Q&A with Graham Hughes – 7 October
This video was recorded on 7th October 2020 and answers some questions put to us from the GHIC community about APS. You can read a transcript of the video below.
Henry: It’s Henry Shelford here, I’m thrilled again to have Professor Graham Hughes for our latest in a line of Q&A’s with him on behalf of the Graham Hughes International Charity. Well without further ado hello Graham
Graham: Hello, thanks for inviting me
Henry: Well you are the founder of the charity and and I think everyone’s always incredibly thrilled to hear from you and it is great to have you up. So last time we were at your home!
Graham: Yes down in the country
Henry: Which was a thrill and I know Leo who is part of the team absolutely loved being being down there and he came back saying ‘I’m sorry for being so late but I was offered lunch, I couldn’t say no!’ It’s so lovely, so he had a wonderful wonderful time and I think everyone learnt a lot on that Q&A. As with all of them we’ve been asking people to send in questions, so those watching if you have any questions, so we’ve had some sent in we’ll work through those and if you’ve got any additional questions just post them in the comments. We’re live on Facebook and on Twitter we’re looking at them both and we’ll pull the questions in as we go. Any problems with the feed or anything do let us know as well, but I think we’ve got some people checking it hopefully it’s all going okay. So the first question we had, I know the answer and it’s a bit sad, so we always get so many comments from people who’ve been touched by you, by what you’ve done, and sometimes have been treated by you, our first question which was sent in Gill was: ‘Does professor Hughes see patients privately, and if so how could I go about booking an appointment?’ I know the answer.
Graham: I’m sorry. Earlier this year when the virus struck we all changed situations and I had to decide whether or not to go one extra year in the clinic and the rules are pretty tight, you can’t if you’re not fully insured etc, but what I do have is the team behind me at London Bridge Hospital and they know which sort of patients I used to see and they will make the appointment for one of the team. What I try and do is discuss with the doctor seeing the patient, and or seeing them you know from a secondary point of view. I’m sorry to give such a negative answer to start with.
Henry: And on the insurance point, the insurance is about you because of COVID and doing the wrong thing and because of your slightly esteemed age, it’s a personal issue that is very difficult for you to be in hospital right right now and treating people. I think what you’ve said though is GHIC actually has a large number of consultants listed on the website who are leaders in their field. There are a number of trustees of the charity and they are exceptional people who you’ve essentially hand-picked to hand over to.
Graham: I mean at London Bridge Hospital where I’ve been working for the last 15 years we have the London Lupus Centre and eight of my team still work there full time, so I call them my mafia because I’m very pleased and proud of them
Henry: And all of them obviously work in the NHS, as you know all of the experts in the UK work in the NHS most of them, and you can always ask your GP for a specific referral. So if you go onto the GHIC website, find a specialist you like, you can request that, we do recommend that. So I know a lot of people still want to meet with you for obvious reasons but that has changed and that’s a part of why you’re putting a lot of your energy into the charity to sort of further communicate around Graham Hughes Syndrome, around APS and teach people.
Graham: That’s right I think the main reason for my starting and getting involved with this charity is education really, anybody who is involved either as a patient or as a family member can learn more
Henry: Caroline has sent in a question which perfectly segues into talking about education. She asks: ‘How can we increase awareness amongst primary care and secondary care specialists and ensure that APS is included as a part of the curriculum in doctor’s foundation and core medical training?’
Graham: Absolutely Caroline this is our biggest concern over the years, I think probably it’s true of every specialist in his own subject or her own subject that it takes a long time to to learn about what essentially seems like a new disease. It’s happened with lupus – 30 years ago patients and doctors didn’t have much information on it. So the answer to your question: we’re giving GP lectures, we have once a year a patients meeting which will certainly be disrupted this year, of course we’ll certainly keep you posted about that, but we publish books on Hughes syndrome, little booklets, and of course doing what we’re doing here with with the TV.
Henry: That’s great and I think the core of that question, right, is that you discovered the syndrome and have essentially been teaching everyone about it ever since because prior to that they obviously didn’t know and so it has been a lifelong passion and work to build up that presence and increase awareness. Part of GHIC’s role is to particularly focus on increasing awareness around if you’re having multiple symptoms. So actually it’ll be useful to to recap on some of the symptoms and warning signs or the potential bells that would suggest someone should go and ask about Graham Hughes Syndrome.
Graham: Yes, well the background is in one way very simple – we call it sticky blood, not quite scientifically exact, but it’s where the blood has a tendency to clot abnormally and so it can affect, for instance, veins in the legs, DVT, but it can sadly also affect major arteries such as heart attack, stroke and so on. In other words it can affect any organ of the body so the full patient getting this syndrome ends up seeing cardiologists, nephrologists, rheumatologists, memory loss the whole thing. The two organs that seem to be most sensitive are the brain, which I’ll come back to, and in pregnancy the placenta, so early clotting of placenta. These women get recurrent miscarriages and that’s a big aspect of the scenario.
Henry: And one of the things we as a charity are working on is to make sure that if someone has recurrent miscarriages that they do automatically go and get a test for Graham Hughes Syndrome
Graham: Yes, it’s the same old thing, it’s an antibody test, fairly simple, gets done in most teaching hospital level laboratories. So we now know for instance the most common, treatable cause of recurrent miscarriage. So any woman for instance who’s had three or four or five miscarriages you know, tragedy, should have been tested or should be tested.
Henry: It’s just an awful situation that has a potential resolution because you’ve treated many people and that’s the result of them being able to carry to term. It’s a big very very important thing and a big part of what GHIC do and why you founded the charity. So those are our first general questions, so we’re going to move on to some about symptoms and side effects and actually you just talked about the brain, so Paula has written in, she said: ‘I was diagnosed with APS five years ago. I have scar tissue due to another DVT in a bleed/tumour on my brain. I’m experiencing worsening symptoms and increasing pain but these concerns haven’t been taken up by my haematologists. Am I right to be concerned, is it normal for symptoms to become more prominent over time?’
Graham: It can happen definitely, normal is a strong word perhaps but it’s certainly a feature of the syndrome. It can come on, the most common age perhaps is between 20 and 40. For instance a lady who has miscarriages at 20 can at the age of 40 start getting severe headaches for example. Obviously with your symptoms that you just told me, you’ve got to be sure whether it’s another diagnosis like an aneurysm or something like that but in general you certainly should be tested. I’ve devised a list of I think 40 symptoms that could be APS and that list we’ll put it together for our people who are involved with the charity so that if you get several of these features you should be strongly suspecting the diagnosis. The brain is a stupid organ because if it doesn’t get enough oxygen you know you get either headaches, migraine is one of the most common features, memory loss, I’ll come back to that if I can because that is a major feature of the syndrome, or even funny terms like seizures. For instance teenagers with epilepsy one of the known causes now is Hughes Syndrome and finally we we have a thing we call the one in five rule. I mean a lot of conditions such as DVT, one in five of those patients have sticky blood which can be treated and diagnosed, one in five early strokes in under forty, one in five coronaries in women and this is a more common disease in women than men but it’s still an important thing not to not to forget.
Henry: Talking of heart attacks in women, in the most recent speech actually given by the Health Secretary Matt Hancock, he talked about one of the problems around the teaching of heart attacks was that they still taught from the perspective of what it feels like for a man to have a heart attack. I think it might be useful for you to tell people what it feels like for a woman to have a heart attack, what are the symptoms?
Graham: Yes well the symptoms are obviously angina which is chest pain on exertion or in the cold and this can lead to an attack which is more serious of course which is chest pain across the centre crushing pain, pain down the arms and of course collapse and all the various more severe things. Now yes, heart attacks traditionally much more common in men than women which is why the people who publish this paper who came from Rome, they found that I think 40 year olds or maybe 45 year old women who had heart attacks, this was a major treatable cause and I make a strong plea, and we’re making it to the cardiology world which is taking note. Incidentally we’re seeing a lot more patients who have angina as their symptom and if you’re a woman aged 30 and getting angina you really should think of this diagnosis because it’s so treatable.
Henry: It’s clearly very very important, that’s a lot of what your fight is about, that Graham Hughes Syndrome can cause a lot of different things between your topic and then help those things. We’ve had a questioning from Jan on Facebook: ‘Can I ask if hyperthyroidism is linked to APS at all?’
Graham: Thyroid problems are yes, linked. APS is what we call an autoimmune disease where your immune system attacks wrongly some of the organs, and in our disease it attacks the membranes of blood vessels as well as the platelets, the clotting platelets, and because it’s an autoimmune disease, in the families of patients with Hughes syndrome we see an increased number of thyroid problems, autoimmune thyroid hashimoto’s and a rise in numbers of other autoimmune diseases such as lupus, multiple sclerosis, not major but definitely an increased incidence. So it’s clearly a genetically determined disease in the background.
Henry: And the link to auto autoimmune diseases?
Graham: Yes, autoimmune diseases are diseases where on the whole the body produces antibodies which normally they should do for various things like flu virus and so on, but the antibodies when the disease happens are wrong if you like, and they start attacking organs. So thyroid autoimmune disease is affected by thyroid antibodies and if you look at a large family with Hughes syndrome you’ll find a number of patients with thyroid autoimmune disease and that can go either way, as in your case overactive thyroid or more cases we see under active.
Henry: Charlotte on Facebook, she said ‘I’m currently on warfarin and suffering strong side effects and symptoms and I’m getting bad infections, joint pain, migraines, visual disturbances and bowel problems. I’m also on Duloxetine to help with the pain but I’m struggling to maintain a quality of life. What would your advice be to help manage this?’
Graham: It sounds like full-blown Hughes Syndrome that you have. Now, many problems but the one I’ll take is the one where you’re having problems with warfarin. Now warfarin is a marvellous drug for patients who are getting early strokes and so on because you’re clear then, you’re theoretically not going to get a stroke because the thickened or sticky blood is effectively neutralized. However warfarin is a pain in the butt because patients often get side effects, it interacts with drugs and in your case one has to ask for the list of painkillers and so on that you’re having to take which can interfere with the warfarin dosage. Food can affect it and two answers to your question; one is of course I would hope you’re under a physician who understands the concept of Hughes Syndrome, in your case very important. The second answer as far as warfarin is concerned is many of my patients who are on warfarin self-test. Now you can buy a machine, that finger prick test, and it tests your INR. Some doctors don’t like this because they think perhaps it’s too much responsibility to give to an individual patient, but in patients who are having difficult times with warfarin I find it very very useful and to do this in collaboration with your own anticoagulant clinic. Many of my patients who seem to be having problems with warfarin, if you look at their little cardex cards that come for the INR it seems to fluctuate up and down like a yo-yo, and many of the cases I see is that the warfarin dose is actually too low and not too high and your INR instead of being three is near a two. Now I’m getting a bit technical there I realized, but in other words you’re not fully treating the patient here, the patient needs to be monitored and may well need to be on a slightly higher dose of warfarin. I hope that’s not too complex an answer.
Henry: I think it’s fantastic I think it’s a very thorough answer. I’m going to risk something terrible and I’m going to jump across my question list of the ones sent in and go out of order, so I will come back but from this point it could all go wrong people but it’s because it’s related to what you’re talking about. Right so Andrea wrote in and said: ‘I’m on warfarin and I currently self-test using the portable handheld machines…’, exactly what we were talking about, ‘…to measure the INR in my blood. Recently I’ve been hearing some conflicting advice about using the machine versus getting a normal blood test. Is one better than the other?
Graham: No, one should be in collaboration with the other. Certainly some patients have a terrible time even with their anticoagulant clinic they don’t get the results until the next day etc and that is improving, people are recognizing anticoagulants, are recognizing Hughes Syndrome sometimes is more tricky just to to treat. So the answer to that one is get to see your physician.
Henry: And why might people think that getting a sort of blood test in hospitals is better than using the handheld device?
Graham: Partly tradition, I mean we’re all brought up on going to the anticoagulant clinic and many patients now have a marvellous time, they can just pop in and they telephone when the results are abnormal, that wasn’t always the case.
Henry: It wasn’t my definition of a marvellous time going to the anticoagulant clinic
Graham: And although some clinics frown on patient self-testing, I think when done intelligently in collaboration it is good. The last little thing I’d say is occasionally the machines don’t seem to work properly, I don’t know why, nobody knows really why and there are some patients who absolutely specifically find that they can’t get a correlation between the hospital results on their own, lots of reasons and I won’t go into them now but it is sometimes a headache.
Henry: Okay and I imagine a hospital blood test there’s much more to it or we may test other things as well, but the the value of the test at home is that you can do it so much more frequently and in doing so really get a measure of what level of treatment you need to do which isn’t possible with a hospital visit.
Graham: I mean you’re right for those who don’t know about warfarin, you have to thin the blood, it’s like the petrol is too rich in the car and you have to thin it for the engine not to stutter. And in this situation, oh I was just trying to think of the number of cases I’ve seen like this, you really need to get the INR. Now the INR is the international ratio, so it’s like comparing full creamed milk with half cream milk I would say, and the INR should be around two in most individuals but in our disease it’s often over three when the headaches disappear, for instance in your case headaches are very good sign of the disease and whether you’re winning or not in your treatment
Henry: Fantastic, right I’m going to now try and go back to where I was in the questions, one thing we are doing is putting in questions obviously from the people posting live. Wendy has posted a question and she’s saying ‘Good morning from South Africa!’ so a global audience which is a wonderful thing, hello! She says thank you for doing these Q&A sessions they are hugely appreciated. ‘My mum has Hughes Syndrome and always seems to have a cerebral spinal fluid leak. Can this affect the APS in any way? She’s also getting severe leg muscle cramps and the only only thing that seems to help is salt’.
Graham: Well that makes sense there are some people where, for instance in your mother it doesn’t sound as if the antiphospholipid syndrome is the major cause, it’s an electrolyte imbalance and salt and cramps, or the lack of one goes with the other. So I don’t think it’s the same.
Henry: So the muscle cramps, don’t think related to APS, but what about the cerebral spinal fluid?
Graham: No I have not come across this I must say, the neurologic neurosurgeons may have had some cases but I haven’t come across it, it’s a hard one to imagine is linked because it’s not a clotting disorder unless there’s been a previous thrombosis in the spine. I’m sorry not to have an easy answer for that one actually.
Henry: No I know also obviously one of the challenges of a disease that is emerging is that level of surety, but you think it very unlikely. Suzanne has sent in a question, she was first diagnosed with APS in 2006, has been taking anticoagulation medication ever since, then three months ago was diagnosed with a non-alcoholic fatty liver. is there any evidence that Hughes Syndrome could have caused this or could it be caused by any specific medications? I think she’s also asking could it be Hughes or could it be the medication?
Graham: Hughes Syndrome certainly has been associated with this in some patients. Of course it can be associated with medication and we’d have to get a clear history together with your doctor for that. Yes the liver is affected sometimes by a poor circulation by sticky blood, sometimes it’s a clot, a patient gets a clot in the liver which sometimes needs even surgery, in other patients the clot is bypassed as it happens in the legs and you get big veins sticking out all over your tummy which is bypassing the liver. So yes it’s now becoming known that this can happen in the liver and the way that the liver starts becoming sick is for fatty change and seen in the liver, so yes, but again don’t forget the drugs, the medications.
Henry: And the medications in your experience will help the Hughes Syndrome which can help reduce the potential for this kind of consequence, but the medication itself?
Graham: Medication itself yes, I was also thinking the opposite where you know there’s a list as long as your arm of drugs that can cause liver function abnormalities, aspirin you know it’s as common as that sometimes.
Henry: Right so Mandy sent in a question: ‘I’m diagnosed with APS a year ago after a small DVT in my calf and I’m currently on 20mgs of rivaroxaban. Numerous APS sites suggest that I should be on warfarin – what’s your opinion?
Graham: Warfarin traditionally has been the main drug for this because it’s tried and tested and although the media hates warfarin, you know they call it rat poison and things
Henry: That’s because it actually is in rat poison right
Graham: Well that’s the reason it works in poor old rats because you give them a massive dose and they eat it and they bleed. So sorry I forgot what the question was talking about right
Henry: But most things in huge quantities are very very bad, too dangerous, caffeine for example, alcohol etc. Anyway she’s asking why would someone be put on rivaroxaban rather than warfarin?
Graham: The answer is quite simple. Over the years we’ve become frustrated by warfarin and the problems of patients who you know can’t take it properly and there’s been fierce competition in the medical pharmaceutical world for new non-warfarin oral anticoagulants and there’s half a dozen around and we were very excited and so about the possibility of changing to these several years ago, there’s very little in the way of published data, there’s a recent big trial from London showing that it probably is not as great a success as warfarin but time will tell on that. So it’s something we’re all hoping for desperately and if in your case you’re doing well and there’s a lack of symptoms or headaches, stick with it! But the data has shown that in severe cases with for instance recurrent mini strokes there have been more relapses on these new drugs than on warfarin so the jury is out on this at the moment.
Henry: And I imagine one of the sentiments to Mandy asking that question is you don’t want to find out your medication is not working by having a stroke, so how can she be confident, what does good feel like and how can she feel confident that she’s on the right medication?
Graham: The patient’s history is very helpful here, very helpful, and if that patient’s for instance gets migraines, which is the 2nd most common feature of Hughes Syndrome, and those attacks start getting more then it makes sense that you’ve got to act as a doctor and treat slightly more aggressively either up the warfarin or the other tablets. Warfarin is easier because you have a measurement with your eye and arm. So her symptoms are worrying and therefore she should be seeing her own doctor or physician in the hospital to monitor.
Henry: Okay so she should look at her more minor symptoms, though a migraine does not feel minor, so more temporary symptoms and see whether they are rising significantly.
Graham: Yes and I said that migraine is the 2nd most common feature and you know the first commonest feature of Hughes syndrome is memory loss and again the brain not liking the lack of oxygen, one of the things that is affected is memory and patients think ‘oh my god I’m the joke of the family I can’t remember names’ and if that suddenly changes or is worsening it’s almost certainly due to ischemia from the sticky blood.
Henry: Great I think that’s very insightful and interesting and important for people to know those alarm signals. Lynn has asked: ‘I’m aware that some medications can cause APS. Do you have any insight into what percentage of APS is caused by Dilantin specifically and how can one pinpoint Dilantin as the cause of APS?’
Graham: Luckily it does seem to be low percentage. There are a few reports of certain medications causing an increase in blood stickiness. I get to see a lot of the reports with my role as the editor of the Lupus Journal and I think the answer is, it’s rare you stop the drug if it’s felt that this is the case. Dilantin luckily is not so widely used anymore but in another disease related to Hughes Syndrome called Lupus there are a small number of drugs that can cause a Lupus-like rash and symptoms as well stopping, the drug stops the disease.
Henry: We’ve had a number of questions, as we do every time, around sort of very specific things which I think is quite useful for people as they look at their own diagnostics. So Lauren has written in, she said: ‘Do the following combined results signal a need for an anticoagulant?
– Positive LAC
– High levels of factor Xa
– High levels Antithrombin iii
– High levels of Protein C activity due to increased consumption’
Graham: Yes they do, they’re very helpful to us and having a full house as it were of those makes once suspect that this is the correct diagnosis for the anti-phospholipid antibodies which is the APL, the true antibody, there are currently three blood tests that the doctor does. You don’t have to bother about them yourself because the lab nowadays should be testing a blood sample with all three and they are an ACL anticardiolipin, Lupus anticoagulant, which is a terrible name because it’s not Lupus but historically it was called that, and the third one is anti-beta2. Now those three sound a bit confusing and I wouldn’t be involved, but the scientists testing these show that in those cases of antibodies if you have all three positive you’re at higher chance of clotting than only two positive or one.
Henry: So in this case, and obviously just to be very clear this is not a medical consultation, this is a trying to provide information and you must talk to your individual consultant. Disclaimer aside, but an increased number of those three particularly if you have all three high then that suggests highly yes. Right we’ve had a couple of questions sent in which we’re going to get to and then that’ll be the end of the medication and treatment section and then we have a question about diagnosis and care. Ali sent in a question on Facebook: ‘Have there been any recent changes or developments in the way MS type symptoms are treated? For the last 14 years I’ve been treated with anticoagulants for a wide range of symptoms but now I’m being told I have obstetric APS and should no longer be treated with anticoagulants. What is the reason for this change?
Graham: I don’t know the reason for that I really don’t. I mean a person who has the features that you have, it’s important to get their diagnosis correct, perhaps I can go back to your question but you say you have MS type symptoms at the moment, I wasn’t sure to be honest.
Henry: She has said that, and what’s she saying when she said she’s got MS type symptoms?
Graham: One of the parts of the body that’s affected by sticky blood is the spinal cord, same as the brain, and if it affects the spinal cord you get loss of sensation in various parts of the body, you’ll get a lot of MS like features, double vision for instance, speech disturbance, balance problems, pins and needles in the arms and legs, they’re all features that MS causes as well but quite a lot of other things do too. So the great excitement was 20 years ago when we found that some of our so-called MS patients were not MS but were Hughes Syndrome opened up a huge interest in research, the story has not been as black and white as one would hope actually, but certainly there are a small number of patients, we have a number of them whose first label was MS but it turned out it was actually Hughes Syndrome. And one of the most remarkable patients, I’m not going to name names obviously, but she was in a wheelchair shaking so badly that she was thrown out of the wheelchair on one occasion and was labelled as MS and in fact nature took a hand, she got a clot I think in the leg and bit by bit the penny dropped – she had Hughes syndrome and all her symptoms have gone on warfarin, she’s on lifelong warfarin, goes to dances, does everything.
Henry: Ali has said that she’s got obstetric APS, what does that mean?
Graham: I think it’s dangerous. Yes there are some doctors in my world of research into APS who who have suggested calling it obstetric APS because it causes the miscarriages etc but the patient doesn’t have any other problem, but sadly we have seen patients like this as I mentioned earlier who go on to get other problems like DVT or whatever so I think it’s a dangerous label to think that it’s a separate disease.
Henry: What would your view be on no longer having anticoagulants?
Graham: I would be worried about stopping I really would, as you say this is not a medical consultation
Henry: This is not a medical consultation but I think your point of passion is that the Graham Hughes Syndrome is usually helped by anticoagulants and that’s an important thing to consider. Okay great now we’ve got two questions sent in, one from Anne who I understand is very keen to ask this question. She first of all says hello, she says you’ve met and she’s been a patient to yours in the past, and as a reminder this is not a medical consultation, we’re not meeting with anyone and we’re trying to provide sort of wider advice which then can be used for other people. So often your question, although you’re asking it, there’s many people who want to know the answer and that’s the point, we’ll also be transcribing them onto the website to provide a sort of more detailed Q&A for people who want to learn about it that way and there’s a good point of which I can say that obviously all of this is only possible through your donations and there is a donation button on the website and anything you can give is incredibly important. We receive no government funds and that is absolutely essential, as you know it’s very hard for all charities so do please consider donating and thank you. And now Anne who’s been waiting patiently: ‘I’m being treated with anti-epileptic meds for seizures. I have lupus, APS and Sjogrens. I am taking aspirin as a blood thinner, I’m worried that no one is looking at the blood clotting side of this. Should anti-epileptic medication be considered or is the risk of blood clots too high?’ So I think there’s an interesting question about anti-epileptic drugs and the interaction with anti-clotting.
Graham: Yes yes very important because we now know that seizures are included as a major feature in some patients with Hughes Syndrome. In fact there’s one study that showed that the same figure, one in five of teenage epileptics is associated with sticky blood, treatable, so it’s a big big story. Now in Anne’s case clearly they’re linked and the fact that she’s getting a lot more seizures possibly means you ought to be looking more strongly at the level of anticoagulation or whether you’re getting any at all significantly. So back to the clinic I think in your case. I’ll tell you a very interesting story – an American lady who came to live in london was referred to me because she had Lupus and my colleague in the States asked her to see me and she had Lupus, which was mild in her case, but her major problem was epilepsy so severe that she was on I think seven anti-epileptic drugs and fate took a hand. She within a week of coming to London got a deep vein thrombosis in her leg because, a very long story short, she was started on warfarin, is now on warfarin. Frequency of epilepsy plummeted and she’s now on one anti-epileptic drug alone. So here’s a lady who, yes she had Lupus, but her main problem was seizures and those effectively stopped when the blood was made thinner.
Henry: Wow, and anti-epileptic drugs; can they cause coagulation or or are these two separate things?
Graham: On the whole they’re separate things
Henry: So the epilepsy could be caused by Hughes Syndrome and I assume there’s also a group of people who have epilepsy for another reason and Graham Hughes Syndrome
Graham: Maybe, yes
Henry: So I hope Anne that’s helpe, it does sound like she should talk to her conversation and refer to this conversation. Jenny has sent in a question: ‘I have very high test results in all three antibodies…’, something we’ve covered already today, ‘…first has tested positive 20 years ago. I’ve had a DVT, recently a stroke while trying to have a baby. My heamatologist wants to transition me from clexane back onto warfarin + baby aspirin. He wants me to eat broccoli every day because he said it helps to balance the INR. Is this a common procedure?’
Graham: So I gather that she was on heparin, now has the pregnancy and they’re talking after delivery of going back into warfarin?
Henry: I think she’s actually currently on clexane
Graham: Which is heparin
Henry: And is considering going back to warfarin, maybe aspirin
Graham: Okay well often following the pregnancy in what we call the Lupus Pregnancy Clinic where we see patients with Hughes Syndrome, once the pregnancy is successful and over then you’d certainly come back off heparin because heparin is a great drug but it’s an injection, you’d only be, if you can, on injections daily for years and years. Maybe I’d like to come back to heparin on that one actually if I may but in her case she should be considered for, depending how bad her history has been in terms of clotting, to go back onto something like warfarin if she’s still getting problems. Did you say she’d had a recent stroke?
Henry: Yes whilst trying to have a baby yes
Graham: That puts her in a in a very strong situation to be considered for anticoagulation, who wants a stroke.
Henry: She’s got a very good question coming up but you want to talk about heparin? And I’ve now become very interested in broccoli!
Graham: Oh broccoli! Well that kind of brings us back to warfarin actually because one of the problems with warfarin is that it interacts with certain foods, and green vegetables especially alter the metabolism of warfarin in the body. So your average person who does one dose Monday to Friday and at the weekend changes their diet a lot, the INR can be knocked out of control.
Henry: In what direction?
Graham: Both directions but it makes the blood thinner I think I’m just going back to that, but some people do that, they take broccoli daily or other things daily. The main problem with warfarin is if you’re an average bloke for instance you know who goes out for a pint or two at the weekend, that alters it, so many things alter the INR, so most of my patients on warfarin tend to know what affects them such as greens, and as long as you’re eating the same amount of broccoli or whatever then there’s no problem, but if it changes that then your dose of warfarin changes. Sounds very technical doesn’t it really, but more nuisance value than anything else.
Henry: And why do you think it would be suggested to eat broccoli every day, would you like to perhaps stabilize it?
Graham: Stabilize it yes, maybe keep you on a lower dose of warfarin you know to stabilize it, but I would just take the right dose of warfarin myself.
Henry: Right I’m going to straighten my tie slightly because this question is clearly going to get clipped for the website to become an important part of it. Right this is Jenny’s second part of her question: ‘What are the medical recommendations for someone like me who is prone to clotting and wanting to get pregnant?’
Graham: You can go ahead and have another pregnancy. The main thing is in the assessment that you’re then seeing someone, probably as you have been, who is an expert in Hughes Syndrome pregnancy, and more and more obstetric clinics now do have special interest in this side of obstetrics. You know the risks, she should be on something for the next pregnancy and that something nowadays is usually a combination of aspirin and heparin and the third thing, which is obvious but it’s so important, is to get yourself under an obstetric team who have links with people, if not them, who are interested in the antiphospholipid syndrome. So I hope that answers your question.
Henry: I think it does, I think that’s a very interesting, important point that you should have a specialist team or person there to talk to who has a particular interest. We’re moving on to some other questions on diagnostics and care then we’ve had a few other questions. Susan asks ‘I think I may have Hughes Syndrome. I’ve so far been diagnosed with sarcoidosis…’, I momentarily stopped because as you know I’m involved with SarcoidosisUK and was myself diagnosed with sarcoidosis a while back. Anyway Susan I’m sorry I’ll focus on you this is not about me! ‘…I think I may have Hughes Syndrome. So far I’ve been diagnosed with SLE, Sarcoidosis and Sjogren’s Syndrome, whilst repeat blood tests show anticardiolipin and Lupus anticoagulant positivity. I have also had 2 miscarriages and suffer frequent ocular migraines, though no history of DVT/pulmonary embolism. Does any of this look like it could be APS?’
Graham: Yes yes yes no doubt about it. Take Lupus and Sjogren’s Syndrome, both very similar. Sjogren’s is in a way like a milder, slightly older aged Lupus but still has the same potential with antibodies and things to cause medical problems, but the same old figure in Lupus is one in five Lupus patients have Hughes Syndrome features, I don’t know why this figure keeps coming up, but you certainly sound as if you have the features of the antiphospholipid syndrome and clearly you should be tested by people who are experts. I should say that there are three tests which we normally use, but for those who are negative to all three, there are rare patients who have got the syndrome when the tests are negative and I think in this case your doctor has to use a clinical judgment. We call this serial negative APS if the tests are negative, very difficult of course because it’s against the odds but it does occur. Sometimes we ask the patient to take aspirin on a trial and error basis, in some way use two weeks of heparin and I’ll come back to that as you mentioned and treatment.
Henry: Okay, we need to say thank you to Suzanne who said ‘thank you for asking Professor Hughes my question…’, I’m glad I’m getting credit it’s really you, ‘…and to thank him I’m making a donation to the charity’, thank you, thank you very much. We can do nothing without you and donations like that so thank you. Charlotte asked the question: ‘Could stress make APS worse?’
Graham: Yes it can do actually but then that’s a very general thing with most diseases. Yes we’ve seen the features coming out in someone going through unexpected stress, how to measure that we don’t really know but the answer is yes.
Graham: And then Alexandra has asked a question. So she had a stem cell transplant last year for Lupus which has made it manageable, let’s start on that, stem cells.
Graham: It started 20 or 30 years ago and been found in some cases of Lupus to help, it’s obviously an expensive, extreme solution and the data has not been totally great in Lupus, some patients have done very well and some not. But to put it in context I think we’ve had 4000 Lupus patients on our books when I was at St Thomas’s and I think we only had one patient who’d had this treatment. So I’m interested in your question.
Henry: ‘Which has made it manageable and lots of symptoms have improved, but the APS has got a lot worse, two new DVT’s on anticoagulants. If APS is autoimmune why did I not get any improvement from the transplant like I did with Lupus?’
Graham: Well you floored me. Very important observations but it may be that stem cell is not the treatment for Hughes Syndrome and judging by your single case it’s difficult. Stem cell has been more effective in certain diseases than others that’s all I can really say on that, but the fact now that you’re getting so severe case of Hughes Syndrome means that you need to be reassessed and hopefully you are seeing somebody or a specialist in Lupus or Hughes Syndrome.
Henry: Ah Alexandra you’ve successfully floored him
Graham: It’s so difficult, but if you look at Lupus patients say the one in five who have Hughes Syndrome. The Hughes Syndrome can be the more severe side of the illness and like my lady from America, whose seizures were her major problem, so and in Lupus patients you know some syndromes called like miscarriage or like, just trying to think of another one, they can be more due to APS in Lupus than any Lupus itself aspects. We once did a study in our Lupus clinic at St Thomas’s and found that Lupus patients who didn’t have the antibody had normal pregnancy history.
Henry: Thank you for that. We’ve had a question from Bernadette: ‘Last week my INR, was 2.8. I do home testing. Prior to testing I had a severe migraine and speech problems. My range was changed to 3.0 this year due to a bleed in my stomach. I am finding it hard to keep a balance and having more migraines and memory problems, due to the tight range. I become symptomatic at 2.9. My range used to be 3.5 to 4 and my symptoms stayed stable. After having the bleed in my stomach, how long will I have to stay in the lower range?’ I think it’d be interesting just to hear your views on the range that people need to sit in.
Graham: Yes I mean it’s such an important question this one. It’s something that we see once a week in our clinic. Major problem with someone who’s had a bleed, what do you do. And it’s different in every case, in your case where there’s a bleed on the stomach, the first question were you on any other tablets such as aspirin that could do that or did it come straight out of the blue. What you say about the level of your INR is so so important and it’s very worrying that you’re getting headaches and so on. In many cases we will add in an anti ulcer medication, I mean adding another tablet but something to stop the acid forming in the stomach that causes ulcers. In others we will try having a holiday from warfarin for a while and trying one of the new anti-coagulants, but the same applies, they can cause thinning of the blood in a dangerously thin area and can cause bleeding. So I think it’s a fine tightrope that we don’t know what to do, we sometimes add in other medications, we’ve even used steroids in one or two patients, but it needs close close collaboration between you and your clinician here.
Henry: And I think we’re coming to the end because you’ve been very generous with your time but I’m sure we’re going to do another one. So any questions we’ve not got to we will keep back and we’ll look to ask them next time. We are also currently scheduling for the chairman of the charity, Arvind, who is also a consultant specialist, to talk. He will also specifically talk more about COVID and updates on that, so Jan we’ll come to your question on that one. Otherwise obviously the default answer is do always talk to your consultant, but he’s going to talk more around that. Thank you Conor for saying thank you, these things are very much appreciated. We’ve got one more question that we need to deal with, Suzanne Jones writes: ‘Someone has a very special birthday coming up next month…’, I believe you’re going to be 80!’
Graham: I’m not sure what age I am
Henry: ‘…My question is how are you going to reduce the huge number of friends, colleagues and former patients who have become friends and your family to help you celebrate when you’re only allowed six!’
Graham: I’m going to hide!
Henry: Very sensible
Graham: Thanks Suzanne
Henry: It has been wonderful as ever, I learn so much through these I know they’re hugely valued, I know we’ve got a lot of people watching, if you’ve got any more questions do add them, we will store them up for for next time. Obviously this is the bit where I have to pitch for the charity again. You know we can do nothing without your donations, it’s incredibly important, you can hear some of what we’re trying to do in particular trying to look towards increasing awareness of the condition, helping people looking to fund research, providing quality information and support as you’re seeing today and so if you feel you can support us and donate that’s hugely appreciated. Then obviously my most important thing is to say thank you to you , thank you for what you’ve done but thank you particularly for this last hour answering all these questions. Thank you so much and I think that just leaves it for me to sign off. So thank you everyone for joining, thank you for your for your time, we will be doing more of these. I will schedule another one with Graham and announce that there’s going to be one with Arvind which I think people will really enjoy. We’re going to bring in more specialists and people. If there’s information you want on the website please let us know, otherwise please donate and thank you very much for joining us. Thank you everyone, thank you again to Graham and goodbye!