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Kath has lots of purple piggies, snake-fish, even more drugs and exciting appointments!

Soooo last update was early September, when I was freshly on a stink load of steroids and energy levels were confusing things. Now down to 25mg (from a giddy high of 60mg!) and really this is basically because it doesn’t do an awful lot for me, other than make me have weird sleeping habits and Eat. All. Of. The. Food. Not even joking, it is scary. Reason for that wack up was because Fester (big toe) tried to be dead again, but the steroids and a hefty wack of antibiotics sorted him out and he remains cold and mottled and thankfully not gone to far over to the dark side…

Week later, my stupid fingers started kicking off, with teensy weensy baby ulcer spots, so same story, stayed on high dose steroids and agreed with Consultant that the daily dose of MMF would get increased too. This is the one I call chemo-lite – used as anti-rejection meds for people following transplants, as well as being a daily form of chemo given to people with auto-immune conditions. I tolerate it pretty well, not too many side effects at all, so decision was made to bump that up from 500mg twice a day to 750mg twice a day and still ticking πŸ˜‰ If anything, none of it seems to be exciting enough as my stupid white count and neutrophils are STILL climbing, from the high early September – this was blamed on increased steroid dose, but now on lower dose and still going up. Constantly looking for infections or ulcers forming, but only other thing I guess it is reflecting is my immune system having fun deciding my blood vessels are all the enemy. Over enthusiastic little git… You would think the stupendous amount of drugs I am taking to flatten my immune system would be doing something. Can you become immune to immunosuppressants??? Anyway, basically all my fingers are now being stupid and freezing cold and I am back to doing everything in mittens except going to the toilet, washing my hands and eating. Mittens = life!

My hip, following the potential pyoderma gangrenosum query had a month of strong steroid cream, as well as the timing of the increased steroids and the antibiotics, so if it WAS pyoderma, it was either going to be very happy and heal, or get worse anyway and form a crater of death. Instead it basically didn’t change at all. For a month. That actually takes some effort. I have had the same open wound now for… Eight months! If you can’t win, at least be proud of sucking so much yeh??

Whilst newly on all the increased steroids and MMF, it was time for the camping party that saw me sitting on the floor with everyone swearing at Death Toe a year ago, in my rather iconic awesome socks. I was verrrrrry apprehensive, not least being a bit wired on the steroids and aware I was not exactly very resistant to the cold… I had epic assistance as per usual from everyone and tried a new sleeping ON the duvet instead of UNDER the duvet thing in the tent and it was SO MUCH WARMER! My sleeping bag finally died, but that is what Christmas is for. I am an only child this year for Christmas as well, as everyone else is sodding off to the other side of the world, so this means I get three times as many presents. True story!

Anyway, I spent a lot of the time in the above fancy dinosaur hat, despite hating both orange and dinosaurs, so bit confused about why that happened… But I also fed blackberries to giant fish, sat around a campfire and had ALCOHOL!!! For the first time since Solfest last year, when I was essentially balancing painkillers and alcohol and completely failing to block out the toe… When you are not in ridiculous pain, turns out alcohol still gets you a bit drunk πŸ˜‰ It was fun being silly, but although my toes were not dying, I was still sore as duck with all the moving and joy. Sleeping in tents also not recommended for being able to move the next day πŸ˜€ WORTH IT. No fingers or toes fell off anyway, which is more than I can say for last year. Also resisted the lake, although not as tempting with it absolutely lashing it down the whole weekend.

Very end of September I had the joy of a trip up to Carlisle, to the same ward that I lived on for two weeks in February. Same ward and staff who sorted out plex round two, cyclophosphamide (urgh) and the destruction of Death Toe (yay!) – some very good eggs! The plan was to do another dose of rituximab; my Consultant had a chat to Addenbrookes to check they didn’t have any other exciting plans and they had confirmed rituximab was best plan. The cyclophosphamide was originally used AFTER the first two doses of rituximab, because I was too unwell and rituximab takes too long to become effective and is a bit too gentle… I have now had four infusions of it and honestly, compared to the cyclophosphamide it is a piece of cake. Doesn’t make me feel sick, just a bit more tired than normal and the drugs they give you with it (hydrocortisone urgh) make me absolutely spiked. No exciting skin rash reaction either last time in April or this time, so that was maybe a one off. I got very used to having awful veins after all the tests and cannulas last year, so warned the doctor I was a nightmare; she got my arm super pumped up and maybe got a bit too enthusiastic, as I bled all over the procedure room and it was hilarious! I have been binge watching Dexter a lot at the moment and it was exactly like one of the more exciting crime scenes, with puddles of blood and smear patterns and fingerprints… Maybe not as funny for her as she had to clean it up because I was plugged in, but it made me giggle. Other than that I basically slept the whole six odd hours curled up in the chair, except for vaguely waking up every 15 mins to get my blood pressure, temp and pulse checked to make sure I wasn’t dead. Very comfy chair! The train there and back went a bit more smoothly this time too –Β I had epic coffee and cake for breakfast when I got to Carlisle, courtesy of my welcome committee/taxi driver πŸ˜‰ Afterwards I was sleepy and dozy, but it wasn’t too late as I bossed the actual infusion, so made it to the train in one bit… Last time I had ended up getting on the train in Carlisle to go home, and somehow got off the train in Carlisle… It was very confusing and I was a bit scared of it happening again, but I did it successfully this time!!

FYI this handsome little fella is my new cat godson Ludo. He is the best cat ever, except for all the cats I am going to have and obviously except Mog, but he is pretty awesome and I am growing him cat grass because… Whilst in animal waffle section of this update, I also decided to get a new fish tank a while back because the old one broke suddenly and I didn’t want to watch all my fish freeze/suffocate to death. This obviously then led to more fish. Being a bigger tank, it has kept leading to more fish, in slow steady fishy increments. The newest are these two awesome golden loaches, called Boggis and Bunce (the yellow snakey guys below). They are eating SO MUCH so my fish food costs have basically doubled from a few pence a week to maybe… 5p a week? Slightly creepier than anticipated; I always swore I wouldn’t get any albino fish and they are just a leeeeetle bit pale and red eyed for my liking, but they have oodles of sass, so have forgiven them. They also have very cool moustache faces!

So ja… Yesterday I had a day off work for a trip back up to Whitehaven to see my Consultant. This was for a routine outpatient follow up, planned before my fingers started kicking off and before the rituximab, so I knew already from many email chains that my Doc had been looking into some new ideas. Getting there was fun – there is basically one main road that goes up the west coast of Cumbria and we had some pretty heavy rain and flooding on Wednesday… So one of the bridges was closed. This led to an hour delay, as I had to take a funky diversion and then timed it perfectly to hit the special pointless uphill standstill queue at Egremont that apparently happens every day. My Doc bumped me to the end of the clinic, but by the time I got there and parked, over an hour late for the appointment, I was a shaky mess of sore muscles and brain mush. My bp was surprisingly not too ridiculous, I was only 145/99!! My hands were shaking though which was quite funny when trying to show off my purple fingers and having to excuse the trembles. Fun new idea that is being investigated is systemic sclerosis. I have had tests before for more general idea of scleroderma, as it would nicely explain the digital ulcers, but was DA DA DAAA! negative… So new batch of bloods have been taken that should pick up a wider range of markers. I don’t exactly fit the typical classic symptoms, but I don’t even give a fig anymore, I just want a bloody name. I keep going through phases of thinking I am being a drama queen and imagining it all and then I have a bad day and can’t move much, or think actually, my fingers are trying to die, or look over old blog posts and remember my toes dying… And yep, my body is trying to kill itself, so a name for the particular flavour of idiocy would be nice. It isn’t likely to change the treatment plan much, but it would be a bit of emotional closure! Given things are trying to do a repeat process anyway, also was decided to go ahead and pretend it is systemic sclerosis for now and change the pentoxyfilline I have been on three times a day since… March? to Sildenafil…

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This is basically the whole reason for this blog update. I am still vastly amused and got to go and pick it up today. Sildenafil…

IS

BASICALLY

THE

PROPER

NAME

FOR

VIAGRA!!!!!

Very amused. Was a bit irked when picking it up today though, as although my Consultant had already written me the prescription, my GP confirmed that she could NOT prescribe it for me. It is a restricted drug, UNLESS it is for men with erectile dysfunction in which case she can write prescriptions all day… So my fingers falling off don’t warrant a drug that has very clear rationale behind the use of it. Not to suggest it is widely used, but systemic sclerosis is not exactly super common and honestly, I don’t exactly full fit the profile. Fun drug paper below anyway for anyone who thinks I am making up crap about viagra potentially keeping my fingers attached…

Paper on management of systemic sclerosis for anyone who cares!

So just to confirm for everyone wondering, I will obviously let you know if I suddenly develop an erection that doesn’t go away, but the main side effects I am likely to experience are the usual crap that comes with basically every drug I am on already – like headaches, getting dizzy, changes in bp, turning pink… Just hoping it doesn’t get as exciting as the horrid iloprost did, as I cannot manage a permanent migraine. No amount of fingers are worth that being my life. FYI I am joking about the erection. If you don’t mind your search history looking a bit weird, research how viagra works. I am not going to suddenly grow a penis either. Or develop uncontrollable sexual urges. Sorry!

Getting home after the Consultant appointment was even more weird. I was a bit spaced out so crashed out in the car park for 20 mins, then set off, knowing I would have a diversion at some point. I do not know what happened (it is a peculiar talent of mine) but I swear I did exactly what the signs and the nice police persons all said… Got as far as… Somewhere and the road ahead was also suddenly closed, with people turning back round and saying that THAT bridge was also now closed… So the only way left was to turn back round. I scoffed at that idea, realised I was somewhere I recognised a bit and headed off up Hardknott Pass! It was just turning dark, so this was maybe not the cleverest idea I have ever had, but it seemed like a genius move at the time. There is no phone signal, ridiculous hairpin bends going all the way up and down a mad crazy mountain road, with added sheep sleeping on the road for funsies. It was pretty fun, in a put your foot down and sing along louder to Aerosmith kind of way. The fact it was dark by then was good, as I couldn’t see the sheer drop beside me… That road takes a LOT longer than you think but I committed hard and eventually made it back to Duddon Bridge and something kind of resembling civilisation and I only nearly drove very slowly into one cow and she didn’t seem too bothered!

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Kath is super wired on all of the drugs oooops! A few gross pictures, because what would an adventure be without feeling a bit icked out…

So… Featured pic is BREAKFAST!

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That is 60mg pred. 60!!! I was on 2.5mg for the whole month of August!!! Absolute fail… The reasoning for this comes in several parts, but needless to say I am quite buzzed. Very rarely had 60mg before, except for when getting a few infusions of methyl pred and although I managed some sleep last night, I also made the spontaneous decision to go to Grasmere Guzzler beer festival to see a few bands and was high as a kite for the whole evening! No, I did not drive. Many thanks to my driver πŸ˜‰ Proper giddy kipper and basically had more energy and talkativeness than I have had in the whole last month combined haha! I think this is going to last a few days at this dose anyway, before tapering down again. Preparing myself mentally for hideous sleep deprivation though, I found it insanely bad in hospital on 30mg daily for the majority of the time, so goodness knows what 60mg is going to be like 😦

Reason number first… This little shit!

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Fester (the big toe next to what was Death Toe and is now Kevin Spacey – ps how healthy and adorable does Kevin look!) has kicked off AGAIN for maybe the… sixth time? Was a little red and tender for a few days and then started waking me up on Friday night. Having not had infected toe pain for a while, it was horrible to experience it again, especially in the middle of the night when my imagination started going wild and life was meaningless and dark rather quickly… It was also very obvious when it crossed that line though, so for that I am grateful as I have been so cautious and worried about whether or not I would catch it quickly enough.

Excuse the toenails by the way – all of the drugs and infections for the last few years have done some serious damage to my nails, but they are growing in quickly and healthier, as you can see near the base! Part of me wonders if that has helped cause the infection, having healthy stronger nail pushing into the side of my toe? Have had many starting in the exact same area though, so it is definitely a dodgy spot anyway and probably weakened to some extent (although not exactly sure how that would hold up factually…).

Reason number next one? Hip hole!! Needs a cool name please, will take suggestions…

This is now (on the left) and when I first had the biospy taken, mid-Feb approx. I have been getting dressing changes and avoiding infection for MONTHS and until maybe a few weeks ago, it was healing over and thought to be on the last few dressings. Then it started to slide backwards and has slowly begun to open up again.

I saw my consultant a week ago Thursday and at the time he asked his dermatology colleague to take a look at my hip. He suggested I had possibly developed something called Pyoderma Gangrenosum… This is NOT gangrene, it is a rare skin condition where your skin breaks down to form ulcers and can be either a condition in its own right, or can develop in existing wounds. It is a little more common in people with other autoimmune diseases and would be a good explanation for why my skin has started to break down. My GP and practice nurse had a good look into it and agree it is best to assume it is this, so I had already started treating it with strong dermovate cream twice a day and it is being checked very frequently for a while to see if it helps. Added another bit to the stupid jigsaw! The high dose pred will also hopefully help with this, as if it is PG then high dose steroids are one of the most effective treatments.

Reason number last bit doesn’t come with a gross picture sadly, but just generally for the past few months I had started to slide back into mega fatigue and all over joint and muscle aches. By the time I saw my Consultant, I was at the point where I said I was now considering it as pain again. You get used to a certain level of background aching and stiffness, which I am glumly assuming will never fully go away. It is so hard to pinpoint when it even began, because it just crept up as slowly starting to feel ‘a bit naff’ all the time. This latest flare up began with much the same resurgence of more and more days feeling like ‘bad days’ when looked at compared to the relatively good few months I had in April and June. I was so giddy after getting Death Toe lopped off and the difference in pain was so overwhelmingly brilliant, that at first I did think I was just starting to be less tolerant of the joint and muscle aches, with nothing worse to mask it. But having now had a toe kick off again and my hip being worse, it really does look like it was the start of a proper flare. Maybe not showing up in my bloods (don’t know yet!) because that would just be a miracle given my record of blood tests, but we are generally advised to be aware of the early symptoms we showed, as they are usually the precursor to flares.

Had to give up Solfest this year – ticket refund was agreed at the last minute (on the day!) as I had the Consultant appointment, but honestly, the bit which upset me most was that compared to last year where I felt determined to do as much as I could despite the pain and feeling crappy, this year I just felt like I didn’t have the physical or mental energy to even want to try. I managed a lovely weekend regardless, with a visit from my baby bro and his lovely lass; they cooked for me and we watched films and it was glorious. That same weekend I was also compensated by going to the wedding of one of my best friends – cried the entire way through the whole ceremony… Apparently weddings make me a mushy puddle of goo! I didn’t manage a lot of the reception, but saw the speeches and cried a bit more, had a lovely time meeting some fabulous people – and the food was ace πŸ˜‰ Going to Grasmere last night and being so hyper on the steroids reminded me how I used to feel at social events, when I would have a little bit of alcohol and be super happy to see all my friends and have the energy to not be focusing on moving around carefully, or being too tired to stand up. I had a bit of a dance!! I have been wondering more and more recently whether I actually fit more into the category of someone who is an introvert; someone who is drained by socialising and interacting with groups, even if they enjoy it. I had always assumed I was an extrovert from the little I have learned about it, although I can’t claim to fully understand it all, and had been a bit confused about how much I didn’t want to see people or leave the house recently. Β I think last night was a bit of a clue that it really is the condition that has made me struggle so much lately with social events – when I have ‘fake energy’ from a massive steroid buzz, I am absolutely over the moon to be running around and talking to everyone and quite happy blathering to strangers in a queue… Apologies to everyone I scared last night haha – I was absolutely wired off my face, it felt like the back of my eyeballs were plugged into giant sockets πŸ˜€

End on a few happy notes? Yes back on lots of drugs for a bit, but have been managing four hours a day of work for the last few months now and it is going nicely. Apart from my beautiful ergonomic keyboard breaking the other day… That was a bit sad. My managers and colleagues have all been really supportive and happy to see me back, which has meant a lot and made it so much easier. I was terrified I would have forgotten everything and be either failing to get the work done to a high standard or be unable to hit the targets, but I actually managed to exceed both my own and work’s expectations which was really lovely. Β Particularly when feeling a bit worse the last few weeks, four hours has been plenty – I am shattered after work most days and it leaves very little energy for anything else, but having a routine and knowing I am managing to work again has been worth it for now.

The other nice thing of the last few months has been my hair starting to grow back very fast – particularly underneath, the lower layers had thinned out dramatically and it has not only quite suddenly stopped falling out, but has started to grow back very quickly. Underneath I have a dense curly undercut coming through, which is amusing me no end, but the most obvious place to see it is around my hairline on my forehead πŸ˜€ Check out the baby fringe below!! I am trying to encourage it to fall in line with the existing curls, but have some rather cheeky misbehaving bits that want to stick straight up allllll the time. I am aware this is largely going to be due to having being on a lower dose of pred for quite a few months (not any moreeeeee!) and also it being a while now since I had the cyclophosphamide infusions (Feb) and the latest rituximab (April). Have been on a steady dose of mycophenolate since April, but apparently although people do commonly experience hair loss on this one, it does also usually settle after a few months. I really never used to lose any hair before being ill – hardly any ever came out in the shower and I never brushed it, so to suddenly lose what for me was a lot every day was scary. I know a lot of people go through worse and a lot more dramatically, but all I can say is although the drugs are very important, don’t downplay the mental impact of the ‘little things’ like hair loss, as they can have more of an emotional impact than the ones you would think would be worse, like getting a toe amputated! Still, when the drugs are necessary, the drugs are necessary.

I also started a different way of looking after my hair lately, about the start of July, at a point where it was still falling out very fast and showing no signs of stopping. I again cannot know how much of an impact this had, due to the drugs, but it was after about three weeks of this method that my hair stopped falling out. As in second picture, my hair is now the curliest and healthiest it has ever been. It has taken a good two months to get into a routine and find what works for me, but it has been worth it and I am eternally grateful and delighted that I found out about this πŸ˜‰ You basically stop using any silicones, sulfates, mineral oils or drying alcohols at all on your hair and only use conditioner that fits those criteria. This means no shampoo! I have not used shampoo for two months and honestly, my hair is the cleanest and happiest it has ever been. No greasy scalp, hair is not weighed down and smells fresh and clean, even if I don’t fully wash it for a few days. If anyone wants to know more, search for ‘the curly girl method’ – works on all hair types, just particularly makes a very obvious difference if you have curly or wavy hair, as it accentuates any natural wave you already have. Now I am going to take some more drugs wooooooo!!!