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Rapid skin thickening


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#1 Vanessa

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Posted 05 December 2011 - 05:36 PM

I have not visited the site for quite a while and I am sending this from Royal Free Hospital.
I came here Friday night with renal crisis which thank goodness is now under control.

I am not 2 and a half years since onset of diffuse scleroderma and I have the renal marker.
I had a mastectomy January 2011 due to cancer and had to stop MMF as Prof Denton felt it could cause a return of the cancer. I had chemo between Feb and May and starting about July my skin began to rapidly harden everywhere except my back. My veins are no longer visible and my skin is extremely painful raging red with inflammation and, as the nerves have been crushed, really painful.

I am taking paracetamol, 2 types of very strong pain medication and Amitriptyline for the pain and it still doesn't stop. It took 5 attempts with a tiny butterfly needle to draw blood for testing and I was screaming because it was so painful. They were unable to get a canula in and I am meant to be going down tomorrow for a central line in the jugular so that I can have iloprost.

The problem is I had iloprost in July, August and October because it's all I can really have since immuno suppressants are not possible for me. I don't think it really made my skin softer and I have been looking up the possible pitfalls of a central line and have decided I am going to tell them tomorrow that I will give it a miss.

It just seems so maddening that my skin is changing so rapidly so far into the illness. I had been told by Prof. Denton that it should plateau then soften at about 2-3 years but mine seems to have really revved up.

I guess I am just feeling pretty fed up all round and I am still forcing myself to do my Pilates because it has kept me mobile but it hurts and feels like I am trying to stretch wood.

Is this normal to go so hard this late in? When will it all stop?

#2 judyt

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Posted 05 December 2011 - 07:30 PM

Hello Vanessa,

So sorry to hear that you are having so much trouble all of a sudden. I myself have Limited so no skin hardening, thank goodness.

I can't really start to imagine how hard it must be for you, and my heart goes out to you.

I hope that others with problems more like yours will chime in and give you tips about how to cope.

Warm wishes from the upside down side of the world,

Judy T

#3 Joelf

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Posted 06 December 2011 - 08:31 AM

Oh Vanessa, I am so sorry to hear about how much you're suffering; as if you haven't had enough to contend with! :(

Like Judy, I have Limited Sclero and very little skin involvement, so can't advise you from my own experience. I've found a few links for you to Diffuse Scleroderma, Skin Fibrosis, Skin Thickness Progression Rate in Systemic Sclerosis with Diffuse Cutaneous Involvement: A Predictor of Outcome and Biomarkers for Skin Involvement which should provide you with a little light reading ;) :lol: and which I hope you'll find helpful.

I can see from your post why you're loathe to try the central line for iloprost and I do hope that you'll soon be able to find some relief from the pain and discomfort with which you're suffering.

:emoticons-group-hug: Sending you my very best wishes,


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#4 miocean

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Posted 06 December 2011 - 08:41 AM

Hi Vanessa,
My skin hardened very rapidly. Within 6 months I was 45 on the Rodnan Skin Score with a scale of 0-50. I suffered sudden renal failure and was on dialysis for 5 1/2 years. Around the 4 year mark my skin started to soften and I am now a 5 seven years later. I never had infusions, it was just natural progression.

I understand your feeling of being so tight and uncomfortable, and those awful needle sticks. I was told once I was like trying to get a needle into a watermelon. :P They were having trouble getting a vein for a nuclear stress test necessary for pre-transplant testing and almost had to put a central line in for that. Once, for a colonoscopy, I had at least four people picking up my arms and legs looking for a place to stick me and I wanted to cry and say "stop handling me like a piece of meat."

Congratulations on continuing to do Pilates through the pain. I was not flexible enough to even bend slightly and my skin hurt so much even the fabric I wore hurt. Before I understood what was going on and being diagnosed I was standing with my feet in the ocean and crying because the waves hurt so much.

I am curious, what is "the renal marker"?

I hope all goes well and you are home soon.

miocean
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#5 Shelley Ensz

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Posted 06 December 2011 - 01:53 PM

Hi Vanessa,

Oh my goodness, I am sorry for the progression you're experiencing right now. I can only imagine what you are going through, and how demoralizing and upsetting it must be. May I send you a whole ton of warm hugs, but with a few tears for you, too?

:emoticon-hug: :crying: :emoticon-hug:

On the positive side, you are to be commended for continuing with your Pilates, despite everything. And it is a huge success to have the renal crisis now under control. We could have lost you, had you not gotten prompt and appropriate care. I know you're probably not in the least mood to do any Sclero Happy Dancing now, but later, when you are out of the hospital and feeling a bit better, please remember to come back here for a happy dance, just for that. It is a major thing to have experienced and averted.

I hope you get a chance soon to discuss your concerns about the infusion with your doctors. Here's hoping you find something effective for the pain, as well.

:emoticons-group-hug:
Warm Hugs,

Shelley Ensz
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#6 Amanda Thorpe

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Posted 06 December 2011 - 02:30 PM

Hello Vanessa

Boy you've been throught the mill for sure! I had major skin tightening in the beginning of my illness but I still remember how awful it was to be unable to have a normal range of motion anywhere because my skin was so tight. I also remember well the itching, pain and general feeling of discomfort that accompanied it. I had Iloprost once and swore off it forever, an then went on MMF, I will never know it either of those or just the disease itself but the skin eventually softened to the point that I now look like someone with limited rather than diffuse scleroderma.

I am now in year 4 and remember in year 1 longing for my skin to change and thinking it never would but it eventually did so yours should too.

Take care.
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#7 Margaret

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Posted 06 December 2011 - 02:46 PM

Miocean ,

I think the renal marker is the Anti-RNA Polymerase 1/111 blood test. Gareth is positive for that and his numbers keep getting higher although no one seems to have any knowledge of what that means. Having this positive blood work is specific to Scleroderma and puts one at a higher risk of renal failure.

Take care, Everyone.
Margaret

#8 Vanessa

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Posted 06 December 2011 - 03:25 PM

Hi everyone.
Firstly thank you for your support and suggestions.
I got very little sleep last night tossing and turning but this morning I shared my fears with the ward sister who suggested that I say I don't want to go ahead with anything until I have had a review with Prof Denton.
Shortly afterwards the rheumatologist registrar came to see me and said that the Prof thought it was best not to have the iloprost anyway now but has suggested that I start hydroxychloroquine sulphate?? Which turned out to be Plaquenil.
I had my first dose this evening. He also suggested a low dose of steroid which has always worried me. My mother had RA and suffered from bone fractures due to steroids. Mind you, that was 30 years ago.
Anyway she said I could discuss it with him tomorrow when he does his ward round.
I think as my skin is so horribly inflamed it might be a solution if the dose is not too high although I would aim to stay on it for the shortest time possible.
I had small doses after my chemo as it is given routinely for a couple of days after each treatment and it made me feel like superman which was really weird because as soon as I was without it I felt like a balloon which had deflated. In other words it is a mask rather than a cure.
The renal marker is polymerase which means that you are more susceptible to renal failure, it is sometimes linked with cancer as mine was. It could be that when the cancer cells were forming in my breast my auto immune system went haywire. Apparently there is less likelihood of lung and heart involvement with the marker and the Sclero plateaus at 2-3 years.
I just can't wait for that plateau.

#9 Joelf

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Posted 07 December 2011 - 10:50 AM

Hi Vanessa,

I've never taken Plaquenil so can't advise you about it from my own experience. However, I know many of our members have taken it successfully and I've found a http://www.sclero.or...xychloroquine/' class='bbc_url' title='External link' rel='nofollow external'>thread on Hydroxychloroquine which I hope will give you some more information.

I do take Prednisolone and also experienced the steroid "high" with it, such as you described; however, I must give you a few words of caution about it. http://www.sclero.or.../steroids.html' class='bbc_url' title='External link' rel='nofollow external'>Steroids can precipitate a renal crisis in Scleroderma sufferers so should be prescribed with caution. Being that you're dealt with by The Royal Free they would be aware of this and also as you've been in renal crisis, hopefully they would be extra careful prescribing them to you.

I've found an interesting link regarding the Anti-RNA Polymerase 111 antibodies.

I do hope these medications will help you to feel better very soon.

Kind regards,

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#10 Vanessa

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Posted 07 December 2011 - 12:37 PM

Yes, steroids scare me in many different ways. I am to have 7.5 mg per day which is a low dose they said.

Hopefully I won't have to stay on them too long.

Does that seem a low dose?

My friend who is here as a day patient and also has the renal marker was on them since February 2010 and she was taking 7.5 and is very gradually reducing it and is now down to 6.

It is such powerful stuff and I forgot to ask her why it's so important to wean off so slowly. What happens during the cut down period?

#11 Joelf

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Posted 07 December 2011 - 02:25 PM

Hi Vanessa,

I'm on 7.5mgs per day of Prednisolone as well. It is considered a low dose but it's very important to wean off the steroids slowly. Here's a useful link on Prednisolone withdrawal which explains it in more detail.

It's definately a medication to be treated with respect!

Kind regards,

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#12 Amanda Thorpe

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Posted 07 December 2011 - 02:26 PM

Hello Vanessa

Have a read of this article at Medicinenet.com which explains that steroids affect the adrenal gland and tapering the drug give the gland the chance to get back to normal whereas sudden withdrawal does not. It states 4 to 6 weeks at least as a withdrawal period but I know of people with scleroderma who take months to withdraw and ultimately your body is going to dictate this.

Take care.
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#13 Vanessa

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Posted 08 December 2011 - 01:11 PM

I started on the steroids today and I don't feel like superman but the inflammation has definitely lessened while the skin on my arms is still very sore as though it were burnt.

I called the hospital to see if I could reduce the dose to 5mg and they said okay, so tomorrow I will take the lower dose from then on as I can't imagine that I would have withdrawal on day 2.

Thanks again..

#14 Shelley Ensz

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Posted 08 December 2011 - 07:53 PM

Hi Vanessa,

I'm glad that you are feeling a little bit better. Yes, 7.5 is a low dose. Don't worry about the dosage yourself, go with whatever your doctors order because you are in the very best of hands. There are general guidelines, which are that doctors are not supposed to merrily throw steroids at scleroderma patients in hopes of an improvement. However, sometimes there are complications for which there really is no alternative. And the best protection there is that you have a doctor who is one of the very tops in the whole world so they are not just tossing steroids at you thoughtlessly!

Personally, I'd go with whatever they recommend. They know the warnings and so do you. They can also assess better what is most likely to help you. Getting inflammation down must be of the utmost importance now. I believe what I heard is that they try to stay under 12 for 2 weeks or less -- and then there is the tapering which can take a very long time. Some people end up taking a very low dose for a very long time.

Just to comfort you a bit, my husband is on 7.5 mg every day for the rest of his life, after lung transplant. He's doing dandy and has had no problems from it at all. (He also does not have scleroderma.)

You are in great hands, so relax into their care and with our comfort. Stress causes scleroderma to worsen, so even relaxing with the idea that you are okay, things will turn around, you have the best medical team there is, and friends who will stand by you always. Just do what your doctors recommend. Who knows, that extra 2.5 might be just what is needed to kick your skin inflammation down! And you know they are already monitoring your kidneys carefully, so you have a safety net there, too.

:emoticons-group-hug:
Warm Hugs,

Shelley Ensz
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The most important thing in the world to know about scleroderma is sclero.org.

#15 Amanda Thorpe

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Posted 09 December 2011 - 04:32 AM

Vanessa, I'm with Shelley on this one, take the dose they are currently recommending! Anything is better that the inflammation you're suffering at present and as Shelley says, you are being treated by a scleroderma expert afterall.

Take care.
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#16 Vanessa

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Posted 11 December 2011 - 04:19 PM

Hi again.
I reduced the dose to 5mg for 2 days and that didn't work at all. Today I went back to 7.5 mg and I am still in a lot of pain with tazer type random shooting pains and the skin on my arms feeling like I have very bad sunburn and in addition sensitive nerve ending sort of pain. It itches badly too.It is getting very cold now but wearing sleeves is completely out of the question.

This flare up has been going since July. That is a very long time and rather than improve it steadily gets worse.
This year actually I have just had so much all round what with the cancer,mastectomy, chemo and all.
Also I have morphed from a glam, slim young looking thing with long blonde hair to a "school dinner lady" brown curly short hair with whole chunks of grey, a big belly since I have gained around 2 stone, and to put the tin hat on it I can't wear a bra because it is just too painful so I have just one droopy breast. Never a good look.

Still it's not all doom and gloom. 1) I am alive 2) I have 2 lovely daughters, a wonderful sister and an extremely kind and considerate partner 3) the cancer is keeping away.

I am blessed.
Thank you all for your support.

#17 judyt

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Posted 11 December 2011 - 05:13 PM

Hi Vanessa,

So sorry to hear that your illness is making you feel so badly about yourself. You are probably right to increase your prednisone back to where the Doctor has suggested if only to try to help the way you are feeling. Here is a bunch of balloons :balloons: and :flowers: some flowers to try to brighten your day.

The one thing about scleroderma is that we know these particulare flares will not last for ever. It may seem a long time away now but time goes by so quickly sometimes and next thing we know we are looking back and saying 'thank goodness that is over'.

I can hardly believe that 43 years of Raynauds is over, I have not had an attack for 2 years now - unbelievable. It is 7 years today since I came home after having part of my stomach removed. I thought the stomach problems were with me forever but now I have almost forgotten what it was like to be in that space.

It is probably no use suggesting that you do something to take your mind off what is happening at the moment because you are so very uncomfortable, but please know that we all understand and any time you want to come on and have a rant about it all we will listen.

Best wishes for a good outcome and a quick improvement.

JudyT

#18 Joelf

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Posted 12 December 2011 - 01:59 AM

Hi Vanessa,

I do hope that you will soon feel a lot better; hopefully the slight increase of Prednisolone will be enough to settle the flare down that you're experiencing. Unfortunately the weight gain around the stomach is one of the downsides of steroids; I do understand because I've joined the "big belly brigade" as well.........boo hoo!!

Hopefully 2012 will be a much better year for you; I'm sending you a very large :emoticon-hug: and also some :flowers: to keep company with the ones Judy's sent you.

My very best wishes to you,

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