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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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