Professor Graham Hughes' April 2013 blog

At long last there is a sense of spring in the air. Earlier this April, it was so cold that even our Schnauzer puppy, Lucy, didn’t want to step outside!

This month saw the latest in the bi-annual international lupus conferences. This year’s meeting, run by a team of South American doctors, was held in Buenos Aires – a great success by all accounts.

I couldn’t go, but was invited to give a ten minute presentation by video at the opening ceremony.

The title was: Antiphospholipid syndrome in 2050: predictions.

A great challenge.

Perhaps optimistically, I planned the 2050 meeting at the Disney Intergalactic Conference Centre, The Moon … Ten minutes allowed.

For what it’s worth, here are my predictions:

1. Antiphospholipid (aPL) testing kits will be available in pharmacies and drug stores worldwide.

2. There will be one, internationally standard test – the dreadful ‘lupus anticoagulant’ test having been superceded.

3. The world of migraine will be totally changed – Hughes syndrome (and aPL antibodies) will prove to be the ‘missing link’ between migraine and stroke – a preventable link.

4. The number of cases of stroke and heart attack will be halved – especially in younger (under 45) women, thanks to wider recognition of Hughes syndrome by neurologists and cardiologists.

5. Many cases of ‘Alzheimer’s’ will be found to have Hughes syndrome. Potentially treatable with anticoagulation.

6. The number of cases of late pregnancy loss – ‘stillbirth’ – will be cut, thanks to the more routine testing of aPL in pregnancy – it is estimated that one in five cases of stillbirth are aPL positive.

Enough for ten minutes, I suppose.

On a less positive note, it seems that there is a vociferous group trying to do away with ‘eponyms’ – diseases named after their discoverer eg. Sjogrens syndrome, Kawaskai disease, Crohns, Wegener’s – and yes, Alzheimer’s. The reasons for this campaign are obscure. I feel that such a move would prove a backward step – especially in student and patient education.

Of course I also feel personally about this. I am proud of the honour of the title ‘Hughes syndrome’ given by my respected co-workers twenty years ago. Of all the jobs I have carried out in my medical career, the detailed description of the antiphospholipid syndrome in over one hundred cases is the one which gives me the most pride; and, more prosaically, the current ‘correct’ title of the syndrome is not the antiphospholipid syndrome, but the ‘anti-cofactor(s) complexed to phospholipid’ syndrome – a fog that would also descend on many other eponymous disease definitions.

My colleague David D’Cruz tells me that there is pressure on medical journals not to accept papers with eponymous names.

Well, there is one journal which won’t be pressured in this way – the international journal LUPUS. I am its editor.

Patient of the Month

A 42 year old IT consultant was referred by a gastroenterology colleague because of abdominal symptoms following meals – especially ‘large’ meals.

He also complained of fatigue, muscle aches and headaches.

Amongst the many possible diagnoses on offer, the gastroenterologist considered Hughes syndrome. Tests for aPL proved strongly positive. Other tests were essentially negative.

How to treat?

I planned to start him on low dose aspirin, but as the patient was concerned about indigestion, we agreed on Plavix (clopidogrel) once daily – a medicine which improves platelet stickiness.

Success! He returned for follow-up four weeks later. Headaches gone (as I half expected) – abdominal discomfort also gone! And something else – the patient, a regular gym-goer had noted an improvement in all his gym exercise data.

What is this patient teaching us?

In this series of blogs, I have included a number of features, possibly secondary to ‘sticky blood’ which have seemingly improved with blood thinning. These have included hip pain, muscle aches, tinnitus, and many forms of memory loss.

During the past few years, I have seen a number of cases where troublesome abdominal pain has improved with such treatment. Whether this is as a result of treating poor gut blood supply (‘abdominal angina’) or is in anyway linked to ‘abdominal migraine’ is not clear.

Interestingly, this patient discovered something else. His scores on the gym cycling and power-testing machines went up by over 20% while he was taking Plavix – and fell when he experimentally stopped the drug.

As befits a good IT consultant, he is now carrying out a series of experiments – comparing his gym kilo-joules (or whatever it is they measure) with variations in treatment. We hope to have some data ready for the next major conference on Hughes syndrome!

[swx3]

Here here!!!

[Hidden]

Agree!

[SueLovett]

I agree with the forementioned.

I used to get abdominal pain after eating but this resolved when I started heparin.

[MaryF Administrator]

Yes - A distraction from the task in hand to remove the name. I am so concerned about the amount of patients who have had serious clinical symptoms, serious ones who have had to be treated, only to be cast aside later on,due to unstable test results or periods of the

tests being negative - that I wonder about doing what some other charities have done.. and have a section for web diaries..with clear interviews etc. I feel very strongly about this. Over and over again, patients who have passed tests in the past only to go through a negative period or those who take years to pass who clearly have the disease.. I remember very very clearly asking if I had Lupus for instance twenty years ago due to my symptoms only to now pass the tests! Perhaps some of us must also get together to write a book those of us who have had clots, and strokes and have every symptom minus a consistent test result.. but with the clear medial evidence and events.

Mary F

[Coppernob]

At a total tangent - I see Prof Hughes has a schnauzer puppy. We're deliberating long and hard about (a) getting a dog and (b) if so, a schnauzer (miniature). I wonder how he's getting on with Lucy???? If we got one, maybe we'd call him Hugh - in the Prof's honour (but only if a boy of course!!).

[GinaD]

Anti cofactor(s) complexed to phospholipid syndrome. Another name!

Once on a return flight over the pond I was moved from one flight to another due to an initial flight delay in Glasgow. The last minute changes resulted in my loosing my extra leg room seating in the regular cheap seats. Noting the number of empty seats in first class I relayed my concerns with sitting all cramped up to the (very rude) stewardess. She accused me (loudly -- I think she was trying to shame me) of being a cheap American and trying ot get an upgrade for nothing. I hand rose from among the seats in front of me. A fellow passenger, rose, turned around and said, "I work at the London Lupus Center with the doctor who first described her condition. This woman knows all the names for her disease: Hughes, APS, APLS, antiphospholipid syndrome, sticky blood syndrome, sludge blood -- she wouldn't know all the names this disaease goes by if she didn't in fact, HAVE the disease. Her concerns are well founded. She needs more leg room. She is at high risk for developing clots."

The stewardess remained tacitern. We arranged a compromise. I crossed the Atlantic laying on the floor outside the lavatory with my feet propped above my head. Charming. But I did not get any DVTs. If this happens again I'll add, -- what was that again? -- "anti cofactor(s) complexed to phospholipid syndrome" to my (now growing) list of monikers. (And should I also add ACCPS?)

And regarding the epynome controversy? A more detailed symptom title may actually cause a resistence among some patients to accept diagnosis. It is easier to say "Crohns" then "uncontrollable bowels."

[overnighthearingloss]

Maybe the naming thing relates to how medical students receive their teaching. If they get to know something by a different name it could possibly then cause confusion.

But it may also be a professional rivalry or embitterment. All a bit kindergartenish really.