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

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.

 

 

Keep on Hoping, Keep on Wishing but Most of all Keep on Believing

Last week I went to A&E because I really thought there was something wrong with  my heart. It felt like someone was sitting on my chest, crushing me and making it hard for me to breathe. I called my GP but I was told to go straight to A&E. I was given a red card on entry which meant I was seen straight away. They put me on a ECG monitor and checked me over. Then I was to wait to see a Doctor. As I was waiting over two hours I figured that they’d worked out that I was not having a heart attack.

I saw the Doctor and after lots of questions and further examination, and a consult with someone higher up the decision was that my pain was due to my condition, Stiff Person Syndrome. There was nothing they could do for me and I had to see my consultant for treatment. They couldn’t contact my consultant so that’s up to me, but I have an appointment in two weeks so I’m not sure they will see me any sooner.

For now I just have to deal with it an accept that this pain is yet another one I am going to have to get used to.

I also have been getting pain in my arms and hands, getting dressed has been really awkward. Again, I’ve been told it’s due to my SPS which is progressing. I was affected from my waist down, now it’s from my neck down. No, from my head down because I also get triginemal neuralgia which is another symptom of SPS and affects my face.

My first reaction to the news was to panic. I sobbed. I don’t want it to progress so soon, I knew it would eventually but it’s not even been two years yet. A lot of people with my condition (which is not a lot really because it’s so rare) have died within three years. The positive side to this news (for me anyway) was that they were not diagnosed in time and did not receive the right treatment. I’m extremely lucky to have been diagnosed so quick, and there are treatments available, so I’m still hopeful. What I am worried about it my consultants lack of concern, he didn’t even follow up to see how my last treatment (in June) went. I’m going to have to be firm with him when I see him next. I can no longer wait six months between appointments, if he’s too busy to fit me in then I will see someone else.

So after my self pity, (I really don’t want to lose my hands and arms, if my legs don’t want to get better I’ll deal with that but I can’t lose my upper limbs, I just can’t) I’ve decided that I’m going to bring out my fighting spirit. I’ve researched all the treatments that might work and I want to try them until I find one that does work. Of course, I would have to get the rarest form of an extremely rare condition, which means my consultant will have fight for the treatments, but I’m determined not to give in.

I also need to be pro-active. I have let my diet slip again and I do get lazy. Activity causes me pain, but if it’s the only way to keep my body doing what I want it to do then I need to be more active. I will walk more, I’m thinking of getting some of those comfortable crutches that support the arm so they don’t feel all the stress of holding me up. I have a friend who is great with alternative medication and although she’s been having a rough time this year with her own issues, I think she’s ready to start advising me again now. I am also in contact with other sufferers which helps a lot.

Sometimes I think that, you, my readers, get fed up of me harping on about my condition. But, this is my place and sometimes I just need to share. It makes me feel better, writing things down can be so cathartic and sharing my story may help someone else in the future. I know that reading another sufferers story as helped me, she’s into her 16th year and it’s been really rough for her, but now she has found the right treatment that works for her and is enjoying life again.

Just a couple of weeks ago my wish would have been to walk again. Now, that’s just a dream, a bonus. My wish now is that I can stop this condition from continuing it’s progression.

One thing is for sure, I won’t give up. I haven’t tried half the treatments that are available yet, and as much as I despise the thought of being on medication for the rest of my life, if it gives me more life then I will take it. I have children who need me. My husband needs me. And I don’t want them to be my carers I want to be the best I can and the only way to do that is to fight this illness and not let it get the better of me. It won’t win, I will! 
School Runs and Shopping Trolleys

Debs Random Writings


Photo by crabtree on Unsplash
Photo by Alex Cagwin on Unsplash

One In A Million

I’ve just finished reading One In a Million by Jaqui Atkinson

I read it in two days, it was really difficult to put down.

Here is the ‘blurb’

Imagine all of your muscles going into spasm, and being unable to move a single part of your body. Imagine being in excruciating pain. Then imagine how it feels when the doctors tell you they don’t know what’s wrong. And they don’t even believe you… It took six years before Jacqui Atkinson was diagnosed with Stiff Person Syndrome, a very rare neurological disorder which affects just one in a million people. This is a remarkable story of courage, of faith, and most of all a story of love. This is Jacqui’s story. She is one in a million.

 

I too am One in a Million.

I’m not as bad as Jacqui, she has a very bad case of Jerking Stiff Person Syndrome with PERM.
I do have PERM though so I know that my condition will get worse.


PERM – progressive encephamyelitis with rigidity and myeclonus


It was fascinating reading someone else’s story, scary at times, emotional at others. In the end though I felt uplifted. This lovely woman has been through so much but still holds on to life and appreciates every day. She also has a very loving and caring family, despite their own difficulties at times.

I can relate to a lot, I too have painful spasms, but mostly in my legs and lower back. Only recently have I been getting them in my arms hands and upper torso.

I can relate to her ability to sit with her feet straight out. She holds a competition with her doctor and her husband to see how long they can hold their legs up and feet in the air while sitting on a chair. Try it yourself, see how long  you can do it for. When my legs spasm I can do this for up to half an hour.

woman in chair with legs stretched out straight

I think Jacqui’s book explains a lot about Stiff Person Syndrome, how difficult it is to get a diagnosis, how people don’t believe you about the pain, how people don’t understand why you can’t move, how people think you are ‘putting it on.” I think I was lucky, although I did look back in my diary and find a page where I was obviously very upset after visiting my GP as I’d been experiencing pain for quite a while and he didn’t believe me. In fact he referred me to a mental health centre. Yes, he thought it was all in my head. However, when I went into hospital and had a paralysing attack right in front of them they did give me thorough testing and keep me in for a couple of weeks until I could walk a little again. I was given a diagnosis of Transverse Myelitis which actually fitted what I was experiencing. It was a year later and after further testing (EMG, NCS and bloods) that I was diagnosed with Stiff Person Syndrome. Most people have to wait a lot longer for a diagnosis.

I related so much to Jacqui’s first story of how she felt when she came home from hospital the first time. It’s such an emotional period which people don’t really understand.

Jacqui has been through a whole range of treatment and finally, after 15 years, she is living her life much better with pain management that suits her and keeps her most severe attacks from happening so frequently. It was really interesting for me to read about the treatments and their affects on her. I know I will face many of these treatments myself at some time. If the money is available and my consultant is understanding. I really hope I can find a treatment that improves my quality of life sooner rather than later. I know there is no cure but I long for days that are free of pain and my being able to walk again, even if it is only between treatments.

I managed to get through to my consultants secretary this week with the help of the hospital’s patient liaison team. I got my appointment brought forward by a month, so now I only have a month to wait rather than two. Thanks to reading Jacqui’s book I have some questions to ask about treatments. I really want to try IVIG again, but it didn’t really make that much improvement apart from give me a little more energy and releasing a little of the stiffness. I do think that with more treatments I might get more relief from my condition, but I’ve accepted that it’s not the miracle I was hoping for before I had my first treatment. I know that’s the reason I got so low afterwards, I’d built myself up thinking that I’d be able to walk again afterwards but it didn’t happen.

Now, I will face whatever there is to come. There are some fantastic treatments out there, I just need to find the one that suits me best.

If you would like to know more about Stiff Person Syndrome then please visit The Tin Man.

Or please take a look at Jacqui’s book on Amazon.

 

Mummy Times Two
School Runs and Shopping Trolleys

Immunoglobulin – My Word of the Week

I could have had a few words for this week, the most obvious one would have been hospital. I’ve been receiving treatment every day in hospital this week and on Wednesday we had to take The Little Man to A&E. He fell over when leaving school and was fine for about an hour, then he was complaining of headache and became drowsy so we couldn’t keep him awake. Then he started being sick so Dad took him straight to A&E where he was checked out. Luckily he was fine, although it ended up being a late night with them not getting home until 11.30p.m.
Of course I could have chosen Election as my word, by the time this is published we will know the results, but for now I can only hope that the party I voted for will win. It has been a very heated election though, particularly with all the media, I remember as a child that people were a lot more private about their political opinions.
The word I have chosen, however, is Immunoglobulin. This is what I have been having by intravenous infusion at the hospital every day. Immunoglobulin is part of your blood’s plasma which is full of anti bodies. When someone gives blood the plasma is extracted to give to people with auto-immune conditions, like me. 
Having talked to other people with my condition who have had Immunoglobulin intravenously (IVIG) I have heard many different results. The best being that it has made a big improvement after the first dose, the worst being that it not only worked but made them very ill with sepsis meningitis. The average being that it works after a couple of months although it’s not that noticeable until the next dose is due and you realise how dependent you are. It’s all a little uncertain.
So far it has made me feel quite ill, starting with massive headaches and evolving into nausea and exhaustion. Maybe once my body gets used to it then I will feel the benefits. I live in hope. If there is no improvement at all then I will have it taken away as it’s an expensive treatment. If there is, some improvement then hopefully I will get to keep it as it will prolong my life if not make me better. 
I am in hospital today having my fifth treatment today. I am hoping that I will still see some improvement, keep your fingers crossed for me please x

The Reading Residence

My New Treatment

On June 5th I am going to hospital as a day patient for five days to try out a new treatment.

I will be getting an intravenous transfusion of immunoglobulin each day.

I am both excited and scared. I’m excited because I’ve heard that this treatment can be really good. It could make me feel more normal again, perhaps even ease my constant pain. Maybe I’ll be able to walk again. I am scared because it might not work at all, I have one of the rarer conditions that IVIG is used for, I even have one of the rarer types of my condition.* Also, as well as not working, the side effects can be very nasty.

I have lots of forms to fill in about what I can and can’t do and what pain I feel. There is even a form about my feelings and emotions. I have already been assessed physically for what I can and can’t do. After the treatment these assessments will be done again to see how well the treatment has worked. This is necessary because the treatment is expensive and it has to be proven that it works so that I can continue to have it.

It’s not a cure, it’s a treatment. If it works then I will have it every three to four weeks.

Since my new diagnosis in January I’ve been feeling really low. Last year I was positive and believed that  I would get better. Then I had the news that I wasn’t going to get better and, in fact, I had a progressive illness that was going to get worse and could be life threatening.

This new treatment has given me hope again. To be pain free for even a week would feel like a miracle. To walk again, even for a short while would be fantastic. My kids would be so happy to have their mum back.

My fingers are tightly crossed that this works.

For the first time this year I feel positive again.

*My condition is Stiff Person Syndrome (SPS), my type is Progressive Encephalomyilitis with Ridigity and Myoclonus (PERM.)

Debs Random Writings

My Random Musings

Cuddle Fairy

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.