Thursday 8th March marked International Women’s Day 2018. For me, it was spent in hospital (Russells Hall, Dudley) undergoing minor surgery. Could be worse, I suppose!
Due to the fact I have Ullrich congenital muscular dystrophy (a muscle-wasting condition), people are often horrified when I tell them how many general anaesthetics I’ve endured throughout my life (at least 10 – honestly, I’ve lost count!).
Although it is obviously best avoided, I’ve personally never encountered any problems or complications as a result of general anaesthesia.
I’m a big believer in knowing your own body and what you, as an individual, are able to withstand. When undergoing any form of surgery, communication is key – particularly when you have a disability.
A Few Tips:
Go prepared: Take all relevant documentation to your pre-operative assessment(s), including names and contacts for all the medical professionals you see regularly.
Meet with your surgeon(s) and anaesthetist: It is not always common practice to see your anaesthetist prior to surgery, but in my case it is essential. Explain your specific requirements and concerns, and don’t be afraid to ask questions.
Ensure everything is in place prior to your admission date: If, for example, you use a Bi-pap or C-pap machine (non-invasive ventilation), tell your medical team – nurses included. Make sure they know your settings and have your NIV machine ready for you to use post-operative.
I usually discharge myself on the day of surgery or, as soon as I know I’m well enough to manage at home (again, I hear you gasp). Anyone with a disability who has spent time as an inpatient will appreciate why I prefer to escape as soon as physically possible.
Don’t get me wrong – I cannot fault the care and conscientiousness of the doctors and nurses. I am a big supporter of the NHS and frankly, I would not be here today without it.
However, the sad fact is, hospitals in the UK are not equipped for those of us with disabilities and complex care needs. Trust me – having been admitted many times, to various hospitals, for various reasons – I am well versed!
On this particular occasion, it was necessary for me to stay in hospital overnight. My Mom was with me all day but went home at around 7pm when it became apparent that I was unfit to leave. This essentially left me alone and stranded in bed (one that didn’t work!), unable to move, reach or sit myself up.
I couldn’t and wouldn’t expect Mom to stay with me all night, in order to assist with my physical care needs. She herself has recently had a full knee replacement and was exhausted.
After a long, uncomfortable night spent clock-watching, I was incredibly relieved when my folks returned at 11am on Friday to take me home. My home is set up for my care needs. Unfortunately, hospitals are not.
It is a progressive, muscle-wasting condition caused by mutations in the COL6A1, COL6A2 and COL6A3 genes.
It is typically inherited in an autosomal recessive pattern (both parents are carriers of the mutated gene). However, in rare cases it can also be inherited in an autosomal dominant pattern (where only one parent has the affected mutated gene).
It frustrates me that so few people, medical professionals included, have heard of Ullrich congenital muscular dystrophy. In my experience, those who are familiar with muscular dystrophy tend to associate it with it Duchenne (the most well-known form).
Many people look at me now – a non-ambulant wheelchair user – and assume that I have always been this way (ie. unable to walk). This is not the case.
In order to raise awareness and familiarity of UCMD, here are a few photos of me growing up with this sadly unrecognised condition.
Above and below: My first wheelchair (manual). Prior to this I used what we, as a family, referred to a “buggy”. At this stage, I was able to walk short distances whilst wearing leg ‘splints’.
Below: In this photo I am around 11 years old. I loved this wheelchair (a manual, Quickie) as it was a sleek, black and purple design.
At age 10, I became unable to weight-bear. My muscles were simply unable to support my growing frame. It was therefore important to find a wheelchair that was comfortable enough to use all day long, whilst also looking half decent!
As you can see, the push handles on this chair were higher than average as all members of my family are tall. You wouldn’t think so, looking at me would you!
I always disliked the unusually high push handles (see above) as they stuck out above my head and were an aesthetic distraction.
Below: My next wheelchair – again a manual. I was unable to self-propel due to elbow contractures and muscle weakness.
Throughout my school years, I always used a manual wheelchair. This is one of the main reasons I hated school so much, since I was reliant on others to push me around. Wherever I was put, I stayed. It was incredibly frustrating.
Below: My Quantum F45 powered wheelchair (this model is no longer in production).
A relatively light-weight, rear-wheel drive with a narrow base, this chair served me well for many years.
This was in fact my second power chair. My first was a Jazzy Pride (front-wheel drive), which was great outdoors. Unfortunately I can’t find any photos to show you.
My Jazzy Pride wheelchair was purchased through public fundraising when I was 10-11 years old. At that time, there was just no way my parents could afford the cost of a powered wheelchair. Our local wheelchair services could not (or rather, would not) provide me with one.
Below: This is my current wheelchair – a Quantum 600, which I have had for almost 8 years. It is mid-wheel drive and VERY heavy!
I have to say – though it is a solid, sturdy chair – I wouldn’t replace it with the same make/model. Unlike my previous powered wheelchairs, it has let me down unexpectedly on various occasions and required quite a few pricey repairs!
It is rapidly falling to bits (literally) and most concerningly, the electrics are now failing. For this reason, I am currently on the lookout for a new chair.
These days, I primarily use a powered wheelchair rather than a manual chair, as it allows me greater independance and freedom of mobility. However, I do also own a Küschall Ultra-Light manual chair, mainly as a backup.
If you have found this blog post useful, I would be grateful if you could share to help spread awareness of Ullrich congenital muscular dystrophy.
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.