The past four months have been brutal. On top of all the ambulance journeys, as I explained in my previous blog post, I can now only lie horizontal with my head touching my pillow for two out of the twenty-four hours in a day because the blood flow to my head gets so cut off and reduced if the surface of my head is touching anything that I start to pass out if I stay on my pillow any longer than that. My head and face get increasingly numb and cut off while I have my head touching my pillow. The rest of the twenty-two hours in the day, I’m straining my whole body and neck into painful and strange positions in order to coax enough blood back into my head to be able to safely go down onto my pillow for two hours. It takes all my concentration. It is a strange feeling being able to actually feel the blood in the blood vessels in your head. The best (but still inadequate) way I can describe it is like after you’ve been sitting on your foot for a long time and it goes numb and then you get the rush of blood back to the foot as the feeling gradually returns. So it’s a tiny bit like that but a much, much slower process. I never regain even 50% of the feeling back in my head, even after twenty-two hours. I’ve forgotten what it was like to be able to rest and have my head on the pillow. Every day I have no choice but to carry on the body-destroying routine of working hard to get enough blood back into my head so that I can safely have the two hours with my head on the pillow without passing out from severely reduced blood flow to the head.
I feel like my body is disintegrating. Given how ill I already was before all of this and how I deteriorate with even the slightest exertion, it has been hell to keep pushing through with the extra physical exertion of being semi-sitting up instead of lying flat, which causes my heart to get even worse. The sleep deprivation has risen to insane levels.
During this time, the idea that I was forming in my head – the ‘Five by Five’ challenge – to raise money for charity, has been the only thing that has kept me going. The execution of the idea seemed like it would be impossible and I knew how much it was going to cost me and pretty much annihilate me but I had to do it. I just had to. Like my friend Tanya wrote to me, ‘you are the only one I know determined enough to be at your worst and decide to do the impossible’.
I’m too ill for anyone to be in my room longer than it takes for essentials (food, water etc.) to be brought in and out but in order to make the ‘Five by Five’ launch video, I had to have my parents in the room for 7 minutes or so. There was also the addition of the light being on and the noise of their voices, all of which are agony for me and cause me to deteriorate. Nevertheless, it was still joyous to see them for the longest amount of time in one go than I had for years and years and, as you can see in the video, I couldn’t wipe the smile off my face, even though I was in extreme pain and physical distress. I tend not to show pain on my face anyway. It has cost me everything and was a one-off but I’m proud that I got through it.
A huge thank you to my parents for making the ‘Five by Five’ launch video possible. Thank you to my Dad for being my voice (I’m unable to speak) and to my Mum for her amazing artwork and for holding the camera phone to film it. Please forgive the mistakes (and the mispronunciation of ‘Myalgic Encephalomyelitis’!); we did the best we could, given that we only had one take to film it in. Thank you to Simon for creating a YouTube channel and uploading the video for me.
So on my birthday, I launched the ‘Five by Five’ challenge. Here is the video explaining it and what it is. I really hope that you all join in and make a video too. This is me pushing back against the darkness, defying everything and choosing to do good, even when all the bad is thrown at you.
The donation page is here: https://mydonate.bt.com/fundraisers/fivebyfive
What is Myalgic Encephalomyelitis?
This is when a virus or enterovirus (or an as-yet-undiscovered retrovirus) causes:
• inflammation of the brain, spinal cord and neurons
• immune system deficiency and dysfunction
• mitochondrial malfunction (the body’s energy is manufactured in the mitochondria), which leads to muscle failure (including heart failure), and heavy exhaustion
• damage to and dysfunction of the heart
• dysfunction of the digestive system, including poor absorption
• extreme sensitivity to noise, light and movement
The hallmark of Myalgic Encephalomyelitis is deterioration upon any slight activity/exertion/movement. Gradually trying to increase your activity can cause permanent damage. The severity of the disease varies greatly. Unfortunately, I have a severe case and am bed-bound, struggle to breathe and swallow, and am unable to speak. My heart is so unstable and my condition so fragile that my body cannot be moved.
What is Vascular Ehlers-Danlos Syndrome (vEDS)?
It is a mutation of the gene COL3A1. This mutation alters the structure, production and processing of a protein in the body called collagen. A significant amount of the body is made up of collagen; it provides structure and holds our bodies together. This is why having faulty collagen is such a big problem and why my body is falling apart in so many ways. As you get older, the condition deteriorates. This is why it is getting increasingly difficult for me to breathe and swallow (dysphagia), why my eyesight is worsening and why I have faulty heart valves.
The vascular type of Ehlers-Danlos Syndrome, which is what I have, is life-threatening and the majority of people with vEDS don’t live beyond their forties. This is because the blood vessels and hollow organs in a person with vEDS are fragile and prone to spontaneous rupture, due to the defective and weakened collagen.
It is important that paramedics and doctors be educated about vEDS so that in an event of a rupture, patients be dealt with correctly and quickly, which is key to our survival. For example, aortic dissection is pretty common among vEDS patients but it can easily be mistaken for a heart attack so it’s essential that awareness amongst the medical profession be raised.