Session 2 – Treatment For Sarcoidosis – The Royal Brompton and Harefield Hospitals and SarcoidosisUK Sarcoidosis Patient Day

Transcript 

Vasilis: It gives me great pleasure to introduce the next chair as well as the speakers. Dr Elizabeth Renzoni is one of our senior consultants being part of our group since 2006. She is very well known for her excellent clinical skills as well as relationships with most of our patients. I’ve learned a lot from her over the years , thank you very much, Elizabeth.

Elizabeth: Thank you, Vasilis, for that very kind introduction, and I just wanted to join the others in complimenting you for putting together such a fantastic day. We hope it will be the first of many, and thank you to SarcoidosisUK for allowing this to happen. It gives me great pleasure to introduce this session which promises to be very interesting. We have three speakers, and, we will have a question and answer session after each speaker, the first speaker is Professor Wells, whom you all know. Athol is a very dear friend. He has been a mentor to all of us, has been a tireless patient advocate for sarcoidosis, has led in clinical guidelines. He’s a Vice President of WASOG, and I think one of his many talents is that of simplifying what could seem to be very complex issues in the management of sarcoidosis. And so we will hear more about this from Athol.

Vasilis: Can you unmute yourself?

Athol: Yes, I’m just grappling with the technology here. Those that know me will not be surprised about that. Can you see my slides?

Elizabeth: Yes, we can. 

Athol: Very, very good. So may I add my congratulations to Vasilis and also to SarcoidosisUK. And I have to say I have to recover, slightly Elizabeth, from your introduction and regather my thoughts. Now what I am going to present today is very simple, and I will try to keep this to about 12 minutes. I was struck by the quality of the questions. I’ve taken out one or two beautiful slides that don’t actually add to the content. And what I’m going to speak to is really terribly simple. It is this sea change, I think, in how we think about introducing treatment in sarcoid. The concepts underpin much of what is said in the BTS clinical statement on pulmonary sarcoid. They also underpin the first world guideline on treatment which is now virtually in the press. And so let me proceed. I’m talking about treatment, and I’m talking about drug treatment. Plainly treatment includes many other actions, and that includes the provision of clear information, reducing stress and anxiety that may in itself drive sarcoid activity. So I am narrowing down to treatment as drug treatment. And let me start by describing the past confusion. Around the world, there was a tendency to over treat. Introducing steroids and second-line agents whenever there was evidence of active sarcoid and continuing therapy in the long term. Driving doses up to suppress all signs of activity. What one gets from the pooled experience, though, is that steroid therapy is a poison that should not be used by the amateur poisoner. So what do I mean by that? There is no doubt that the overuse of steroids at high doses for too long has led to a tsunami of steroid-linked problems, and we now see this in sarcoid patients with diabetes high blood pressure. They have issues with their weight, osteoporosis; I can go on. But the compelling information now recently emerging is the sharp linkage with these disorders between past overuse of steroids. But by contrast, a neo-holistic approach in one centre was well known for this, of not intervening at all when the disease burden is huge or promises to become so is also a huge problem. So we have an issue of balance here. And it struck me more than 20 years ago; we are dealing with the complex disease. We are dealing with drugs that create major issues in some people if overused in the long term. How do we find the right balance because this is not the 1960s? Sarcoid patients cannot be expected to collaborate with longer-term treatment approaches in this setting. And that’s the logic of treatment; the goals of treatment are crystal clear. And so what came to mind, first of all, is what does anyone need from a treatment in a chronic illness? And one plays around with more complex statements, more detailed statements, but in the end, what anyone wants with a chronic illness is to live longer and to feel better. And feeling better may be a benefit without side effects. There may be side effects, but there needs to be a benefit that greatly outweighs the side effects. Therefore if we’re going to construct logical reasons to treat and we’re going to do this in partnership with a shared logic, we must base our reasons to treat on this dichotomy. And from this comes the treatment indications in sarcoidosis. Living longer, so identifying those people who are in danger. Maybe you’re talking about a shortening of life expectancy? But I have to say as well the danger covers permanent disability because of damage to major organs. You may not have a shortened life if you have that problem, but you do certainly have a reduced life and then, by contrast, unacceptable loss of quality of life. And you have to separate these problems in sarcoid because, in many people, they don’t link now; in many diseases, they do. You will find that those people in danger are those people by and large that have unacceptable loss of quality of life. But sarcoid is one of those disorders where this is uncoupled. There are people whose quality of life is good, but they have a danger. And you have people who have unacceptable loss of quality of life in the short term, but they don’t have the sort of danger I’m describing. Now let me break this down a little because how we break this down influence critically how doctors and patients speak. So it is very helpful to start by excluding danger because if danger is present, you’ve got a rock-solid reason to discuss treatment. And knowledge on this really comes from medical expertise. You’ll see why I’m saying this when I get to quality of life. But you are looking at tests that establish severity in major organs and tests that establish high future risk from major organ involvement. Now treatment decisions here obviously remain a partnership, but this is a situation where key information is sought by the doctor provided by to the patient with the shared logic in why treatment should be used. Now I make these points because it’s radically different when it comes to choice made based on quality of life. Because there is only one expert in the room at that point. I cannot do a blood test to find that somebody has 17.73 units of fatigue. So the question you’re asking, I often ask people to take four weeks to think about it, think about their life from day today. But is the quality of life so reduced that something needs to be done. And while some patients still want a doctor to decide for them and they have a right to have this happen, I think many other people prefer to decide themselves on this question. But they need to know that danger’s been taken out of the equation because otherwise, you look at loss of quality of life, you think it actually signals danger, and you cannot think clearly about this separate question. And the approach to what you do? I’m not going to talk about different drugs. Obviously, that’s the next talk. But the level of therapy differs radically. Now in dangerous disease, this is what is generally described as typical treatment in sarcoid, and I’m afraid many people with loss of quality of life have been subjected to this approach, with a net production and quality of life due to the side effects. But you are talking about taking control. You switch off the disease, and then you find often that a civilised dose of therapy in the longer term that could never have taken control maintains control. And you’re finding the minim longer-term dose that preserves the gains. Now, by contrast, and I speak for many people. I make 20 different deals with 50 different patients in finding the right approach to treating losses. I have somehow lost my screen at this key moment. I don’t think I did anything, but I will do a new share and try to recover my equanimity.

Vasilis: We can still see your slides. 

Athol: That’s very good, I couldn’t. Now time forbids detail, but there will be a negotiated start of therapy. Very often, you’re talking about finding out whether a very civilised dose looked at in the short term transform somebody’s life. And then you’re encouraging the patient to experiment with levels of low dose to find the best balance between benefits and side effects because that’s very individual. You can’t do a doctor knows best on that question. But you better be sure there isn’t danger when you embark on this pathway. And I think my second to last slide is entitled traps for young players. So not all quality of life problems in sarcoid can be defeated using standard drugs. And I would mention fatigue which really needs a different approach. Depression and I think if you’ve got a major illness with great anxiety, the normal human reaction is to fear; the abnormal human reaction I have to say is to be ecstatic about this. Sleep loss, lack of attention to whole-life health and this problem of brain fog, so I’m just listing some of the issues that you can’t cure with a drug menu. And finally, many chronic symptoms in people with sarcoid are not actually due to sarcoid, and they shouldn’t be attacked with steroids and other drugs. There’s a long list, and I’m only listed frequent culprits, and part of the job of the sarcoid physician is to work out those things that are a problem that are actually not sarcoidosis. So I’m going to summarise. I’m afraid this has taken a little longer than I had hoped. But in summary, I think we can say half of patients with sarcoid do not need to be treated. Now that’s not half of people coming to the state. You’re here because you have more problems with sarcoid. But you should not treat in sarcoid unless you take one of the two drugs as danger and quality of life. I’ve said what I want to say about bullet two, three and four, and finally, your need for long-term treatment should always be challenged, and sometimes that means you end up on a lower dose, but you don’t come off treatment immediately. Thank you very much.

Elizabeth: Thank you very much, Athol, for that excellent summary, and we have about only five minutes for questions. So I’m going to move to a few, I’ll try and summarise some themes that I’m seeing from the chat and from the previously submitted questions. One concerns stopping treatment, and how do we decide whether it’s okay to stop treatment?

Athol: Case by case. You’ve got to be very clear indeed about danger versus quality of life. I think you should always experiment with time, and that sarcoid activity often does burn out. For quality of life indications, you’re simply reducing the dose and finding the minimum dose that is required. It may mean stopping treatment. It may mean reducing, then reducing, then stopping treatment, but you find that out. For danger, the problem is activity is suppressed, so you can’t see it still there until you reduce therapy, but you should do that periodically. And plainly, you will be less likely to do that sooner if somebody’s in a high-risk situation. More likely to do that sooner if somebody has good reserve and you’ve treated because you anticipate there will be trouble at the time of introducing treatment if you don’t treat. So look, this is case by case, but the guiding principle is that if you can come off therapy, you should come off therapy. But you shouldn’t rush into it in the first year or two if the if the sarcoid burden is serious. And one final point if you reduce too soon, you actually increase the average dose of steroid because you have to go back to higher doses.

Elizabeth: Okay, and I thank you for that, and a link question which several have asked in in different ways is, once you have a return of symptoms let’s say systemic symptoms a specific symptoms, how can you know and how can we test to tell us whether it is sarcoid activity coming back or it’s something completely unrelated?

Athol: I’d say there are three aspects to this, and each will be a sentence. First of all, patients know how it feels to have active sarcoid, and they can often tell you that this sarcoid is active, and you should trust that if in doubt, I believe. Secondly, you are looking for clusters of symptoms that together are decisive, whereas one individual symptom might not be. Lastly, if you are in doubt, you can do selected tests to demonstrate that sarcoid is active.

Elizabeth: Thank you. Thank you, Athol. We’re getting loads of questions also on the chat, a few questions relate to the new biologics and the fact that steroids so often cause side effects. So rather than go for steroid side effects, can we not just treat with a newer and of course Anjali will also be speaking to this, but what is your view?

Athol: Very simple, actually. To talk about steroids as if all doses are the same drug is a sad mistake. So we’re all on prednisolone 7.5 milligrams daily. If you have extra steroids, you reduce what you make. We all have spits of higher dose that we make naturally, so a low dose of steroid does verge on natural therapy. Whereas high doses of steroids are highly unnatural because they subvert body mechanisms that attune to low doses of steroid. So we’re not talking about steroids on block versus other therapies. And I think you have to have a hit list of the nastiness of therapies. So I think the nastiest is hydro steroid in the long term. The nicest therapy on average is a very low-dose steroid plus or minus hydroxychloroquine. And the other drugs are somewhere in between. So if your dose of steroid is going to be unacceptably high or you have a particular problem, you move early to other things. If, in fact, you’re tolerating low dose therapy and it’s doing the job, why would you go to therapies that are more toxic? And then sometimes people have particular wishes not to use steroid, and you shouldn’t go on arguing at that point, but you should consider going straight to a second-line agent. But I would go to methotrexate before biologics because I think the trouble associated with biologics, they feel like they’re a miracle of the space age, but actually, the grief associated with them tends to be higher than low to medium dose methotrexate, so.

Elizabeth: Thank you, Athol. Unfortunately, we’re only going to have time for a final question because we only have one minute. There are several questions on vitamin D. Why is it not recommended? What do you do if you have osteoporosis and have low vitamin D but also have sarcoid? Very quickly, in like 30 seconds.

Athol: Well, you’ve occupied the question time in asking the question. Look, I’m going to say there are four problems with vitamin D, and I don’t have time to say what they are. I’m going to say that just occasionally, low vitamin D can drive sarcoid, and that’s terribly rare. And I’m going to say that once in a while, you must use vitamin D for bad osteoporosis. But all I will say because there isn’t time is that there are four separate issues with vitamin D. Four separate problems that it can drive in sarcoid. Therefore it’s another poison not to be used by the amateur poisoner.

Elizabeth: Thank you very much, Athol. I leave it there, and it’s now my pleasure instead to introduce Dr Anjali Crawshaw, who is working  as a consultant in University Hospital Birmingham, completely focused on ILD and sarcoidosis. She leads a regional multidisciplinary sarcoidosis service and has a lot of experience. So we will hear from her about the new and old therapies for sarcoidosis. What medication to choose when treating? And the floor is yours, Anjali.

Anjali: Okay. Thank you very much to you and Vasilis and SarcoidosisUK for asking me to speak, and also for giving me the slot after Professor Wells. He’s always a hard act to follow. So I’m going to take you through   the medications of the treatments that we can offer for sarcoidosis assuming that you and your physician have come to an agreement that  treatment is at this stage required. There are many different options. And when you first come to clinic, it can be very overwhelming to hear about these and so learning about them early gives you the opportunity to think about things and help in the decision-making process. I’m going to talk about steroids and then the more classical immunosuppressants. A little bit about other options such as biologics, anti-fibrotics and other things we can do and offer for treatment. Before talking about the medications, I think, one of the questions we’re often asked quite rightly is, what’s the evidence for one drug over another drug? why should I be choosing this? Why are you choosing this? why are you recommending these things to me? When we are learning about new drugs, the ideal way to learn about a drug and whether it’s going to be helpful in treatment is doing a clinical trial. How do we do a clinical trial? We take a group of standardised patients with the same set of symptoms, the same level of disease activity, the same makeup, same gender and, split them into two groups. Give one half the treatment and one half no treatment or a placebo, but actually, in sarcoidosis, we all know that’s not the case. The disease is so different presents differently in different people, level of organ damage, level of disease activity, the symptom burden. And this makes it really difficult to design a study for sarcoidosis patients and for medications in sarcoidosis. And much of our evidence comes from either small series of patients or people that when you pick at the details of the study, actually the people entered into those studies or trials were actually having very different  disease. So deciding whether a drug  is good for sarcoidosis or not becomes more difficult. Much of our recommendations on sarcoidosis is using these studies  in context and with expert  consensus. So we’ve all heard about steroids, and in the UK and prednisolone is the steroid that we use most frequently. What does it do? Well, it reduces inflammation, and it suppresses some of the chemicals  that are produced by the inflammatory cells that  Simon talked to us about earlier. , the study suggests that there is some short-term improvement in both symptoms and the biomarkers but we know that biomarkers or the chemicals the blood tests that we do don’t necessarily correlate with either how patients are feeling or the level of damage occurring. Despite the years it’s been used there’s still actually a remarkable lack of current evidence to demonstrate long-term benefit for use of steroids,  any clear alteration in the natural history of sarcoidosis and also, importantly, a lack of evidence to suggest and improve mortality. So when do we start steroids? Well, we start steroids as  Prof Wells has suggested if there is dangerous or potentially dangerous disease, and that includes  cardiac sarcoidosis, neurosarcoidosis or sarcoidosis affecting the eyes, and I understand there are talks about this later, so I won’t go into that much further. But also when we feel that the risk of damage or the burden of symptoms outweigh the risk of the treatments that we’re offering. So steroids, and I will touch on this in a moment, come with a come with a set of risks, a set of side effects, and it’s important just to weigh up the relative risks. For lung sarcoidosis, we use breathing tests as a guide, and there are specific parameters that we can help guide us, but then it’s different for every patient. And it’s important to remember that the breathing markers in different people vary greatly. So actually, often, it’s a drop from baseline that we’re looking at. What dose of prednisolone do we use? Well, traditionally, people were started on very high doses of steroids, but actually, we find that’s probably not required, and a dose of around 20 to 40 milligrams is probably enough. We’d use lower doses in patients whose sarcoidosis has been grumbling along; it’s not presented with that bang presentation that  Simon presented earlier but more gradual progressive, and we finally say well the balance is tipped in terms of starting treatment. And we also get very much higher doses, sometimes intravenously if we feel there’s life-threatening or organ threatening disease. So the side effects of steroids. Now there’s a long list of them, and for many people, they can be very problematic. Now that’s not to say that everybody who started on treatment has steroids. It has side effects. And not everybody has all of the side effects. The one of the most frequently concerning to people is the increase in appetite and subsequent weight gain and associated indigestion. But other important things include difficulty in sleeping changes in mood and behaviour which really can be for some people quite disabling they can feel irritable or anxious. There’s also increased risk of infections the affect on potential blood sugar or diabetes blood pressure. And if you’re talking about more long-term steroids, risk to bone thinning to your eyes and a condition called Cushing Syndrome. I think some of the questions proposed earlier were saying, well  I’ve been on steroids or started them, how long do I continue them? and when can I get off them? that’s the question is you know as soon as we start talking about treatment we want to know well how long is this for. Well, we continue it based on treatment response. If they’re working, great if they’re not, we might need to think about moving to other things. We would want to constantly re-evaluate the need for treatment. There’s no one thing that tells us yes, your sarcoidosis is active. There’s no one blood test. There’s no one parameter that we look at. And it’s important to understand that actually, in a study of patients looking at how long their disease lasts; we know that around 40% of patients will go into remission without treatment at all. So when you’re feeling well on treatment, what we need to understand but is difficult to know is am I feeling better because I’m on treatment or is it I’m feeling better because my disease is in remission. There’s no clear way of knowing that, and actually, this is why consider constant consideration for withdrawal of the medication is really important. We’re aiming for the lowest possible maintenance dose of steroids, ideally five to ten milligrams. And then balancing the risk of  treatment so the side effects that we get with steroids versus the risk of triggering or allowing irreversible disease progression. Ideally, we should consider attempting withdrawal at 6 to 12 months but continue the treatment for if there’s relapse with either dose reduction or withdrawal. Understanding that actually from for many prolonged treatment is often required. I saw a question in the chat, I think about  inhaled corticosteroids, and we know from the sarcoidosis registry that around 8-10% of patients are on inhaled steroids but actually, the evidence for this is not great. The idea that you’re getting steroids to where it’s needed is, an attractive one, but so far we don’t have the evidence to support that. So beyond steroids, well, what do we do next? So this is when, as Professor Wells said, actually, perhaps we should consider  being seen in a specialist centre or by someone in your local centre who has a clear understanding of the secondary medications that we can use. The first thing we need to check is that, you and your clinician have the same understanding of the dosage that’s being taken. Often people quite rightly feel that they’re on, a dose that they don’t are not happy with, or other things have happened in the interim, and the dose may not be the same as what as the dose your doctor understands you to be on. And that can affect what we decide to do next so making sure that everybody knows what dose you’re on and the problems that you’ve been having is really important. When would we add treatment or refer you to specialist care, a specialist centre? Well, if there’s progression of disease or, there’s an unacceptable symptom burden if the side effects from steroids are intolerable or we’re unable to reduce the steroids below 10 to 15 milligrams a day because that’s the dose at which we find the side effects become more problematic particularly long term. Or if there are other considerations comorbidities that really make steroid use a problem such as diabetes, obesity, osteoporosis or high blood pressure. Or if there is a patient strong preference to you against using steroids. So what are these other drugs that we use? Well, there are also drugs that suppress the immune system, the overactive cells in sarcoidosis. Well, what’s our evidence for using these? Unfortunately, it’s a similar story to that seen in steroids. Small-scale studies in patients that are not necessarily so similar to be able to draw strong conclusions. We talk about methotrexate, azathioprine, mycophenolate and the Leflunamide and I’ll go through those briefly so that we have  heard of these medications to give you that that power to be able to  understand and participate in discussions  when you’re talking to your clinician. Second-line immune suppression also has risks of toxicity, different to steroids. Risk of increased infection, marrow suppression issues and for people of childbearing age conception planning issues. Many are not suitable for people with chronic viral infection or reduced or liver or kidney function, and they require regular blood monitoring, which for many can be a significant side effect. So methotrexate, this is one of the most commonly used second-line agents worldwide. It inhibits the blood cell synthesis, and there’s no evidence to suggest that methotrexate is better than azathioprine. Its  efficacy or how good it is reported to be around 50%, with a response rate, so the number of people who respond being around 40 to 60% and that’s based on looking at breathing tests. It can be taken as a tablet and also usefully for many can be used as taken subcutaneously so as an injection. You need to have folic acid supplementation with this to prevent toxicity. And the dose is usually something around five to ten milligrams to start off with increasing up to fifteen to twenty milligrams as required and tolerated. Side effects include nausea, lung inflammation but importantly not lung fibrosis, some problems with blood count, nausea, sometimes hair loss and skin rashes. Azathioprine is the other drug very commonly used. There’s less less evidence supporting it, but it’s about as effective as methotrexate. It again inhibits blood cell production, and , there are no randomised controlled trials looking at this. It’s open-label studies, so series of patients suggest that there’s a modest improvement using azathioprine. A dose of around 50 milligrams once a day, increasing up every two to three weeks till a dose of between 100 and 150 milligrams is usually used. And again, similar side effects in terms of nausea dyspepsia are often seen. It can have problems with low blood counts, and it can be difficult to differentiate below blood count issues seen as a result of azathioprine with that low blood count seen in sarcoidosis itself. We would do a blood test before giving you azathioprine to determine the risk of toxicity. There’s possibly a signal that there’s more opportunistic infections with azathioprine. The rest of the medications are used less frequently, and I’m going to go through them slightly more quickly. We use a drug. We often use mycophenolate, and the evidence for that is again even less than azathioprine and methotrexate. It’s widely used in other inflammatory conditions, and our understanding has been extrapolated from those conditions, and we use a dose of one to one and a half grams a day. Similar side effects diarrhoea, leukopenia, low blood count. Leflunomide, again our understanding about this is extrapolated from rheumatoid disease. Can be better tolerated, but our experience in sarcoidosis is limited. Cyclophosphamides is a very old-fashioned agent used for inflammatory disease, but it’s rarely used in sarcoidosis, so it’s just to know if it exists. Hydroxychloroquine we do use quite frequently. It inhibits blood cell activity the way the inflammatory cells work in sarcoidosis and can be helpful in skin disease, joint disease, and  high calcium levels. Also, I’m telling you.

Elizabeth: Sorry just to interrupt you, but, we only would have four minutes for questions now. As we just to give a few questions. 

Anjali: Okay. Fine. Sorry . I’ve only got a couple more slides, actually. It was just a few of the questions have been about biologics. Why aren’t we using them? Is money an issue? And actually, biologics are antibodies that target the inflammatory pathway. They’re widely used in other inflammatory conditions but not routinely available in the UK for sarcoidosis, and that’s because the evidence isn’t great. The small studies that have shown, looking at the  biologic agents don’t really show a large amount of benefit and so being able to give those is they’re not yet funded. Anti-fibrotic agents. A study last year suggested they could be used in sarcoidosis. It’s important to treat coexisting conditions, and I think those are going to be discussed later by my colleagues. I won’t talk about future research because I know that’s coming up. So really, what I wanted to get to was your take to your consultation messages. And the most important thing is decision to treat is based on multiple factors. And that’s how you’re feeling, what the side effects of the treatments are and really the balance between the two. The trial evidence for various treatments is sparse and based on expert consensus, and while steroids are usually the first choice, there are other options, and so thank you for listening and sorry to have to hurry the end of that.

Elizabeth: Yes, thank you very much, Anjali, for a very thorough, comprehensive review of treatment. I’m sorry to rush you. It was just to give you the opportunity to answer a few questions. We’ve got two minutes, so, a few of the questions concern a Covid. One is how does the Covid pandemic change our choice of treatment? And I imagine the question is how worried should we be about using immunosuppression during a Covid pandemic? And another series of questions also in the chat concerns the similarities between long Covid and sarcoid. Could it be that some of the patients that have long Covid actually have sarcoidosis?

Anjali: Oh, so in terms of choices of medication in the context of a Covid pandemic. Actually, we really need to be looking at the same factors, which is how dangerous is the disease? And really, we should only really be considering treatment if the disease is dangerous or there’s an unacceptable burden of symptoms. And so in that context, really the balance would favour treatment even allowing for the Covid pandemic. I don’t know what you would say Elizabeth but I think really we have to be certain that the disease is going to cause more danger than not treating did, and then the other question was regarding long Covid, and I think there’s another talk about  Covid and sarcoidosis later, but yes, I agree that the symptoms can be similar and I don’t think the jury’s out on that front, really.

Elizabeth: Thank you. Yes I would agree with what you’ve said about the immunosuppression during the Covid pandemic. If needed, one would go ahead and continue with that treatment. Some of the comments were, you know it was a shame that we rushed you in the last bit of your talk. So in terms of the biologics, for example, infliximab, would there be particular subsets of sarcoidosis where you would consider them? You know when would you consider the use of biologics? As I rushed you through that and it seems to have been an interest.

Anjali:  Yeah, so there are groups of patients that we give infliximab to, for example, we’ve got very good experience for patients with cardiac sarcoidosis and neurosarcoidosis who’ve  not responded to conventional therapy, so steroids and then a second-line agent, we found that they’ve had very inflammatory disease, and treatment with  infliximab has caused that disease to melt away very quickly. So I think those groups of people. And also, significant skin sarcoidosis again can respond. Lung disease, there’s much, much less evidence for that.

Elizabeth: Thank you, Anjali. There’s lots of questions on the chat. I don’t know if you want to have a look at that. They’re coming very quickly. But everyone says really appreciated your talk. So thank you very much, and we moved to the last talk which it will be given by Charlotte here she is. So Charlotte Hogben, , our lead clinical nurse specialist here at the Brompton, together with  Benedict Davitry and Melissa, do a fantastic job in keeping us in check and communicating with all of our patients. And she’s going to be talking about the role of the clinical nurse specialist in care delivery. And I would say that in one-word indispensable, but I will leave Charlotte to detail it further.

Charlotte: Thanks Elizabeth. Let me just get my screen going. Okay, is that showing the slide in a way that it should be? I hope.

Elizabeth: Yes, it is.

Charlotte: So, the role that we play in care delivery it will be different across the country in terms of who is available in your local centre and exactly what the nurse specialists do. And I can only talk really from our perspective, but I think there are there are some ways to try and find additional support if you haven’t  got someone that you know of in this in this role. But the key things that we’re involved in doing is, first of all, being a point of contact for both patients and GPs. Being a link person who can liaise with all of the multiple specialities and the consultants. We’re heavily involved in drug initiation and monitoring. Giving advice on side effects. Symptom management and also onward referral to other services. our team, hopefully for those of you who are under the under the Brompton you would know how to contact us if you’ve needed us in the past and we have phone numbers and email addresses on our letterheads. and hopefully, I know across the patient group we have a great degree of variety in terms of how much support people need. Some people don’t need to contact us on a frequent basis at all and simply talk to the consultant regularly in clinic and don’t need anything additional from the nursing team. Whereas there are other people who have to contact us frequently due to medication difficulties, problems with blood, problems with symptoms. So there’s a great deal of variety in terms of how much support you might need from a nursing team. But I would suggest that if you don’t know who your local nursing team is, having a look on your letterheads, see if there’s any contact details, a phone number and email. If there’s nothing specifically for a nursing team, then maybe for a secretarial team. And you can always find out from them if there is a nurse you can contact. And most importantly, if you’re having a consultation in clinic and you haven’t previously been aware of anybody who you can contact in between those consultations, ask the doctor. Ask the doctor who your point of contact should be, and they hopefully will be able to let you know. The other way to try and find out for your local area is via any support groups and patient groups. There may be other people who are under the care of those services who are able to help point you in the right direction, but as I say we have all our details on our letterheads phone numbers and email addresses to contact, and we are here Monday to Friday every week. And any call or email that comes into us, we will get back to you as soon as possible. Usually, the same day if it’s a simple answer or if someone’s  needing a really urgent answer. But if someone is asking a question when we need to do a bit more research and get a bit more information, it might take a bit longer before we get back to them. One of the key benefits for patients really in terms of having a contact with a nursing team is that we can link in with all the different specialities. As sarcoidosis is a multi-system condition, you might be under different teams for management of different elements, and we can help coordinate some of that and contact different teams. Or if you develop new symptoms, we can liaise, for instance, with a dermatologist in order to see if they feel that, a rash you have, for instance, could be related to sarcoid. So contacting us for issues like that, we have those links, and we have that ability to liaise with those different teams. As I mentioned, one of the key things that we do and what that we spend a lot of our time doing is helping manage the medications that were previously being spoken about. In particular, the most common treatments of the immunosuppressant treatment because, as we were told they need regular blood monitoring, they have lots of side effects potentially associated with them and often GP practices are either unable to prescribe them or are unfamiliar with the medications and need support from us in helping manage that, because many GPs practices are unable to prescribe medications, you can’t get them by the usual pharmacy. It might be that we’re involved in setting up home delivery of medications. We would also be speaking to you about potential side effects, about the blood monitoring that’s required and the kind of things to look out for in terms of difficulties that you might have. And we will also liaise closely with the GP about the blood test. Now for our patient group coming to the Brompton you may well be spread quite far from the centre. So whereas we would find it very easy to arrange blood tests for you here, it’s not going to be easy for you to be travelling here for the blood test. So we tend to ask the GP or local teams for help in arranging blood monitoring, and we give the GPs our secure email address that they can send through any blood results that they have, any concerns about any results and we’re very happy to advise on those in conjunction with the consultants. And that’s one of the one of the key things that we’re involved with. Also, very specific to here because it could be very different in your local area. One of the other things that we’re involved with and that we would potentially contact patients about is arranging IV therapies. So where people are having in those sort of limited circumstances biologic therapies or where we’re arranging iv steroids as more urgent treatment. The phone calls and email address, the phone number and email addresses they’re really to be able to offer advice on managing symptoms, whether that’s side effects from medications helping identify whether symptoms you have are related to sarcoid or whether they need further investigation by the GP or local services. And liaising with your consultant in order to advise on any adjustments of doses that might be required of your medication depending on your symptoms and discussing any concerns that you have in between your consultations because it’s obviously not possible to directly get in touch with your consultant on a day-to-day basis between your clinic appointments. But that’s what our number’s there for is to contact us with any concerns you’ve gotten then we can take those to the consultant and arrange any earlier follow-up earlier tests or any changes of medication that are required or offer advice back to the GP or local services to carry things forward. We would also be involved in making referrals to other services. So we would potentially point someone in the direction of patient groups and some support groups, so particularly if someone’s just been newly diagnosed they may not be aware of SarcoidosisUK, so we would point them in the right direction and also got to encourage people to get involved. We also, if someone has lung involvement, we might be suggesting that they do pulmonary rehabilitation courses and we can make those referrals directly. If someone’s got severe disease and fibrosis, we might need to arrange oxygen assessment. If someone’s having difficulty with weight loss or significant weight gain, we may help get dietitians involved in order to help advise on that. And we can also make referrals to psychology services for additional support. Here at the Brompton, we’re sort of divided into two groups. We in the ILD team and my team are primarily the lung involvement advice and can help with other elements as well. But Sue Gilmore is the heart failure nurse who has the particular focus on cardiac sarcoid and those who do develop heart failure. She sent me some information. Now, this isn’t my area at all, but she, unfortunately, was unable to attend today, but she sent me a few slides just to run through. So as she points out, some but not all patients with cardiac sarcoid have an element of heart failure. And her role is to act as a point of contact in a similar way to us to give advice on their condition, providing education and support adjusting medications in order to manage the condition, as she says, the immunosuppression treatment and monitoring is done by our team the respiratory team. These are some of the medications that she might be involved in in monitoring and adjusting treatment doses, ACE inhibitors, beta-blockers, diuresis, but if you are in this position and have cardiac sarcoid and you’re using these types of medications, you probably already are in contact with her or someone similar in a similar role as a heart failure nurse. The type of education people need obviously varies according to patient, but she would educate on being aware of symptoms to look out for, monitoring weights and when to seek additional help. She’d been making changes to medications based on blood tests based on other parameters, and particularly monitoring kidney function. And some of that monitoring does overlap with our team and we might be offering joint advice on how to manage the condition. I have saved  a couple of bits and pieces for some of the questions. I know they’ve been very specific, so I’ll hand to Dr Renzoni to go through the questions that have come in.

Elizabeth: Thank you very much, Charlotte. Thank you, and , if we don’t manage to answer all the questions, they are all listed in the chat, so you can, but you know many people are also expressing  thanks for your help with them  throughout the year, but , to go through some of the questions  one  is an import very important one. I mean, they’re all important, but this one Why aren’t there more nurses trained in sarcoidosis and how to help patients with the condition that they can talk to?

Charlotte: I think one of the key difficulties or the complication that comes with sarcoidosis obviously is because it’s a multi-system condition and the way nurses tend to go through their training as they start to specialise in a particular area such as respiratory such as cardiac and so the nurses that are there to help on within the sarcoid teams are generally embedded within another area. So, for instance, Sue, who would help with cardiac sarcoid issues, is embedded within the general heart failure team. And we, as the respiratory nurses, we’re within the wider ILD team but are very much focused on sarcoidosis as well. So the nurses might not be labelled a sarcoidosis nurse as such, but there are lots of different people involved who can offer the advice and guidance. Of course, that’s going to be different across the country, and certain areas will have better levels of support than others. And I think it’s something that can always be, campaigned for through your patient groups to get better funding for more nurse support. It is certainly something we would advocate. We always feel that there should be more nursing support available.

Elizabeth: Definitely, I can only echo that because, of course, it would be it will be important to have more and more specialist nurse support in across the different areas in the UK. Whereas probably there is still a lot of heterogeneity and the type of support and level of support you can get. Another very good question is, what is the role of patient organisations in the delivery of care? Just for some increase of health literacy, or is it part of the therapy?

Charlotte: I think it’s part of therapy because peer support is so important, you know. As much as we can advise on drug therapies and potential side effects to look out for, we don’t know ourselves how it feels to have your sarcoidosis symptoms. We don’t know how the fatigue feels. We can’t even begin to imagine that. So being able to talk to people who are in a similar situation as yourself and being able to join support groups and have those discussions with people who experience the same things is really important and every individual will have little tips about how to manage their symptoms and how to how to help themselves outside of the medication side of things and you can only really get that from your peers that’s not something that you can you can get as thoroughly from us because I said we don’t we don’t have those symptoms.

Elizabeth: Thank you, Charlotte. And, another question which you have already touched on but just if you wanted to underline. What questions and queries can or should a patient bring to specialist nurses rather than waiting to check with the GP? When should we come to you? When should we approach the GP?

Charlotte: So I think I mean the difficulty that people will always have when you have a diagnosis like sarcoidosis is often you’ll go to the GP, and they will perhaps suggest you speak to your sarcoidosis service because there’s an assumption that it’s related. And if that’s the case and you’ve spoken to the GP and they have asked you to contact us, that’s fine. If we don’t feel it’s sarcoidosis related we’ll contact the GP get back to them, and ask them to further investigate. But the key thing for me really is that yes, you know side effects from medications we’re probably more experienced in in managing those, if you’re having a flare of your symptoms that are very similar to what you’ve had previously. And you recognise them as your sarcoid symptoms, or if you just need general advice in managing your care we’re really happy for you to get in touch with us. The only time I don’t want someone to get in touch with me is if it’s an urgent emergency issue. We’re not an emergency service. We’re here Monday to Friday. We can’t necessarily answer a call immediately, and if you have got sudden changes in your symptoms like you’re suddenly much more breathless suddenly having chest pains, then that’s not something to call us about. You need to contact emergency services, whether that’s nhs111 contacting 999 or calling your GP for an urgent call out. They can then liaise with us for advice. We’re not expecting them to manage your care, particularly if it is related to your sarcoid. But, it’s always a worry when we have a message that’s been left on a Friday evening with someone who’s saying they’re much more breathless, and we haven’t picked it up until 10 o’clock on a Monday morning. So, certainly those kind of things should go by the GP. If you’ve got a simple issue like you know you’ve got a chest infection you’ve had before the GP can manage that. Or if you feel it’s unrelated to your sarcoid, of course, approach your GP first if you if you can.

Elizabeth: Thank you very much, Charlotte, very comprehensive answers and as I said there are there are lots of messages on the chat. I think we don’t have time for more, but for all the panellists, as I said, there are several questions. A few will probably be dealt with in the next sessions. There are several questions about what should pulmonary physicians be screening for at presentation for other types of organ involvement? And I’m sure we’ll hear more about that in the next session, where specific forms of sarcoid will be discussed, including cardiac sarcoidosis. So, we now have a 10-minute break, and then we are back for that session which will be start chaired by Peter George and started by Vasilis. Thank you very much.