blog post, please do share!

Thank you

My Open Letter to Personal Care Assistants | Muscular Dystrophy Trailblazers

All my life I’ve required care, whether it be from family members, friends or paid employees. For over a decade now I have been hiring assistants to help me with an array of tasks, including personal care. I have always chosen to recruit my own staff rather than use agency workers. This has given me much more flexibility in terms of when, how and for the duration of time I use my PAs. It also means that I know exactly who will be providing my care, which is not always the case when going down the agency route. However, with this comes the added responsibility of being an employer, which in itself can be rather daunting and stressful.

I’m in the fortunate position of having been gifted the best family I could ever hope for. I do appreciate though, that not everyone has the invaluable support of relatives to rely on. For these individuals their only option is to pay others, often strangers, to assist with their needs. Like me, they might advertise, interview and hire independently, paying for their care with council funded Direct Payments (available in England, Scotland and Wales). Alternatively they may decide to use an agency.

For others though, in times of desperation, they have no choice but to leave their residence and spend time in respite care. I know of cases where young people in their 20s have been placed in nursing homes for the elderly, where staff have no knowledge or experience of their condition and specialist needs. Personally I can’t imagine such an experience and count myself lucky that I’ve never had to resort to this.

Over the years I’ve had several carers (or personal assistants) – whichever label you prefer. For the most part, I have found them through friends, associates or word of mouth.

Several months back my longest serving employee had to leave for personal reasons. It came as quite a shock but couldn’t be helped. She worked for me for eight years and had seen me at my worst and most vulnerable. She is a good friend close to my age, whom I trusted and relied on, and so the news of her resignation was somewhat distressing. Thankfully she was good enough to stay until her position had been filled, which she was under no obligation to do. Nevertheless, I was abruptly faced with the immediate and unavoidable task of advertising for her replacement.

I was under no illusion that finding someone who could and would meet my needs was going to be a simple endeavour. It certainly was not. I’ve been casually told social workers, who carry out my annual Needs Assessment, to simply advertise and hire, as and when needed. As if I’ll be flooded by pools of applicants to choose from. Then again, I guess these social workers have never had to find people willing to drag them from their pit every morning and get them ready for the day ahead. Trust me it’s no easy undertaking when job seekers are sadly too often put off to discover that personal care does in fact mean personal care!

I placed ad’s everywhere I could think of; online and locally. After several weeks of limited interest, I arranged interviews with each candidate in the hope that at least one would be suitable. Most were let downs, failing to turn up without notice or changing their mind after showing initial enthusiasm. My expectations were raised only to be shattered.

I was surprised by the casual disregard and lack of consideration from some of the applicants. I spent whole days at home waiting for interviewees to arrive, as if I have nothing better to do. Is it really that difficult to send a text message or make a quick call to say you cannot attend for whatever reason?

Time was ongoing and I was increasingly aware that I would have to find someone – anyone – as soon as possible. I live rurally in a town populated by less than 10,000 and so inevitably I wasn’t getting as much interest as I might if I lived in a city. This was an incredibly tense and stressful time for me.

Although my carer had said she would remain with me until a replacement could be found, I knew it was too much to expect her to stay as long as it was taking. I couldn’t be without the care I needed to live my life – to simply exist. Yet at the same time I couldn’t find anyone to provide this care. I was facing an almost impossible challenge.

In the end it was once again through friends of friends and frankly sheer luck that I found someone to take on the essential role. I won’t lie, it’s been a difficult transition and my daily routine has had to adapt. But, several months on, things seem to be coming together and all the initial doubts and struggles have been ironed out. I do still worry about the future prospect of having to go through the whole hiring process once again. It’s an unenviable task but one that is an essential and unavoidable part of life for those of us with a disability.

I realise it’s difficult for those applying for positions as PAs to empathise with our unusual and complex situation. If you’ve never needed care yourself it’s understandably difficult to grasp the necessity and importance of the role of caregiver.

For this reason, I have written an open letter to carers and prospective PCAs (personal care assistants). It has been published on the Muscular Dystrophy Trailblazers website. If you’re interested to read the edited version of my letter, click here.


Open letter to carers

On behalf of all of us who require personal or social care, I invite anyone considering taking on the role of personal assistant to think carefully about what it really means before you do apply.

Firstly, this is not a choice for us. We’re not, for example, hiring a cleaner because we’re too busy or too lazy to clean our own homes. When we advertise for carers, it’s because we NEED them and not necessarily because we want them.

As physically disabled individuals, many of us cannot independently carry out essential everyday tasks such as washing, dressing and toileting. To have no option but to entrust such intimate activities to another person – a stranger – is unnatural and unnerving. We are in effect placing our lives in your hands when you take on the vital role of personal carer.

Recruiting carers can be a lengthy and extremely stressful process for us. There’s the initial worry over whether there will be any applicants at all, followed by the dreaded interview process.

We often find ourselves waiting around for interviewees to attend, only for them to carelessly fail to show without any notification. Please do bear in mind that just because we are disabled, like you we have lives too, so don’t waste our time. We appreciate there are valid reasons for failing to attend job interviews, but it’s no hardship making a quick phone call or sending a text message to let us know in advance. As you would with any potential employer, be professional and courteous.

If and when we are able to successfully recruit, it can be incredibly frustrating and disheartening when that person flippantly decides to resign days later. You may wonder how and why this can happen but the sad fact is that for many disabled people it is a reality. We are not afforded the luxury of being able to manage until a replacement is found. No, we can’t simply wait for the right person to show up.

Some of us even have to resort to respite and residential homes in the meantime, thereby taking us away from our own homes and everything we hold dear. Try to imagine if you will, how demoralising and distressing such a situation would be if it happened to you. I therefore reiterate how important it is to think before applying for a role as a personal carer.

Are you trustworthy, reliable, willing and able? Ask yourself: are you entering this area of work for the right reasons? Your role will involve a range of tasks and you will be responsible for the safety and wellbeing of your potentially vulnerable employer. So, if your attitude to care work is casual and indifferent, then this is most definitely not the job for you!