Guest Post: The Rag Doll Disease by Hayley Steed

It was in my second year of university I started noticing my body struggling. The first thing was that I couldn’t use a straw, my mouth just wouldn’t close around it but I didn’t think it was a big deal. Then it was paralysis of my face – I’d be doing my makeup and I just couldn’t move my forehead or make my lips meet. It got worse when my speech started to slur and I began to choke on my food. I went to my university doctor about 5 times in 3 months, asking for help to be told it was stress, anxiety, migranes etc. It wasn’t until I went home to my local surgery that someone realised something was wrong. I was referred to a neurologist but the waiting list was 2 months – in that time I dropped to 6 stone from not being able to eat and I have vivid memories of having to leave restaurants with my boyfriend from everyone staring at me whilst I choked, to physically putting my fingers down my throat and picking out food I was choking on all whilst holding my breath.

My neurologist, Dr A as we like to call him, sent me for MRIs and CAT scans immediately before I was diagnosed with Myasthenia Gravis, nicknamed the Rag Doll disease. It’s an auto immune illness meaning severe muscle weakness. Essentially my body’s immune system attacks itself and sends antibodies to kill the messages from my brain to my muscles, so they just don’t receive the message and don’t move. That’s anything from my eyes drooping, to not being able to smile, to my swallowing muscles failing, to my lungs struggling. Dr A wanted to admit me but only let me go home as my Mum is a registered nurse. He told me to cancel mine and my boyfriend’s first holiday together which was just 3 weeks later, and cancel the trip to America my family had planned for several months later in the year, which was when I knew how serious it was. One of the hardest parts still was sitting outside that hospital and ringing my boyfriend to tell him I was ill.

I started on medication and in December 2013 at the age of 20 I had a transsternal thymectomy (they cut open my chest and took out my thymus gland which had a tumour.) I was his youngest ever patient and they told me it had 1/3 chance of making me slightly better, but not curing, 1/3 chance I’d be exactly the same and 1/3 chance it’d make me worse. It’s such a rare disease, they know so little about it. They don’t know what causes it, or how to really fix it. Either way I’d have the scar which runs the length of my breastbone and three ‘bullet’ hole scars underneath from where they fed drains into my lungs, which they took out when I was awake two days later. The only thing I’m going to tell you about that operation is that because my illness is all about muscle weakness, they couldn’t let me have any painkillers before I woke up. Usually they’ve already hooked you up to a morphine drip so you wake up with painkillers inside you – I didn’t have that in case it meant I didn’t wake up. I woke up feeling the full effects of the operation, screaming.

A couple of months before my 21st birthday they found a blood clot in my leg so I was on more treatment for that, meaning I was not allowed to drink for my 21st. I nearly had to cancel my birthday trip to Rome but I pretty much told them that wasn’t happening unless I could die doing so. I went. I’ve had two relapses, one when my eye closed for about 2 months, I’ve no idea why. I had to cancel all my work experience and just stay at home for that time. The other was last summer. I had a week where I kept waking up in the night feeling very phlegmy, to the point I couldn’t lay on my back because I felt like I couldn’t breathe. Then one morning I woke up and I couldn’t breathe standing up. I went to A&E, was met by ICU and strapped up to IVIG, a drug which essentially restarts your immune system. I had that over a course of 5 days which left me exhausted but it worked. That’s the only time I’ve come close to dying from it. (I had to cancel another holiday me and my boyfriend were supposed to be going on).

Something that came alongside the physical illness, was the anxiety. It’s better now, but for the first couple of years of diagnosis, I just wouldn’t go out. I was terrified it would make me more ill, or if I went for a meal, I would choke and either everyone would stare or I’d die. I didn’t like people seeing me when they knew I was ill because I thought they’d be looking for signs of it and I didn’t want to see anyone who didn’t know I was ill and have to explain why I couldn’t speak properly all of a sudden. Now, even though most people know I’m ill and I rarely choke at meals, I still get panicky when I’m going to events or meeting an author that my speech or face will just drop and they’ll wonder why. It’s a vicious cycle because the anxiety and stress makes my symptoms worse. Sometimes I try to joke about it so if I’m with my friends for a special occasion I always take my pills and say ‘if we’re taking photos, let’s do it now whilst my face works’. My worst bout was after my second relapse – I’d wake up every morning in a hot sweat, panicking in fear of waking up and not being able to breathe again like before.

My friends now tell me how snappy I was after being diagnosed and I was – I was angry. I was angry at the hand I’d been dealt, angry that I wasn’t like them anymore, that I couldn’t be ‘normal’ and angry that none of them seemed to think it was a big deal. An illness like mine, one which can’t be cured, brings a lot of frustration with it. It dictates every moment of my life – I decided I still wanted to work, which I do, and to live my life, which I do. But if I have an event in the evening after work, it will tire me out for the next few days. I can’t go to a different bookish event each night like I wish I could, I have to carefully plan. I can’t go to bed late, because if I’m tired, my muscles will be so much weaker. Numerous times I’ll be mid-way up a flight of stairs and my legs will give way – I just have to sit and let everyone walk by me until I’ve rested enough to go again. Most of the day I can’t smile, because my cheeks aren’t strong enough. I frequently look liked I’m mid-stroke and my medication heavily increases my risk of cancer. Rest is the biggest word in my life and it’s so frustrating, as a 20-23 year old to be told you can’t when you have always been able to and that’s always what I’ve struggled with most. If someone who knew me in my pre-illness years saw me now they’d be shocked. I used to love going out, I loved drinking and clubbing, I was loud and brash. I’m still quite brash, but if I go out, which is rare, I don’t drink because it’s a depressant and makes my muscles weaker. Even without alcohol, I can’t do stuff every day like ‘normal’ people can, I always have to consider what will tire me out, what will be too much first. It’s constant.

My scar then and now

Most importantly I think we need to stop putting illnesses on a scale. I had so many comments after being diagnosed like ‘at least it isn’t cancer’ or ‘at least it isn’t a brain tumour’ and yes, I’m so thankful I haven’t had to go through either, but does that make mine any less valid or difficult to live with? My own friends thought my illness wasn’t a big deal, until they nearly saw me die from it. But people can die from depression, from cancer, from Crohn’s, from epilepsy, from anxiety. So many people have a story like mine, of struggle and pain and difficulty. Mental and physical health don’t need to be measured or met with judgment of how bad they are.

This has probably been a quite depressive post so I’m going to list some good bits:
• It’s been nearly 3 years since I was diagnosed and I’ve gone from 40 pills a day to 8. I’m stable and have no signs of relapsing anytime soon *fingers crossed*.
• This was all going on in my 2nd and 3rd year of uni. I’d get extra time to finish my work after my operation and blood clot in 3rd year, and I got a First. I’m damn proud of that.
• I’ve been on several fantastic holidays which I haven’t had to cancel including driving the coast of California with my family and Rome for my 21st birthday.
• My incredible boyfriend has been with me since day 1 and has stayed throughout the most difficult times, even when I was freaking out about my ‘ugly’ scar.
• I’ve never been so close to my family – I had to call my Mum at 3am from my bed after coming home from the op to come and spoon feed me morphine because it hurt too much to move. After an experience like ours, you value everything so much more and we’re pretty much best friends. • I’m really happy – I got a job in publishing and my illness is settled enough to live with. I’ve got used to my life being toned down.
• Since my op I have skydived and abseiled to raise money for our charity – Myaware. This year I’m taking part in an attempt to beat a world record of skydivers and my Mum is jumping with me. If you want to sponsor me you can find my page at www.justgiving.com/HayleySteed3

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