10 Facts About My Condition

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

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’

Lucy At Home UK parenting blogger

Keeping Well

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

Healing from the Inside, Repairing a Leaky Gut

I have been sick for just over two years now. I have an auto-immune disease and I’m pretty sure it’s made worse because of my leaky gut and my weight.

I have decided that by dealing with my weight and thinking about what I put inside my body is the path I must take to start healing.

Leaky Gut

I have a poorly gut. I’m no doctor but I am convinced it’s either the cause of my issues or it exacerbates them. Before I got sick I was having a lot of problems with bloating, stomach pain, constipation etc. I even had a CT scan which showed that I had a lot more gas inside me than was normal, it was leaking into my womb. I was going to seek further help but then I ended up in hospital. I know the problem is still there, but I’ve decided I am the one who can tackle it.

What is a leaky gut? The medical term is ‘Intestinal permeability’ and it’s when the lining of the small intestine becomes damaged causing the things it contains to leak through into the blood stream. This can cause an autoimmunse response.

Symptoms of a leaky gut include migraines, irritable bowel, eczema, chronic fatigue, food allergies and even rheumatoid arthritis.

It sounds like a pretty serious condition but it’s not mentioned often. In fact a lot of medical professionals don’t believe it exists.

A leaky gut can be caused by food and/or medication. A particular culprit is over-the-counter pain relief that contains aspirin.

Repairing a Leaky Gut and Healing from the Inside

The obvious way to repair a leaky gut is to think about what you are putting in your body and improve your diet.

Paleo

Last year, after I’d put on a lot of extra weight during steroid treatment, I decided to try a Paleo Diet.

What does the Paleo diet involve?

You should avoid, grains, rice, beans and pulses, dairy products, processed foods, sugar, artificial sweeteners, soft drinks, ready meals, refined vegetable oils, chocolate and sweets, refined table salt.

You can have, meat,  fish, seafood, vegetables, nuts, seeds, fresh fruit, olive oil, coconut oil, nut oil, honey, coconut sugar, nut and seed milks, and even coffee and alcohol in moderation.

It’s not that bad is it?

I lasted a few months but it became a little too difficult. I liked my bread and rice too much. I have taken a few things from the diet though. My dairy intake is very limited, I’m a big fan of almond milk. I have cut down considerably on sugar, I do have sweet treats, but not regularly. I no longer eat pasta, but I wasn’t so keen on it anyway. I have never been a fan of processed foods so I although I have them, I don’t indulge often. I also try to eat gluten free whenever I can. I’ve never used table salt although I do add salt, usually rock salt, while cooking.

a healthy meal of fruit and juice

Time to Lose weight

Although I lost a little bit of weight last year, eleven pound to be precise, my body mass index (BMI) is still 30. I am still suffering from bloating and constipation, so I know my gut is still leaking. I’ve decided that it’s time to lose weight and I think I’ve found something that works for me.

Herbalife

I met Charlie in hospital while we were both having infusions. He has MS and is a true warrior. He was diagnosed 10 years ago and was in wheelchair. With a lot of work he know walks and drives and is living his life as well as he can. To get this far he has tried everything, and has worked out a combination of things which have increased his mobility and given him his life back.

One of the things that Charlie is enthusiastic about is Herbalife. We had a chat and I decided to try some. I chose the vanilla shake and substitute one of my meals in father of a shake with added fruit. Each shake contains a good amount of vitamins and minerals. I’ve recently suffered from a lack of vitamin D, vit. C and Vit. B12. I’ve also taken supplements of magnesium which help with my leg cramps. a Herbalife shake contains all  of these and more so I no longer need to take any supplements.

Since staring Herbalife I have felt a little better and I have lost 7lb. I’m convinced that the shake is helping and I’ve just ordered my second tub.

Mindful Chef

I have also decided to eat more healthily and instead of having the hassle of researching meals and getting in what I need, as well as all the shopping I need for the rest of the family, I have decided to try a food box. With health in mind I went for Mindful Chef. These boxes are delivered with everything you need and all the food is reduced in sugar and refined carbs. You can choose whatever meals you want and when you want it. I have two meals, which are for two people, so I split them up and make myself four meals. It works for me and in combination with my herbalife shake for lunch and a healthy (gluten free) breakfast, I am hoping for some good results.

If you would like to try Mindful Chef you can get £20 off your first box by giving my e-mail address when you sign up. (okesanne@gmail.com)

a plate of food, sweet potato chips, haddock and a small salad of tomato and lambs lettuce

Fish and chips, Mindful Chef style

My Goal

First and foremost I want to heal my leaky gut, and I also want to lose weight. I am hoping to lose the two stone I put on since being sick. As I can’t exercise much I need to rely on what I eat. I hope that once the weather gets a little better I can go for some outdoor exercise with my walker. I can’t go very far, but hopefully I’ll be able to build up to do a little more.

 

Have you heard of a leaky gut?

What do you do to keep healthy?

 

A Medical Zebra, a Post for Rare Disease Day 2018

Where it all began

When I was in hospital two years ago I became a medical guinea pig. Most of the time I didn’t mind, it was a teaching hospital and the young doctors needed to learn. I was a rare case and difficult to diagnose.

When I first arrived at the hospital the first words uttered by a doctor were Multiple Sclerosis. My symptoms were very similar to MS but the doctor made a classic mistake of looking for horses when in fact I was a zebra. It’s a strange medical term but basically it means that most doctors will go for the obvious diagnosis (horses) which are more common, but some cases are not so obvious (zebras) and more rare. My condition is very rare, one in a million worldwide!

At the time, after days and days of testing, podding a poking to an extreme extent, my diagnosis was Transverse Mylelitis, another rare condition. This was later changed after more podding and poking and blood tests to Stiff Person Syndrome with PERM, but more about that later.

An MRI Scan had ruled out MS as I had no problem with my brain (although some may disagree with that but they are not doctors.) A Lumber Puncture had ruled out further, more obvious conditions. I had my legs pricked with little pins so many times that I looked like a pin cushion. The defining test had been the planter reflex, or in laymans terms, the tickling of my feet. When an object, usually a pen but they did have ‘proper’ tools for this, was brushed up the soles of my feet I didn’t feel a thing, but my toes shot upwards. This, my friends, is not normal. I had no control over my little tootsies, and still don’t but tickle my feet and they’ll shoot up. This is called the Babinski sign and is supposed to indicate a problem with the spine and that’s where my diagnosis of Transverse Myelitis came from and it means inflammation of the spinal cord. There is also a test on the hand, the Hoffman Sign, which is indicated by an involuntary flex of the thumb or forefinger when your middle finger is flicked. Oh, it’s all very technical you see.

Back to the student doctors. They were there every day and most times I agreed to let them take a pod and poke of my poor lifeless legs. Some days I was not so willing and I’d tell them to piss off. Well, I’m sorry, but when you are ill you just don’t care. On one particular day I had ten, yes TEN, students around my bed, with the curtains drawn it felt very claustrophobic. My consultant had visited me beforehand and asked me to stay quiet unless I was asked any questions. I think he had become a little fed up of my saying, no, you don’t do it like that, or try doing this, because I believed I’d become quite an expert in all the tests. Well, you would do if they were performed on you several times a day for a fortnight. Not only that but as soon as a doctor had performed a test and left I’d be on google finding out why. That’s how come I know about the Babinski and Hoffman signs.

So, I’m lying there like a good little guinea pig while these students do their tests and basically they haven’t got a clue what they are doing…but I did as promised and stayed quiet. I tried to concentrate on things like how smooth my legs looked considering they hadn’t been shaved for two weeks. I put up with having my legs lifted and dropped, I think this was fun for them because they all seemed keen to have a go, and they pricked me with pins, tested my reflexes (they got the Hoffman’s completely wrong) and pinched me after asking me to shut my eyes, oh they were mean! Then the consultant asked them what they thought was wrong with me and pointed to them one by one for their diagnosis. The first guy said Transverse Myelitis, but the second say Guillain Barre, after that the rest of them said Guillain Barre, talk about a pack of sheep. I so wanted to congratulate the first guy but I stayed quiet.

I stopped allowing students after that, there is only so much you can take and I’d had enough. Plus, I was starting to feel better, it’s one thing being poked, prodded, pricked and pinched while you are immobile and numb, but once I was feeling more I just couldn’t take it.

I left hospital with my Transverse Myelitis diagnosis and the assurance that I would be recovered in a couple of months, if not, then six months at most.

The New Diagnosis

So when I went back to my consultant eight months later with no recovery at all then I was sent for more tests. I had more blood tests then a EMG and NCS.
The EMG, or Electromyography, involves long needles pushed into the muscles to record any evidence of abnormal activity. Some people hate this procedure, but I didn’t feel a thing. Then came the NCS or Nerve Conduction Study, this is where they electrically stimulate the nerves to see if they react correctly. Again, I didn’t feel anything in my legs but apparently they behaved appropriately. Then they did my arms and boy did I feel that, it was just like having electric shocks…don’t touch a plug with wet hands! But, I was distracted from the pain as each shock made my fingers move like a robot, it was really fascinating.

Once all the results where collated my consultant decided that I had a form of Stiff Person Syndrome, Progressive Encephamyelitis with Rigidity and Myoclonus. I’m still not hundred percent sure that he’s right, I talk to other people with the condition and I can’t relate entirely with them…but then they don’t relate to each other, it seems each one of us is different but we are all diagnosed by anti bodies found in our blood. I guess if the condition was not so rare then there would be more similarities between patients, who knows?

Back to the Present

Since my diagnosis of Stiff Person Syndrome I have been trying out treatments that could make a difference. First I was given a big dose of steriods, intravenously for a week then in tablet form. The only thing they did was make me blow up like a balloon. So my consultant put me forward for IVIG, (Intravenous Immunoglobulin) which I’ve been having for nine months now. The first couple I only had a minor improvement but the third one was much better. I think mostly because I stopped expecting a miracle and settled for improvement instead. I have a LOT of symptoms which I won’t bore you with now (well done if you’ve got this far!) I have to be grateful for the lessening of any of these symptoms and the IVIG does help. It just doesn’t make me walk again and that is my biggest wish, my dream. I won’t give up on it, ever. I know my condition is progressive, but I’m not going to give in just yet.

Rare Disease Day 2018

28th February is Rare Disease Day, when those of us with rare medical conditions shout out loud, look at us, we are ill too. Find us a cure!

I’m one in a million. I’m a medical zebra!

I wish I was a horse!

a zebra in a field of horses

 

School Runs and Shopping Trolleys
Naptime Natter

A little Note About Positive Reviews on Raisie Bay

A little Note About Positive Reviews on Raisie Bay

Some people only write reviews when things go wrong with products, which is good because it lets people know that there could be potential problems. I’ve also seen negative feedback with say things like, I had to return this item because the colour did not suit me…is this useful?

I write reviews on most items I buy because I like to give genuine feedback. If I have a genuine problem with a product I will write my review in the appropriate place.

I write reviews on my blog too, but they are mostly positive. Why? Because I only write reviews for the things I’ve loved. If I don’t love them I let the person who sent me them know with details why and then let them decided if they would rather me write a negative review or not write one at all. It’s always the latter.

This is my blog, my place and I’ll let you know about the things I love. If you want to find out what other people have hated about the product then you will need to look elsewhere.

My reviews may all be positive, but they are still genuine.