Professor Graham Hughes' Monthly Blog November 2016

BLOG – November 2016

Good and bad! November saw the publication of the 2nd edition of my book “Hughes Syndrome. A patient’s Guide”. Wonderful stories of two of my patients – one from the Scottish Isles, and one from Romania.

The first patient from the Scottish Isles presented with severe neurological problems. Treated by Dr Hoda El-Mahrouki with heparin and then warfarin (INR 3.5-4) and now completely well. The Romanian patient is this month’s patient of the month.

The bad news – at least for me, is the decision of the Trustees of the Hughes Syndrome Foundation to drop the name “Hughes Syndrome” from the charity.

The epithet ‘Hughes Syndrome’ was an honour conferred on me by colleagues (experts) at the 4th International Congress on Antiphospholipid Antibodies” held in Louvain in 1989. (1)

Coincidentally, this month’s ‘patient of the month’ features an outstanding review entitled “Hughes Syndrome and epilepsy : when to test for antiphospholipid antibodies?” (2)

Patient of the month

Miss A.J., aged 18, came to see me from Romania, Eastern Europe. And she came by train as her mother, who accompanied her, was afraid of flying.

Miss A.J. had been diagnosed with lupus in the hospital in her home town. She had complained of joint pains, a marked butterfly rash on the cheeks, and hair loss.

However, she had also suffered from 3-4 seizures and some odd psychiatric symptoms. Her anti-DNA test was positive.

In view of her seizures and odd psychiatric features, she was diagnosed as neuropsychiatric lupus and was started on steroids. High dose steroids (60mgs daily). Unfortunately, she suffered further ‘brain’ episodes and the steroids were continued at a high dose.

When she arrived at my clinic, she had gross “Cushings” – bloated, red face, weak – and her problems seemed now to be more steroid-related than lupus related. Then the test results came back – DNA antibody weakly positive, kidneys normal, but aPL tests for ‘sticky blood’ extremely high. Brain MRI showed several small ‘dots’. Perhaps APS was the major player rather than the lupus. A planned programme of steroid reduction, coupled with warfarin anticoagulation was started.

Two years later she returned to England – again by train. Off steroids, on warfarin. Completely transformed. No more steroid effects – face and body back to normal. And no more ‘cerebral’ features.

I have photographs in my clinic of the enormous improvement in her appearance, and in her life.

Postscript : Last month, some 20 years later, she returned to London (on her own and by Air). It was a social visit, but she took time to pay a visit to London Bridge Hospital. Well-controlled on warfarin and very, very well.

The importance of the correct diagnosis. And the joy of medicine!

Reference:

1. Hughes GRV. Tales of a Flying Doctor

Hayward Medical communications ISBN 978-0-9542022-1-7

2. Noureldine M et al. Hughes Syndrome and epilepsy : when to test for

14 Replies

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  • As I read this, it is not a wonder why I am always "nagging" about being on Warfarin with a steady, therapeutic level.

    For me Warfarin has been my lifesaver and I can only thank Prof Hughes for what he has done and today continue to do for us here.

    Kerstin in Stockholm

  • I agree with you! MaryF x

  • I am very sad for Prof Hughes to have Hughes Syndrome Foundation change their name, I owe him my life and refer to myself as having 'Hughes' - and always will.

    My severe neurological symptoms also vanish with an inr of 3.5-4 - returning only when I fall below this level - which my GP didnt really believe I don't think until I printed off some literature to prove it and next week when I see him I will also show him this months video for 'question of the month'.

    Thank you to Prof Hughes, so many of us would not be as well as we are today without you (and warfarin!) x

  • Yes we were sad too but there is no fear that the name will ever change here and we will always refer to it as Hughes(Antiphospholipid) Syndrome, the name he actually gave it before it was changed to honour him. Its also internationally known as that far more often by most Doctors and we have stats that bear that out.

    Its actually rather funny, as the reason of "not using eponyms anymore" is rather strange. Sjogrens and Autoimmune Thyroid -"Hashimotos", is often referred as the "big trio" with Hughes, and both of those along with Parkinson's, Graves, Alzheimer's, Hodgkins, Cushings and Crohns are never going to be stopped from being used, plus I could find lots more! Its frankly an embarrassing joke!

  • How do I find the video. I'm new to this and not good with technology. Can I find it via my phone or does it need to be on a computer? X

  • Thank you very much.

    I hope today's a good day for you!

    I'm wondering how I can add a photo to my profile.

  • HI there, go to your profile, then account settings, then edit profile, you should then be able to add an image, MaryF

  • I'm sorry I'm just hopeless at this sort of thing. I went to edit profile and have added other information but can't see the option to upload a photo. Never mind it doesn't really matter. At least I'm here. Thank you xx

  • Though I have never hard the option of seeing Prof. Hughes I am in debt to him ashis hard work has saved my life and the lives of countless others. Its a shame that they are taking away the name, his recognition for searching and finding answers for so many patients whom have been rejected by dr.s that still do not understand Hughes symdrome. Thank you Prof. Hughes for all your hard work to find out what was killing and/disabling so many. Thank you for my life and for the thousands of patients just like me who where constantly told there was nothing wrong with us and that its n our heads. Thank u saving me from death or dementia and giving me the chanceto raise my children. I, we are forever in your debt.

  • His name continues to be well known, don't worry, and some centres bearing his name have opened recently in Europe. A lot of doctors and medical professionals know it by both names. MaryF

  • That's good around here most dr.s don't know what it is or are still refusing to consider or test for it.

  • This is Dr Hoda El-Mahrouki. There actually are several stories of success from the Western Isles managed on Warfarin with an INR target of 3.5-4.5.Also cases from the South of Scoctland I will share with you a couple in the new year as I have just started them on Fragmin and hopefully Warfarin. Correct diagnosis and management with simple anticoagulation certainly transform lives. I will be back next year. Happy Christmas and wonderful new year for everyone.

  • I will send a copy of this to Professor Hughes, thank you for your feedback! MaryF

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