So… I spent quite a while today untangling above messy wool awesomness and feeling very lost. I was going to write a short one, as I was knackered and emotionally destroyed, but instead decided what the heck and put together a very ‘Kath’ email to the Dr at Addenbrookes I have been trying to get a referral to now for 11 days. I spent a lot of the day in a lot of pain weeping, having been told that my options are let the dead part of Death Toe naturally fall off or go the whole hog and take it right down to the base. I was then abandoned in a lot of pain on my bed with my brain gone blank until the incredible ward nurses came and rescued me, drugged me up, bandaged me and between them spent quite a while calming me down.
The below was put together largely completely off all opioids, except towards the end where I again reached as long as I could handle letting the lidocaine patches have a turn and had some oramorph. Which will hopefully now send me to sleep and I will wake up to find a pumpkin carriage is awaiting outside. After breakfast of course. Lets be civilised!!
[The names of all hospitals, persons and toes have been hidden to protect their identity and reputation… Plus if you call your toe Death Toe in an email, you generally don’t get a response…]
Dear Dr xxxxxxxx
My name is Kath – forgive the dramatic email following, but I am actually begging you to read this.
I am 27 years old, nearing the end of my third week in xxxxxxxx Hospital in xxxxxxand was told in a brutal ten minute experience today that I would be expected to decide between myself and my Dr if I wanted to amputate my whole toe or leave the necrotic part to drop off. This was by a vascular surgeon who failed to introduce himself and then only looked at the toe no one had seen for four days – and that he had never previously seen – when I demanded that he do so (but advised before and afterwards that it wouldn’t change his two recommendations). He then left my foot propped on pillows on my bed in a shared ward, when I was telling him that the cold air on it was excruciating and went back to his department without advising a nurse that I needed immediate pain relief and a new dressing. Thankfully my bell was within reach, but today was a very painful day and I have reached the end of my tether and do not want that man anywhere near my toe at any point in the future.
I am hopeful that you have already encountered my name. You may even have already replied to my Rheumatologist, Dr xxxxxx, at xxxxxxxxxx – in which case, I will hopefully be having an incredibly welcome discussion with her later on this afternoon. Dr xxxxxx admitted me three weeks ago as an inpatient with some input from Dr xxxxxx in xxxxxxx, as I had spent about a month not leaving the house, unable to put my shoe on and sleeping about an hour a night due to the pain in the toe. Since being admitted, I have had six days of iloprost (my third week of this since February) and an infection confirmed in a toe that had had inandine dressings on it since late June, had prompted three months of methotrexate that frankly did jack all, and was refusing to acknowledge the existence of painkillers. My toe has gone downhill rapidly since I came in, although my infection levels in my blood were (as of Monday) showing normal white count after seven days of iv benzyl penicillin. Lidocaine patches on my foot are failing to help and the only thing really working is oramorph – tramadol was dropped as it may as well have been tic tacs. I am throwing regular dipsticks at the staff and the latest one this morning was ok, so I am not in immediate fear of losing a kidney at least.
I first discussed Addenbrookes with Dr xxxxxxxx a few months ago, but only got to the point of formally requesting that she refer me to you on the 17th, whilst in floods of tears and quite frankly wacked on oramorph, with my long-time ischaemic toe having just been skinned, toe nail and all, by a dressing removal that morning.
She advised that an email had been sent that afternoon and a message left, but after a progressively terrifying and PAINFUL week where the toe went from pink and skinned with small necrotic areas to now being necrotic over a good third from the top down, I was advised this Monday 24th that she had spelt ‘addenbrookes’ incorrectly in your email address and had corrected her spelling and resent it that morning. Now I have been in constant daily contact with a number of your patients and contacts through the Vasculitis UK online support group for at least two months now and when I mentioned this, xxxxxx was alarmed and advised that THIS was your email address – ‘addenbrookes’ not involved. I passed this on to Dr xxxxxxx on Tuesday and left a voicemail for xxxxxxxxx, but as yet have heard nothing back. I am of the understanding that xxxxx cornered you at a recent conference, where she would have mentioned a 27 year old female with cryo who was wanting to be referred to you. I have also spoken to Dr xxxxxxxx, with whom you share the care of xxxxxxxx and I am under the impression he has also passed on my name and details to your office and asked that you consider becoming involved in my care.
I am in no way medically trained, but am intelligent, have always been a very curious, cheerful and optimistic sort of person and am now very much losing the latter two of those traits. Since becoming obviously chronically ill at the start of 2015 and then being told at the start of this summer that I had a diagnosis of cryoglobulinaemic vasculitis, I have learned as much as I can about the disease and have always tried to be actively involved in my treatment and researched each step. I was told this last Monday that suddenly my diagnosis is in question due to a negative cryo test and that whilst it could still be cryo, I was not able to start the rituximab that had been planned until the diagnosis was firmed up. I have also been requesting plasmapheresis for quite a number of weeks and was advised that it was rather invasive and usually considered at the point of severe kidney damage. Personally I am considering the removal of my toe to be a pretty invasive thing to undergo… From a pedestrian understanding, if I have cryo then surely it would make sense when starting a new treatment to at the same time remove the cryoglobulins already clogging my system so as to prevent recurring damage and blockages from them? I have made it pretty clear that I wanted plasmapheresis but then Monday’s revelation has side-lined that, with today’s vascular toe removal opinion further muddying the field in terms of treatment priorities and what I can expect to be allowed to have here.
Blood tests were taken Tuesday morning for the usual suspects, including a repeat cryo test and a lupus anti-coagulant and a beta 2 glycoprotein 1 for anti-phospholipid syndrome as this was being considered as another option (despite many past lupus anti-coagulant tests being negative, most recently that one taken Tuesday morning!) My beta 2 glycoprotein 1 test is not back yet as of today that I am aware, as it was sent to Sheffield and I have also had no result from the cryo test. The cryo test that I was advised would be put into a flask and walked down to the lab and tested under the strictest protocol, yet I when I observed the lack of flask the phlebotomists laughed and said they always just held it in their hands. I am ashamed for not speaking up at the time, but I was in a lot of pain and was still reeling from my six month and counting diagnosis having been pulled from me the day before. Dr xxxxxxx’s junior doctor has been advised of this and did suggest they would have it repeated if it was negative again, but as above, we are now at the point of my first toe being removed and I would quite like a diagnosis as soon as possible without becoming a case study in a protocol war between rheumatology and phlebotomy. The other thing I have been told is that if the diagnosis of cryo and aps do not come back as certain, I will be currently considered to have undifferentiated small vessel vasculitis, for which the suggested treatment is cyclophosphamide. As mentioned, I am 27, have not had any children to date and am very concerned about the thought of such a strong drug being used to carpet-bomb my immune system, if rituximab would instead be appropriate to at least try properly first.
In terms of progression, as far as I know I fit the textbook diagnosis of cryo – I have widespread purplish livedoid mottling over my whole body that comes and goes in the cold especially, but particularly on my hands and feet, first noticed when I was 17. I am sitting typing this in mittens for example, covering my very mottled hands, with thick loose fluffy socks covering my mottled ischaemic feet. I also have a ridiculous history of infections, which includes a LOT of cellulitis, kidney infections and pneumonia when I was about 21. My symptoms kicked off in the very early 2015 with the first of what is now five ischaemic digital ulcers (one finger, four toes), all of which have become infected and required some level of debriding and now amputation being considered. This particular toe I might add is by far the worst so far and has responded to nothing, now having turned quite exceptionally black over a very short space of time.
I started having very sore aching muscles and sore large joints, particularly lower back, knees and hips, was absolutely shattered with an almost narcoleptic tendency to fall asleep very suddenly and started experiencing horrific brain fog. Think putting tea bags into the fish tank… By summer 2015 I had been referred to Dr xxxxxxxxx by my GP and the podiatrist, who recognised an autoimmune process of some sort. Dr xxxxxx started me on hydroxychloroquine and we kept going through many negative blood tests and treating the ulcers as and when they presented with long courses of antibiotics. That would be until the cryo diagnosis approximately four months ago, when I started MTX and went onto a varied steroid regime to try and control this toe.
I am taking the presumptuous route of emailing you directly as I am frankly becoming terrified. Dr xxxxxxx is due to go on holiday next week and whilst I know she would be doing a handover to her colleague, I have been really struggling to accept the level of patient-Dr contact (I last had a proper conversation with her on Monday and have seen only members of her team since then). No one is around at all at the weekend to even take bloods and I am feeling increasingly ridiculous worrying about my kidneys and temp spikes in the face of ward staff who are shrugging off my concerns and pointing to stable temperatures as if they are the sole indicator of an undefeated infection. No one seems to be urgently alarmed that my toe has gone black except for me and whilst I am MORE than delighted to get it removed, having had quite enough of the pain and social isolation and fear, I am also aware that if I have cryo I would much prefer to be operated on somewhere that had experience of cryo in an operating theatre.
This is not in any sense meant to be an email bashing the expertise or care of Dr xxxxxxx; she has never in any way been disrespectful or patronised me and has kept me up to date at each stage of her receiving new information. The staff on this ward are also lovely and caring and although they seem bemused by how interested I am in what the latest blood tests are for, they are tolerating my questions and my increasing fits of crying for six hours a night about losing a toe that I was never particularly fond of anyway. I am also typing until past midnight each night, as it is one of the few ways I have found to disconnect my brain from following the pain in my toe and foot, which again I think is looked on as being borderline insane. I am struggling with facing an amputation, being in hospital for an open-ended duration at the age of 27 on a ward where the average age will be a least in the late 80’s and the VAST majority of the patients have dementia, feeling increasingly like a hypochondriac for wanting to know the details of four hourly observations and knowing that come tomorrow evening if you have not contacted Dr xxxxxxxxxx, I will have a weekend of no contact with a Dr at all, unless I gnaw my toe off myself and get sent downstairs to A&E.
If you would care to get involved – preferably in a knight in shining armour, sweeping me down to Addenbrookes in a flurry of concern sort of way, I would be eternally grateful.
I hope I haven’t scared you into thinking I am a nutcase; I am just a very scared 27 year old in quite a lot of pain, becoming increasingly freaked out about the progression of my disease and treatment. I would also ask that you please talk to xxxxxxxxx or xxxxxxxx if in any doubt as to how scared I am or how… slow my treatment has been, as I gather that you have a good and long-standing relationship with them both. xxxxxxx in particular has a very good understanding of my situation and was on the phone to me for over half an hour today helping me come to terms with an amputation.
Thank you for your time and consideration.
…and now I go to bed and hopefully don’t regret this in the morning!!