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Points for Reflection

This storyteller relates tales of some of the poor, unthinking, perhaps even unethical, treatment she has received over the past forty years. From being given inappropriate treatments that caused physical damage when in childhood, to perhaps well-intentioned but unthinking attitudes in adulthood.

There are three variants of spinal muscular atrophy that affect children, differentiated as Type I (severe Werdnig-Hoffmann type), Type II (intermediate) and Type III (mild, also known as Kugelberg-Welander disease). It is possible that the clinicians at initial diagnosis assumed incorrectly that this client had Type I, in which children rarely survive past aged two. In fact, her symptoms would suggest Type II, as Type III usually onsets in adolescence. Are there instances in your professional practice of different variants of a disease that have different prognoses?

Activities

1. This storyteller argues that training in ethics and disability awareness should be part of the pre-registration curriculum for health care professionals. It is the case that some higher education institutes include disability awareness training in their courses, and there are several post-registration courses available in ethics and disability awareness. In your place of professional practice, do you know of any training in ethical issues and/or disability awareness that you could attend if you wanted? Are there policies in place that address these issues?

2. If you don't already know, find out what is the legal situation in the UK regarding care and treatment of clients with a physical disability?

3. There are two examples of unethical behaviour in the story when medics refused to see the client. If you were a nurse on duty, how would you act if the medical staff refused to treat a patient in these circumstances? What is your professional obligation according to your Code of Practice?

4. Further reading: In “Jennifer’s story” Anita Macaulay describes the difficulties the family went through before getting a diagnosis for their daughter (Type 1, SMA); how she was labelled a ‘neurotic mother’ and that some healthcare staff refused to see them In: Marteau T, Richards M (eds) The troubled helix Cambridge University Press,Cambridge, p39-43

Quotes

“On our return to Manchester a helpful local medic even suggested putting me in a home, forgetting about me and starting again.”

“The surgery went well but two days later a careless, young nurse broke my leg by attempting to straighten it. Nobody had ever taught her that some people have joints which don’t flex the way they should.”

“Assumptions about disabled people abound, for example, that we can’t possibly be in a sexual relationship: I have had dozens of x-rays throughout my life but never once have I been asked if I could be pregnant (despite being married).”

Further information

The Jennifer Trust for Spinal Muscular Atrophy: http://www.jtsma.org.uk/

US National Library of Medicine, Genetics Home Reference - spinal muscular atrophy.


Location: The Stories
Transcript

You can download the full transcript here

Nightmares from a disability perspective
Suzy’s account of living with spinal muscular atrophy

I was born in Manchester, in 1965. It was apparent to my mother, almost from the beginning, that something was amiss with her new baby. Doctors repeatedly insisted that nothing was wrong and implied that my mother was fussing. As I grew older and failed to roll over, sit up or crawl it became obvious that there was, indeed, something wrong. The NHS was at a loss to explain what. By the time I was two my parents were so desperate that they took out a second mortgage to pay for tests to be run on me as an inpatient at a children’s specialist hospital. I was diagnosed with spinal muscular atrophy (SMA). My parents were told that I probably wouldn't live beyond the age of three and were sent home with no further advice or support. On our return to Manchester a helpful local medic even suggested putting me in a home, forgetting me and starting again. It was then that many years of inappropriate treatment began.

I was referred to the local hospital for weekly physiotherapy. The hospital staff had never heard of SMA and attempted to treat me with the same techniques used on children with cerebral palsy, in whom the hospital specialised. This treatment was so inappropriate, painful and humiliating that by the age of three I used to scream hysterically every time I saw the hospital gates.

When I reached school age I was transferred to an orthopaedic doctor at another local hospital. He had me put in callipers and tried to get me to walk which was painful and upsetting as I repeatedly fell over and it was destined to fail. However, I had a regular physiotherapist who got to know me and with whom I was happy. Unfortunately, when she was on leave, a much less sympathetic, though much more senior and experienced, physiotherapist took over my case. When she overstretched my achilles tendons and I screamed in pain she accused me of being “naughty” and said that if I didn't “shut up” she would “smack” me. I screamed and pleaded but she was convinced I was throwing a tantrum and she carried on until my tendons were so badly torn that my ankles had to be splinted for over a year.

Around this period, about the age of seven, the spinal scoliosis (characteristic of SMA) began. I was prescribed a range of moulded, wraparound spinal supports. As I grew, my spine collapsed further and it became obvious that the back supports were having no effect. My parents looked around to see if any better treatment was available. Somebody recommended an orthopaedic surgeon based at a hospital in Liverpool. He was very keen on Milwaukee back supports and prescribed one immediately. I have since been told that these work on able-bodied youngsters by “encouraging” the person wearing the support to stretch up thus exercising the spinal muscles and correcting the scoliosis. As I didn't have the muscles to do this, I slumped into my support and it caused painful pressure sores but, despite this, and the fact that my scoliosis continued to worsen, the Professor was convinced that this was the right treatment.

When I was 9 I broke my leg. My parents took me to the accident and emergency unit in Stockport. My old doctor was on duty. He was so piqued that my parents had taken me to a different specialist that he refused to see me. Nobody would examine my broken leg until the shift changed and a different doctor came on duty. His ego was so enormous that I was left waiting, aged 9, with a fractured femur, for several hours without so much as an aspirin.

Twelve hours after arriving in casualty I went down to theatre and my leg was set. I was put on a general medical ward where the nursing staff had no idea how to look after a disabled child. I was not given any help to eat or drink and I became badly dehydrated. I developed a massive kidney infection and became quite seriously ill. My excellent GP had the good sense to get me out of the hospital and back home where he and my mother could look after me properly.

Less than a year later I had to be rushed to hospital in North Manchester, with acute appendicitis. My appendix was removed by a surgeon who was very caring and took great interest in all her cases. The surgery went well but two days later a careless, young nurse broke my leg by attempting to straighten it. Nobody had ever taught her that some people have joints which don't flex the way they should.

My old orthopaedic surgeon retired and was replaced by someone keen on surgery. He tried to persuade my parents that it was in my best interest to put me on traction for six months and then operate to straighten my spine. My parents were very worried about this as they knew that my muscles would waste during the traction. They got an appointment for me to see someone in Newcastle who specialised in neuromuscular impairments. He warned against this course of action. However, the orthopaedic surgeon told my parents that if I didn't have the operation my hipbone would press into my ribs so badly that my ribs would break and puncture my lung. Horrified, my parents consented to the operation. By this time I was terrified.

I was admitted to hospital and forced to undergo “medical photography”, stripped to the waist, for “before and after pictures” of my spinal deformity. This was a very traumatic experience for a girl of just 14. A “halo” was fitted to my skull to provide an anchoring point for the traction. Unfortunately, during this procedure, I collapsed in theatre. I woke up in intensive care on a ventilator. The nurses on that unit refused to look after me as they were only used to the “hole in the heart” babies in which the hospital specialised. An off duty nurse from my ward volunteered to give up her free time and look after me.

After this disaster, the surgeon spent a week deciding what to do with me. In the end, the halo was removed and I was sent home. When I went to see the professor for my regular, six-monthly check-up, a few months later, my family and I were kept waiting for eight hours until the clinic was over. He refused to see us. He walked right past us in the hospital corridor and would not speak when we tried to ask him what was going on.

As I grew up my problems with NHS doctors became less specific and more a matter of general ignorance. Medical staff do have an annoying tendency to handle disabled patients without first asking permission and consulting-rooms are often filled with extra (anonymous) staff and students without anyone asking if we'd prefer a little privacy. Assumptions about disabled people abound, for example, that we can't possibly be in a sexual relationship: I have had dozens of x-rays throughout my life but never once have I been asked if I could be pregnant (despite being married). Lack of sensitivity is also rife: when I was warned that pregnancy would be life-threatening for me (due to my restricted breathing) the doctor looked surprised and embarrassed when I cried. A newly-wed, able-bodied twenty-something would have been dealt with much more sensitively. Also, virtually every time I attend hospital, I am asked to stand on scales to be weighed. I say that I can't and I invariably get the reply, “Not even if I help you?” When are nurses going to be taught that some people can't stand?

When the American government failed the population of New Orleans so badly in the aftermath of hurricane Katrina, the Rev. Jesse Jackson said that white Americans saw black Americans as only three fifths as human as themselves. This is how it feels to be disabled: I know that most able-bodied people don't see me as just as fully human as they are. The NHS is peopled by able-bodied staff who are not separate from the rest of society: they have the same attitudes and bigotries as their peers. I truly believe that medical staff should not be allowed to qualify without undergoing rigorous disability awareness training and passing a course in ethics.

Incidentally, my spine was never straightened but my hipbone has yet to break my ribs and I am now 40.