Professor Graham Hughes' Monthly Blog December 2016

BLOG – December 2016

“Treatment Choices, Treatment Failures”

I am writing this blog on the last day of the year – an eventful year both in politics and, for me, in medicine. For me the highlight of the year was the opening of the Graham Hughes Autoimmune Diseases clinic in Madeira. This honour was especially cherished as it came from another country, Madeira, Portugal and arrived completely out of the blue.

December’s diary included a short visit to a meeting of the Lupus Academy in Dubai. The academy is an international collection of lupus experts, aimed at furthering knowledge of lupus and related topics. This particular meeting was intended for doctors from the Middle East (including Iraq and Syria), and my role was to hold two “think tanks” (or “round tables”) on treatment of Hughes syndrome. Totally stimulating! As you might guess, their clinical success and failure very much mirrors -our own here in the UK, which brings me to the title of this month’s blog – “Treatment Choices, Treatment Failures”

Patient of the Month

Dear Professor Hughes,

Re: Mrs B.D.

I would be very grateful if you would see this 47 year old lady. In summary, she has a diagnosis of Hughes syndrome, but sadly, is responding poorly to treatment.

Her past medical history includes hospital treatment for low platelets at the age of 13 (platelets now normal), and a 30 year history of migraine headaches. She was investigated at one point for infertility, and has no children.

Some five years ago she started daily aspirin (75mg) but with only partial success.

During the past 2-3 years, the headaches have worsened, sometimes producing transient ischaemic attacks. One year ago she started developing balance problems and severe memory disturbance. Investigations in her local hospital confirmed the presence of high titre anticardiolipin antibodies, and a brain MR showed multiple focal lesions. At this point she was started on Warfarin. However, despite initial improvement the response has been disappointing…..

What is this patient teaching us?

In my clinical practice at The London Lupus Centre, this doctor’s letter is not uncommon. What could be the problem?

1. Firstly, of course, the diagnosis. Is it correct? In the case of Mrs B.D., she has been carefully investigated and the clinical picture, together with the

frequently repeated high levels of antiphospholipid antibodies, suggested Hughes syndrome.

2. Poor response to aspirin. Although the antiplatelet drug aspirin is helpful in many cases of Hughes syndrome, it is not always so. It is recognised, for example, that some patients are aspirin resistant. In others, the response is poor – for reasons that are poorly understood.

3. Poor response to warfarin. And here, the reason is often surprisingly clear. Take a look at the patient’s INR book – INR 2.6, 2.1, 2.5, 3.0, 2.3, 3.2, etc. The patient is not being treated! So many Hughes syndrome patients – especially those with neurological symptoms – seem to require a higher INR – for instance in the 3.5-4 range. Interestingly, the patient usually finds his or her ‘best’ range by trial and error. Such patients often face a struggle in the anticoagulation clinics – which more usually deal with elderly patients with atrial fibrillation, doing well with an INR of 2, for example.

But Mrs B.D. has Hughes syndrome, and is at high risk of developing a stroke. When the appropriate INR level is achieved (many of my patients also self-test their INR’s), the result is clinically very rewarding. Yes, higher levels (e.g. over 4) increase the risks of bleeding, but ‘sticky blood’ is a clotting disorder, not a bleeding disorder.

Professor Graham R V Hughes MD FRCP

Head of The London Lupus Centre

London Bridge Hospital

MaryF

16 Replies

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  • P Hughes is asking the right questions. Let's hope answers bring forward better choices of treatment and relief for sufferers.

  • Yes as always. MaryF

  • I almost feel ashamed when always nagging about this; The INR must be kept stable at a high INR to work for us.

    Also APS is a clotting disorder - not a bleeding disorder.

    Thank you for telling us this prof Hughes!

    Thanks Mary!

    Kerstin

  • Hi Kerstin, and a big 'yes' to that. MaryF

  • I conquer, thank you Dr Hughes

  • Seems I am not being treated then as my INR is up n down .since November it has been 2.4 2.5 2.9 3.2 Next test is Monday I have been feeling rubbish but you can't tell my consultant as he just put in letter to other doctors that APS is stable.

  • Print out the blog and give it to them both, worth a try. MaryF

  • Sounds good

  • Best of luck, I issued all doctors and consultants attached to mine and my children's cases with books written by Professor Hughes! MaryF

  • See if you can get your consultant's email address and forward him Professor Hughes response to "Nov question of the month". Good luck!

  • From reading the blog is seems I'm not being treated as the haematologist and rheumatologist have agreed that my INR should be between 2 and 3, I've queried this on numerous occasions, so much that I sound like a stuck record but keep being told that is my range. I will print off the blog and give each of them a copy when I see them next which unfortunately isn't for several months. I also have a Transverse Myelitis so I'll give a copy to my neurologist when I see him at the end of the month.

  • Good for you, also bear in mind this connection: ncbi.nlm.nih.gov/pubmed/119...

    MaryF

  • Thanks Mary, they checked my vitamin B12 when I was first diagnosed and GP checks it regularly and its been fine all the time. Just have see what they all have to say when I give them a printout of Professor Hughes blog

  • Thanks Mary, just wish my home town haematologist for the warfarin clinic could read this as well as the anticoagulation team Pam

  • Please just print it off and ask politely that they do. Use the analogy of treating a patient with not enough insulin as most Dr's and nurses will understand that. If you get the old adage that they don't want you to bleed, remind them you have a clotting disease and your blood is too thick!! Ask them NOT to compare you to AF patients as there is no comparison.

  • These Blogs from Prof Hughes are so crucial to our learning and understanding, I am so grateful to read these. One thing I have never really asked at my appointments is what my titres are? I have primary APS, more recently told by Prof D'Cruz that I am now triple positive but I have never asked what my titres are? What level is considered high & would they tell you without asking? I also have Lupus etc oh yes too many 'secondary conditions' to mention, but thankfully with APS have not had a clot. Hope you all have a well day xx

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