CAPS/Asherson's survivors

[disloyalorder]

If there are any other people on here who have experienced Catastrophic Antiphospholipid Syndrome (CAPS), sometimes known as Asherson's Syndrome, would you be prepared to share some details about your experience/treatment?

You may or may not have seen earlier in the year, Mary posted some information about EURORDIS (European Organisation for Rare Diseases), who have sponsored a research study into CAPS and are interested in producing better guidelines for treating this APS variant. 

healthunlocked.com/hughes-s...

I am due to meet with a number of the CAPS consultants next Wednesday to discuss their findings and, using this information, begin to produce some patient guidelines/recommendations. 

I can advise based on my experiences, but if there is anyone else on here who has survived this too and would be prepared to talk to me either on here or via DM so I could incorporate their advice/opinions, that would be really appreciated. My aim is to provide an accurate but inclusive patient perspective, so the more relevant voices the better. Any advice would obviously be credited to you.

Thanks!

eurordis.org/

ncbi.nlm.nih.gov/pmc/articl...

I

[MaryF Administrator]

Hi I think you got responses last time, and we have more members now, so I hope you get some more.     MaryF

[Hidden]

Hello disloyalorder, this is the first time that I have I have posted on this forum, but this is my journey to an eventual diagnoses of CAPS 

I am 78 years old who is inflicted with this horrible disease.   In 1995 I was 55 years old and was taken to St Thomas's Hospital  A&E  with bad stomach pains. After several test nothing could be found but because I was bleeding from the back passage a surgeon decided that he would take me to theatre to do an exploratory operation which he found a DVT in my bowel this lead to a removal of part of my bowel and colon, four days later I had breathing problems and it was discovered that I had another DVT that had lodged in my left arm I was taken back to theatre and was then put in an induced coma which I was in for a month , a haematologist Doctor Beverley Hunt  

[Hidden]

I am sorry I have accidently sent you my post that was not finished, I will continue

Doctor Beverley Hunt is now Professor Beverley Hunt who has taken care of me for the last twenty one years, to cut a long story short I was diagnosed with multiple DVT whilst in an induced coma and was then diagnosed by Doctor Hunt with anti thrombin 3 deficiency which I was injected with by her and then was brought out of my coma.

I was put on Warfarin 1mg INR level was to be at 3.5 I was then allowed to leave hospital but continued to see Professor Hunt regularly and on one of these visits I was told that the diagnoses had been changed from Anti Thrombin 3 to Anti phospholipid syndrome. 

 I continued my life and eventually went back to work.    In 2010 my consultant was changed to Dr Karen Breen and she told me that the APS was now catastrophic APS.

Unfortunately in 2012 I had an angiogram at my local hospital on the coast where I now live and this lead to an aneurysm in my femoral artery with this I had septicaemia also  and so was taken into that hospital  for treatment. Because I was not correctly anticoagulated my INR shot up to twelve and was then given an emergency injection of Vitamin  K, because of this a DVT blocked an artery in my leg which unfortunately lead to the amputation of my lower right limb. However I have survived this and am coping well with my CAPS and have regular INR checks by my GP. I have to say that I have not noticed much difference in my well being between APS and CAPS although the devastation of this horrible conditions caused me to lose my limb and the fact that if one is not anti coagulated correctly  the effect can be life changing.

Brimac

[Lure2 in reply to Hidden]

What an extraordinary experience. You are a strong woman of 78 years. But it has cost you a great deal. You are lucky to have met Prof Beverly Hunt at that rather early stage of your journey. You had her for 15 years behind your back. Sorry you had to change your Consultant.

I am 72 in June and live in Sweden. I agree with you that if you are not anticoagulated correctly the effect can be life changing. Important to have an APS-Specialist behind your back also. Have you tried selftesting?

Best wishes to you from Kerstin in Stockholm

[Hidden in reply to Lure2]

Hi Lure2

First I need to let you know that I wrote the post for my husband whose computer had broken . He is the one with CAPS.

He now has another laptop and he his signed onto this forum as brimac . your post has come to me I am with another forum under the name flipflop66 sorry to confuse you but I did sign into this forum under my husbands name brimac but for some reason all his post have come to me under flipflop66.

I could not agree more with you that he is strong in fact his Haematologist Professor Hunt calls him the man with nine lives which I feel are being used up very quickly unless the medical fraternity in some hospitals start to take this condition very seriously.

what was very scary was when we moved from London to the coast Joined a new  GP surgery where they knew very little about Hughes Syndrome let alone CAPS, The nurses there were totally ignorant of it and when I tried to explain how serious and life threatening it could be  I was told that they could not be expected to know everything, The only word that I could think of was, Bizarre or sheer madness. However he is now under the protection of Professor Beverley Hunt who is a leading light in CAPS and Hughes Syndrome we do take regular trips to London to see her.

Take care and thank you for your post.

  flipflop66 on behalf of brimac

[disloyalorder in reply to Hidden]

Thank you so much for sharing your story. Your strength and coping ability is very impressive and so admirable, I'm very sorry to hear of all the hard times you've had to go through. I hope that there are no more in the future. 

When you say you weren't correctly anticoagulated, would you mind elaborating on this a bit? Do you mean that (in your opinion) you were given the wrong doseage of warfarin, or a different anticoagulant, or that you weren't monitored enough? 

One of the areas that is being looked at is how effective the various methods of treating CAPS are - anticoagulants are one of them, and the outcomes of the treatments. In my case, I feel it was poor management of my anticoagulation while in hospital that triggered CAPS, so I'm interested that we seem to have that commonality. 

[Hidden in reply to disloyalorder]

Hello again, first I need to let you know that it is my husband who uses the name Brimac on this forum and it is I who answered your  post as his computer is not working at the time , I logged in with my husband details but for some reason the post has now come to me. I am on a different forum for Atrial Fibrillation under flipflop66.

Can all replies please be sent to Brimac as he now has another laptop.

To reply to your question now, his caps was diagnosed after that awful event in hospital on the coast as he was very badly managed by not being monitored properly or being anti coagulated correctly on warfarin.

His INR shot up to 12. and after a vitamin K injection his INR went down suddenly to 1.4 I had my husband transferred into the care of Professor Beverley Hunt at St Thomas's in London as he seemed so near to death and after a major operation to try and save his leg which was unfortunately unsuccessfully but, at least his life was saved    . His INR was managed correctly  at St Thomas's under the care of Professor Hunt and was bought up to its correct level of 3.5. he still continues to be monitored by her

It has been quiet a journey for both of us and we do tread on egg shells every day . More Hospital, and nurses including doctors need to be trained better on this condition as it is and always will be life threatening unless more is done which you seem to be doing so thank you.

If there is anything that we can do to help you please do not hesitate to ask.

Brimac 

[lloydyuk]

Hi there

I'm not sure if I've missed the boat as I only just spotted this thread.

I'm a CAPS survivor and have written about my experiences here on an old post. I too contacted the European study however was told they had someone which I'm guessing is probably yourself!!

If I can help I'm more than happy to. I was diagnosed with APS in 1995 age 22 and stayed pretty well  stable until 2013. In hindsight my INR was kept low (2.5) & infrequently monitored according to most others with knowledge of Hughes Syndrome. 

Over Christmas of 2012 I had what I thought was a winter bug until I collapsed in the bathroom during the early hours of New Year's Day and got rushed to the nearby hospital which fortunately was very near.

I'd suffered a cerebellar haemorrhage and the fun was just starting! All in all I didn't go home until the end of June 2013. 4 months hospital in patient and 2 months rehab unit. 

If you'd like to contact me directly with anything I can help with that is fine or I might tell you loads of things you don't necessarily need.

Best wishes

Danny 

[disloyalorder in reply to lloydyuk]

Hi Danny,

I think we did speak briefly in the previous thread, thanks for sharing your story. The meeting is on Wednesday, so you haven't missed anything. As far as I'm concerned, any information can potentially be helpful. Particularly if you have any information about what you were treated with, and your general thoughts on the treatment you received - whether there were parts you think could've been done differently or better. 

Thanks again for your help

Lisa

[lloydyuk]

Hi Lisa I know some bits but as it was to do with my brain I was out of it for the majority I've been told some parts tho. I know I had numerous plasma exchanges, 16 I believe and Rituximab afterwards. My kidneys suffered infarcts but recovered but fully and my adrenal cortex was killed off, I'm now addisonian, in addition I had a bur hole in my scull and a shunt later revised several times with an ommaya reservoir which remains until now due to infections namely  hydrocephalus & ventriculitis I think my kidney damage made it difficult to treat these as the use of anti biotics were limited. I'm now left with ataxia - balance, coordination and ongoing fatigue issues together with Addison's disease and of course APS.

Danny 

[disloyalorder]

Me again!

The meeting on Wednesday was incredibly productive - roughly 9 hours of intense discussion about potential treatment recommendations for patients who present with symptoms of CAPS. Even with such an experienced panel (... and, uh, me) a lot of the treatments required intense debate. As such, we weren't able to get through everything on the agenda, and the guideline drafting process is still ongoing. It does mean that there is time to review the points that were discussed so far - anti-coagulation, plasma exchange, rituximab, corticosteroids, antiplatelet therapy. 

These are a couple of questions that were posed either directly or indirectly, I'm interested to know any other CAPS patient's opinions. I do appreciate that they're quite bleak!

- Would you feel safer if the doctor who was treating you began treatment (for example plasma exchange or rituximab) as soon as possible even if the treatment wasn't the most appropriate, or would you prefer if your doctor had access to the contact details of a CAPS specialist who would advise, even if this meant that it might delay your treatment by a day or two?

- If you were offered a treatment that could potentially treat CAPS but also has a risk of causing long term disability, would you accept the treatment? Would you want to be the one making that decision? 

If you received plasma exchange, was it available at your hospital or did you have to be transferred? If you were transferred, who made that decision? Do you think this affected your recovery positively/negatively? 

If anyone feels able to provide some input from a fresh perspective, I'd certainly find it very helpful.