Life after Sydenham’s chorea

[Adrian] OK, I will share a  few comments. So I put out a request on Facebook for ideas, what people wanted to talk about at this meeting today and a lot of the the comments came down to what happens later, and Michael has already said that there is this proportion of people who have more persistent, chronic problems.

And he’s also pointed out that actually, it sounds like maybe people don’t go looking that hard for persisting and ongoing problems, at least when it comes to writing up things in in journals and in the literature.

So I thought I’ll share these with you and I’m very happy for people to jump in and say what they think, although of course I can’t see people while I’m on this screen.

“The basic problem is that no one seems to know anything about Sydenham’s chorea in adults. There’s no effective medicine, and it’s so rare that funds for research don’t seem to exist.”

So that’s obviously quite a negative perspective and and that is this individual’s experience.

Part of the reason we have our charity is that we can fund research and we have partly funded the work that Michael has done, but also previous work, so it’s not entirely true that there isn’t the potential to do some research.

But I think, from what we’ve heard people say, is that it’s it’s already hard enough to find doctors who know anything about Sydenham’s chorea in children, let alone in adults.

Another comment was, “mental health related issues are very rarely talked about or discussed and not easy to get info on – my 11 year old was diagnosed 2 years ago, just had a relapse and I thought they were going to put him in psych [I think psychiatry], it got so bad at the hospital.”

So there is this thing of… you see these physical symptoms. But it is well recognised you do get these more psychiatric symptoms. And Nadine is really the expert on this.  Michael showed that emotional problems, anxiety, irritability, hyperactivity were actually pretty common, but there was a long list of other mental health symptoms that can happen in the child. And then of course, what then happens going forward?

[Nadine] And I think he made a really important point, Adrian, about saying that that the symptoms need reassessed when when the symptoms gets better, because I know, we, as part of our study where we looked at the 12 cases that we had, two of the children met the threshold for a diagnosis of autism. Now autism is a lifelong condition. It’s really important you get it right. But actually, as they recovered, their symptoms settled, those symptoms, were no longer present, so they wouldn’t have met the threshold for a diagnosis. So it can be really intense, I think, at the point when the symptoms are, you know, really unwell. So you’ve got the the physical symptoms, but you can also have these really striking psychiatric symptoms of lots of things, from tics, to high levels of separation anxiety, that then feeds into, you know, poor sleep, poor diet. So it’s really, really important that you’re thinking of the whole presentation and not just the physical side.

I think I said earlier, it’s really important that the child has someone to communicate with about how they’re feeling, whether that’s the school nurse or a counsellor just a member of the family or one of the health professionals at the hospital when they’re going for their their follow-ups. It’s really important that the child has a space to be able to explore their thoughts and feelings because it can be really quite scary and a lot of you have talked about young children. It can be really quite scary, if you lose your speech or you lose the ability to feed yourself or to walk, just having some space to reflect and think about that alongside the the psychiatric symptoms that you might be seeing.

[Adrian] And I think you did set up a support group, Nadine, in Glasgow.

[Nadine] We have, we had a little group of parents and and patients that we supported. We were really fortunate in Glasgow that we had the liaison psychiatry team, not every General Hospital or Children’s Hospital will have a liaison psychiatry team. And I think the criteria for those teams have changed a lot since I’ve moved away from the [Royal Hospital for] Sick Kids as well. Like I’ve mentioned, there’s lots of crisis work going on, criteria can change as well. So if we’re dealing with 16 to 18 year olds, it can make it harder for families to families to get in.

I work at the minute in CAMHS [Child & Adolescent Mental Health Service], or SIPPS as we call it, and we very rarely get cases coming through. We have had them, but trying to meet the threshold for community mental health services can be really hard. So exploring what counselling services are out there, what services are in the hospitals really important. Most hospitals will have a paediatric psychology team as well that might be able to visit when you’re on the ward but also to signpost you in the direction of appropriate support.

The educational psychologist can be really important. They’ll have different names throughout the world, but for us they are the psychologists that go into school and help when children can’t attend. We had lots of non attendance at school or reduced timetables. Or children needing to take wheelchairs in into school. It was lots of hindrance for just being able to access education or their absolute school refusal, because it was just all too overwhelming. So having key people in school, like the the special education needs team or the educational psychologist, it’s really, really important. But you should hopefully be able to get that advice within the General Hospital, but if not, you can always phone the local CAMHS teams as well for advice.

[Michael Morton] I wanted to say something about adults and long term difficulties.  I think there’s something very difficult about a condition that can fluctuate.  Services for adults tend to assume that you are who you are and that’s how it is. And outside of maybe epilepsy and psychiatry and one or two other conditions, there isn’t really an expectation of things fluctuating. But that’s exactly what happens with a lot of people who have chronic Sydenham’s symptoms, but I think the the key has to be this word “rehabilitation” that Michael used a bit.

And the best models for rehabilitation are probably in brain injury, and you can almost think of what happens to a brain with Sydenham’s when the problem doesn’t go away. Whether you could find an injury on a scan, you might well, but you might not, but it would be minor – but you are left with someone who has got something different about the way they’re made as a result of their illness. And that requires lifelong rehabilitation for some individuals, but fortunately not very many. The mental health part is really important, but actually it’s the whole person. And I can only think that using that word rehabilitation with professionals can sometimes tune in a way of thinking which can be helpful. But there’s no one service that sits there that has the skills unfortunately, it’s about each family putting together around an affected person what they need.

That said, we’ve talked a bit about chronic treatment and you know there are lots of ways of trying to treat chronic conditions, and it’s knowing when to try something new, and when to decide to stop, given the side effects of drugs and the problems there can be. So having a medical guide through this process is important.

But I don’t know whether Michael has thoughts from the research about the the really difficult chronic conditions where people have the paralytic type, symptoms are quite disabling, as well as the mental health side, which I think I can see how mental health services should be able to grapple with, if they can get their heads around rehabilitation, but from a neurological point of view, there are children like we were hearing earlier, who are still unable to walk two years later and what can be done? That is a very interesting challenge.

[Michael Eyre] Yeah, I’m not sure of anything to add beyond, definitely, it’s a rehabilitation approach, isn’t it? A multidisciplinary approach? I’ve never managed any patient like that myself, so I wouldn’t be speaking from experience, well, not a patient like that with chorea. But extrapolating from other illnesses, yeah, intensive rehabilitation is often needed in a setting where that can be provided on a daily basis sometimes, for a period.

[Nadine] So then it’s sometimes the the parent that needs to be that facilitator of the team of experts that’s around your child. And then as you become an adult, whilst you’re having these relapses in times of being unwell, trying to hold that coordination together can be quite tricky, I imagine. So it’s about having a network and people around who recognise when things are getting a bit tricky, that can step in and help.

Because when we talk about a team of experts, we could be talking about a neurologist, we could be talking about a speech and language therapist. You might have a counsellor. There’s lots of different layers of of treatment. It could be the cardiologist, it could be the physiotherapist. There’s lots of different specialists that feed into the treatment of Sydenham’s chorea to have a successful recovery when it is chronic, that takes a lot of coordinating and takes a lot of time. So I know from the Facebook group we see a lot of parents end up sadly having to give up work because they need to focus on the care needs of their their child while they’re trying to coordinate all of this, and it can be exhausting at times, because you’re knocking on doors and not always getting the answers that you want, or you finally coordinate together and you have that success. But then as soon as that team pulls away, relapses can happen that set you back – then trying to get back into services and trying to get those resources again can be really tricky.

[Adrian] I believe, Stina, you particularly had issues with trying to get the the relevant supports or the appropriate support. I don’t know if you wanted to add something.

[Stina] No, it’s just surprising how difficult it is to get people to cooperate. The doctors. So I think we have received some good help in some cases. But then when actually healthcare is supposed to be carried out, it doesn’t work. Really, to get people to cooperate, and I think as a parent, you have to be very strong and you have to keep fighting and it’s just difficult to never get any recognition. And I think it’s so strange. And then you get different diagnoses, you get different types of treatment. It should be easy. And I haven’t had the feeling that people are cooperating – I think it should be in the interests of the child, and #1 should be to get the child back to health and to get them back to school again.

[Nadine] Lisha, your story touched on that a little bit as well, just the frustration, the communication between services, and that is absolutely the reality. You know each team will have their funding, their aims and objectives that they need to meet, to achieve and successfully keep that funding from the government, and it becomes an absolute minefield of what you’re navigating. And I think that does get a lot worse as you move into adult services. The services seem to separate even further. An example of that would be autism and ADHD, which aren’t managed together in adult services. They’re managed very, very separately, whereas in children they all come under the umbrella of children’s mental health services. So it’s really tricky sometimes for parents and children and families to understand that, because we as professionals don’t always understand that.

[Lisha] On that  – it was very difficult for us. The only way we got it was was going private and you know, God forbid, we had the money to do that and I know a lot of people don’t… I never would have been diagnosed. And I’ve been told by a lot of doctors that it was all in my head, which even now today I carry that with me… like, “get over it, you’re just making it up…” and that’s a really scary, difficult thing, because I genuinely for many, many years thought, I must be such a terrible person to be making this all up, and seeing what this is doing to my family.  And I was told as well that it was FND [functional neurological disorder], I’ve been told that all girls before the age of 16 have seizures, I’ve been told, “oh, it’s the stress of your GCSEs [exams]. Like, you’re putting that all on, you’re making it up a lot of times, and that’s very hard to hear. And, you know, thankfully, I have a family who didn’t believe that. But I’m sure in a lot of cases people do believe that and just, you know, go with that, and I think it’s very difficult because there’s not a lot of research on Sydenham’s chorea, and there’s not a lot of awareness, but how much of that is a vicious cycle, because a lot of people probably are misdiagnosed and a lot of people probably have Sydenham’s chorea, but don’t get diagnosed. Therefore there’s no awareness. Therefore the cycle continues.

So yes, I just wanted to to add that in and thank you so much for doing stuff like this.

[Mel] So when she was 15 and and she got diagnosed with FND, basically, that really did traumatise her more, and so we just left the NHS for probably, well, for six years until last year. And then I read up about PANDAS again. So when she was 15, we asked for a PANDAS test and this is how we ended up finding out she had Sydenham’s chorea, and the doctor said no, it’s not that.

We were treating her for Lyme disease, and we were doing herbal and various other things like that. And it was only a year ago that I got the PANDAS test done. And then I found Doctor Ubhi on Facebook, typing in PANDAS. And then we went to him, and that’s where we’ve ended up, where we are now.

[Nikki] What I was gonna say is, I think once you’ve got the medical side sorted and obviously your child is on medication, it is also asking the questions about what happens after, like, getting Gracie back to walkign again, speech therapy. But you’ve got to keep asking the people within the hospital. I mean, this is all new to me, like it was to them. But yeah, when I see now the treatment, what I’ve asked for for Gracie, for physiotherapy (because obviously there are movements and the leg movements) to get her back to walking, get her out of a wheelchair, speech therapy, physiotherapy, neuro, whatever. We went to Oxford, so they did tests because I was concerned obviously, because it affected her brain, did it have any impact with her learning? Because Grace’s mathematical side really deteriorated. Was it the math side of her brain, was it also affected with Sydenham’s? So I I pushed for every possible test going, to make sure that she was 100% and I think that’s what you have to do. Just push it. Everything. Yeah. If you don’t ask, you won’t get, because you have to ask.

[Nadine] And one of the reasons we designed our information leaflet, was to share with schools, so that you could go into school and say, look, this is what it is, this is what we need support with. Because I think sometimes schools will think, well, they’ve been in primary care and in a hospital, and they don’t get that the impact on them even just from a tiredness perspective, of needing some reasonable adjustments to their school day, so that they can successfully manage and successfully get back to school. So we’re more than happy that that’s there to share. And the lovely team in Italy have translated it and use it as well, which we were very, very honoured about.

So I’m aware of the time and I’m aware that we were moving on to questions and answers with Doctor Morton and I is there any questions out there that you were really want to ask when you signed up for this event, that you really wanted to to share with with the group?

[Katie] The only thing that I’ve wanted to know is whether, because I tried to find studies about symptoms and as we all know, there’s nothing that actually exists. I tried to make use of my uni database as well. And even then, it’s like really limited, what’s actually on there. But I wanted to know if anyone knew, if there are any links between having Sydenham’s as a child and then ending up with some kind of autoimmune disorder. ‘Cause basically I’ve had like pain since I had Sydenham’s as a kid, and we were sort of brushed off for, like, 20 years. And it’s only really recently that people have started taking an interest, and it’s only since then that I’ve had the diagnosis, but I just didn’t know if anyone was aware of any other sort of studies or any patterns of other adults that have ended up with something similar?

[Michael Eyre] Yeah, I think there is an association. That people who are susceptible to Sydenham’s, there must be genetic factors there. We don’t know what they are, but they’re probably shared genetic factors with other inflammatory conditions. Anti-phospholipid syndrome is something that I’ve seen in association with Sydenham’s, so I think it’s definitely plausible that there are associations, and if you ask about the family history of patients with Sydenham’s, they are more likely to have a family history of inflammatory conditions than a random person.

[Stina] I’m just wondering when it was mentioned, regarding pain, I don’t have experience of how to treat this, because my daughter had severe pain in her joints and generally in her legs, and also of course headaches and stuff. But I think the pain in her legs is what she’s complaining mostly,  sometimes big joints and sometimes the smaller joints (fingers and hands), but the legs are quite severe pain.

Any particular recommendation for medication or anything else that could help her?

[Nadine] Does she take over the counter paracetamol [acetominophen] and Brufen [ibuprofen]?

[Stina] Yes, yes.You make it better, but I think she’s still in pain and has inflammation, so it’s just if there’s anything else you would recommend.

[Nadine] So part of me would say asking for physiotherapy as well. I think it’s really important. There’s the physiotherapist and there’s the occupational therapist. I think they can always help if you know if you’re not wanting to rely on medication alone.

And my other thoughts would be obviously having someone to talk to about how she’s feeling because I know when you’re feeling uncomfortable, when you’re feeling in pain, having somebody to talk to and to share that with can can be helpful, because distraction is one of the biggest things that can help. But so is communication. And obviously if she’s sleeping well, as well.

[Stina] Yeah, during the night. But it’s difficult to relax and I feel like the the pain is making it difficult for her to move, and therefore she doesn’t like to go to physiotherapist because I’ve been trying to take her to physiotherapist – she always complained the pain is too bad, so she doesn’t want to be there.

[Michael Morton] Yeah, I suppose I just wanted to say that we tend to forget when we talk about Sydenham’s chorea, that rheumatic fever gets its name because of rheumatic [joint] pain, and the original condition presented with pain. And then people began to see the association between pain and chorea, and pain and carditis (heart disease), and what we now know as rheumatic fever grew out of a historical process of linking these things together.

So we have to think about pain in this story, and I suppose pain management is a very skilled task, but it starts by understanding that pain is part of something. But that doesn’t necessarily mean that there’s a something that can be treated, other than the pain, and this is the real challenge with pain, that it’s a sign of something going wrong. But once it becomes chronic, the treatment is often not trying to treat the underlying cause.

Trying to manage the pain, pain management, can be a very complex task involving all these things. Nadine’s talking about from movement to conversation to medication. Basically, rehabilitation approaches again, so it’s disentangling what can be done about the Sydenham’s story part of the pain, and what can be done about the pain that is now here, as opposed, maybe, the pain that was there when it all began, because pain can get very stuck. So it’s a rich and complex world… And I’m sorry, I can’t be more helpful, but I I think the main thing is to keep a very broad approach and be open to whatever helps.

[Annie] Yeah, it’s not actually a question. It’s just I wanted to attest, Amanda, her pain got really, really much better when she got IVIG [Intravenous immunoglobulin]. And now after one year with the IVIG every 6th week, we now reduce her NSAID [non steroidal anti-inflammatory] in half, and her pain isn’t coming back and it’s just much, much better. All her limbs, the pain in the limbs, are so much better with IVIG. So I hope you get the same results.

[Stina] Yeah, that sounds really good. We have actually tried it before and that’s what happened the last time, but they stopped it and then the pain came back. So she was moving and she was feeling quite OK at the beginning of this year, but now we haven’t had any IVIG for five months. And now she’s in severe pain again. So I I hope this will bring her back to where we were in the beginning of the year.

[Nadine] And I think the key thing there, just building on what Michael was saying, is pain can change. So sometimes it’ll be that the feeling of pain, but pain is a really broad thing, and it’s sometimes understanding what is the pain that’s being described, and particularly in children that can be tricky because pain is an expression of distress. Sometimes it’s a way of saying, “Mummy, I’m not OK.”, without necessarily maybe having that intense strong feeling in the body.

[Stina] No. I can feel her joints. It’s definitely actually complaining about the joints and I can feel that they are soft. So there is a joint problem.

[Annie] I just have one. Has anyone noticed a problem, my daughter, she has severe problem of speaking for herself? She turns to me, she has the separation anxiety. But she’s, in certain situations, especially meeting doctors, she’s like a stone face, you know, like, no expression. And I’m afraid that they’re going to start thinking that she has autism. It’s not the case because it’s, you know it, it doesn’t defer in that way in autism, but it kind of scares me. Have you had that experience, that they just can’t..  she can tell me, “I want to speak, but I can’t”. It’s like a blocking.

[Nadine] It’s really, really common, and in our approach in liaison [psychiatry] would be that Doctor Morton would take me along with him, or one of the other nurses, andyou would have your first session together, but then the expectation was that we would separate and come back at the end. So then your daughter would get time with the nurse or Doctor Morton, just depending, and the parent would spend time with the other, and then we would come back together at the end to agree a plan from both sides, you know. The importance of everybody’s opinion and expectations. But I I can’t stress enough that communication is really important and in liaison psychiatry, we didn’t tend to put labels on things, we didn’t tend to talk about “separation anxiety” and “anxiety” with the children. It was more just that they had this space to explore, and it was a confidential space where they could explore. So sometimes we might have been.. I remember, one of the other nurses very much would play games and be very active, tended to use pencils and colouring and some of that non-directive approach to exploring how they were feeling. Each session might be different. I could have went into a session with lots of, you know, theory and books and ideas, and come out it with a totally different session. But the main thing is about that protected space, and just allowing the child the time to have a voice… sometimes then I would need to be that voice when we went back in the room with the big scary doctor, to be able to communicate.

Or sometimes they didn’t want to let their parents down by saying how they were feeling. There’s lots of things that can be going on in the dynamic of the room, but I do think it’s really important that the child has somebody who they can have some communication with away from maybe the doctor and the parent, and for parents that can be a scary thing. A really scary thing, to allow someone else to speak to your child, and not really be 100% sure about what’s been said. So there’s an element of trust that comes into it as well.

[Adrian] I’m sure there’s there’s other things that people want to say. I know Sadiq had to leave and he’s had experience of chronic pain with his son. So just to say thank you to our speakers. Thank you, Nikki, so much for sharing your story. Thank you, Michael, for your your presentation, but also all of that hard work you’ve been doing, seems like the last two years, maybe more now. Thank you, Nadine, for for sharing. And it’s been a real pleasure to see you all here. And to hear your contributions. And I wish you all the best for the future and and and please do stay in touch.