So, What’s Wrong With You Then?
(Please don’t ask a disabled person this, unless you’re at a pretty intimate level of friendship. But I’m happy to share, seeing as it’s you…)
I have a number of rare neurological conditions, including: –
– Chiari Malformation,
– Syringomyelia,
– Autonomic Dysfunction,
– low Cerebrospinal Fluid volume issues (possible CSF leak),
– Brain Injury.
I’ve explained a little about each of these conditions below, and how they affect my life…
I share this information, not for attention or sympathy – I’m a normal person leading a normal life, I’m well adjusted to my conditions and lead a pretty happy existence, despite obvious difficulties – but because I was once someone looking for information on these conditions, seeking a sense of belonging amongst others who also deal with them, and a longing for more information about other disabled lives.
Plus knowing more about the lives of others that might be different to our own, helps us to become better people, friends and community members.
Chiari Malformation is a condition where the flow of Cerebrospinal Fluid (CSF) – the fluid which surrounds the brain and spinal cord – has been blocked or impaired.
CSF is important as it acts as a cushion between the brain and the skull, protecting the brain from impact injuries. It also plays a role in the brain’s immune response, helps regulate blood flow around the grey matter, circulates certain substances and clears waste.
When there is too much CSF, too little, or CSF doesn’t flow properly, the brain often doesn’t function well and it can cause all sorts of symptoms and knock-on issues. In 2017 I had brain surgery to try to rectify these flow issues, which has helped somewhat (through this sort of surgery is generally performed to try to stop things getting worse, rather than with a hope of winding issues back).
Syringomyelia… Chiari Malformation can cause knock-on issues elsewhere in the CSF system. If flow of CSF is impaired in the head area, this can cause a ‘mis-flowing’ of CSF lower down, around the spine too. This changed flow can cause Syringomyelia – which is where a CSF-filled cyst (a ‘syrinx’) forms inside the spinal cord. Syrinxes can compress the spinal cord tissue, temporarily blocking nerve messages, or damaging the spinal cord permanently.
My syrinx was discovered in 2013 when I reported to my GP that I was having trouble with a number of seemingly unconnected health issues – hand tremors, frequent urinary tract infections, arm numbness, balance issues, headaches, problems with my vision, etc.
An MRI scan to investigate the possibility of Multiple Sclerosis found a large bubble of CSF fluid growing inside of my spinal cord, squashing it from within. I was told that surgery had to happen urgently, as it was threatening my bodies function from the neck down. This discovery began a period of several years, over which other interconnected health issues were discovered, causing me to go through a number of surgeries and procedures.
My syrinx has decreased in size somewhat due to surgical treatment, but I have been left with permanent nerve damage that affects a lot of different systems in my body. My syrinx is at the bottom of my neck, so I have issues from that level down in a similar way to someone with an incomplete spinal cord injury from something like a traumatic injury (although not quite the same, as generally different areas of the spinal cord will have received damage).
This causes me to have constant body-wide nerve pain, issues with sensing heat, cold, pain, and with sensing the position of my joints. It causes my larynx to spasm and stick (making me intermittently unable to make speech, and sometimes causing issues with breathing), arm and leg weakness, balance and coordination issues, muscle spasms, tremors, occasional temporary paralysis of limbs, etc.
My Autonomic Dysfunction can arise from injury to the brain and spine, and can mean your body doesn’t regulate a number of automatically controlled systems appropriately.
In my case, my heart rate tends to always be on the high side – even when I’m calm and resting. It can make even moving slowly extremely exhausting, really impacting daily life, and causes chest pains.
My blood pressure can spike or drop suddenly with no reason. My digestion can slow or stop altogether (for hours, or days), meaning undigested food just sits in my stomach, and I’m unable to eat more to get proper nutrition and energy. One of the most debilitating issues is that I pass out very often, with little or no warning, so have to always be mindful of where I am and what I’m doing, and generally only go out with someone else.
Autonomic dysfunction has effect things as desperate and seemly unconnected as your eyes being dry / watering excessively, not being able to adjust your eyes to changing light levels, reacting inappropriately to temperatures (shivering and feeling cold in hot weather / not being able to cool down or feeling cold in hot spaces, feeling short of breath, not being able to void your bladder, etc. I’ve found this can effect everything from my ability to drive a car, do regular exercise, sleep or rest comfortably, have enough energy to do vital things, go out and about independently.
Low Cerebrospinal Fluid Volume / Intercranial Hypertension / CSF Leak... Because of the brain and spine surgeries I’ve had in the past, I have ongoing issues with the pressure / volume of CSF around my brain.
Not having enough CSF causes severe headaches, nausea and vomiting, tinnitus and hearing issues, eyesight problems and sensitivity to light, as well as being very sensitive to other stimuli (like touch and sound).
It means I’ve basically had a headache every day for the best part of a decade. Simple things like sneezing or going over potholes in a car can be excruciatingly painful.
When the head pain and symptoms are moderate to severe, the only way to really help it is to lie very flat and still. This eases the pain a little an helps stabilise the symptoms somewhat, by taking pressure off certain structures of the brain. This bed rest can last a few hours to two weeks or more at a time, lying still in bed and only getting up for the bathroom. I also consume very large amounts fluids every day to ensure that my body isn’t prevented from making plenty of CSF to replenish the system, alongside using strong painkilling medications and other drugs to help ease the conditions effects. If you see me in sunglasses, rest assured that I am not cool, the world is simply a very glaringly bright place to me!
Brain Injury
Because of the trauma my brain has gone through, perhaps unsurprisingly, my brain has received some permanent damage over time. This causes issues with language, word finding, numbers, memory, coordination, multitasking and means I get overstimulated easily and fatigue quickly. The more tired or otherwise taxed I am, the more these issues tend to show up.
Things other people take for granted, like being in busy shops or restaurants, can be excruciating for me. I often don’t cope well with all the stimuli – noise, movement, light, people talking to me, navigating spaces and moving around safely, etc, on top of dealing with pain, tiredness, discomfort, etc. My brain becomes easily overwhelmed and then looses its ability to be able to think clearly, coordinate or concentrate. It can be annoying, such as not being able to make a decision or form an opinion, but it can also put me in danger, like not being able to judge to cross a road safely.
I always find concentrating hard, and multi-tasking extremely difficult. My family are used to me saying ‘hold on’ if they approach me, as if they interrupt what I am doing it’s very likely that I will forget what I am saying / doing / noting down / etc. Following recipes is hard, as I muddle the steps, or miss one out.
I had to give up driving some years ago, as I began to forget what different controls did whilst in the process of driving, and also struggled to concentrate – which was very scary!
It causes practical issues, such as leaving the hob on, forgetting passwords, having difficulty learning new information, forgetting if I’ve taken medication, loosing things… I often I avoid social situations – I can very good at covering up with cognitive difficulties (even with everything going on, you’d be surprised, and even though that that’s very exhausting to do), but doesn’t stop me being nervous about sounding silly, being lost for words, or about meeting new people. It also means I miss out on a lot of opportunities. People often have no clue just how tricky I’m finding things, except my husband, kids and very closest friends.
But also, the emotional effects of a brain injury can’t be underestimated – it has a big psychological effect. You *are* your memories and the things that you are good at – start chipping away at that and you start to dismantle and diminish your very sense of self.
One reason for this website is an active ‘yah-boo’ to the effects of brain injury, and a reminder of all the things I push hard to keep participating in and enjoying. Every activity – from making art, wobbly fossil hunting, to working my language centre by writing poetry – plays an important part in exercising my grey matter, fighting against psychical and emotional decline, and forming an identity that is beyond being ill.
As I always end my posts on Instagram, my mottos are…
#LiveLifeInTechnicolour
(variety, vibrancy, creativity, art)
#StayCurious
(never stop learning, never stop exploring)
Please do not mistake this information for medical advice. If you are looking for information about or support with these conditions, I recommend the Brain and Spine Foundation website and helplines.
