August 7th 2017: Friedreich Ataxia’s News included the following definitions for Scoliosis and Heart disease—
Weakened core and leg muscles can lead to aggressive scoliosis or curvature of the spine in many patients.
Around three-quarters of people with Friedreich’s ataxia will develop some form of heart disease, usually hypertrophic cardiomyopathy which is when the heart muscle becomes thickened.”
At the age of 14, I was diagnosed with Friedreich’s Ataxia. My parents were growing increasingly concerned about the curvature of my spine, and the main reason for their call was to seek a medical answer for it. It was a frightening picture for my family, because I had in fact, been developing severe scoliosis. This physical attribute of Freidreich’s Ataxia was the tipping point which led to my diagnosis.
When I was 25 my scoliosis began to seriously impede on my physical condition. The curvature was profound, with a 30 degree angle that bent in the middle. After undergoing numerous checkups and experiencing countless worries about the condition of my spine, I decided to have an operation to support my spine. This procedure involved the insertion of stainless steel rods in my spine, from A1 to Coxic. It was a dangerous procedure for me, due to the anaesthetic dose, and I was told by neurological surgeons that I had a 50/50 chance of survival. I took these chances anyway, and pulled out of the operation miraculously. But this is not to say that I came out completely unscathed.
I knew the operation may have a severe outcome, but knowing this did not make it any easier. My life was not made easier as I had hoped, because my condition still had debilitating effects on me. But, this only served to make me more determined.
The operation served as a catalyst for my academic career. With a newfound sense of determination, at 25, I started with an associate diploma in Accounting and then completed a double degree from Monash University. This was followed by a Master of Arts from Monash University and finally, a Doctorate of Philosophy from the University of Melbourne.
My spine has played a significant role in my academic achievements. Whilst studying, I had to also develop new methods of study that did not interfere with my spine – most of these methods were targeted at alleviating the pain. Taking medication for this may have had devastating side effects, most importantly in terms of reducing my intellectual capabilities. I was certainly not going to compromise my intellect, so I pushed forward to the best of my abilities, despite the hindrances it has caused.
It has also taught me a valuable lesson. Today, I am in a position to understand my disability through a pragmatic perspective. I am confident in what I know, and how to manage my disability to achieve the best outcomes for me.
According to Wikipedia, “91% of people (with Friedreich’s Ataxia) develop heart problems such as cardiomegaly (up to dilated cardiomyopathy), symmetrical hypertrophy, heart murmurs, atrial fibrillation, tachycardia (fast heart rate), hypertrophic cardiomyopathy) and conduction defects”
At 25 I was given more devastating news. At this time, when I sought medical checkups for Scoliosis, I was told that if my spine continues to bend, it would also severely damage my heart even more.
This prompted me to get continuous check-ups throughout the years for my heart as well. In fact, since 2003, I have had a yearly check-up for my heart at the Friedreich’s Ataxia Clinic.
The knowledge of my poor heart condition has been burdensome to me, but even more so in recent times. I attribute this to my move into shared support accommodation; this event, and some related events which occurred thereafter, have definitely become the stimulus for my worsening heart condition. I have experienced a gradual loss of control, and the stress and frustration that comes with not having an adequate say in my support needs.
In 2017, I was admitted into hospital after an incident (described below) which occurred in this shared support accommodation. In hospital, there were many tests conducted, including one for my heart. My doctor prescribed Minax, to slow my racing heart. The following is a recount of this incident, as it appears in a previous blogpost:
“So then, the big feature: the worst day of this weekend. Yesterday in the afternoon, I stooped out of my wheelchair. Of course by this stage I was feeling very tired, and was thinking the only way for me to feel relaxed was to pull myself by the sides of the table to make the wheelchair fall; this was to put me in a more relaxed position. Again, I had to do this by myself. When I fell, my feet pulled the buzzer cord, which alerted the staff.
This has all had an extraordinary effect on my heart, which is something I will have to have more check-ups about this week. Hence, this weekend has been a manifestation of the loss of control that can lead to me feeling punished. It is a result of the lack of dignity and choice I have, living in a group home.”
I talk about social issues and disability in my book, 6 and a ½ Years on a Dunghill: Life in Specialist Disability Accommodation.
You can also order directly from me.
Many thanks to my friend and colleague Bruce Wearne, who has inspired me greatly in all my work and who has been my prolific ghost writer. My gratitude also extends to my phenomenal team of academic support workers. Firstly, Christina Irugalbandara whose work means so much to me. Daniella De Bruin whose brilliant and caring nature means so much to me. Ikemi Ivara whose ability to command and control the situation with kindness. Lastly but definitely not least Mercy Ndegwa who’s continually teaching me how to be a better person.