February 2013
Recently, the Times of London featured a couple who had tragically lost a baby late in pregnancy. A 'stillbirth'. The Times railed against the unacceptably high rates of stillbirth in the UK and asked for more to be done to reduce its prevalence.
Well...... a recent collaborative study, presented at the American College of Rheumatology, came up with a figure of 1 in 5 of all stillbirths being associated with antiphospholipid antibodies. Potentially preventable.
I'm pleased to say that the 'blogs' have not gone totally unnoticed. INOVA, a leading kit-testing company based in San Diego, is about to publish the collection of the first three years of "Patient of the Month" as a small book which they plan to hand out at various medical meetings. I'll keep you posted.
Patient of the Month
A short trial of heparin.
Mrs JP, a 47 year old school secretary, was referred for a second opinion. Her main complaint was of headaches - often severe - almost daily. She had been investigated thoroughly and test results included a normal brain MRI. Her doctor had considered a diagnosis of Hughes syndrome, but antiphospholipid antibody (aPL) tests were negative and the diagnosis was ruled out.
The history was complicated. As a teenager she had suffered frequent migraines. Whilst in college, she had developed suspected glandular fever which had left her fatigued for many months.
In her mid-twenties, the headaches subsided and she had a successful pregnancy. No miscarriages.
At the age of thirty, she developed a large DVT and was treated for nine months with warfarin. A year later she developed another thrombosis, this time in the arm. At this stage, a full clotting screen was normal, with one exception - a weak positive test for aPL. Another short course of anticoagulants.
Ten years later, at the age of forty, the headaches returned - almost daily. In addition, she developed worrying lapses in memory - especially with word-finding. She had also developed another new set of symptoms - dryness in the eyes, mouth and vagina, together with bladder irritation.
On examination she had the telltale sign of skin livedo. She also had very dry eyes on testing. My own feeling was that here we had the common combination of Sjogrens' syndrome (dry eyes etc.) and Hughes syndrome.
Where to go next?
A trial of aspirin was unhelpful. In this situation, my own practice is a 2-4 week trial of heparin - the safe 'low molecular weight' heparin (in this case 'Fragmin' (dalteparin) 10,000 units by injection once a day).
The result? Dramatic. Total disappearance of the headaches. Gradual improvement in word-finding. Delighted patient.
What is this patient teaching us?
Firstly, follow your clinical instincts. Because the aPL results were 'borderline', a number of the physicians had doubted the diagnosis. Yet the clinical clues were there. One day, we'll doubtless have more sensitive blood tests. In the meantime, treatment choices have to be based on those clues available.
Some years ago I introduced a 'heparin trial'. Clinical experience had taught us that a number of our Hughes syndrome patients became headache symptom-free when treatment with anticoagulation was started - for example, warfarin following a DVT or heparin during pregnancy.
Low molecular weight (LMW) heparin is safe and effective against thrombosis. Its major drawback is that it comes only as an injection, not a pill.
Nevertheless, when we try a patient such as Mrs JP on a short course of heparin, we often get a very clear idea of whether we are on the right track.
In this patient's case, not only did the 'heparin trial' (*) prove strikingly effective, leading to long-term, stable warfarin treatment, but it also brought back her confidence - confidence that had been sorely dented by the long history of clinical doubts.
* Reference - Hughes GRV. (2012). Heparin, antiphospholipid antibodies and the brain. LUPUS Vol 21 pp 1039-1040
Do confirmatory tests have to be positive for Hughes Syndrome?
Finally got the Hughes diagnosis!
Hughes syndrome, clots & headaches
My girlfriend thinks she has Hughes syndrome
Haematologist still says my migraines have nothing to do with APS/Hughes
