My Quantum 600 powered wheelchair, which has been my legs for almost 8 years, is gradually falling to pieces. I have patched it up no end with DIY repairs, and attempted to keep it going for as long as possible. But the electrics are now failing and so the chair is becoming unreliable. Consequently, I have no option but to start the search for a replacement.
As the wheelchair-users out there will know, this is never a simple task! It is a BIG decision, not least because wheelchairs are so ridiculously expensive. More so than a new car!
Throughout my life, I have had no choice but to privately fund all my wheelchairs – both manual and powered – since those offered by wheelchair services are wholly inadequate for my needs (and I suspect, most people’s).
So before committing to a purchase, I need to be absolutely certain that the wheelchair I opt for will be the right one for me.
My new wheelchair must:
– Have rise and tilt
– Be as compact as possible for indoor use
– Be durable outdoors as I live rurally
I have an appointment with my local wheelchair services on Friday 16th February. So I’m hoping they will be able to offer some useful advice and guidance, along with a voucher towards the cost.
A representative from Motus Medical has already visited my home to demo two mid-wheel drive (MWD) chairs:
– The Quickie Salsa M2 Mini – The Quickie Jive M
I found the Quickie Salsa M2 Mini to be an ideal size (the base is only 52cm wide, with a turning circle of 110cm). However, when tested outdoors over gravel and uneven terrain, it did not perform particularly well.
The Quickie Jive M was too large for the contours of my home (overall width 62-66cm). Furthermore, I felt that it didn’t compare well with my current Quantum 600 in terms of outdoor ability.
So that’s two tried, tested and crossed off the list!
I will continue to keep you updated, following Friday’s appointment.
*Disclaimer* Please be aware, this is in no way an official, definitive guide for parents of children with neuromuscular conditions. Here I offer support and advice along with my personal perspective, having lived with Ullrich congenital muscular dystrophy for 29 years.
– I am a UK based disability blogger
– I have Ullrich congenital muscular dystrophy
– Born in 1988, I am now 29 years old
– Born with ‘floppiness’ and dislocated hips
– Slow rate of progression: I didn’t start to walk until 19 months old and ‘bum-shuffled’ until then
– I was able to walk short distances, wearing custom-made leg splints, until age 10
– I would fall over a lot as a child
– I could never walk up or down steps or stairs
– I am now completely non-ambulant and use a powered wheelchair
– I have contractures in all joints
– I have a severe ‘S’ shaped scoliosis (curvature of the spine), which has not been surgically corrected
– Over the years, my condition has deteriorated
– As an adult, my primary concern is respiratory-related
– Education: I attended mainstream school, achieved A-Levels and a degree with Honours.
I would first like to say, a life with muscular dystrophy need not be an unfulfilled life! Many people with neuromuscular conditions lead full and active lives, whilst pursuing demanding careers.
Though severely disabling, muscular dystrophy is not synonymous with suffering or a poor quality of life.
It can, of course, be shocking and extremely distressing to learn your child has a muscle-wasting condition. In some cases there may be a family history, whereas for others like me it could be completely unexpected and unfamiliar.
I am the only member of my family with any form of muscle-wasting condition. So when I was initially diagnosed, aged 4, with congenital muscular dystrophy following a muscle biopsy, my parents were venturing into the unknown.
The year was 1992, there was no Internet, no Google, few resources and more to the point – they had never even heard of muscular dystrophy!
It may be helpful to talk to other parents of children with a similar condition. If this is something you think would be beneficial for you, ask your care advisor or neuromuscular consultant who will assist.
If you are not ready to speak to other families, remember this is always an option if you change your mind in the future.
How and when to tell your child
There is no right or wrong answer. Only you can decide what is best for your child. It is also important to consider siblings, as they should be included in any discussions you have as a family. However, I would strongly advise against telling siblings more about the condition than the affected child. Any information you choose to share with your children should be fair and equal, regardless of the gap in age.
When deciding if, when and how to tell your child of their diagnosis, consider:
– Their age
– Their own awareness of their disability – Emotional maturity
– Your ability to answer any questions they might have
From a personal perspective, I recommend an open and honest policy from the very beginning.
I cannot recall any formal discussion or disclosure of my condition, whereby my parents sat and explained the ins and outs of it all to me and my older brothers.
I was officially diagnosed with congenital muscular dystrophy at age 4 (specified as Ullrich CMD many years later), but I was always aware of my disability. I recognised that I was different from my peers – I was unable to walk, run and climb steps. I also looked different – I was very thin as a child, I had contractures and scoliosis causing asymmetry of the torso.
My parents told us that I have something called muscular dystrophy, meaning I have much weaker muscles than other children. At that age, this was enough knowledge for me.
For many years, when other children would ask the inevitable question, ‘what’s wrong with you?’
I would simply answer, ‘I’ve got muscular dystrophy so I can’t walk like you’.
Whenever and however you decide to approach this, I would urge you to emphasise above all else that your child is loved and supported by you and your family. Sometimes the comfort of a loving hug from your parents is all the reassurance you want and need.
– Do your research: Unlike when I was a child, these days there are many reliable resources of information. Of course, ask any medical professionals your child is seeing, including consultants, physiotherapists and occupational therapists. However, I will say that not all children with the same form of muscular dystrophy will experience exactly the same symptoms or at the same rate. For this reason, I recommend talking to those directly affected, such as other parents and adults with muscle-wasting conditions who, like me, have a lifetime of experiences to draw from.
– Always trust your instincts! As the saying goes, a mother knows best. This has certainly proved true in my case. My mother, noticing my delayed progression as a baby, sought medical help and was told she was an “over-reactive mother”. Thankfully, she persisted and met with a neuromuscular specialist who almost immediately identified my condition.
– Ensure your child receives the Flu vaccine EVERY year, as well as the pneumonia vaccination. You, as primary caregivers, will also be eligible for the Flu vaccine – take it!
Some children are diagnosed well before they reach nursery age, whilst others like me, will have already started school when they learn they have a muscle-wasting condition.
– Starting school can be daunting for any child: Being parted from their parents, away from home and surrounded by lots of unfamiliar faces. But for a child with a disability like muscular dystrophy, this transition can be even more challenging.
– Particularly in this day and age, there is no reason why a child with a muscle-wasting condition shouldn’t attend mainstream school. However, you must consider your child as an individual and decide whether or not you feel this would be best for them. Indeed, your child may have a preference. Some would rather attend a school for special educational needs.
– I suggest making several visits to any nurseries or schools you have shortlisted. Check that everywhere is fully accessible. One visit is not enough as we often forget to ask certain questions and fail to spot things on first impressions.
– It is essential to ensure staff are fully aware of your child’s needs and abilities. Meet with the special educational needs co-ordinator (SENCo) well in advance and if possible, meet and introduce your child to their new teacher and *teaching assistant/support worker (*where applicable). This will help them feel more comfortable and confident when their first day of school arrives.
– Ask your child’s physiotherapist and/or occupational therapist to visit the school and meet with staff to share their knowledge and expertise.
– Be positive, be optimistic, be encouraging. BUT don’t make promises you can’t keep: As your child grows and interacts with other children, they will become increasingly aware of their differences. This will inevitably lead to questions. They may ask why they can’t stand, walk or run like their friends. Furthermore, if your child has a progressive form of muscular dystrophy, they may ask you if their symptoms will get worse as they get older.
For instance, I was able to walk short distances until the age of 10. We were never told if I would continue to do so as I grew. Therefore, I would often ask, ‘will I always be able to walk or will I have to use a wheelchair?’
I appreciate why many parents would be reluctant to overshare and reveal what the future may hold in terms of deterioration, in fear of scaring their child. However, I feel strongly that it is important to be honest and unambiguous. If you don’t know the answer, say you don’t know. Don’t try to comfort your child by telling them it will all be okay and things will not get worse. Knowledge and preparation is power!
– For most children with muscular dystrophy, the biggest changes occur during puberty.
– Growth spurts put extra strain on weakening muscles.
– If able to weight-bear, your child could lose this ability resulting in the need for a wheelchair.
– It is likely that your child will accept having to use a wheelchair more than you. Children are very adaptable. From personal experience, I can tell you that using a wheelchair is a great relief compared to exerting all your strength, energy and reserves on standing and walking. A wheelchair offers mobility and freedom. So please keep this in mind.
– Growth also leads to contractures becoming more severe. Regular physiotherapy (ie. stretching) will help maintain flexibility and movement.
– If untreated, spinal curvature (scoliosis) will increase resulting in asymmetry of the torso, a tilted pelvis, pressure sores and discomfort.
– Scoliosis, along with muscle deterioration, impacts on respiratory function. Should your child feel overly tired, nap during the day, or experience regular headaches, particularly on first waking, you must see a specialist respiratory consultant. It might be necessary to introduce noninvasive ventilation nocturnally.
– Noninvasive ventilation (NIV) comes in the form of either a CPAP or BiPAP machine (usually the latter).
– Bilevel positive airway pressure (BiPAP): a face mask is worn (many different styles are available) and air is delivered from a machine through a tube to support breathing.
– Other respiratory equipment your teenager may benefit from, especially when ill: Nebuliser and cough assist machine (seek advice and information from your respiratory consultant and respiratory physiotherapist).
Other Family Members
Some forms of muscular dystrophy are inherited. This can obviously be a concern if relatives are planning families of their own.
A few years ago, my brother and his wife decided they wanted to have children. Because of my condition, he asked his GP to refer them to a genetic counsellor. They took with them a copy of my medical record.
In their case, it was determined that my brother is highly likely to be a carrier, while his wife is highly unlikely. Once pregnant, they were offered a test which involved inserting a needle into the developing embryo to ascertain if the baby would have muscular dystrophy. They decided against this due to the invasive nature.
I’m happy to report they now have a happy and healthy baby boy.
How to cope
As a parent, it is understandable that you will focus all your thoughts and energy into your children. Raising a child with muscular dystrophy brings with it many more challenges and emotional turmoil. This can have a huge affect on you, your health and relationships. It is therefore crucial to take care of yourself too.
Remember, it is okay to cry, scream and shout! There will be times you struggle, lose your patience and indeed you will have to fight your child’s corner in different situations.
It’s good to talk: whether you choose to confide in relatives, friends, fellow parents of disabled children, or healthcare professionals – don’t bottle up your concerns and frustrations. This will ultimately have a negative effect on your wellbeing and your family.
Make time for yourself:‘what time?!’ – I know, I know. I appreciate that caring for a child with any disability is a full-time job. But you must allow yourself some form of relief. Even if it is just dedicating yourself to an hour or two with friends for coffee each week. You need space. Your child needs space. It will be good for all of you – trust me!
If you have any specific questions, please leave a comment or contact me directly. I am more than happy to talk privately.
If you like this blog post, I would be grateful if you would share so that others may learn from it.
As some of you may know, my very first ceiling track hoist was *finally* fitted on Monday 11th December.
I now have a straight track in my bedroom and a separate H-frame in my ensuite bathroom.
Why I need a ceiling hoist
I am 29 and completely non-ambulant due to Ullrich congenital muscular dystrophy. Being rather petite, I have always been manually transferred (yes, lifted by family and carers) rather than hoisted. This method has always been preferable since it’s much quicker and frankly less faff. But, we’re all getting older and more frail.
I live with my parents who are both in their 60s. They are my primary source of support, though I do employ a carer part-time. My mother underwent a full knee replacement in August 2017, and is therefore limited in how much she is able to help me. Consequently, we have reached a stage where a ceiling hoist is a necessity.
A long and frustrating process!
Back in October 2016, Mom was told she needed a full knee replacement. The following Spring, my only carer announced she would be leaving within the next few months to pursue a career as a paramedic. With this in mind, I contacted my local community occupatinal therapy team to request an assessment. I was told they’re vastly understaffed and, with an extensive waiting list, I would need to be in a terminal condition in order to be seen. I appreciate their predicament, I really do, but I was unwilling to be fobbed off so easily.
I was instructed, over the phone by an OT I had never met, to “camp out”, meaning I should wash, dress and be toileted on my bed. Yes, for a prolonged and indefinite period of time, I should go without a shower and simply not wash my hair. (Due to my physical limitations and my wheelchair, there’s no way I could wash my hair over the sink).
Disgusted at her casual disregard, I asked my neuromuscular consultant to issue a letter of support. On receipt of this, an OT suddenly found time to visit me in my home for an assessment. Following this, representatives from Prism Medical and TPG DisableAids attended separately to advise, measure up and draw plans. Both rep’s then submitted quotes to the purse holder at County Council who, of course, approved the cheapest option.
NB: A portable hoist was trialled but proved unusable with the layout of my room and the type of bath in situ.
We were expecting Prism to arrive at 9am on 24th October 2017, as arranged. Having waited over an over with no sign of anyone, I called only to be told they weren’t coming because of a “technical issue”.
To cut a long story short, Prism claimed they couldn’t connect the track from my bedroom to that in my ensuite bathroom. This is despite consulting with occupational therapists and agreeing to do the job. Prism also claim they left telephone messages for both myself and the OT’s, on the previous Friday, to inform us that they wouldn’t be attending. Neither I, nor the community OT’s received any messages. I call bullshit!
Later, I learnt that Prism have similarly disappointed several others, resulting in formal complaints being issued against them. So when the purse holder at County Council told me she would renegotiate with Prism rather than approve funding for TPG to carry out the work, I insisted otherwise.
Having to fight for your rights and basic needs is, unfortunately, very much part and parcel of having a disability. ‘Tell, don’t ask!’ This is my motto. In my experience, if you are not clued-up and assertive, those in authority simply fob you off.
Thankfully, Funding was approved after a different OT, accompanied by the rep from TPG, visited to discuss and re-evaluate the situation.
Rather than trying to connect the single rail in the bedroom to the H-frame in the ensuite bathroom, it was decided that two separate hoists would be best.
My carer had by then handed in her notice and would be leaving at the end of the month. I was seriously starting to worry the hoist would not be in place before Christmas.
But much to my relief, TPG (who, compared to Prism, were infinitely more professional and efficient throughout) booked in for the 4th December.
Then, just my luck, we were hit by the worst snow in 7 years! It was like flipping Narnia.
I tried to remain optimistic though in reality I knew there was no way TPG would be able to make the journey from Hereford. And they didn’t.
So, it was third time lucky, on the following Monday that the long-awaited ceiling hoist was installed. I no longer need to worry about hiring new carers as lifting is not an issue. Furthermore, the pressure is off Mom – literally! And, the thing I am perhaps the most happy about – my dealings with community OTs and the County Council are over.
For now at least…
(Apologies for the poor quality of the images. All were taken by myself on a Samsung S5!)
As promised, here are the developments following on from my previous life update…
Having re-advertised for a part-time carer to replace my current PCA, I interviewed seven applicants, plus one who’s interested in ad-hoc cover. All were enthusiastic, though as any employer will know, things often change in the days that follow.
There was only one no-show which actually isn’t bad at all compared to my previous attempts to recruit new carers.
One of the seven ladies later asked to be a backup as she decided she couldn’t do every weekday. From the six remaining, I invited three to shadow, knowing that at least one would change their mind. I was right; they did.
The first was a no-show (yes, another)! After I contacted her to ask if she was going to attend, she simply replied, “I forgot”. Needless to say she was scrubbed from the list.
Number two messaged me a few days before shadowing, to say she had reconsidered and felt there weren’t enough hours. This left me with one applicant.
Thankfully she did show up. Seemingly reliable and keen to take on the role, I offered her the job on a trial basis from Monday 30th October.
My current PCA is leaving in December to pursue a career as a paramedic. So, fingers crossed all goes well with the newbie…
Ceiling track hoist
To my surprise, I was contacted a couple of weeks ago by Prism Medical to arrange a date for installation. Finally, after waiting for so long and constantly pushing the matter, I would get the much needed ceiling hoist on Tuesday 24th October.
In preparation, my Dad had to remove the glass intersection above my bathroom door to allow through-access.
When the day arrived, we cleared the room and waited for the workmen to arrive, as expected, at 9am. An hour later and still no sign. Becoming impatient, I called Prism Medical but was told they wouldn’t be coming due to a technical issue.
What?! What technical issue?
Prism Medical claim they left a voice message, on the previous Friday, explaining they couldn’t connect the single rail going from my bed to the bathroom door, with the H-frame in the bathroom. We received no voice message. They also claim to have contacted the Community OT’s. They too have had no calls or messages from Prism.
A rep from Prism previously visited my home to take measurements, draw up plans and provide quotes. They then corresponded with a Community OT (or so I’m told) and agreed to install the ceiling track hoist. Yet despite all this, they have suddenly decided they’re unable to carry out the work. Excuse my language, but what the actual fuck?!
As you might expect, the remainder of Tuesday was spent on the phone: trying to get hold of an occupational therapist, complaining to and about Prism Medical, and negotiating with County Council Equipment Services.
I’m hoping to get funding approval for TPG to do the work, as they too had sent out a rep to provide a quote.
Naturally the County Council opted for the cheaper quote from Prism. That’s worked out really well, hasn’t it!
Today is Thursday 26th October. There has been no notable progress since Tuesday. Essentially, I am back to square one – harassing the OT’s daily to ensure my case is not ignored. Unless you constantly pursue the issue yourself, frankly nothing happens.
I am so angry and disappointed with the whole cock-up, though sadly not overly surprised. In my experience, unless you’re prepared to self-fund, this is the service (or lack thereof) you can expect!
I will be putting in a formal complaint and am seriously considering writing to my local MP. If we allow companies and organisations to get away with such failures and blatant disregard, nothing will change.
So, once again the saga continues. I will keep you updated – *Keep an eye on Twitter and my Facebook page*
Thank you so much to each and every one of you who has offered advice and support!
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.
It’s now been one year since my blog, Life on the Slow Lane, was created. I finally bit the bullet on 5th October, with my first (proper!) post going live on 11th October.
As you can see from that first shabby entry, back then I lacked a specific objective. I just wanted to get going after contemplating blogging for many months prior.
Over time I have (I hope) managed to focus the aims and purpose of my blog.
I now concentrate primarily on disability issues, specifically those relating to muscular dystrophy. Here I share my thoughts and experiences, having lived my entire life with Ullrich congenital muscular dystrophy.
Life on the Slow Lane has enabled me to interact with many other disability bloggers. The amazing Simply Emma generously invited me to guest-blog for her, which I did in May and July. Then more recently I collaborated with the lovely Gemma over at Wheelescapades.
My blogging highlight of the year
A particular highlight for me was the overwhelming response I received for the piece I wrote about my life with UCMD. The fact that it is an especially personal and exposing piece makes the positive feedback all the more touching. To date, this, my most popular blog-post has been viewed over 11,700 times.
Admittedly, it’s been challenging and time-consuming. I have so many thoughts and ideas, yet not enough time to execute them all.
I often feel like I’m playing catch-up, mistakenly comparing myself to other much more established disability bloggers.
I’m not a big fan of computers and otherwise avoid them if at all possible. Initially, I had no clue how to even set up a blog, let alone edit and customise one.
I realise many of my peers are adept with design software, graphics and managing websites. But I’m just not. Computers are in no way of any interest to me. I’m old school! So this, along with social media, is something I have had to familiarise myself with.
Blogging goals for the next year
• Continue to upload regular posts
• Reach a wider audience and increase my readership
• Raise awareness of Ullrich congenital muscular dystrophy
• Meet other like-minded disability bloggers
Thanks to you
I’d like to thank each and every person out there who has read any of my articles. I truly appreciate your feedback and support!
As long as Life on the Slow Lane attracts an audience, however small, I will continue to write.
I feel like it’s been a while since I blogged about the goings-on of my day-to-day life. Not a particularly exciting post, granted. But I thought it might be useful to share these ‘goings-on’ with you, as I’m sure there are some of you facing similar struggles.
I have for the past few months been occupied with life crap – specifically, disability-related life crap – which has meant that blogging has unfortunately had to take a backseat.
Righty right, I’ll try and keep it brief…
As some of you may know, I live with my parents who are my primary source of support. I do have a part-time carer who I employ, but otherwise my Mom (yes, I say Mom vs Mum) is my main caregiver. Sadly she herself suffers with progressive osteoarthritis, and following exploratory surgery in October, it was decided that she needed a full knee replacement.
This in fact took place on Sunday 20th August, although it wasn’t until a couple of months ago that Mom was given a date for surgery. However, prior to this I had to put in place provision for my care needs. This involved recruiting a second carer and ensuring I have all the equipment I would need.
For the past 4 months I have searched for a second carer. I advertised everywhere and anywhere – newspapers, news agents, local shops and the post office, job sites, Facebook and so on. The response has really surprised and frustrated me – so many no-shows, let downs and people failing to read or understand the basic job specification.
I ask very little of applicants. I don’t request references, qualifications, experience or even a CRB/DBS (criminal records check). I interview informally in my own home, and with employees I am flexible, easy going and more than fair, taking into consideration their individual circumstances.
However, despite the fact I am completely non-ambulatory, I have never used a hoist. Thus far, family and carers have always preferred to lift me manually as it’s much quicker and frankly less faff! I’m only tiny – approximately 5 feet tall and 5.5 stone in weight. So until very recently, it has always suited to go without a hoist.
Understandably this is off-putting to potential applicants. But, every carer I’ve ever employed has openly admitted that working for me is a breeze compared to any other job they’ve had, and that for them the lifting is a non-issue. Nevertheless, I appreciate that most would prefer not to lift – that’s fair enough.
With this in mind, I instigated the process of applying for a ceiling track hoist to be installed in my ground-floor bedroom/ensuite bathroom. I will need a H-frame in the bathroom and a short track from my bed to the bathroom.
Not a huge ask really, particularly as I have never received any support in the way of equipment. Everything I have – wheelchairs, bed, bath lift etc. has been self-funded. The post-code lottery is a very real and unjust thing, people! But that’s a topic for another day…
Dealing with Community Occupational Therapists
I contacted the community Occupational Therapists, explained the situation and requested a needs assessment. I was initially fobbed off with the excuse that they’re vastly understaffed and that I would need to be terminally ill in order to qualify. When I asked how they suggest I manage after Mom’s operation, the OT replied that I should “camp out” and be dressed, bathed and toileted on my bed!
Disgusted at her casual disregard, I asked how she would feel having all her personal care needs carried out on the bed she sleeps in. “Oh well, this is the situation we’re in. It can’t be helped”, was her insensitive response.
I then contacted my neuromuscular consultant who wrote a letter of support. On receipt of this letter, the OT’s suddenly found time to carry out a needs assessment in my home – shocker! (It’s not what you know, but who you know, right!?)
Following this, two reps – one from TPG, the other from Prism Medical – came and measured up in order to provide quotes for the ceiling hoist. I have since learned that the second quote is unusable, which frankly is no surprise, as he clearly had no clue what he was doing; at one point asking to see the gas meter. Even the OT who accompanied him questioned his experience.
In the meantime I have been issued with a portable hoist, though it has taken many weeks to receive a usable sling. Rather than measuring me, then visiting me in my home with a variety of slings to try, the OT’s insisted on sending one at a time. After much harassment from me, a community OT finally conceded and actually attended to properly assess me for a sling.
Honestly, they complain that they have a backlog of work and no time, and yet they waste so much. The sling issue could have been carried out in one appointment. Simple, done, move on. But instead, they chose to drag it out for weeks simply because they wouldn’t visit or listen to the patient.
Today is Sunday 10th September, and no further progress has been made with the ceiling hoist. Yet again I will have to chase the OT’s, otherwise nothing will ever happen. Sad but true.
I had taken on a second carer who began shadowing at the beginning of August. She was very enthusiastic, supportive and accommodating – said all the right things. Then whilst on my way to visit mom in the hospital, two days after her surgery, I received a message from the new carer, who was due to work that evening. She issued a stream of excuses as to why she couldn’t (translate: wouldn’t) do the job.
Until then, my current carer had always been present. Essentially it turns out she was happy to come and get paid to watch someone else do the job. She just didn’t want to have to do any work herself. Now I know why she’s had so many jobs!
So, as it stands I am managing as best I can with my one part-time PCA, though she is planning to leave in late October to train as a paramedic; thus posing yet another obstacle.
Having realised how long this post is, I think I will leave it there for today, though there is much more to tell. Suffice to say, the saga continues…
The important issue of independent toileting is often discussed within the disabled community. I regularly see the topic arise on social media.
As a wheelchair user, this is something I have struggled with my whole life. Believe me I have tried every method and contraption available.
In 2011, after careful consideration, I opted for a suprapubic catheter. Following many requests for information and advice, I have written about my experience. As it is a sensitive and personal subject, I have decided to attach the file below rather than to upload as a regular post.
*Disclaimer* This is my experience and in no way represents that of any other. Your individual circumstances will invariably affect the way in which your body responds to a suprapubic catheter.
As a precursor; I have Ullrich congenital muscular dystrophy. I am now 28 years old and unable to weight-bear. I have full sensation and an otherwise healthy, fully-functioning bladder. I have never suffered from urinary tract infections. My reasons for choosing a suprapubic catheter are purely practical.
If you require further information or wish to ask a question, please do contact me.
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.
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
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.
Born with dislocated hips and ‘floppiness’. At 2 weeks of age I was put in plaster for 12 weeks to realign my hips.
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.
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.
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!
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!
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!