Sticky Blood-Hughes Syndrome Support

Aziophrine

Aziophrine dose was recently increased from 150 mg 3 tablets to 200 mg 4 tablets daily

Since then felt like I have hang over and don’t feel well at all

Blood was tested 4 days ago for liver function etc but also my ck level which came back slightly elevated 480 or something I think 🤔 my go called me and told me results and was puzzled as you would aspect my ck level go down not up 🤔

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Hi there, I presume you have more appointments coming up? I know these things take time to adjust, so maybe it will all settle down, but do please get in touch with your main consultant if you feel unwell. Perhaps in a few days things will feel a bit better. MaryF

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Thank you 👍

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I felt like that, even worse in fact when I went from 50-100. I stopped for a while then restarted at 50, then up to 75 where I have stayed and felt ok. Can you call the hospital and ask what to do? Maybe you need to step back down and try the increase again later, in smaller steps?

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Your dose is weight dependent?

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Another question: are you on a scheduled dose titration upwards, depending on scheduled labs? What is the scheduled titration intervals? ( assuming labs look good?)

Also: I assume Dr Paul Holmes prescribed initially the azathioprine at the initial dose. Who now is taking that over? Your GP? Your Rheumatologist? Do they just follow the protocol dr Holmes has set forth and tie in with him via email?

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Who is doctor Holmes ??

I’m in London Great Britain 🇬🇧

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Neurologist in London- who has an interest in working with Azathioprine in cerebral APS in patients who’s INR is consistently in range ( high enough) but continue to display neurological symptoms non the less.

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Dr Holmes suggested azathioprine

Kath mummery at the national neuro hospital suggested trying rituximab

My haemo Beverley Hunt prescribed the aza. It’s normal to start low then if it’s tokerated, increase the dose until some relief is felt

So I did 50-100 stopped completely

Started again at 50 then 75 and have paused on that dose, which is very low

I do feel quite good st the moment. A bit like being properly awake and alert for the first time in ages

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I have blood tests every 4-6 weeks via my gp

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I’m so happy for you! Wow! I totally can understand how nice that must feel- I feel mentally sluggish- but I’m on seizure meds and dysautonomia meds that contain methscololomine bromide... so. I’m not sure I can pull apart why. The neurologist and PhD why is your equivalent of Cath Mummery who administers the neuro cog tests tells me it is not the meds/ but GP isn’t quite as convinced. My INR is “ in range” unless you look at the CFX test. Chromogenic Factor Ten Assay activity level for those of us with the lupus Anticoagulant Antibody. ( now I’m throwing out a controversial (?) not FDA ( USA standard) test.

It actually can more truly reflect the actual INR. So if your vein value says 3.9, your cfx, a percentage, can then be calculated. You might only be , say 3.2...

My hematologists are mixed in their opinions on this.

I’ve had my finger prick be in high 5’s, vein be in low 4’s and cfx show actual INR at 3.2. ( I brought home checker with me to hospital lab each time. Blood drawn at same time from vein for tests. )

I’m not really understanding the pros and cons of this CFX Assay- but it’s only for LA positive patients. ( I’m triple positive and a crazy aggressive clotter. Seizures as well.)

I’m a patient of Professor Natasha Jordan and I was lucky enough that Prof Hughes was in town and available to sit in on my consult with her when I was in about a year ago exactly.

I probably just need to come back and add a good Hematologist appointment to the mix- and Paul Holmes. Texas ( where I live) is just not sure how to handle all this or how to put the puzzle pieces together.

I certainly don’t know how to.

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Think I will have to 👍

Many thanks for your reply 🤗

Called 111 nhs non emergency number to day at 4.18 pm as I was feeling so I’m well now nearly 7pm now one has called me back !!! Because of my history ie stroke etc think I may of got a quicker response 😮

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All the best... when you feel up to it- we would be grateful to hear how you are. Looking forward to you feeling better, Tim.

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Azathiaprine is adjusted for the patient at maximum 2.5mg/kg/day. Regular blood tests are necessary to see if the body tolerates it. Mine doesn't due to a defective enzyme which didn't break it down and excrete via the kidney, so I became ill and it was stopped. It is used as a cortico-steroid-sparing drug for those with autoimmune diseases such as SLE, as I am sure you already know, Tim. I 'm just explaining for others here. Only a consultant can prescribe, but your GP can and should intervene ie contact your consultant to express concern, especially if your muscles are being affected.

The muscle enzymes consist of proteins that exist in high concentrations in muscles.

The main one is creatine kinase, or CK. When muscle is damaged or degenerates,

muscles leak CK into the bloodstream. In someone with myositis,

the CK level in the blood may be elevated, thus when an elevated CK level

is found, it is an important clue that something is wrong with the muscle.

However, sometimes medications can elevate the CK levels, which is why I would either contact your consultant with all your latest blood tests, or ask your GP to contact him/her, especially if you are feeling worse.

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

Muscle pain /weakness. Arthralgias, myalgias are noted, therefore, I would see your GP.

With good wishes,

Ros

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Thank you 👍

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Hi Ros

I wonder what the defect enzyme is in you that you speak of?

I started azathioprine last week and so far I am tolerating it without issue. However I’ve had to be started on a much lower dose as my TPMT test came back low and I’m in the ‘carrier range’.

My Dr has advised I probably will never get above 75mg otherwise I could suffer severe side effects.

I just wondered if this is the same thing you had?

Thanks

Kelly

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Kelly, I was wondering the same, but it doesn’t sound like Ros is talking about the TPMT gene mutation. That, as I understand it, has more to do with risk of bone marrow suppression.

( Ros, FYI, my TPMT Test just came back negative - so no problem if it should be decided it could be a good option for my particular APS situation to start azathioprine.)

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Defective enzyme and the TPMT Test, which I did not have prior to starting AZA. see below:

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Having said what I did, above. I may be wrong - as I said I am not a medical doctor and I wasn't entirely clear what you were saying. I did not have the test prior to starting AZA - I was under Dr Hughes - but reading more about it, this may in fact be the case. I was taking 1 tablet a day and the toxicity increased because my body could not excrete the drug via the kidney. This was a very long time ago - about 20 years ago.

My research indicates:

Approximately 0.3% of the population lack thiopurine s-methyltransferase (TPMT), an enzyme which helps remove thiopurine drugs such as azathioprine from the body. In addition approximately 11% of the population have measurable but reduced levels of TPMT (so called intermediate group). Individuals with no TPMT activity can become severely ill if treated with normal doses of thiopurine drugs. These patients develop bone marrow suppression with a reduction in blood cells, which causes anaemia, infection and abnormal bleeding. Patients from the intermediate group of TPMT deficiency may in some cases have less marked effects. Such side effects can be avoided if TPMT is measured before starting treatment.

I became severely anaemic (within a month) and my white count etc affected. While I cannot be certain because I have not had this test, I can infer I'm within this 0.3%.

We are a complicated lot. Unfortunately, I have had to have 2 spinal operations, the result of chronic inflammation, which attacked the spine ie lumbar. As a consequence of different tests, apart from low platelets, which is common in SLE & quite manageable, unless one takes a serrated blade and start slicing the body, the bleeding will not be cause for concern. What I was also told, was that I had a form of thalassemia, which explained while suddenly, my Hg drops. I have inherited this condition ie genetic and is Middle Eastern.

All this is quite fascinating, but I would rather not be "fascinating" but quite ordinarily boring!

So, thank you Yllek - because I have now learned something from you. This alone shows how important support groups can be, although one must always refer to a doctor! My GP telephoned me as a result of the latest hospital tests, showing I had thalassemia. No treatment necessary, unless severe anaemia, which is why I have iron infusions from time to time.

With good wishes,

Ros

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Oh I’m so glad you got a different drug that did help you.

It does indeed sound like you are either in the 0.3 or 10% group. I’m in the 10% group which can cause toxicity as you describe above.

However by starting with a lower dose the Drs are hopeful I will tolerate it.

Sorry to gate crash your post Tim. Hope you’re doing better.

Kelly

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I was unable to excrete which meant high levels of toxicity. My blood tests indicated it and I was very unwell.

I was put onto Azathiaprine because I was very sick taking Methotrexate orally. Fortunately, the U.K. just allowed the injections so after 2 weeks coming off Azathiaprine I was prescribed MTX.

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Another option if you test positive for TMPT is CellCept according to my Hematologist... so Ros, if ever you find you need to “ switch?” For whatever reason? You might ask you doc about this possibility?

Tim, perhaps this might apply here also to you? I’m not sure. I’d like to here what your hematologists and Rheumatologists think about this in the UK as an alternative?

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Cellcept Generic Name: mycophenolate mofetil, is suitable for those with kidney involvement, ie lupus nephritis, which doesn't apply to me.

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Azathioprine was suggested by prof D'Cruz. To treat my Lupus

As u say the dose is weight dependant so the dose each of us takes means the same amount is in our system

I was and still am on steroids

Quickly my liver enzymes the Sjogrens had caused problems with, returned to normal

Since on azathioprine I've been diagnosed with APS

I came off a short while and went on Methatrexate I was so sick even on injections that I went back on Azathioprine instead

The Azathioprine affects my INR and I'm hardly in range

I don't know if it helps my conditions or not. Good luck 💋

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Thank you 👍

Just been on the phone to 111 nhs non emergency help line said I’ve been waiting since 4.15 pm this afternoon to get a call back

First operator put phone down on me said it’s normal to wait over 5 hours for a call back I said what even with my medical history of a major stroke and mini stroke plus now micro clots on brain!!!

So I rang back and spoke to a different operator told her I had been cut off

She said I shouldn’t waited that long but then changed her mind and said it was normal to wait up to 6 hours for a call back given the information I had given the first operator!!! She said a doctor would be ringing me soon I told her not to bother I ring st Thomas on Monday

What a joke 🤕

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The more I see/hear of the NHS the more scared I'm getting. 💋

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Don't call NHS Direct! Either your GP or go to A& E! Phone St Thomas and speak with the Consultant or Senior Registrar. Not a junior doctor who knows less than you, unless S/he promises to call back after consulting your Consultant!

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Not unusual to have both SLE and APS/Hughes - depending on surveys it's in the region of 25-30%.

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No...

Well, I can only suggest extra liquids- and Ros. She seems to know quite a lot. ( I’m guessing she will quite sensibly tell you to take the offer of a doctor ringing you back in 6 hours. It’s safer than waiting until Monday- and quicker. )

It’s easy to get quickly disheartened when you feel so unwell with a disease that’s not well understood by main stream medical personnel. Do you feel you are ok to wait until Monday to call your GP? Are you better as day progressed? Worse? Same?

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London Bridge doc gave me a leaflet about aziophrine. But that was it aziophrine wise. maybe I m meant to look at the leaflet twice a day before meals.

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🤣

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