Week 3; Day 15 & 16… A curveball, a real adventure and firm evidence of the institutionalisation of Death Toe!!!

Now anyone who gives half a fig would have maybe noticed there was no Death Toe update posted at 12.30 am this morning (Tuesday 25th)… To those who noticed and were miffed, I can only sincerely apologise and suggest that you keep a saucy paranormal romance book by your bed for the days that I fail you 😉

I had a very busy day yesterday and gave myself a break as my mind was fizzing… With it being a Monday, I had been waiting all weekend for answers, with my mind on overload and was then thrown a bit of a curve ball to put it bluntly…!!

Monday mostly – news bomb!

I saw my Dr during morning rounds – she came to see me last (she gave me a heads up!) which was because she had A LOT to discuss.

The upshot of the lengthy conversation we had in the morning is that basically when a cryo test was sent away most recently to type it (e.g. find out more exact info about the specific nature of the little sticky ghost blobs in my blood that cause all of the problems), the test came back negative… Now I had a postive test early summer and my symptom progression and presentation are absolutely textbook for cryo, so there was no reason to doubt this as a diagnosis. Until this stupid negative test result yesterday. I was talked through several points as below… and then given some time to settle my head and think of questions before later that afternoon when we would do a recap… Where further progress has been sorted today I have said, so as not to blow anyone’s mind with frustrated anticipation. Also trying to tread the line between too technical and too patronising haha 😀

 

The (rather tricky) cryo test was redone first thing this morning for cryo. Cryo is basically still Plan A – and treatment is still planned to be rituximab once infection nailed/immediately if toe much worse when next seen.

The problem – and why she wanted to discuss fully now with me, so it didn’t come as a shock if she suddenly had to pull the plug on Plan A, is that they cannot give rituximab if not definitely cryo, because its too ‘gentle’ and too specific a treatment in what it works effectively against/what it is licensed for use with.

If this test comes back negative, then we are basically going to have to go back to a diagnosis of undifferentiated small vessel vasculitis (alternatives grouped under Plan B). Enough of the symptoms – hell all of them! (ulcers, mottling, fatigue, muscle pain, ischemia, peripheral neuropathy, doppler tests, ultrasounds of arterial pulses in all four limbs, blood pressure issues) fit small vessel vasculitis (which does also cover cryo), so this would still be the strongest contender in terms of a wider diagnosis. Undifferentiated just means not yet pinned down!

If this is the case, Death Toe and other symptoms (such as high blood pressure and ischemia in fingers and toes), are bad enough that my Dr said she really wouldn’t want to wait for treatment.

…below by the way, is how secondary reynauds presents – showing the kind of mottling/ischemia I get in toes and fingers that eventually leads to ulcers. It comes and goes during the day, is very cold sensitive and also coincides with cold peripheries and is painful and or tingly/numb. It just looked especially neat at one point today, so I snapped a shot for my med student minions! (My fingers are NOT that chubby. They are just short. And at an awkward angle. Stop judging!!!)

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Because the meth has worked so pathetically, the plan for Plan B would be to blast on with a chemotherapy drug called cyclophosphomide in a carpet-bomb type approach. Cyclo is an immunosuppressant that works differently from rituximab; it is much more toxic and stronger and would basically flatten my immune system so that whatever disease process is underlying doesn’t stand a chance. It is used for a LOT of types of vasculitis. But it has more side effects – and I have a history of getting infected constantly at the drop of a hat, so it is dangerous in that sense as I would have no protection against infections. It also tends to make women infertile and I am 27 and think babies are pretty bloody awesome 😦

If its a case of babies or limbs/kidneys then… Fair enough. But until we have narrowed this down more/ruled out cryo, I have asked that she tells me nothing further than I already knew about cyclo, as the day I have to accept cyclo I will feel like I am kissing baby Kaths goodbye, even if infertility isn’t guarunteed. Anyone muttering about egg storage, bear in mind I have pcos so the fertility drugs will quite potentially cause some serious cyst rupture issues. Anyway. Basically trying not to dwell on this bit right now!

The OTHER diagnosis option (Plan C!) would possibly be anti-phospholipid syndrome. Again, been tested for this since I was 17 due to mottling. Again, always negative… (except the one cheeky lupus anti-coagulant postive result once, always negative since). Again, all possible bloods done again this morning. Including a weird one called Beta 2 glycoprotein 1 – which has to be sent to Sheffield and wasn’t widely available until recently. Also known as sticky blood syndrome – cool name ja?! – or Hughes syndrome and could potentially be dealt with with ritux and plasmapherisis according to many of the studies I have found. N5o point looking into this (for me anyway), until we have a blood test confirming it, as until then it will go down as undifferentiated small vessel vasculitis if not cryo!

There are three aspects to all of this. Death Toe is an end symptom. Sorry sunshine, but you are. The underlying condition (e.g. cryo…) is the cause. The infection is a shitty gatecrasher. The underlying condition cannot be dealt with properly/aggresively until infection gone. So infection blood tests were taken Monday. Swab was taken on Sunday if you will recall. Neither of these are back yet as of Tuesday midnight :/ I have now had a day of no antibiotics and am hoping this won’t have caused too much damage if I am still riddled! Every single blood test possible for kidney and liver function, full blood count, crp (indicator of inflammation), immunoglobulins was also redone this morning. My Dr went back over every single bloody (ha!) blood test in the last… five years since I moved to England, before she spoke to me, with them always all being negative, except for the cheeky one mentioned above.

Lets have a postive sideline!! The infection kicking off has meant no meth since a week today and my brain has not felt so sharp in four months!!! 😀 Even on oramorph!!!

(Downside, no meth has also meant nothing squishing the autoimmune process and I am noticing more stiffness and muscle twitches, even on substantial painkillers and steroids… The twitching is getting quite funny again! Speaking of steroids, short term plans put into immediate effect whilst waiting for blood tests back have included pred from 20mg/day to 30mg/day 😦 ) No one likes pred. You also don’t want to be on pred long term as it is damaging AND is harder to taper off. 30mg isn’t ridiculous though, so I am NOT panicking. As this picture below clearly shows. Jim – inside-friend let me contaminate your hat… Suck it! This is me not panicking very shortly after the news bomb!)

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We then went through The Gallery of Death (not sure the name actually received enough credit at the time!!) and discussed various tactics to deal with the necrosis.

The below would be knitting-friend’s response today to a picture of Death Toe which sadly nails my feelings exactly, but was funny enough I had to hobble out and wave it at some med students to let them share the giggles 😀

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My Dr came back Monday late afternoon, once I had had a chance to work out a list of questions, have some food and a rest. I didn’t cry in the interim, but I strongly suspect I was in shock as I was insanely cheerful (again see above pic with hat…) and really shouldn’t have been… I also strongly suspect everyone was told to be careful in case I had a meltdown – OR I was being really weird, as staff were treating me like a delicately iced cupcake yesterday!!

– and we had an HOUR long chat, which frankly was awesome. I have been dead grumpy about having had so much unanswered the last few weeks and not being able to just chop off Death Toe and start ritux. Mainly as I have been absolutely shitting terrified to be blunt!!! But the total lack of access to any answers over the weekend and being surrounded by people who don’t particularly have time or the knowledge to deal with all my obscure questions for the majority of each day stresses me out!

She is essentially forgiven in that firstly we had a one on one chat UNTIL I RAN OUT OF QUESTIONS – this does NOT happen with me 😀 For one thing, I am insanely curious and have always adored biology to quite a technical extent – I understand a lot more than most people take away from high school biology anyway, simply because it always interested me… and then I applied my brain and a lot of time to learning all about cryo and to a certain extent, the other possible options.

We made a massive list of all the answers to all of the questions and I was advised of immediate plans going into effect in case I can’t be immunosuppressed very shortly (which would be due to the gatecrashing infection having not buggered off).

This included nerve conduction study – more below! Also included starting ramipril to lower blood pressure, which I began this morning and has POSSIBLY already had an effect! (Its an ACE inhibitor – it basically relaxes the muscles that control the small arteries, so perfect for small vessel vasculitis. Essentially, beefed up nifedipine!) Also steroids, as discussed above. If Death Toe is worse next time someone makes eye contact with it – bloody better be tomorrow – then iloprost again instantly as an emergency measure, as it has a short term noticeable effect on the mottling, but just doesn’t last at all long term sadly. Drs will also then consider a one off shot of something like clexane or similar, which is an anti-coagulant – the one people stuck in bed in hospital get injected once daily in their tummy. I don’t have to, because I am fabulous.

A little bit more Tuesday centred…

Monday night I had woken up and put together some more technical notes at about 3 am (- I kept waking with Death Toe being an idiot last night :/ he is in big trouble if he is more black today!! He has stayed clothed today (last stripped off on Sunday) so not too sure how necrotic we are talking now…) Anyway, I updated family and one or two peeps by msg and then had conked out without blogging as I knew I needed sleep.

I woke up and got surrounded by med students within an hour – they can smell the excitement! – they were of the 2nd year variety and they were using me as a case study for their seven weekly appraisal. I say using – I was quite hyper this morning and my brain was all meth-free and fizzing, so I feel quite sorry for them as they had to deal with a LOT of talking. They presented my case to other rheumy Dr during rounds – he had no further info, but I know my Dr will have updated him fully by the END of today as she is off next week on leave… and he said I was the boss of cryo knowledge (or words to that effect – I won’t lie, I had a smug face) and basically told them to be wary of ever neglecting rare conditions – and that they are never in reality as rare as statistics portray them to be. They also don’t feel rare when you have it yourself!!

I kept reminding them I was high on morphine haha and gave them possibly the most info they will ever again come across from one patient on history, blood tests, symptoms, other aspects like depression… Even!! Haha we even discussed how being in hospital changes your ‘pootine’ – or poo routine (for which I want all of the royalties…) and how it feels having to answer questions like “Have you opened your bowels today?” EVERY SINGLE DAY! Hence the above pic of my ‘watching you poo’ dude I made last year at Magic Orchard, leaving him and his buddies on the back of the festival loos haha 😀

Death Toe’s adventure was being bundled into an ambulance transport about midday. I have not gone further than 100m max from my bed in over two weeks – suddenly I am being taken to my local health centre about a 15 min drive away for a nerve conduction study… There were trees and roadworks and other cars and a LOT of different people… It was very exciting! The study itself was fun as you get to watch your limbs flail around without any concious control and have dramatic finger spasms 😀 So far from what the gentleman said earlier I didn’t show any actual nerve damage in my arms, so what I am dealing with is the classic vasculitis small vessel peripheral neuropathy I guess, which I am already tackling by fighting underlying cause and blood pressure issue… By the time I was done, my return transporting van was ready, but I had also realised that it was a LOT colder than my lava hot ward… and remembered just how cold sensitive and pain inducing anything other than than toasty warm is on my hands and feet. Let alone a foot already experiencing a nerve fire-storm! So yeh. Death Toe couldn’t wait to get back and I have then spent the rest of the day in a LOT of pain. Well done NHS, my toe is institutionalised.

The plan tomorrow (mine anyway!!) is to get the infection swab and bloods urgently so they know if they have to get me back on antibiotics immediately. The normal kidney, liver, inflammation bloods should also be back soon… Death Toe really does need immediate tackling to remove necrotic tissue, but the tissue viability nurse wasn’t around when I was today and it is down to him really to advise on what type of dressing to use/how to remove dead tissue/how much to remove. The necrotic tissue also needs to go asap as it is the perfect harbouring ground for the infection to dig its nasty little toenails into…

 

Soz came to see me t’other day and brought this incredible portrayal of how she would deal with Death Toe, with her army of cabbage and brussel sprouts (BAWK!)…SHE loves them, the absolute weirdo!!

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Oo! I also saw the pain nurse again and can start lidocaine patches on my foot tomorrow as a non-opiod based option oaf painkilling! I had specifically asked her to look into this on the advice of one of the ladies in the Vasculitis UK charity and it looks like she has been happy to check out and implement a suggestion! I have high hopes!

SO. I currently may or may not have cryo… and if not, it is going to be undifferentiated small vessel vasculitis unless we get positive results for the two tests being done for anti-phospholipid syndrome… and for those keen to know, nothing back yet from Addenbrookes – which is beyond frustrating, but I can’t do much about that at 1am, so I am going to leave it be for now 😉

 

End technical overload… (My brain feels SO good for having got all that out 😀 )

In Bay Life updates… (Damn that makes a good name for a feature 😛 )

P may be leaving us tomorrow and I am really going to miss her, as are a lot of the staff. I am also slightly nervous about who else may come in, as in terms of fellow inmates, we have by FAR the nicest bay on the entire ward; no one takes their clothing off or starts screaming in the middle of the night, no one is very argumentative… Age wise, four out of five of us will be the youngest (women at least) and only one of us has any level of dementia!! There is no way this can stay as civilised and ladylike 😉

 

Speaking of tendancies to remove clothing, J has been put on fall watch due to a slightly wobbly landing the other day and she HATES it, as she loves nothing more than busting out of ‘jail’ as previously mentioned… When I went in to see my great great Auntie today, J has now been sat with a fall sensor mat between her and the rest of the room, so if she touches it an alarm sounds and the staff can move in to help her or guide her back to her seat. She doesn’t quite seem to gather that the alarm isn’t preventing an escape as such – which I maybe a touch wickedly found quite amusing, as at least ten times in an hour saw her stand up, set off the alarm and sulkily sit down. At one point she quite loudly said “Awwwwww rats!” – and both I and my Auntie had a smirk – and my elderly Auntie gave a naughty “Ooooooo!” in the most classroom manner you can imagine 😀

M meanwhile was last heard up near my end of the ward saying “I think we are going to avoid bombs tonight…” Which is always a good way to go to sleep!

3 thoughts on “Week 3; Day 15 & 16… A curveball, a real adventure and firm evidence of the institutionalisation of Death Toe!!!

  1. Another brilliant blog Kath!! Your knowledge of Vasculitis and your art of describing it are so informative in a really interesting and entertaining way!!! Considering how much pain you are suffering —it’s beyond me–how you can find the energy and will power to carry this through!! Well done you!! Your trip out must have been a real highlight in your week!! Look forward to the next episode!! Lots of love and hugs—–Peggy!

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  2. Christine says:

    Hi Kath, I have sticky blood. Been on warfarin for 15yrs.
    I also had cyclo at the beginning. I had 3 courses then they said I wasn’t allowed anymore or it would kill me. Yikes!
    Then I had azothioprine for years until they said they couldn’t raise the dose anymore. Then I had the rituximab which seems to have done the trick.
    I’ve been on steroids for 15yrs, not a good thing. I am now a steroid induced diabetic! I’m only on a maintainence dose of 5mg now but I will never get off it as I am steroid dependent 😦
    I hope your tests come back quickly so they can sort you out.
    Did your card from me arrive. I wasn’t sure I’d sent it to the right place xx

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    • Ahhh I am kind of hoping if it is Cryo they will keep to the ritux plan and if it is aps then why not skip straight to ritu?! 😉 I am gathering quite a few papers confirming ritux is a perfectly acceptable treatment! I haven’t been to ask for post yet sorry, there are maybe a few things so I will need to ask toemorow!! HAHAHA 😀 Thank you 🙂 xx

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