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Posts Tagged ‘Still’s Disease’

After emailing my Rheumatologist with an update yesterday, he replied to say that the referral to start the Stem Cell Therapy and Bone Marrow Transplant procedure had gone through.

Yikes. That conversation was real then.

I’m not commited to anything at this point, nor am I even close to making a decision about it; I have simply agreed to hear about what the procedure entails as a treatment option for Still’s Disease.  It has been agreed that we have exhausted all other options and the medications are just not working for me. There was a suggestion that maybe I could try a combination of biologics, which is unchartered territory but perhaps less risky than the SC/BMT. I need to revisit this idea with him at some point as I forgot to ask about it during our last conversation; the focus becoming SC/BMT.

There is only one hospital in the North of England that carries out the procedure and that is in Sheffield, about 90 miles away from home. That’s going to make it tough spending any long amount of time there. The funny thing is, that the only Haematologist to carry out the procedure also happens to be my Rheumatologist’s brother, which makes me feel I will be in safe hands. There shouldn’t be any communication issues between the two at least!

The first step is to see a Rheumatologist called Dr Akil at Sheffield Hospital. My Rheumy thinks it’ll be about four weeks before I get an appointment, but I wonder if even that is optimistic. It doesn’t sound very long and I’m kinda nervous about seeing a new doctor after all these years. I’m wondering if he will have access to my huge volume of notes, or if he will want to do some tests and investigations himself.

I was also sent some information about the procedure, although it is so rare that he hasn’t been able to find any patient-based info. Instead, it is a 21 page article full of medical jargon. I couldn’t face reading it straight away, it was enough to have it and realise that all this is very real. Today though, I decided there was no point sticking my head in the sand and that I needed to face it head on. It is going to be a massive decision and so I’m going to need as much information as possible to make it properly, plus enough time to process and take it all in and to address any questions I have about it all. Better to start now then.

And start I did, but after two and a half hours of reading I am only six pages in and have decided to leave it there for the day. Because I want to fully understand what’s in front of me, I’m constantly checking up on word meanings and acronyms and making notes in a separate document. It’s like being at school again, which would be great if it wasn’t such a personally daunting subject. My GP, (who popped round earlier to see how I am getting on since leaving hospital), has kindly offered to interpret anything I don’t understand though at least.

So what have I learnt so far?

  • Only 3000 of these procedures have been carried out for people with autoimmune disease since 1995 – worldwide; about 175 a year.
  • It will be an ‘Autologous’ Stem Cell Transplant, which means harvesting and replacing my own cells.
  • Remission is possible, both longterm and temporary; if temporary, returning symptoms tend to be easier to treat.
  • It is also possible that there will be no change and there is, of course, risk of death.
  • With Still’s Disease, the main risk of death comes from Macrophage Activation Syndrome, which can occur at either stage of the procedure. This is something I have already suffered from, I discovered recently.
  • My Still’s medication would be stopped as early as possible and I would have to rely on Steroids to prevent any further flaring.
  • I would need chemotherapy – I guess that might seem obvious but seeing it in writing makes it real.

And that’s about as far as I’ve got really, wading through all the medical jargon. I’ll read some more tomorrow and keep posting about it here. Since it is such a rare procedure, I think it is a good idea to keep a record of it for others that may need it in the future, even if I don’t go the whole way. Because it doesn’t just stop at understanding the procedure itself, or even coming to terms with the risks – it’s the whole impact on my life and future, the future I share with my fiance and any plans that we might have to start a family etc.

So much to think about,

L

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I had an appointment with the dental surgeon today, regarding my wisdom teeth, which came through malformed and black. The two on the left have now started to break apart and so my dentist felt that they needed to be removed. They’re not really causing me any pain, but they are providing an infection risk because of my Still’s medication.

The doctor I saw was really lovely. I can put up with doctors poking needles into various joints and performing all sorts of procedures on me… but when it comes to the dentist I’m an anxious wreck! She explained how my bottom tooth was vertically impacted and very close to my jaw bone, so she would have to remove a substantial bit of gum and bone, splitting the tooth root into two before removing it. I had an xray to check the position of the facial nerve; there’s a risk that they can damage this and cause drooping to the lip and numbness of the tongue. I’m hoping the risk is small and she was just covering herself, as I don’t want any more problems. Removing the top tooth, thankfully, appears to be more straight forward.

After telling me about gum splitting and bone cracking, that I would be bruised and swollen for about three weeks, she then asked me whether I would prefer a local or a general anaesthetic… guess what I picked –

General. I’d much rather be blissfully unaware as they torture my mouth thank you very much.

I had my last two teeth out via General Anaesthetic too, so I’m hoping this won’t be a problem. She seemed to think there may be an issue surrounding my Tacchycardia, but I seem to remember them saying it was easier for them to monitor this during a General Anaestheti, where you’re hooked up to monitor… and trust me, if I were awake it would only make my heart rate rocket even more than normal!

So, all the paper work is done, I just have to wait for them to get in touch with a date now. In the meantime, I’ll be trying to book a holiday before its due, so I don’t look hideous on any holiday photos!

L

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It’s the day after my first Tocilizumab infusion and I’m feeling a bit rotten but Im guessing that is more to do with the long day, sitting in uncomfortable chairs and so on, rather than the drug itself. Thankfully, there has been no further signs of reaction so I’m hoping that was just a one off.

I had an early wound care appointment at the clinic and then I planned to spend the rest of the day taking it easy, to save some energy for tomorrow.  The wound is starting to close up at the ends and turn a nice shade of purple there, but is still overgranulating along one edge, meaning it is not healing as it should. Today the nurse covered it with a double pressure pad to encourage the excess tissue back where it belongs. I don’t think it is a major problem and it isn’t bothering me in anyway, but the scarring will be much neater if it stops overgranulating and heals as planned.

By the time I got back home, the postman had been and I had a letter waiting for me. Inside where two things: Firstly, a copy of the Occupational Health report on my current condition and then an appointment date for my next hearing with the Head and Human Resources – a week today.

The doctor’s report was only a paragraph long but it was still hard to take in his view of me. I’m forever trying to look on the bright side and convince myself that things aren’t that bad, so when I see things in writing from someone else, it comes as a bit of a shock. Basically, he says I suffer from severe Still’s Disease and am failing to respond to potent medications, meaning it is difficult to predict if/when the condition will be stabilised enough for me to return to work. *sigh*

Don’t get me wrong, I’m still sure that we will find a medication that will stabilise things as we have done in the past; I had a good few years with only occassional flareups and minimal joint involvement so I know it is not impossible. But, I also know that it is becoming more difficult and taking longer, and I need to allow myself that time to stabilise without feeling guilty about work.

Which brings me on to my next point: The Stage 2 Capability Hearing. I don’t know what my management is thinking about the current situation, but I imagine that they want someone to be doing my job and would rather terminate my contract than leave the situation long and drawn out. However, I’d also guess that they don’t want to risk terminating my contract in case there was some comeback from the Equality Act, (especially since I work at a SENs School). Then I’m torn between fighting for my job and feeling that I could do without the niggling guilt and added stress (like capability hearings) that come with having it right now.

Even having to think about it all is more than I want to do right now; it is so hard to make decisions and handle situations like this when you feel so flat.

L

Related Posts:

Preparing For DoomWork and Other Worries, Work Issues,  Occupational Health Appointment.

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Apologies if this post is a bit rough around the edges, but I wrote most of it post-surgery and I’m feeling rough around the edges too!

Infusion days always feel full of hope at first, that today could be the day that things turn around. We arrived on the ward at the normal time and had the usual pre-infusion checks – Blood test, BP, Pulse, Temp etc.  The nurse asked about any new symptoms / pains, so I had to mention the stitch-like pain beneath my ribcage, even though it could disappear as fast as it came – there have been no obvious side effects from the Orencia, thankfully.  With the cannula fitted easily, it was back to waiting for the test results and the go ahead for pharmacy to mix the infusion.

My rheumatologist came to see me a couple of hours later and I filled him in on how things had been since the last infusion – to cut it short, that I’d been having too many days when I couldn’t even get out of bed on my own and so had increased my Prednisolone to 20mg again, and that I’d been more stable since but still not brilliant.  My blood test results told the same story, I was still anaemic with a haemoglobin of 8.3, but there was a slight improvement in my inflammation markers, with my CRP down from 112 to 80ish, thanks to the extra steroid.

For the first time since starting Orencia, I am beginning to lose my enthusiasm for it and my rheumy pretty much admitted the same thing.  We both want to give it a full opportunity to work though, so are going to stick with the infusions a bit longer. I mentioned that it has definitely helped with the fatigue, which is the difference that I noticed straight away and what got my hopes up initially, but it just hasn’t reached the joints yet and I’m not sure if it will.

The infusion itself went fine, no line resistance thanks to the vein having time to recover and sticking to those guidelines too of course! I didn’t feel the rush I had felt after my first few infusions but then I’d had a late night and was pretty shattered anyway.  I’m trying not to read too much into it, but it is getting harder to believe things could turn around at this point.  I just don’t want to be negative but at the same time I need to be realistic.

My next infusion is in three weeks, rather than four, to fit in with my trip to New York, and in the mean time I need to double the dose of Methotrexate to 15mg – ugh.  I am definitely not enthusiastic about that but I’m willing to give it a go if it gives the Orencia a better chance.  It is still smaller than the dose I was on all those years ago; I just hope I don’t get hit with terrible nausea when things have been so good so far – I don’t want to be sick on the plane or in New York(or anywhere for that matter!)

Speaking of New York, my rheumy seemed really pleased that I was going and said it was one of his favourite places.  We made a plan to try and make sure I will be as well as possible for going, including the early infusion and then a couple of joint injections to give me some relief/movement in this left arm, and maybe another increase in the Prednisolone.  I felt really pleased that he was supporting me, because it reaffirms that I can’t let the Still’s Disease get in the way of enjoying my life.

So for now, I’m just trying to take it a day at a time and not look too far ahead. Ce sera sera.

L

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I started this blog at the beginning of June this year, with lots to say but not really sure if anybody would be interested in reading it.  I knew there were plenty of people out there with Still’s Disease, but finding them was a different matter – we tend to blend in with the RA crowd, or there is confusion regarding the interchangable term Systemic JIA.  Then slowly, but surely, more and more people found their way here, through links, facebook and search engines… and today we reached 1000 hits.

The Top Ten Posts so far have been:

  1. Home Page
  2. My Story
  3. A Slightly Better Day
  4. Still’s Disease
  5. Confused About Still’s Disease?
  6. Articles Section
  7. First Orencia / Abatacept Infusion
  8. Round Two
  9. Poor Veins For IVs Blood Tests
  10. Two Cortisone Shots Later

I hope that the information I post has been useful to some people; it is difficult to find info that corresponds directly to Still’s Disease rather than RA, but it is out there and I’ll keep on posting what I find.  My intention was always to use this blog as a stepping stone to creating a UK Still’s Disease website and I’m hoping to soon make that a reality, so watch this space.

Thank you for reading,

L

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I was surprised to find six new articles about Still’s Disease in my inbox this morning, thanks to the drug Tocilizumab being approved in the treatment of children with Systemic Juvenile Idiopathic Arthritis.  This drug is being hailed as a wonder drug in the treatment of Still’s (sJIA), with patients so far showing a 90% increase in wellbeing; as these articles state, even children that were previously wheelchair bound have been given a new lease of life.  What I didn’t know, is that Tocilizumab is actually the first drug to be approved to specifially treat sJIA/Still’s.

The first article from the Daily Mirror tells of a young girl with sJIA, who was bullied for being in a wheelchair and was crippled by pain, even with Morphine, but is now leading a ‘normal’ life thanks to Tocilizumab; she talks about how it has literally transformed her life, which is what we all want.

The next article  from the Daily Mail is my particular favourite, since it features my Paediatric RD Eileen Baildam, who played a major part in the drug trials:

“Dr Eileen Baildam, consultant paediatric rheumatologist and triallist at Alder Hey Children’s Hospital, Liverpool, has treated 12 children with the drug and seen them all make a remarkable recovery.  She said ‘These are very sick children, the disease affects every single joint in their bodies as well as heart and lungs. They can die from heart failure and other conditions if they don’t get treated. It’s much worse than rheumatoid arthritis in adults.”

The article goes on to say how two thirds of patients treated with Tocilizumab had a 70% improvement after just three month, with this increasing to a 90% improvement after a year of treatment, with little or no side effects.  The drug costs the same as similar biologic therapies available, but the NHS is currently assessing whether this will prove cost effective in the treatment of children.  Dr Baildam says:

 ‘I hope and expect it will be approved and I think it should be given to children as soon as they are diagnosed to limit the disability caused by this dreadful disease.’

And I would agree with this – surely in offering this drug to children as soon as they are diagnosed will limit the amount of damage the disease causes, mean less hospital admissions, tests and procedures, plus shorten the length of time spent by doctors trying different medications, many of which have serious side effects that could in turn lead to hospital treatment being required.  All these things would suggest that early treatment is absolutely cost effective.

The other articles are all pretty similar but I’ll link to them anyway: The Independant Health News and The Liverpool Daily Post; the sixth one was very basic so I’ll leave that out.

All of this is extremely good news for Still’s patients, whatever their age.

L

Ps. To see why the terms Still’s Disease and sJIA appear to be interchangable in this article, please read my earlier post ‘Confused About Still’s Disease?‘  This interchangability causes much confusion.

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As I mentioned in my last post, I have had more good days than bad this week and am feeling much better in myself, even if the joint pain and swelling is still there.

This could be down to the Orencia and Methotrexate combination I started at the end of June; I have had three infusions so far and my next one is scheduled for August 25th.  I was sure that I felt a difference after the first, I had more energy and even managed to have a dance at my friend’s wedding a couple of days later. However, at my last infusion, my rheumy was concerned that my test results were actually getting worse, which of course doesn’t fit.  I was confused  but decided to listen to my body and take every day for what it was; time would tell in the end.

I have read of other people who experienced an increase in wellbeing and energy levels, whilst being treated with Orencia, despite still having active disease and joint involvement.  Perhaps this is the case for me, or maybe it just takes little longer to dampen down the inflammation?  But there is another factor that could explain my current ‘wellness’ and that is where the dilemma comes into play:

Both times I have been on the better side I have been taking 20mg of Prednisolone, which I initially tried to taper off.  The most recent increase to 20mg was just under two weeks ago and I’m still currently taking it, grateful for some relief.  We all know how effective Prednisolone is at providing this short-term relief so it would seem that this is obviously to thank for my good days (in part at the very least). 

But my worry is, how are we supposed to know how well a new drug is working for us if we ‘mask’ our symptoms and disease activity by taking Prednisolone and even pain killers?  I know that the theory is to taper off the steroids after so long on the new treatment to see if it ‘holds’ things at bay, but how disappointing to go through months of treatment, convinced that it is working and find out that it was only the Prednisolone doing its stuff, not to mention the frustration of having to do it all over again with another drug.

I’m not suggesting we shouldn’t use steroids and pain killers to tide us over the worst times; in fact, I don’t even want to think how bad I’d feel without them!  I just wish there was an easier way.  It’s hard to explain to people that see me looking a little better and want to enthuse about my new treatment, that it might not be down to that after all – just a pred boost.  Speaking of which, I saw my manager while I was out yesterday and immediately felt guilt ridden.  There I am, out and about, yet unable to let them know even a general idea of when I’ll be fit to return to work.

This doesnt mean I have given up on the Orencia working for me.  I’m probably being a bit impatient; after all, I was told it could take 3-6 months to really feel any benefits and I’m not even at the two month mark yet.  I just don’t want to have to depend on the Prednisolone for good days over the next few months and I want to be able to tell whether my new treatment is helping or not – even a general idea would be good. If only scientists could produce a similar drug to Prednisolone, without all the horrible side effects, all of our problems would be solved.

It’s a battle of wills, but we must keep fighting.

L

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According to the article, the drug known as RoActemra or Tocilizumab has been approved for usage in children over the age of 2, who are suffering from Systemic Juvenile Idiopathic Arthritis, or Still’s Disease, in the EU.

This is very good news, as I have heard nothing but good things about the drug so far. I also have a fellow Still’s buddy who is doing very well on it.  Only recently, I posted about an article on a young girl who had been refused funding for Tocilizumab by her local PCT, one reason being due to her age.

I hope that the news gives this family, and others, another treatent option and, more importantly, some relief.

L

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Following on from yesterday’s retrospective post, I spent the night on the Short Stay Ward with a view to have a CT scan and a couple of other tests today.  Before I carry on, I would like to point out that I have no problem staying in hospital and I’m not one of these people that look for a reason to complain or be awkward.

I’ve mentioned here before that I often struggle in the mornings at the moment, ranging from needing help to sit myself up in bed, to waiting for my painkillers to work before I can function well enough to shower, dress and generally move about better.  This morning was one of those days that I woke up unable to really move thanks to pain and stiffness, not even to lift the blanket away – as a young person, this is difficult to admit and I would certainly never exaggerate or play on it in any way.

The breakfast lady woke me and put a bowl of cereal and a hot drink on the bedside table; I told her that I needed some help to sit up and eat and she said she’d let someone know.  Twenty minutes later, someone else came and took my uneaten breakfast away and placed my tablets on the table instead. Again, I tried to explain that I hadn’t eaten because I needed help to get myself up and would do to take my tablets too, she said she’d let someone know but later came back with a wash bowl and told me to have a wash as ‘it would make me feel better’. I tried to explain that I couldn’t get myself up AGAIN but she just huffed and went away before I had chance to finish.

I was starting to really hurt now, being stuck as I was in the same position.  Then half an hour or so later, two nurses came to change the bed and told me I’d have to get up. I tried to explain again that I would need some help sitting up; their response was to stand and stare then say ‘Just swing your legs round and sit yourself up’. Not very helpful but I kept calm and tried to explain that I couldn’t as I was in a lot of pain. ‘So why haven’t you taken your painkillers then?’ they asked me, quite sharp by this point. I couldn’t believe they had to ask. ‘Well it’s not like we can lift you up, you’re not a baby’. The other one spat.

I was just trying to explain that all I was asking was for a bit of a push/ support on my back to prop me up in a seated position, from which I’d eventually be able to unfurl my locked arms and legs, when the first nurse grabbed me by the (extremely painful) arms and started to drag me to the edge of the bed; because I ended up horizontal across it, the second nurse proceeded to grab my ankles and pull me by them too. I don’t know if they were expecting a miraculous recovery, for me to sit up and say ‘okay I give in, I can actually sit up I just wanted to make a fuss…’ but if it had carried on a few seconds more I’d have ended up on the floor.

Thankfully, at that point, a doctor appeared and asked them what was going on.  I just burst into tears and in all my life, in all the times I’ve spent in hospital, I have never cried. But then I have never been made to feel so humiliated either.  The doctor calmed me down and asked me what the problem was; as soon as I told him he gently gave me the right support to prop me up in bed so that I could take my painkillers and told the nurses that it was actually in my admission notes that I was having some mobility problems and might need some help.

As always, I was moving much better after a couple of hours and had to then listen to the two above nurses discussing how I’d had a ‘miraculous recovery’, at the end of my bed of all places! When I tried to explain to them the reality of living with Still’s Disease and flares, I was met with dirty looks and they promptly walked off, but I had to put up with petty, snide remarks for the remainder of their shift.

You can probably tell that this incident upset me but I’d been told that my CT Scan was booked for 2.45pm and that I could go home if it was clear, so I just kept my head down reading until that time, still thinking it best to get the scan done.  Sure enough, at 2.45pm I was taken down to the scanning room, only for them to tell me that I was supposed to have a green canula fitted to inject the contrast that would show up my blood vessels.  The ward knew this but nobody had even tried. The doctor there did try to insert one, but because of my bad veins she couldn’t manage and so I was sent back to the ward with the promise that someone would insert the canula there and take me back down before it closed at 5pm. Funnily enough, nobody did.

It became obvious to me that I wouldn’t be getting the scan until the next day and I couldn’t bear another morning like this one, so I made the decision to discharge myself against their advice. The ward matron was on duty by that point and wanted to know my reasons and so I told her about my experience.  She did apologise and said she had a friend with Still’s Disease; I hope that at some point she stands up for this friend and myself, by educating the two ignorant nurses in question.

Anyway, I have never been so relieved to be away from a hospital. What I have accounted here is my own main experiences but I also witnessed a lot of other mistakes and mistreatment of patients that I will be including in a formal complaint to the hospital.  Now, my doctors might think me clingy, but this had made me feel even more certain that I don’t want to be treated anywhere else but at my own hospital, with people that know my background; people I trust.

Oh, and I never did get the blood transfusion!

L

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Today is the last day of July and Juvenile Arthritis Awareness Month; that doesn’t mean that I think we should relax our attempts to raise awareness but I still wanted to mark it by looking back at my own experiences. In a way it follows on from my post a few days ago, on children learning to cope with Juvenile Arthritis.

Sometimes, I look back at photographs of me as a small child smiling at the camera and feel sad, not for myself but for the person that little girl could have become; for all that I know she will have to face in her future.

I was too young to really understand the significance of what was going on throughout my first phase of illness; I just remember feeling unwell in exactly the same way I do now – with the fevers, fatigue and pain.  I must have known that I was different to other children in certain ways; after all, not every four year old needs a buggy because they can’t walk long distances, or wears a neck brace and leg splints, but I felt happy and secure in my infant world.

As a teenager and young adult it became more difficult; I mourned the loss of the life I’d had in my years of remission, the activities I had to give up and the new experiences I felt I was missing out on. I’d always had a head full of dreams and ambitions but I had to learn to adapt them to fit in with my limitations – instead of being active and sporty, I became academic; career plans and dreams of world travel and charity work fell on the back burner, but I found a job that I enjoyed and visited beautiful places closer to home. While other people sailed through life I plodded along slowly, a few steps behind, but always reaching my goals.

But it wasn’t the life I’d dreamed of and at times I was consumed by frustration at everything that this illness had taken away from me, before I’d even had chance to experience it.  I would find myself wondering where that little girl in the photograph, the younger me, would be if it wasn’t for Still’s Disease; what type of person would she be now if I’d never got sick? Surely, her life would be better without all this? Maybe she’d be prettier, more confident and successful – sailing through life with everyone else…

But then another part of me, (the part that’s like a slap in the face and tries to make me see sense when I’m feeling low), tells me that this isn’t neccessarily true and besides, if it wasn’t for everything that I’ve been through over the years I wouldn’t be the person I am today – and you know what, I actually quite like that person.  How different do I really want to be, truly?

Living with this disease has opened my eyes to so much that I may have failed to notice otherwise. Over the years, I’ve learnt to value the things that are really important to me in life, to try not to worry too much about the things that aren’t; to appreciate beauty and pleasure in the simplest things, to find the silver lining in every cloud and to treasure the most important thing of all – life itself.  I hope I’m right in saying that it’s made me a better, stronger person in many ways.

So I ask myself again, what would I really be like if I hadn’t gone through it all and would my life really be any better?  Of course, we’ll never know for sure, but there’s an equal chance that I may be worse off; that I wouldn’t be as content as I am right now, or as determined; that I wouldn’t be surrounded by so many wonderful people both in real life and online, know the value of real friendship and love. 

Perhaps things haven’t turned out too badly for that little girl after all.

After rethinking things in this way, I look at that photograph again and instead of feeling sadness I feel something closer to pride; I think of everything that little girl has had to face and yet she’s survived and come through it all, and most of the time she’s still smiling.

And I hope that others can learn to view things in this way too. Many people deal with chronic illness every day, we’ve all experienced that sense of loss and wondered what if? But we are who we are because of the things we go through. It may sound cliche, but every scar tells a story and whatever doesn’t kill us, really can make us stronger. Now if only we could transform that into physical strength, we’d all be superheroes.

L

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