What a long winter! For three months it has been wet, cold, windy and … miserable. Darwin must have had something to say about hibernation.
Three lectures this month – two GP lectures and one specialist. The specialist talk was in Barcelona – to their annual ‘Ten Topics’ meeting (even my one day in Barcelona was cold and wet). This meeting, voted last year as the most popular Spanish medical meeting, follows on the success of the London meeting – but to a larger audience of 300 attendees. As with the St Thomas’ meeting, the emphasis in Barcelona is heavily on lupus and Hughes Syndrome, and the level of both content and discussion is high.
Each year the venue is the same – the hotel Sants – a large, functional hotel over the main railway station in Barcelona, well equipped for meetings and conferences – of many varieties. Unforgettable was the ‘Ten Topics’ meeting five years ago when we shared the conference rooms with an annual ‘sex industry’ conference. Wandering around the hotel foyer were two specimens of attendee – the ‘Ten Topics in Rheumatology’ doctors with their conservative conference bags, and the undoubtedly more exotic ‘sex industry’ conference delegates with (one can only presume) their more unorthodox kits!
The two GP meetings here at London Bridge Hospital were, as always, memorable. The second meeting held on a Saturday morning, attracted 100 GPs. The post-lecture discussion again opened ones eyes to the importance of our syndrome in general practice – the migraine, the miscarriages, the memory and balance symptoms, and even the more severe complications of untreated Hughes Syndrome – the angina and the strokes.
As always, the discussion centred around ‘how many cases have we in our practice?’ and ‘how common is the syndrome in reality?’ And as always the answer remains uncertain.
It is one of my ambitions to attempt a realistic survey to try to answer some of these questions. It is a project which could be fairly easily carried out, given the funding, say, for a research graduate.
John Wolffe and his family have done much to help our charity, and also recently took part in a BBC Breakfast TV programme about Hughes Syndrome. I was interviewed and again asked ‘why does the public, the media and the medical profession know so little about Hughes Syndrome?’ I sometimes feel that the media would like specialists such as myself to criticise GPs for their apparent lack of knowledge of the syndrome. That I would never do, having been a summer locum GP for nearly fifteen years.
Why, then, the lack of general knowledge about Hughes Syndrome?
Perhaps, surprisingly, it is not easy to answer. I am reminded of the same lack of public awareness of lupus back in the early 1970s when I set up my lupus clinic. At least that has changed with the weekly lupus diagnosis on the TV programme ‘House’.
My colleagues at St Thomas’ are starting a trial of the new anticoagulant ‘dabigatran’ (who thinks of these names?). As you know, we have waited a long time for a genuinely new drug that might prove an alternative to heparin or warfarin. This drug has passed its preliminary hurdles and looks promising. (The joy of not having to check INRs!). Such studies are painfully slow, but with the large clinical experience at St Thomas’, we hope to get an answer.
Two interesting studies appeared in January. The first, from the Lupus pregnancy clinic at St Thomas’, compared the pregnancy outcome in antiphospholipid antibody (aPL) positive pregnancies in women with or without a previous thrombosis. As might be expected, those with previous thrombosis had a poorer success rate.
On a similar theme – Hughes Syndrome pregnancy – a group in Singapore (Mak et al, Rheumatology 2010, 49, 281) found in a survey of five major trials that a combination of heparin and aspirin is superior to aspirin alone in pregnancy success rates of aPL positive women with recurrent pregnancy loss.
Case of the Month
A 57 year old solicitor developed visual disturbance, diagnosed as optic neuritis. He quickly went on to develop weakness in the limbs, balance problems, and patchy loss of sensation.
He was diagnosed as having multiple sclerosis. Over the next two to three years he deteriorated and was largely confined to a wheelchair.
His local physician, however, didn’t let things rest. Blood tests showed a high aCL test (very high, in fact, at over 70 units). The neurologist at first stuck to the MS diagnosis (“positive aCL tests are sometimes seen in MS”), but the patient and his physician were dogged and he was referred to our centre.
On examination, the neurological features of course suggested MS. But there were two small clues – firstly, the long history of frequent headaches, and secondly, the borderline low platelet count (107,000 – corrected to 130,000 on manual checking).
Eventually, it was decided to start a trial of warfarin treatment. Nothing to lose perhaps. And for six months, nothing much changed. At follow ups, the warfarin anticoagulant INR card read 2.1, 2.5, 2.3, 2.6 ….. The dose was increased (the patient now self-testing) ….. 3.5, 3.7, 3.8, 3.9. And clinical improvement! For the first time in years, the patient is mobile and, under physio guidance, is beginning to regain limb strength.
What is this patient teaching us?
I make no apology for coming back to MS. Such a difficult diagnosis. And with the odds stacked against recovery in severe cases. And – it must be said – with a reluctance in some quarters to accept that some cases of APS may closely mimic the disease.
In this month’s patient, time will tell. But for me there are two – maybe three – lessons.
Firstly, that Hughes Syndrome, if it starves the brain of oxygen, can mimic almost any neurological condition.
Secondly, that warfarin under-treatment in some cases of Hughes Syndrome is a waste of time. Rather like treating an under-active thyroid with half the dose of thyroxine, or treating diabetes with half the dose of insulin.
Thirdly, the platelets may be important players in the ‘sticky blood’ syndrome. A low platelet count in the machine used for testing can sometimes be ‘artificially’ low due to platelet stickiness. A small clue, perhaps, but good medicine is based heavily on good detective work.
Professor Graham R V Hughes MD FRCP