Q&A with Graham Hughes – 4 November

Charlene: Good morning everyone, my name is Charlene Pink, I’m joined today by Professor Graham Hughes who is going to be doing another Q&A for us. He’ll be answering all of your questions that you’ve been submitting online around APS, we’ve got some questions around COVID today that we’re going to be addressing and if you have got any more questions that you would like answered please do submit them during the live, we will be checking up on them and trying to answer as many as we can. So hello Graham thank you for joining us today!

Graham: It’s a pleasure

Charlene: Lovely to have you with us before the lockdown, I’m sure we’re all feeling a bit anxious about it but hopefully we can get some questions answered today. I will just say that any donations to GHIC would be greatly appreciated and it really does make the world of difference and to the charity and all the work that Graham has done so far, both in raising awareness of APS and of course as you all know he is the founder of GHIC and he is the one that discovered the syndrome, so as I said any donations would be extremely appreciated and you’ll be able to do that over on our website. Without further ado we’ll get through to the questions, we have got a lot of them have come in and as I said if you’ve got anything else you would like answered then do just let us know during the live stream. So the first question is from Jan, I believe she posted this last time actually when you were here previously for the Q&A and we said we’d get around to it this time, so it’s around COVID and I’m sure she is of the opinion as many others are that they’re concerned about COVID and the outcomes for APS patients is quite a worry for them. In your opinion should they currently be shielding?

Graham: I don’t think so, so far as we know there’s not been an increased chance of of getting ill from COVID if you have APS, the big worry for me anyway is that one of the features of the patients who are quite ill with COVID is thrombosis, so much so that most centres dealing with COVID emergencies give anticoagulants. So the obvious question for me and I’m sure for you is are those patients who are getting thrombosis patients without underlying clotting disturbance. I used to think there was no evidence for this but apparently there is a paper coming out soon in one of the medical journals that suggests a few of those patients getting clotting during COVID are positive for antiphospholipid antibodies. I’ll pass that back on to my colleague so when he comes around again, Arvind, he’s working on the COVID ward in his hospital, will know if that paper is out yet. To cut a long answer short I don’t think there’s any extra risk for people with antiphospholipid down from the general population.

Charlene: Thank you Graham, so yes as Graham mentioned we will be doing another Q&A with Arvind next month and he’s probably going to be answering a lot more questions around COVID that you might have. So we’ll be ready to welcome him then and kind of get an extended answer but definitely for the time being the government haven’t reintroduced shielding, obviously it’s still advised to be taking the precautions we all have been all along, making sure that we are washing hands and keeping our distance and doing everything that you can to stay safe, but at the moment it seems that there is no urgent need for additional shielding measures as such. So we’ve had another question around COVID come in, this is from Claire Harris on Facebook and she has said that ‘I have noticed the symptoms of long COVID seem to be similar to APS’. So I’m sure it’s quite rare but long COVID is for people who experience symptoms over a prolonged period, so for many people it will be quite short-lived they might recover within a few weeks but for some people they are experiencing symptoms possibly even up to six months after contracting the virus. So Claire asks ‘Does the professor have any thoughts on this, could treatment for long-COVID symptoms be the same as treatment for APS symptoms?

Graham: Well that’s a very good question I totally agree with the sentiments behind this question. Yes you’re right some of the features of long COVID are those that we see in the antiphospholipid syndrome and they relate to circulatory changes, the brain not getting enough oxygen, what we call Raynaud’s, the cold white fingers, those are all features of long COVID and yes it may well be that this, the mechanism, is rather similar. There has been lack of perfect circulation in some of the organs that have been affected, to my knowledge there’s no big positivity in antiphospholipid tests in long COVID patients it’s an obvious question to answer and I don’t think it has been shown that it’s antiphospholipid alone.

Charlene: Thank you, we’ve got another question around COVID, this is from Jillian Sinclair and she has said ‘I have other family members with other autoimmune diseases who have received letters from the NHS advising them to take vitamin D supplements. Should APS patients be doing the same?

Graham: I think vitamin D has become the the big star of medicine, it seems to be vital to protect against immunological problems. Certainly in the old days when we were looking at vitamin D and lupus it was almost universal for them to have low vitamin D levels and colleagues of mine working in vitamin D first of all take vitamin D themselves because they’re so convinced and secondly there is evidence that it’s very harmless, you really don’t overdose in most cases. Normal packages give you the right instructions but vitamin D does affect immunity so yes I think it’s one of the things you can be on the safe side with.

Charlene: Okay so it’s the case of not necessarily having to take them but there would be no real harm if someone did want to increase their their vitamin D intake at this time either via supplements or via high vitamin D foods and such?

Graham: Yes absolutely I mean you can get a vitamin D blood test if you really are keen but on the whole we don’t need that routinely.

Charlene: Okay great so I think that’s definitely something we’d all be considering even non-COVID times just for the winter season, making sure we’re all keeping our immune systems up to scratch and the best they can be. As I said we will be addressing some more COVID questions when Arvid comes in next month and if you do have any other questions around COVID do comment them on the live stream and we can get to them a bit later on. But for the moment we’ve got some questions around pregnancy, now I know Graham that this is something that you’ve spoken at quite depth about and you’ve said previously about the higher chances of miscarriage, so it’s something that I think you’re quite keen to talk around this area. We’ve had a question come in from Lucy and she firstly just says ‘Thank you so much to Professor Hughes for taking the time to do these Q&A’s it’s absolutely amazing that we’re able to reach out to him from around the world with questions our doctors are struggling to answer’. So yes we’re so grateful that you’re able to do these Q&A’s with us and it really does just make the world of difference I think for people who are struggling to find answers due to the rarity of APS and a lot of doctors maybe don’t know the disease as well as they should, so it’s great that we’re able to hold these Q&A’s. So her question was ‘In what circumstances should a woman with APS be advised to not become pregnant?

Graham: If she’s known to be APS, if she’s had the disease or symptoms or clotting problems and is positive on the tests she should be seen when the pregnancy test comes positive by physicians and obstetricians who know about this. We started a centre at St Thomas’s Hospital years ago looking at what we call them The Lupus Pregnancy Clinic but that was mainly lupus patients but of course it rapidly became overtaken by Hughes Syndrome so they should definitely seek advice from doctors, obstetricians and physicians who specialise in this. What should they do, certainly the old days they should not be advised against pregnancy if that’s what they want. I think there’s every reason to be very firm on that unless there’s some structural or other unrelated problem. So what happens the the young lady gets seen in the antenatal clinic or the advice clinic by a midwife or a doctor and it is tested – now it’s until recently not all hospitals even tested for APL which is really amazing to me because it’s been going now for, was it 34-35 years, that is changing I think. One of my jobs is as editor of the Lupus Journal and we’re getting more and more papers on research in Hughes Syndrome which is encouraging. So they get tested, if the test is positive and they have not had an abnormal pregnancy history many doctors advise just carrying on. Normally I’m a little bit proactive myself, I think unless there’s an obvious indication against then aspirin, low dose aspirin, 75 milligrams daily has been shown to help, not definitively but statistically the number of successful pregnancies. I know that patients often are not tested until they’ve had three or more failed pregnancies and this is one of the big tragedies of APS, you know if your own wife or daughter was pregnant and there’s a hint of anything to do with Hughes Syndrome such as migraine you’d want to be tested, why have three pregnancy miscarriages? So I’ve written down, I don’t know how many people know this, but I think there should be a standard questionnaire by the midwife or doctor on the first visit and that question is number one, ‘Have you had previous miscarriage?’, number two is ‘Do you suffer from migraines?’ and the third one is ‘Do you have a family history of autoimmune disease?’ and that includes rheumatoid, lupus, multiple sclerosis and particularly thyroid, they run in families. So those three questions will help push a doctor on towards testing for antiphospholipid syndrome.

Charlene: Okay great so we’ve also just had another further comment from Lucy who’s watching us live, hello Lucy thank you for joining. She’s just said that she’s been told that women are not tested whilst pregnant, so this is not before they become pregnant it’s whilst they are pregnant, as this can affect the result. Do you know anything about that, whether there’s truth in that?

Graham: I don’t think so, there are odd cases but that’s wrong.

Charlene: Okay thank you for clarifying. So we’ve also had another question around pregnancy, it’s clearly a topic people are keen to know more about. Jenny says ‘Is it possible for APS to only be present during pregnancy? Lots of specialists will not test for antibodies during pregnancy or shortly after miscarriage so I’m worried in case I have undiagnosed APS.’ In your opinion should she push further then to get tested even though some specialists are refusing to do so during pregnancy?

Graham: Yes I think so, what I’ve given to the charity is a list of symptoms, I think there are 40 on the list and if you add up to 10 of those or more as a suspicion of Hughes Syndrome, I know that it’s rather difficult to put these lists on paper and every patient is different but you could test at any time including pregnancy.

Charlene: Okay great thanks for clarifying. So as Graham said he did give us that big list of symptoms which you can find on our website and there is a scoring technique to it, so each different symptom has a different number of points allocated to it based on their importance and relevance in APS and you can actually go onto our website, the GHIC website and do a checklist yourself and see which symptoms you do match. It’s by no means a full comprehensive list and you should always speak to your individual consultant or doctor, whoever you’re dealing with, to get a more official diagnosis but this will just give you a rough idea if your symptoms do match up or if you score highly on the point system. So for example 10 out of 50 on the point system is classed as possible suspicion of APS whereas if you scored higher up in the 20s and 30s there’d be a strong suspicion is that correct?

Graham: Yes it is, I’d like to play down in some ways the importance of this list. You know the patients out there that I haven’t seen could go to a list like this and say oh gosh I’ve got some awful disease and I was in two minds about putting it out there because I think there are patients very much on their own who need help and people can throw this back at me and say it’s not very sensitive or specific but we’ll see.

Charlene: Okay so yes those are our questions on pregnancy and we will have some more information upcoming on the website and things to do keep an eye on around that. This is also a good time for me to just say that this video will be put onto our website as well, so if you’ve not been able to watch the whole thing live or if you’re not able to still tune in for the rest of it, it is available on the website and we’ll also have the transcript as well as all the previous Q&A’s that Graham has so kindly done for us, they are available on the website for you to re-watch.

Graham: I should say that the history of pregnancy and antiphospholipid syndrome has been staggering. Before patients were tested for this syndrome the success rate and pregnancy in patients with antiphospholipid was less than 20%, it’s now over 90% in most big centres so that’s totally revolutionized looking at early pregnancy.

Charlene: Would you be able to talk a bit more just around the research that has been done thus far, you said there still needs to be a lot more work done around the miscarriage and the pregnancy side of things yes.

Graham: The reason for the failed pregnancy was originally thought to be clotting in the womb, clotting of the blood vessels to the baby and the infant in the tummy doesn’t get the oxygen required and the patient miscarriages. That’s probably true mostly but there are probably other mechanisms as well, these antibodies can mess up other parts of the placenta and supply of blood to the baby so what’s been very striking in experience has been that the combination of heparin which is an injection and baby aspirin during the pregnancy months has been the best solution for those women who’ve had bad this medical history. There is another thing we haven’t talked about and that’s late miscarriage. Most miscarriages are at three months but late miscarriage, which an article once in The Times put down as the greatest tragedy known to man, which is losing the baby at eight months or thereabouts. We now know as you might expect that antiphospholipid syndrome is linked to stillbirth, not dramatically like early pregnancy loss but there is a link, it’s about twice the chance of still birth in patients not on anticoagulants and that’s another strong reason for testing early in the pregnancy, to find a woman who’s had half a dozen miscarriages and then later a stillbirth is just medically not acceptable these days. Patients should be monitored for that, so that’s been another great thing for us anyway to see this success.

Charlene: We’ve had another question from Kathleen and this is around side effects and symptoms and she says: ‘My mother was told that she has had a silent stroke and the Professor previously diagnosed APS on symptoms as there is a very strong family history of APS. Her current doctors are reluctant to lay the blame at the feet of APS as she has had negative blood tests for APS and she also has lupus which is in remission. What advice does the Professor have in this situation?’

Graham: I must say the clues are there, the fact that she’s had lupus – one in five lupus patients have got features of APS, that’s the current teaching to med students – so a positive history would worry you about this being a clot rather than any other thing but as age increases there are other causes of stroke, obviously major causes, blood pressure, abnormal blood vessels in the brain and so on, but coming back to the history which is so important. The features the previous doctor diagnosed, okay the tests are negative but it’s something we’ll come back to a little bit later.

Charlene: And then just another question in terms of side effects and symptoms. Marianne firstly says ‘Greetings from Finland’ so it’s wonderful to know that we’ve got a global audience, everyone’s tuning in from around the world and Marianne said: ‘When my INR drops under 3 I get difficult memory problems, pins and needles and numbness in limbs, sometimes balance problems and headaches. Recently I was diagnosed with mild sleep apnea. Can it make these symptoms worse because of lack of oxygen?’

Graham: Yes very much so, it’s very interesting in the days since we’ve been using warfarin as an anticoagulant that the INR, some of you may not know this, but the INR is the ratio. Think of it like Tesco’s milk, ratio of one is normal milk, ratio of two is half condensed cream milk, ratio of three is skimmed milk. You need if you have brain problems with APS to and you’re needing warfarin to monitor carefully that the routine use is up to let’s say about an INR of three but a lot of patients and doctors even are scared of that. I think the dangers of bleeding on warfarin statistically have been well worked out and they are 4 or over so this lady clearly is getting problems when her INR is below 3. My normal advice if you can get it is to get your own INR finger prick testing machine and to collaborate with your clinic. Remember that the antiphospholipid syndrome is a much more clotting disorder than many of the other reasons for giving warfarin and it’s very common for us to see a patient who’s headaches and memory loss return when the INR is say 3.2 instead of 2.8. So it’s as fine as that and your patient in Finland is absolutely correct with this observation.

Charlene: Thank you and we’ve also had some questions around the medication side of things. So this first one comes from Dee and she says ‘Does APS and lifelong warfarin increase your risk of getting a blood cancer like polycythaemia?

Graham: The answer is no, blood cancer’s not linked to this disease as far as we have found out so far.

Charlene: Okay and then in terms of another question we’ve had come in from Mandy Harrison she says ‘I’ve been told I can’t be tested for lupus because I’m on rivaroxaban, is this correct?’

Graham: Wrong, test for lupus it’s a separate test. Anti-DNA it’s called.

Charlene: And the medication has no bearing it will not give a positive outcome or a negative outcome and the test won’t impact it. So we’ve had another question come in around medication and different treatments so this one from Mavis she says: ‘I’ve heard about a drug called diprodol, could this be used to treat APS?’ Have you heard of it?
Graham: Pass, I think it’s one of the family of anticoagulants and I have no experience with it. We get quite a lot of questions about the new oral anticoagulants and I can speak to those if you like in a minute.

Charlene: Yes it’d be great to get some more insight on that and see whether it could possibly be something or whether there’s no no use in that. So we had another one from around the world so Kelly says ‘Hello from Texas’ and she has actually made your acquaintance already, she says ‘You were kind enough to co-consult on my case along with Professor Jordan in 2016 and I’ve continued to clock through multiple anticoagulation regimes including a noxoprine warfarin. Now on rituximab, fondaparinux has been suggested as a possibility as APS patients seem to do well on it, especially those with neurological aspects like me. I failed Dr Jill Schofield’s Apixiban trial, documented by scanned DVT’s, migraines, seizures, and TIA symptoms. How is fondaparinux any different than Apixiban? Does it have an advantage?’

Graham: Not to my knowledge but I may be wrong on that one. Let’s discuss treatment briefly; there’s two drugs being mentioned here, particularly rituximab and the anti-clotting drugs. Rituximab is a fabulous drug it’s been around now for diseases like lupus where the immune system goes mad, goes overboard and rituximab is one of the new monoclonal antibodies, the new wonder biologics if you like that’s been found to be very useful in lupus and in other autoimmune diseases. For very severe cases of Hughes Syndrome where the clotting seems to go on despite anticoagulants, there has been trials of rituximab and we’ve used it in a small number of patients ourselves with mixed results. In some patients there’s been clear response, the immune system is calming down on rituximab but as yet it’s not the great drug that we’d hoped for. In the antiphospholipid syndrome concerning anti-clotting the main treatment is something that makes the blood thinner, first of all there was aspirin then came heparin and then warfarin. More recently we’ve had anti-clotting drugs which have been marketed and have been very exciting for us. Sadly I think the truth at the moment is that the overall results haven’t been as good as expected and the trials are going on, there’s a big trial here in London which showed that for people getting stroke and other major diseases they were not as effective in general as warfarin. So I’m afraid I’d still watch that space as far as these drugs are concerned.

Charlene: Yes it’s very interesting and as we’ve said before in previous Q&A’s things may well change in the future, it’s constantly something that we hope is going to be looked into more and it could always be a case of different circumstances in the future. Jenny asks ‘When should immunotherapy be considered to lower APS antibody levels?’

Graham: Yes that’s the question I was answering in the previous talk, you know high antibody levels means that there’s something wrong with your immune system and APS is one example of disease where antibodies mess things up. You’d logically think that immunosuppression would be the right treatment but as I said with rituximab the results have been mixed, it’s not premised but if you’re desperate, if the patient and her doctors are desperate, this is one line of approach which is being taken.

Charlene: Okay interesting and Sue has commented, thank you for your question Sue, she says ‘I was off warfarin and on a pixaban for a year and had some problems on it, I don’t know whether it might be related or a coincidence but symptoms include some new pain, increased facial tightening, light sensitivity, struggle with memory’. Lots of symptoms we’ve talked about before and one seizure episode as well she says on warfarin her target INR was 1.7 to 2.5, she requested that to be increased and it is now 2.5 to 3. Might these symptoms be helped with a higher INR do you think?

Graham: Yes definitely, you know assuming there are no other contraindications when a patient walks in the room and says I’m on warfarin but I’m still getting headaches, memory loss, balance problems, speech disturbance, all brain things then the first thing I do is can I see your little booklet which has your INR in it and then you read out 1.9, 2.8, 3.4, that patient is not getting treated properly. She’s picked up the fact that the symptoms of Hughes Syndrome are returning whatever the drug is but with warfarin you can monitor it and the INR in her case is not good enough.

Charlene: So Linda has asked ‘Can any genetic testing be done whilst on warfarin?’

Graham: I’m not aware that there have been genetic studies of APS to see if there’s a gene that figures as there are in lupus, I don’t think that any genetic helping has become routinely used, I think I would have heard about it. I hope I’m wrong because I hope that that will come in the future but it’s definitely a genetic disease in that there are family histories which are very strong. But in terms of strength of your genetics it’s not that strong.

Charlene: Well we’ll go onto our next topic and we’ve had some questions around diagnosis and testing. Marie has asked ‘After having a history of dialysis and having two kidney transplants I was diagnosed with APS this year after having a clot and a lupus flare’. Would you say that this is rare considering her medical history?

Graham: No I don’t think so, I think anyform of lupus can be associated with this. We don’t know in her case whether she was tested for APS early on, it’s a routine test now in any lupus clinic to have sticky blood, come to think of it many of the features of lupus may not be just lupus but APS, the miscarriages, the clot, the DVT, the memory loss, even I hate to say the stroke that some people get is more likely to be from sticky blood than lupus.

Charlene: Okay and then we’ve had a question again around testing and Sanjita has said ‘If someone has raised APTT with normal PT, and increased platelets, should they be tested for LA with other factors? Or could you suggest anything else?’ Especially since they’ve got occasional chest discomfort and shortness of breath. Graham: We don’t know how high the platelet count is do we?

Charlene: No we don’t

Graham: It sounds a different story the platelet counts in APS are normal or low, they can be very low occasionally that can cause bleeding which is a horrific combination. So it doesn’t sound like APS but certainly that combination is, hopefully a haematology colleague is looking after the patient. There are other clotting disorders I should say, there is one where you have very high platelets and they can cause clotting but that’s a different scenario.

Charlene: Okay thank you Sanjita for watching, she’s watching us live at the moment, she’s also just posted another question and said ‘Does DRVVT being higher than the last blood test indicate APS is worsening?’

Graham: Not to my knowledge. Let’s take the simple test for APS, the anticardiolipin or an antiphospholipid is the terrible word for an APL, some patients call it the apple test. APL it can go up and down and it does and then we see patients over the years, rarely but it does happen, the antibody disappears totally and that answers a number of questions for patients, there are patients who have the whole figure of the disease but the tests are negative and of course they have a very hard time with it because some people don’t believe that they’ve got Hughes Syndrome.

Charlene: I see, so Linda has commented online she is also watching us live and she asks ‘Have you seen that APS patients also have Addison’s?’

Graham: Yes we reported very early on a small number of patients with Addison’s, it’s very interesting there was a history, was it the 50th or 100th I don’t know, anniversary of Addison describing his disease. There was a conference in Guys on Addison’s disease and one of my team went along to that conference and put his hand up and said we’ve got a number of cases of antiphospholipid, presumably clotting in the adrenal gland that’s caused it, so the answer is yes.

Charlene: Okay that’s very interesting, but you wouldn’t say that’s something that’s particularly common?

Graham: Rare, it’s really quite rare

Charlene: Okay so we’ve got lots of people watching online thank you very much for tuning in as we’ve said previously this video will be available on the website if you do want to watch it back or haven’t been able to listen to the whole thing. Just another mention as well that GHIC really would appreciate any donations that are able to be made and is important to be able to continue the vital work that Graham started and that’s what we’re trying to continue here so any donations would be greatly appreciated and you can do so on the website. So we’ve had another question coming from Wendy who’s also watching us live, thank you Wendy. Wendy asks ‘Can liver disease negatively affect APS or can APS flare up liver disease?’

Graham: Yes the liver can be affected in APS no question about it. There are patients who’ve clotted in the liver and sometimes that’s acute, it’s like clotting in the kidney, so yes is the answer. Some patients have very mild liver involvement and have very slightly abnormal liver function tests and we’ve certainly seen that it’s not very big or common a story, but definitely is on our textbook list.

Charlene: Okay fantastic, we’ve also had Charlotte watching us live thank you for your question Charlotte and she says ‘Hello from Denmark!’ so another another country we can take off the list, Charlotte says I have Hughes and Factor II. Does Factor II increase my Hughes Symptoms?’

Graham: I don’t know the answer to that question but possibly one we can check back on that.

Charlene: Yes alright and then we’ve also had a question from Anna, also watching us live hello Anna thank you for joining, she says ‘With warfarin does the INR have to be at 3?’ As far as my understanding is, it’s different for each person isn’t it?

Graham: The warfarin dose varies depending on each individual circumstance yes you’re correct, if you go to a warfarin clinic, I mean I must say a lot of the patients attending a warfarin clinic are little older patients who had maybe a DVT or an arrhythmia of the heart that’s what warfarin is widely used for, but in Hughes Syndrome everyone is different, many patients feel fantastic when they start warfarin; headaches gone, balance issues gone, memory sharper. But often we find as I said in a previous answer that the the dose ought to be pushed up high and the doctor and the patient working together should not be afraid of that, a patient may have total improvement converting an INR from 3 to 3.2 it can be as fine-tuned as that, very important to know that.

Charlene: Thank you and we’ve had some more comments online so Tracy asks ‘Why does APS fall between rheumatology and haematology, does it sit somewhere best in between? Is there a particular reason for that?

Graham: All these things have more history than anything else, we were rheumatologists even I was more of a general medicine doctor than a rheumatologist. We ran lupus clinics and it was through lupus that we discovered APS so historically rheumatologists have all been interested. When we do collaborate with haematologists for instance at St Thomas’s we have a combined clinic.

Charlene: And we’ve just had a comment from Jen, hello Jen thank you for joining in. She just said sorry if I missed the question if it’s already been asked, she asked about INR levels and we did previously answer that question Jen and we said that INR is different for each person, there is no one set level it really depends on each person, Hughes Syndrome can impact people differently and thus your INR is going to be different from someone else and so it’s best to talk to your individual consultant on that.

Graham: There are patients who really tell you a lesson, I’ve got one lady, she’s been INR 4 for the last two, three, four years completely well with no symptoms. Previously getting regular seizures so that’s a high level but you know some patients do require, for reasons we don’t understand, higher INR.

Charlene: Okay thank you, we’ve had a question around symptoms and more side effects and so we’ve had someone say that they are suffering with migraines, movement disorder as well and she’s had several attacks this year and also sometimes talks gibberish through an attack. Would you say that’s quite common?

Graham: Definitely apparent in the syndrome yes

Charlene: You’ve previously spoken about migraines as well as being one of the most common?

Graham: Movement disorders, ticks, jerks, balance problems, all brain things and it’s the brain that seems to be most sensitive to a lack of oxygen. But the movement disorders quite interesting, I had a lady with severe tick going back many years with the arm jerking like this and gone now on anticoagulants.

Charlene: Oh okay was there a particular anticoagulant that she was on?

Graham: No it’s warfarin, if the brain is involved historically anyway we’ve tended to go for safety and although the media don’t like warfarin as I said the fact is it’s the one drug which the patient has control of their own dosage and body.

Charlene: So Linda has picked up on the different side effects and symptoms and said ‘Are headaches and kaleidoscope vision more common with APS or with lupus?’

Graham: APS more maybe and if you have a lupus with visual problems of that sort you really have to test for APS because the treatment’s different, lupus steroids etc different treatment

Charlene: Yes I think that’s very interesting we have also spoken about how some people are actually misdiagnosed at the start and they could actually be thought to have MS related symptoms or lupus.

Graham: Well it’s not surprising if you think of a patient with balanced disturbance, visual flashings, loss of vision in one eye, pins and needles in the arms and legs MS is likely to be high on the list of possibilities but they’re also high on the list of Hughes Syndrome and we certainly in my clinic have seen a small number of definite patients who were attending MS clinics who are now free of anticoagulants.

Charlene: Great, thank you everyone for tuning in again we’ve had a comment from Susan, so Susan has actually said ‘Can I have APS with sarcoidosis?’ So this is interesting actually, I believe we might have had a comment like this last time from someone because as you know Graham we’re involved with the SarcoidosisUK charity. Is it possible that you can have both?

Graham: I’ve not come across it, they’re both rare diseases one possibility and I’m just hypothesizing here is that sarcoid has many features in common with a disease called Sjogren’s which is a relation of lupus. So misdiagnosis does come into it as sarcoid patients will tell us that they’ve not been diagnosed, they’ve got big glands and so on. I’ve not in my whole career come across that combination.

Charlene: Okay thank you for yeah for explaining that, we’ve had also a comment coming from Wendy and thank you for tuning in Wendy, she has said that her mother’s vitamin B levels have shot up and she said it’s shot to 1107, she was between 107 and 443 the last time she asks if the increase in vitamin B could cause an APS flare-up or could it aggravate the APS in any way?

Graham: I’ve not come across this at all. We do measure if any neurological features are there of course and I don’t know of it being published as a study that it’s affecting. I think the answer is no.

Charlene: Okay thank you and Ali has asked as well, thank you for joining in Ali, she’s commented live and has said ‘Can you tell me if aches and pains in the joints and the muscles flare, is this caused by Hughes Syndrome or fibromyalgia?’ So it appears she suffers from both and is finding it hard to tell which one it’s coming from.

Graham: Well I’ll tell you my prejudice here, I think if a patient with Hughes Syndrome gets fibromyalgia like symptoms they should be investigated and there’s a danger as I believe anyway with fibromyalgia being a kind of bus stop – doctors don’t go any further than giving a label, what does it mean? It means you’ve got aches and pains really and you could well have associated mild lupus or mild surgeries, those are the two we see very commonly. So yes it’s a feature we see in the new patient coming up we’d test for dry eyes, so the patient have the tear impairment which is a feature of sugar allergies, rashes, all the features of what we call mild lupus which is often very responsive to queening of all things plaquemines hydroxychloroquine tonic you know like gin and tonic but a big bigger dose as a tablet. Very effective in the aches and pains situation that we see like I think this lady has.

Charlene: Okay Wendy also, just to follow on to her previous question, asked ‘Do low levels of hemotricht red cell count and MCV aggravate APS, is there any treatment for that?’

Graham: Yes so low levels hemoglobin is a straight anemia but other levels did you say the red cell count?

Charlene: Yes red cell count and MCV

Graham: MCV is the size of the red cell.

Charlene: So one is the count and one is the size

Graham: There are things that make the red cells go bigger they need investigating. A normal red cell, say seven, that won’t go into actual measurements, if it goes up 10 or 11 there are things that cause it. One is allergy to certain drugs so you need to investigate that and again that could be different per person, obviously everyone could be affected differently if people have different allergens and so on. Med students have to learn a dozen or fifteen causes of raised MCB’s, it’s an important observation.

Charlene: Definitely. Another question from Sanjita she has asked ‘Can general infections flare up APS?’

Graham: It can be disastrous. It’s one of the things that we’ve seen the patient’s fairly stable for a number of years on anticoagulants, goes down with a bad infection and everything out of kilter. There’s a very rare condition called catastrophic APS which you may have heard about which is a good description of it where you blood clot in different organs of the body, intensive care treatment and some can be fatal and that’s thought to follow severe infection. This was something we thought we might see with the COVID pandemic but I don’t think it’s quite come out that way.

Charlene: A lot of people are commenting live as well that might have missed the start of the video, asking if they are more susceptible to COVID but our understanding at the moment is they might not be more susceptible to it but if they were to catch it then the side effects from it that they might be impacted in a different way or in a more severe way to someone who didn’t have the syndrome.

Graham: That’s right so yes you’re not at increased risk from catching COVID, more from if you were to catch it then you’ve got a clotting danger then it would be an increased risk.

Charlene: Yes so I think that’s an important one to note, I know obviously a lot of people as I said have been concerned about COVID and as we said previously in the video if you missed the start we’re just suggesting that you take your precautions that you were, shielding hasn’t been recommenced it’s just obviously the national lockdown as of tomorrow but making sure that everyone’s keeping themselves safe, but you’re not necessarily at an increased risk. We’ve had some more people comment from around the world, we’ve got people tuning in from Switzerland and other parts of Europe, from Australia as well, hello to everyone thank you so much for tuning in it’s greatly appreciated. Jenny is from Australia and says ‘Thank you so much for the opportunity to ask Professor Hughes a question. Can APS cause positive and antinuclear antibodies?’

Graham: It doesn’t cause antinuclear antibodies that’s other autoimmune diseases but as we said in the last question there’s a risk of association with other autoimmune diseases like thyroid, Sjogren’s lupus and it’s quite common for us to have APL patients positive with a positive antinuclear. A lot depends on how strong that level of antibody is, if it’s one in 40 forget it, but if it’s one in 200 well maybe that patient’s got aches and pains and a few other overlapped features of Sjogren’s.

Charlene: Ali has said thank you for answering my question, so we answered the previous one of hers about the aches and pains and just following up on that she’s commented live and said that her doctors took her off Plaquenil about a year ago. Regarding the pain could it be worth going back on to it do you think?

Graham: Difficult to answer a specific question like that I’d be in big trouble if I did

Charlene: Of course, I’ll take this moment to just say we do this as obviously a kind of rough guide and we use it more to give general advice, obviously if there are very specific questions you do need to speak to your individual consultant or doctor.

Graham: Yes well Plaquenil is a fabulous medicine, it’s got a high degree of safety and the dose is, around the world I think, the dose is 200 mgs or milligrams and that is now standard in lupus but it’s very effective for the mild symptoms, the aches and pains and fatigue. Higher doses can cause retinal damage in the eye and that’s a big scare but the doses we advocate which is one tablet a day or even less, three tablets a week often over a period of time start getting benefits for fatigue and aches and pains very useful to think about. And yes maybe it’s a wise idea for her doctor to think about or her to think about going back on Plaquenil. Slow to act, takes a couple of months to build up but it’s worth the wait.

Charlene: Is that the same case with other medications as well that some can be slow to act, are there others that you’d see immediate effects?

Graham: Yes huge variation, heparin immediate and that’s why we do two weeks heparin test and many of our patients for diagnosis see if their memory loss goes away. But with queenie or Plaquenil it’s two months or so before you get a reality so it’s kind of a more prolonged thing to see the effects in that. One of the nicest things I’ve found in clinical practice is a patient who started on one a day Plaquenil or even a patient who’s worried about it to start on one a week, you know you don’t grow horns you feel better, you start taking two a week and when that patient comes back in three months it’s so often the case, ‘how are you doing?’ – ‘fantastic, my fatigue is much much better’.

Charlene: It’s definitely great when people find the right medication that works for them and alleviate the symptoms. We’ve had a question come in from Ruth, she says ‘How does Factor II Leiden relate to APS?’ Should she be tested for APS?

Graham: No they’re separate causes of clotting

Charlene: So just for my own understanding and people watching if some people aren’t aware, what exactly is Factor II Leiden and Factor V Leiden?

Graham: I think it was discovered in Leiden and it’s one of the clotting proteins. Clotting is very complicated and Factor V Leiden is one of the processes and it’s been found that if you have abnormal levels of artefact of anti-factor Factor V Leiden there is a danger of thrombosis. But it’s a totally different disease from Hughes Syndrome as a cause of thrombosis. For instance Hughes Syndrome can cause artery clotting which is devastating whereas Factor V tends not to.

Charlene: Okay so that’s the main difference, so in Ruth’s case you do recommend that she should be tested for APS just to act on the side of caution or is there not a need?

Graham: It’s not needing to, it depends on the history. If she’s had multiple classic things then there’s a standard clotting screen APS and Factor V should be part of that screen.

Charlene: Okay and I believe we’ve had a similar question to this one coming before, this is from Gemma Parks thank you Gemma for commenting, and she asks ‘What percentage of APS patients would then go on to develop lupus?’. She said that she’s had APS for eight years, it’s been treated with warfarin but she also has some clinical features of lupus and she is described as lupus by her consultant. I’m not sure that we know the percentage exactly? Graham: I should say it’s very rare for primary APS to move into lupus.

Charlene: Is it more common the other way around for lupus to move?

Graham: No and that’s one of the things that should be known by patients that if they come along at the age of 18 or whatever it is and test positive but no evidence of lupus, in my experience of what 35 years of APS it does not move into lupus. For the woman who’s had multiple miscarriages, lupus tests are negative at 18 or 20 they tend to remain negative. What you’re talking about or your patient is talking about is aches and pains, mild lupus possibly right from the start but APS is her main feature.

Charlene: Brilliant so we’ve got about 10 minutes left with the questions and I think we’ve tried to answer as many as we can, as I previously said if you do have any questions that we haven’t managed to get round to this time we will be doing another Q&A next month with Arvind, we’re very lucky to be joined by him next month and we’ll try address more but we’ll squeeze in the last few now. So Sanjita has asked another question and has said ‘Can hydroxychloroquin reduce ESR and CRP?’

Graham: Yes it can reduce, it’s got a number of effects which makes it an interesting drug from our point of view but one of them is anti-inflammatory and that’s where the aches and pains come in, that improves.

Charlene: So we’ve had quite a few questions today around medication and things and this one is that a lady’s APS is being treated with aspirin currently, she also says possible lupas-like symptoms should she try prednisone or hydroxychloroquine first, which one would you recommend as being the first port of call for the aches and pains?

Graham: Is that her just for APS?

Charlene: She said she’s currently being treated with aspirin but I’m assuming she’s having some increased symptoms so out of the two is there a preference for which one to go to first?

Graham: It depends what she’s got, I’m sorry to be on the fence on this one but there’s no question that hydroxychloroquine first, steroids last. If it’s mild and if a patient comes in with SOS then you may go for strong drugs first.

Charlene: Jen has commented saying that she has APS and lupus, hashimoto’s, lesions on the brain and absent seizures currently controlled with keppra. She says I have hearing loss and tinnitus, she does have hearing aids, is this a symptom of APS or lupus?

Graham: APS more likely, all those features are seen in APS and it’s very serious and presumably she’s under a clinic.

Charlene: Funny you should say that, she does go on to say that she’s currently seeing a neurologist and a rheumatologist, she said she can’t find a GP who understands APS which I think a lot of people come into the same type of quarrel don’t, she asks should she involve a haematologist or an immunologist as well?

Graham: Yes it depends on how much joy she’s getting from her current physicians but she’s seriously ill with the brain getting attacked by poor circulation, therefore yes.

Charlene: As we’ve said it’s something quite common that people struggle to find someone who understands APS and the effects that it can have. I think that’s why this charity is here really, to educate as best we can the colleagues and patients and relatives and so on. So as I said before any donations would be extremely appreciated, it would mean we can keep continuing to do our work, raising awareness, spreading the news. Our main aim is of course to make sure that we can eventually find a cure, that there’s more treatment available and an increased understanding for people who are suffering at the moment. So it really is of the utmost importance and if we are able to get any donations that would be greatly appreciated. Right we’re down to our final minutes, I will just sneak in a couple more questions before we’ll have to end the live Q&A but thank you everyone for joining once again. We’ve had a question from Charlotte who says ‘Are steroids not good for Hughes?’ She is currently being treated with steroid cream, she has psoriasis.

Graham: Yes steroid creams are widely used for psoriasis which is different from APS itself for clotting disorder conventionally does not need steroids

Charlene: Okay so not for that one thank you and Marion says ‘What is recommended for facial rash associated with APS? It can be worse sometimes with small raised bumps mostly over the cheeks and nose’.

Graham: We would tend to look at lesions in the skin obviously we get a haematologist involved but our friend Plaquenil is very useful for skin manifestations of autoimmune disease that would be one thought for me.

Charlene: Well we’re going to have to conclude the video here, we’ve had some final comments from people it seems everyone’s absolutely loved the video today Graham so thank you so much for coming in. We’ve had a comment from Jenny saying ‘This is absolutely amazing thank you so much Professor Hughes’, Mavis says ‘Thank you Professor, I’ve learned a lot today’ and so everyone is extremely grateful for your time, we really appreciate you coming in obviously the day before lockdown it’s great that we were able to do this one more time in person, though socially distanced, and as I said we will be doing another Q&A next month to end the year off so we’ll be answering any questions we didn’t get to this time. But I can only say our greatest thanks for coming in today Graham, we really do appreciate it and it would be great if everyone can go to go to the GHIC website, donate if you can to allow us to keep doing more videos like these. Thank you so much Graham, I’ll leave it at that and thank you everyone for watching!