stiff person syndrome

My Story in the Paper

August 13, 2018 by Anne Sweet 4 Comments

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’

Two and a Half Years (and a couple of weeks)

June 13, 2018 by Anne Sweet 2 Comments

In the Beginning

Two and a half years and a couple of weeks ago I woke up with pins and needles and ended up in hospital paralysed. It seemingly came from nowhere, but I now believe I did have hints that something was happening with my body.

After two weeks in hospital I came home with a zimmer frame and the promise that after six weeks physio therapy I would be fine. Nine months later when I saw my consultant next I turned up in a wheelchair, things were not fine.

I’d had an MRI in hospital which hadn’t told them much about what was wrong with me, so my consultant did blood tests, muscle tests and nerve tests and I ended up with a diagnosis of Stiff Person Syndrome, or a specific type of SPS known as Progressive Encephemyelitis with Rigidity and Myoclonus, or PERM.

So for the first year I was expecting to get well, but exactly a year after my hospital admission I found out that not only was I not going to recover, but my illness was progressive.

Year Two

I am still in a wheelchair although I can walk for short periods with aids. I walk around my home but I always have something to hold on to. I don’t walk outside very often, I’ve tried sticks, a rollator (walker with wheels) and crutches. They all help with balance but nothing can stop the pain and fatigue. I get so far and my body just doesn’t want to move any further.

I take a cocktail of drugs every day, but the pain is always there, I may be able to dull it, but it never goes away. Sometimes my body freezes up into a complete spasm. Sometimes I can’t swallow properly, sometimes I can’t breath properly, it’s unrelenting.

Apart from the drugs I’ve had intravenous steroids which helped in the first instance, but did little to help after the second round. I have regular doses of Intravenous Immunoglobulins (IVIG) but it doesn’t help much. I get reduced spasms and it helps the myoclonus (jerking) but it’s not a miracle cure as I’d first hoped it would be.

Parts of my body are always stiff, parts are always in pain, parts always have pins and needles or neurological pain. It affects my sleep, it affect my life, each and every day.

I also have startle reflex, so a loud noise or something to make me jump, or even an intense emotion can make me go stiff, if I’m standing I may fall, if I’m sitting or lying, it just hurts.

I also have vertigo which can come at any time and last anything from minute to hours. This makes me feel like I’ve just downed a bottle of vodka, but not in a good way. Double vision, dizziness, loss of balance, the rotten stuff.

I could go on and on with symptoms but I don’t want to bore you.

Year Three

Yes, I’m half way through my third year, my youngest can’t even remember the times when I could walk. When I used to chase him around the school playground while waiting for my older girls to come out of school.

People get fed up of you when you are sick for a long time. At first it’s concern;

“How are you?”

“Are you feeling any better?”

“When’s your next hospital appointment?”

“How did you get on at hospital?”

Then they get bored;

“Are you not well yet?”

“I thought you’d be walking again by now.”

I’ve even been accused of moaning too much and not getting on enjoying my life.

I’ve even been accused of competing with others for attention.

Yes, people have had enough of me and my sickness.

Do they not think that it would be my greatest joy to announce that I’ve had a treatment that has worked, that my pain has gone and I can walk again? No, for some reason I’m just a miserable old cow because I haven’t gotten any better.

The Future

Despite what some people think of me I have always been a positive person and I’ve approached each treatment with excitement and determination. Yes, it does get me down when it doesn’t work, I don’t think there is anyone who wouldn’t feel the same. I do get into some very dark places sometimes when I can see is the pain and disability and the hurt I cause others. Sometimes I think my family would be better off without me.

Then I bounce back and fill my life with happy things. I start projects that I can get excited about, something to look forward too. Plans, future plans. Even if things don’t get better I can cope if they stay the same. If I get worse then I’ll deal with that too, one day at a time.

I am working with some other Stiff Person Syndrome sufferers to gain awareness for our condition and hopefully trigger more research into diagnosis, treatments and even cures. I am reading studies, finding out all I can and most importantly, learning from others who are living with it. It seems daunting the amount of research I am doing, but it gives me a purpose and hope.

I have also decided on what I want to ask my consultant when I next see him. I’ve found out that there is a drug out there that have helped people just like me to get back some life. I don’t know if he will approve it but I’m going to ask all the same. Also, I will give up the IVIG, yes, it helps me a little but I know it’s in short supply and others gain more from it than me.

If you’ve read this far, then thank you, I know there are people out there who genuinely care. Just as I know I will never give up hoping to get well again.

A Medical Zebra, a Post for Rare Disease Day 2018

February 26, 2018 by Anne Sweet 15 Comments

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!

Word of the Week – Intravenous

January 12, 2018 by Anne Sweet 9 Comments

As you can guess, it’s treatment week for me as I go to hospital each day to receive my IVIG. (Intravenous Imunoglobulin) That leaves me very little time for anything else.

Today is my last day and I’m feeling okay, no side affects as of yet and a decrease in pain…fingers tightly crossed.

Another thing that happened this week was that my kids went back to school. It was a little stressful at first but more for me getting everything ready for them, than for them. They were excited to get back and have been great.

So that’s my week in a nutshell! How’s your week been?

Happy New Year!

January 2, 2018 by Anne Sweet 8 Comments

Happy New Year to all my readers!

I know I’m a little late but we had an awful Christmas and New Year’s Eve, so last night we had our traditional munchies, film and family time and it was much more relaxed and finally we can celebrate the New Year.

Although it was a really tough week I’m happy to report that the kids had a great time. Their little face on Christmas day where all I needed to make Christmas special. There is nothing like that feeling and I will miss it when my young ones grow up.

So, with Christmas over it’s time to start thinking of the New Year and what it will bring. Yesterday marked my second anniversary of being ill. Next week is the one year anniversary of my actual diagnosis, Stiff Person Syndrome with Progressive Encephamyelitis with Rigidity and Myoclonus.

Next week I go into hospital for the third round of IVIG (Intravenous Immunoglobulin.) The last time it left me with the most horrid migraines that I thought I was dying. Seriously, I’ve been a migraine sufferer for over 30 years and I’d never experienced anything like it. It was also a hemiplegic migraine which meant I also suffered paralysis and my poor hubby thought I was having a stroke and took me straight to A&E. Next week I am going to be more prepared, plenty of water (at least 3 ltrs a day) and regular pain relief. I am staying positive. The first time I had IVIG I thought it was going to be my miracle treatment and when it didn’t work I plunged into depression. The second time, after the awful migraines had stopped, I did see some improvement. It wasn’t the miracle I seek, but a little relief from some of my symptoms was well received. Unfortunately they didn’t last. I can see myself getting worse all the time and this scares me so much. The life expectancy for someone with my condition is 3 years and I am now into my third year! The good news is that the condition is incredibly rare so it’s difficult to go by the original life expectancy because most of the deaths were in the early days before they had worked out any kind of treatment. Also, most of them had other conditions as well, cancer, diabetes and epilepsy. I don’t have any underlying condition that is life threatening.

Next week I am hoping for less side effects and more positive results, those who have IVIG regularly tell me that it works better each time (but it also stops working after time.) I have to stay positive!

I’ll be honest with you, my positivity has not been around for the past week or so. I wanted to take a break from my blog and social media because I felt that being negative was not the impression I like to give. I like to show my positivity, I’m faced with so much trauma every day but I find sharing positive blog posts and messages helps to keep me focused. However, when you are feeling low and close to wishing this life would end right now, it’s so hard to express exactly how you feel without it looking like you are seeking sympathy, or even worse, trying to make others feel bad.

Sometimes, though, you have to be honest, and honestly I’ve been feeling CRAP. Everyone around me, apart from my little ones, has made me feel sad and low. My illness has been awful, I have felt so so ill, and all the time all I’ve wanted to do is enjoy the holiday and feel the Christmas spirit. And my family has just made me feel worse. I’ve put up with so much sh*t in the past week, too much. If it wasn’t for my little ones I think I would have just given up on everything.

But, my little ones have been my joy in the madness. They have been so happy and carefree. I may have been frustrated with them a couple of times but it was nothing that I’m not used to. Also, my little Star became a young lady over the holiday. She’s really growing up now. I know she is behind in maturity, but some things you can’t delay, and a 5ft 5 inches you cannot think of her as a little child anymore.

So, today is a new day and despite the fact that my arm has gone into an incredibly painful spasm that has now lasted more than 12 hours, I am going to keep plodding on and dig deep because my positivity is in there somewhere. I cannot allow myself to go into this year feeling so low.

I have much to look forward to this year. I am staying hopeful that next weeks treatment will be a good one. Then in April I am going on a fabulous Blogger Retreat which I’m really looking forward too. In August we are having our first 7 day holiday in many years (We usually just have a short 4 day break) the kids are going to be so excited. There are places I want to go, things I want to see, memories I so want to create. If this is going to be my last year it’s going to be great! But of course, it’s not going to be my last year, I’m not ready for this stupid condition to beat me yet. I’m ready for a fight!

It doesn’t matter how many times I fall, I will always pick myself up and carry on. When I did the Activation Game I exercised being the best possible version of me for a day. It’s always a great thing to strive for, being a better version of yourself, and something that I often try to practice.

How about you?

Happy New Year