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Raisie Bay

Stiff Person Syndrome

I’m no Superwoman

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Superwoman in Shenyang China, hanging from a building

Photo by Harry cao on Unsplash

It’s been three years and three months. I look back, no, I am forced to look back by bloody Facebook, on the things I said three years ago.

It’s okay, it’s not bad, I can do this, I’m not going to let this beat me.

I can’t do this anymore.

I’ve said that so many times. It’s bloody hard waking up in pain day, after day, after day. It’s even harder trying to fall asleep in pain, night, after night, after night.

The pain is constant, it never leaves, it’s part of me now, something I just have to deal with.

Does that make me special, am I superwoman because I can cope with pain every day and still make a joke, say something positive, or even smile?

Hilarious

I laugh sometimes, especially at the Impractical Jokers (Comedy Central.) Yes, Sal, Murray, Quin and Joe have me in stitches, even though it’s not the kind of humour I usually enjoy. Sometimes it’s nice to have a little stupidity in your life. I’m going to see them in October, Live! I’m so excited.

It’s also a depressing tale. My daughter bought the tickets while I was really ill a while ago. She came up to my room and told me. I spent the rest of the evening (night, day, I don’t know, I was ill,) worrying about it. She’d booked front row tickets for us, spent a fortune.

I can’t sit in the front row, I’m disabled. I have to sit in one of the special places for wheelchairs. My daughter was annoyed, she insisted that I’d be able to walk in and sit at the front. I walk around the house after all. Even if I could I wouldn’t be safe in an emergency, I would be breaking violations. I’m not allowed to sit there.

It was then I realised that no-one, not even my family members understand.

I can’t do this Anymore

I am in pain, constantly. Every step I take hurts so bad I’m longing to be done with taking steps. I get up to put my phone on charge on the other side of the living room. Yes, I can do it, but no-one understands the effort and pain.

I can help my husband cook a meal in the kitchen, I have a stool, I have counters to lean on. Taking something to the sink or getting something out of the fridge is a lot of effort. At the end of cooking I can’t wait to sit down. I don’t enjoy my meal because I’m hurting.

This happens in front of my family on a daily basis, but they still don’t understand how I feel, how much it hurts.

I can walk from the living room to the kitchen and visa versa. I don’t want to do it twice though. I get up to answer the door when someone knocks. I’m exhausted by the time I get there, from the pain and the effort. I still do these things because if I don’t I’m giving up.

I could give up, boy, I so want to sometimes. I just don’t want to do this thing anymore, this life, this existence, any of it.

I want My Life Back

I want the old me back, I want to be able to run around after my kids, pop to the shops when I feel like it, walk up the garden, or around the park, drive a car, visit people who have doorsteps.

I want to go to the dentist and the GP on my own. I don’t want to go to the hospital any more.

I want to be able to visit my Mother’s grave, tend to it, clean away the dead flowers and polish up the headstone.

I want to be a Mother again, take my kids to school, go to parents evening, take them out, play with them.

I’m no Superwoman

I hate this, I hate the me I am. I’m no superwoman just because I post something positive on Facebook, or here on my blog.

I have always been a positive person and that’s not going to change. Because despite everything, if I don’t stay positive then what is there?

When I say I don’t want to do this anymore, it makes people angry. I have no right to give up, I have family, I have a home, I have a lot more than a lot of people. Hey, I might even have a future if I’m lucky.

And Cats, Cats are nice

So, I swallow up the bile, live with the pain, and just do it. I get up out of bed (sometimes I really don’t want to, the pain is so bad, but even though I’ve begged for help it’s not happened, so I just get on with it.)

And that’s it, that’s my life.

I’m no Superwoman, I’ve got no choice.

I don’t want sympathy, I don’t want to know how amazing I am, I don’t want people to know what I’m really thinking.

I didn’t chose this to happen, I’ve done everything I can to heal myself but nothing has worked.

And I don’t know if I can do it.

Oh no I’ve said too much,

I haven’t said enough

R.E.M Losing My Religion

I won’t give up, I can’t give up. I know what it’s like to lose a parent, it’s hard at any age and my youngest is only eight (the age I was when my Dad died.) I have to keep fighting. I have no choice. But wouldn’t it be so nice to be allowed to sleep ……

And Dream of Sleep

Little light shining
Little light will guide them to me 
My face is all lit up
My face is all lit up
If they find me racing white horses 
They’ll not take me for a buoy

Let me be weak, let me sleep and dream of sheep


Oh I’ll wake up to any sound of engines
Every gull a seeking craft
I can’t keep my eyes open 
Wish I had my radio 
I’d tune into some friendly voices Talking ’bout stupid things
I can’t be left to my imagination


Let me be weak, let me sleep and dream of sheep 
Ooh, their breath is warm
And they smell like sleep
And they say they take me home 
Like poppies, heavy with seed 
They take me deeper and deeper

Songwriter: Kate Bush

“Reflections

Show Your Rare – Make Them Listen

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Today is Rare Disease Day. I’ve written lots on my rare condition and also Star’s condition, but today I wanted to write about getting help when you are rare.

Star

Way back in 2013, Star woke up one day with a wonky neck. I took her to the GP who said to take her to A&E. We were told she had torticollis and that it would get better itself. It didn’t and we were back in A&E three more times before they scanned her and discovered a rotary subluxation of a disc in her neck. She was admitted.

A week later she was sent home again after having nothing but pain relief and muscle relaxants. She was given physio therapy but the therapist stopped treating her as she was sure she was getting worse not better.

I’ll cut a long story short, over the next eighteen months I had to fight to get the treatment my daughter needed to fix her neck. Each step was slow and, for her, painful. It seemed that none of her doctors where listening to me. She was finally diagnosed with a rare condition called Elhers Danlos Syndrome.

Her condition means that her ligaments are lax and she is prone to subluxations. Her bones can slip out of place and back again, causing a lasting pain. She has also dislocated her ankle a couple of times and dislocated her wrist. It was also, most likely, the cause of the subluxation in her neck. Which is why none of the treatments worked until she was permanently fixed with plates and screws.

She has many more issues and even now, with a diagnosis, we still have to keep on at doctors until they listen. Her condition is rare and it makes it more difficult for doctors to treat her with traditional methods.

Me

I had problems for a few years before I was really sick, but no doctor could work out what was wrong with me. My mental health was questioned on more than one occasion.

Then I became so sick I was paralysed and admitted to hospital. Again, no doctor really knew what was wrong with me. I was in a teaching hospital and each day students came in to question and examine me. They were clueless too. I did get a diagnosis of Transverse Myelitis, but I knew that my doctor was not absolutely sure of that.

A year later, I had learned to walk again (in a fashion..I know how to put one foot in front of the other, but balance and gait are something I’ve never mastered.) After more tests I was given the diagnosis of Stiff Person Syndrome. Again, a diagnosis does not mean that treatment is available, or that doctors understand my condition.

Each time I go to hospital it’s always the same, they don’t know how to treat me. So long as my blood pressure, temperature and heart rate are okay, they send me home and tell me it’s just part of my condition. I tend to not bother going to hospital now if I feel really ill, what’s the point?

Make Them Listen

Days like Rare Disease Day, and Charities like RareDisease.org help raise awareness. Without this then future generations will also go on not knowing anything about these rare diseases.

Why?

Building awareness of rare diseases is so important because 1 in 20 people will live with a rare disease at some point in their life. Despite this, there is no cure for the majority of rare diseases and many go undiagnosed. Rare Disease Day improves knowledge amongst the general public of rare diseases while encouraging researchers and decision makers to address the needs of those living with rare diseases.

Rare Disease Day


Disease or Condition?

  • A  disease is a pathophysiological response to internal or external factors.
  • A disorder is a disruption to regular bodily structure and function.
  • A syndrome is a collection of signs and symptoms associated with a specific health-related cause.
  • A condition is an abnormal state of health that interferes with normal or regular feelings of wellbeing.

As you can see from the chart above, even though you can give a different description to disease, disorder, syndrome and condition you can also see that they are all similar. Each one causes the patients body to react in a way that a normal healthy person doesn’t.

Both my daughter and I have syndromes, but we also have an abnormal state of health (condition,) a disruption to regular bodily structure and function (disorder) and our symptoms where caused by a response to internal or external factors (disease.)

Never Give Up

If I leave you with one thing it has to be never give up. There are so many rare diseases out there and the doctors really don’t know about all of them. There is a saying amongst the medical profession,

If you hear hoofbeats look for horses not zebras

This means that doctors are looking for the obvious not the rare.

This is why many people with a rare disease/condition/syndrome/disorder call themselves medical zebras.

Awareness is needed so that the Doctors will recognise those zebras!

zebra

10 Facts about Stiff Person Syndrome

Rare Disease Day 2018

The Day My Diagnosis Changed (Stiff Person Syndrome)

When it’s Not All Black and White (Elhers Danlos Syndrome)

My Angel (Star’s first Halo)

An Update on Star (Star’s Surgery)

10 Facts About My Condition

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I have Stiff Person Syndrome, I am one in a million

Stiff Person Syndrome Awareness Ribbon

Today’s Blogtober prompt is 10 Fact About Yourself. I’m turning this around a little and using it as a little awareness for my condition. In just five days times it will be our Awareness Day for Stiff Person Syndrome so this is quite timely.

I have this stupid Stiff Person Syndrome which is a condition which does exactly what it says, it turns it’s victims stiff. But it’s an extremely rare condition, believed to be one in a million sufferers world wide, and each person has different symptoms and reacts to different treatments. No doctor really knows what to do. And whenever I end up in hospital and say I have Stiff Person Syndrome I just get blank looks like I’m making it up. 

Let’s get on with some facts about Stiff Person Syndrome….

  1. Stiff-Person syndrome is characterised by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms.
  2. People with Stiff-Person syndrome can be too disabled to walk or move, or they are afraid to leave the house because street noises, such as the sound of a horn, can trigger spasms and falls.
  3. Stiff Person Syndrome is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia
  4. There are some rare forms of Stiff Person Syndrome that are due to Paraneoplastic Syndrome. The person has a cancer and might not know it yet. The immune system Ramps up to fight the cancer and goes haywire attacking other organ. In SPS case it’s the central nervous system: brain and spinal cord.
  5. Most, but not all, of patients with Stiff Person Syndrome have elevated GAD65 levels in the blood indicates that the body is creating an antibody that attacks GAD, impairing the body’s ability to make GABA.
  6. Some people with Stiff person syndrome have the glycine receptor anti-body which is generally an indication of the PERM type of the condition.
  7. Stiff Person Syndrome can cause swallowing problems to the point of medical crisis. The patient then develops severe anticipatory anxiety about swallowing and starts to avoid eating.
  8. Other disorders that can cause symptoms similar to Stiff Person Syndrome include hyperekplexia, multiple sclerosis, transverse myelitis, occult vascular malformations, neuromyotonia (Isaac’s syndrome), Schwartz-Jampel syndrome, muscular dystrophies, and metabolic myopathies.
  9. Stiff Person Syndrome was originally named Moersch-Woltman Syndrome after the doctors that discovered it.
  10. There are 5 types of Stiff Person Syndrome:

-classic stiff person syndrome
-focal stiff person syndrome
-jerking stiff person syndrome
-progressive encephalomyelitis with rigidity and myoclonus (PERM)
-paraneoplastic-related stiff person syndrome


Stiff Person Syndrome and Me

I first fell ill on 1st January 2016, but I realise now that I’d been showing symptoms for about a year previously. When I did get ill I was admitted to hospital unable to move. Intravenous steroids over a 5 day course got things working again and then I had physio to help me learn to walk before returning home 13 days later.

I was originally diagnosed with Transverse Myelitis after an MRI and lumbar puncture had ruled out MS. A year later, further testing of my muscles, nerves and blood showed that although I was GAD negative (see 5 above) I did have the Glycine Receptor anti body and was diagnosed with Stiff Person Syndrome plus PERM (6)

Next week I’ll be promoting the new Stiff Person Syndrome UK website and Youtube channel. I’ve been preparing lots of things to share on our Twitter account and I’ve made a video from clips of sufferers all over the world. The more people that know about our condition the easier it will be for future diagnosis and treatments to be made, so awareness is extremely important. 

Thank you for reading, and if you ever want to know more about Stiff Person Syndrome then please ask. 

A zebra with text "The zebra stripe ribbon for stiff person syndrome refers to training in medical school where they teach future doctors that "when you hear hoofbeats, think of horses not zebras!"
Please watch out for my zebra pictures on Twitter next week 
#Blogtober18

My Story in the Paper

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These days it’s pretty easy to get your story in the news, just go online and submit it and if they like it they will use it. 

It’s a little hard on the self conscious though. Do you really want everyone to know your true story? Will it be published correctly? Will they change it to suit? What will people think?

My Story

Recently, I was approached to tell my story. I was asked, I didn’t put it through my self. I had several telephone conversations with the lady and after she had written my story she read it back to me. I was happy, everything seemed just as I had told.

Then my story was published on the Daily Mail online and will be appearing in a woman’s magazine in print. There was no turning back now. It took me three days to tell anyone, even my husband. Then he posted it on Facebook.

My reaction was to cringe, at the headlines, at the photos. Even though I’d shared the photos before, the thought of more people seeing them made me squirm. The headline made me squirm too.

The Mother of Three that is turning into a ‘Human Statue.’

When the condition, Stiff Person Syndrome, was first discovered, it was found that a common feature was a stiffness that turned it’s sufferers into statues. This seems to have stuck throughout time, although most of us with Stiff Person Syndrome do not see it like this anymore. Many medications have been found that can prevent the condition from progressing to this stage, and some have even found their condition goes into remission. Still, it sounds dramatic doesn’t it?

Many people with Stiff Person Syndrome have spasms which can stiffen parts or all of their body. When I have a spasm I do go stiff, but it’s not board like, I often tend to curl up and I’m unable to straighten again until the spasm passes. This can last anything from a minute to 20 minutes. I also have smaller spasms that affect my feet, hands or legs, sending them stiff. But eventually they relax again.

I do have stiff muscles that never relax in my back, legs and feet. These are what contribute to my inability to walk.

I have to say though, they did manage to stick to the truth, the things I told them. There was a little sensationalism thanks to the highlighted headlines, not anything I’d not said.

The Reason for Sharing.

My main reason for sharing my story is to raise awareness. I’ve recently become part of a small group of Stiff Person Syndrome sufferers that are trying to make our condition more widely known. Very few doctors or medical professionals have heard of the condition. Even fewer general members of the public. With awareness comes better diagnosis and treatment. And maybe even one day a cure.

I’m doing a little research myself and I’ve already noticed that those with paroneoplastic Stiff Person Syndrome have responded to a particular chemotherapy drug. SPS can be a side effect of cancer, and when the cancer is treated, the symptoms of SPS are resolved too. Maybe, in the future, the drug could be tested on other SPS patients to see if it makes their symptoms go away too. It’s just my theory for now, but who knows what could happen. 

So, as cringy as I find it, my story needs to be told, as do many others. One day maybe lots of people will have heard of SPS and treatment will become readily available. 

Reactions to My Story

I was surprised at how little many of my friends knew about my story and my condition. I guess, I must not overshare as I’d originally thought (or was led to believe.) I don’t want sympathy, but what about my family? Maybe they deserve a little sympathy for what they are going through. I hate my children seeing me sick, I’ve become an expert at hiding my pain and my feelings. My husband is more observant and is constantly asking me how I am doing. It’s good to know that people care, but I also find it awkward. 

Now that I have been forced to look at the bigger picture though, my feelings have been put aside. Yes, I may feel embarrassed but if my story helps to raise awareness then I will continue to tell it for as long as I am able.

Maybe I should be more honest from now on. I was slated for it recently and it really made me hide back in my shell. But now I don’t care. So what if a few people decide to mock me or ignore me, there is a bigger picture here that I am part of. 

So, here goes, the link to the first article about me as it appears online.

Birmingham Woman is Turning Into a ‘Human Statue’

Sticky Mud and Belly Laughs

Lucy At Home UK parenting blogger

Keeping Well

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It’s not been the best of time for me health wise, after catching the kid’s sickness bug I seem to have been knocked off my feet, almost literally!

It’s made me think a lot about my illness and how it’s progressing. I don’t like it, and I don’t like my family seeing me like it either.

This is not my usual state of mind. I probably get on a lot of people’s nerves but I’m really a ‘cup half full’ type of person. It’s strange really when my life hasn’t really strolled along the easy path, it’s definitely been a slow mountain climb with lots of crumbling rocks on the way.

Maybe that’s why I’m a positive thinking person. If I focus on the negative then I’d probably be a very depressed person right now. That doesn’t mean that I’ve never suffered depression, I have quite a few times, but these days I am well aware of the warning signs and I know the best ways to keep it at bay.

So, what have I been up to this week?

Well, I’ve got a new hobby! I have always been interested in papercraft but I’ve never been the arty type. I’ve decided that I’ll give it a go though and I’m making some cards. I’m using my favourite resource, Pinterest, to get some inspiration and I’ve been buying some bits and pieces to get me started. Okay, I know you don’t absolutely need a die cutter for starters, but I couldn’t resist treating myself to one.

Here’s my first attempt.

 

I’ve even ordered myself a cabinet to keep my bits and pieces in. With this and my crochet I’m going to be keeping very busy.

Talking about crochet, here is my latest creation.

 

The request for these keep coming so I can see myself making more. I’m also working on a big blanket with a CAL (crochet along) but that’s a year long project. I am loving it already though and can’t wait for the finished product. I can’t decide whether I prefer big projects or little ones. Last night I spent about an hour crocheting this jammy dodger tape measure cover.

 

I think now you may be able to see how I cope with my illness…I occupy my brain and my hands 🙂

 

Debs Random Writings