Welcome to NHS England health inequalities improvement programs first sickle-cell podcast series. We have created the series to coincide with black history month.
Stephanie George – patient
Hi everyone, my name is Stephanie George. I am a sickle cell warrior.
Amanda Cope – Birmingham Women’s and Children’s NHS Foundation Trust
Hi, I’m Amanda. I am an advanced nurse practitioner in haemoglobinopathies at Birmingham Children’s Hospital. Part of my role I see patients when they come in unwell, as in-patients on the ward, I also do outpatient clinics and get to know all of our lovely families in the outpatient setting as well. I’m one of the lead nurses for the HCC (haemoglobinopothy coordination centres) in paediatrics.
Isabel Adams – Birmingham Women’s and Children’s NHS Foundation Trust
Hi, I’m Isabel. Most people call me Izzy. I’m an education nurse for haemoglobinopathy across our HCC region, which is the West Midlands. I spend a lot of my time teaching now, previous to that I was a clinical nurse specialist.
Dr Dianne Addei – NHS England
Hello everyone, I’m Dr Dianne Addei, I’m a consultant in public health medicine and I’m the lead for delivering NHS England quality improvement program for sickle cell services.
Stephanie George
On this podcast we are looking at sickle cell and care in childhood.
Amanda, [please] describe the signs to look out for to indicate when to get help for a child who is in a sickle cell crisis.
Amanda Cope
Sickle cell anaemia is often the name of the condition. So, some people call it sickle. Some people call it sickle cell disease. Some people call it sickle cell anaemia.
It’s generally like a broad term for the condition.
Sickle Cell is a genetic condition. Both parents have to be carriers to pass it on to their child, or have the condition themselves. And in the UK, we pick this condition up from our neonatal screening tests, when babies are born. If patients come to us that haven’t been born in the UK, then we may pick it up at a later date when they attend hospital, for example, or they may have been diagnosed in another country.
The condition itself affects the red blood cells and your red blood cells help to carry oxygen around your body. In sickle cell, these red cells, are misshapen, and they become a crescent shape usually, and that crescent shape is why it’s called sickle cell, because the crescent shape is very similar to a farmer’s sickle. Those cells can get tangled or trapped in the small blood vessels.
So, when we talk about patients having a sickle cell crisis, the red blood cells have got trapped or tangled together. And those cells then cause pain. And that pain can be anywhere in the body, but most commonly in the hands, feet, limbs and often in the back. This could be in isolation, or it could be alongside another complication and there are lots of different complications of sickle cell. Patients can have chest problems, they can become anaemic due to things like viruses or infection. Anaemia itself means having a low haemoglobin. Generally, if we’re saying that somebody with sickle cell has become anaemic, it means that their hemoglobin has become significantly lower than what is normal for them. Patients could also have problems with their spleen or even things like stroke. So, sickle cell anaemia is kind of generally used as a broad term and a sickle cell crisis is usually just sort of the term used to describe pain.
Some children are very good at explaining that there’s something wrong. They may point to where the problem is. They may complain of pain, or sometimes parents might notice that their children are unusually quiet or not right. Occasionally you can have swelling to the limbs, particularly in very small babies. You can have swellings in the feet, in the fingers, in the toes. But also, just because there’s no swelling doesn’t mean that there’s no crisis. So it can be quite tricky. But parents usually realise that there’s something not quite right with their child, or the child is unusually miserable. Sometimes children will refuse to put their feet on the ground and just decide that they’re not walking because it hurts, or they’ll stop using a particular arm or stop using their hands to do things. So, it can present in a in a variety of ways.
I would just advise parents to seek help if they’re ever worried. Parents are experts in their own children, they know what their children are usually like, they know how they usually behave. I think some kind of routine kind of basic guidelines would be to seek help if your child is having any high fevers or high temperatures; if the pain that they’re experiencing us unmanageable at home, you may have tried paracetamol and ibuprofen for example. If that’s not working, then get in contact with your team, and it may be different in different areas as to who is the best person to contact. It may be the nurses who put you in contact with the medical team. You may have direct access to a ward and it does work differently throughout the country, so I think that’s kind of a local Team that should explain who to contact and when.
I would also advise parents to get advice if their child is having any difficulty in breathing, any loss of consciousness, or even if they’re increasingly lethargic, or a lot more tired than normal, physical weakness or any visual disturbances.
The other thing I would say is, if you get in contact with us, we would rather see a child and reassure you that everything is OK, than have you worrying at home that
there’s something not quite right. And equally, let us know what’s worrying you because it may be that something may be more serious than you first thought.
And we just need to kind of work with you to make sure that your child is safe.
So if you feel that your child is not right, we would much rather hear from you than you sit at home worrying.
Dr Dianne Addei – NHS England
Thank you to Amanda.
Stephanie, what early memories do you have of how your sickle cell was managed in school?
Stephanie George
I know we had a lot of support with the paediatricians, and the specialist nurse. I remember them writing to our school and saying, ‘They do need to be wrapped up warm’, ‘They may have a lot more toilet breaks than other students, because obviously they need to drink plenty of fluids. So primary school was easier. You have one teacher who follows you around school.
Isabel Adams
It is a real milestone when your children start school, but it should be really exciting and positive milestone and we hope to create that for parents by giving them the confidence to let their children go to school and have all the exciting opportunities other children do.
Stephanie George
So, I remember primary school being really, really efficient. They would make sure that we had regular toilet breaks. We would eat fruit in between classes. The head teacher was so, so, supportive. And the letters did help.
Isabel Adams
So the way that we do that is by engaging with schools. Start a conversation with teachers and other support staff at the school. With the parent will put together a care plan. That will outline what the child’s condition is, what medication they’re on, what staff need to look out for in terms of the child being unwell. Who to contact should they have any concerns when to contact parents.
Stephanie George
So, primary school was easier, but secondary school was completely different. Because obviously you have different classes, different teachers. Even though my parents made sure letters were being written and school received the letters, there were times where, teachers just didn’t want to accept what was happening. There was a time where I really needed to go toilet. And she was just like ‘no’. I was like ‘I really need to go, and I’m gonna go toilet, I can’t just sit here I need to go and wee. You’re just going to have to talk to my parents about it, if your going to out me into trouble.’ And I did go and speak to my parents about it, when I got home.
They would put the AC [air temperature control] on or open the windows, and you’d want to put your scarf on, or sometimes put your jacket on, and they would get…
‘No you can’t do that! You need to take your scarf off, you need to take your jacket off’.
If I don’t leave these things on, then I am going to get sick. Which is then going take time off of me at school, then I’m gonna be behind.
Dr Dianne Addei
Stephanie, I am so sorry that even some of the smallest acts of compassion were ignored by the school staff.
Stephanie George
Sometimes, it was really, really hard.
I’m talking about, 15 or 20 years ago. So, I don’t know how far forward that has come for children who are growing up now, who have it?
Isabel Adams
Most schools, we find are really keen to make sure they get everything right for those children. A lot of places around the country do school care plans.
And it’s something we should definitely strive to do. As the child gets older as well, those care plans will really change. So as their medication changes, secondary school, primary schools send their kids swimming; different advice needs to happen at different points during the child’s life. So, it’s really important to keep it up to date.
Alongside that care plan is we provide like a training online kind of session for the teachers. The parent can join if they want to as well. And that gives sort of further explanation and it also allows the school to ask questions, and that can be really useful because you don’t necessarily get everything from a piece of paper. You want to have a chat, say, oh, we do this at our school, is that OK? Or I’m worried about this, what should we do about it? It gives that opportunity for that conversation to happen.
Stephanie George
So, my parents have been very active in my life, and they used to come to so many of our paediatric appointments, because I have it and my sister has it. So sometimes we would have them together.
Isabel Adams
We want to empower the family to be able to have an open dialogue with the school about things like how much has my child had to drink today. Did you remember to put their gloves and their hat on when they went outside so they didn’t get too cold?
You know, we want them to be able to have that daily conversation with that teacher who’s on the door as they take their child in and out each day. So they can feel confident that things are going OK. If for any reason if things aren’t going quite as the family had hoped, or they’re worried about something, then the clinical nurse specialist can get involved again and have more dialogue with the school.
Do some more training or make sure that things are put in place.
Stephanie George
Thank you, that’s good to know.
Dr Dianne Addei
To help patients to receive rapid access to specialists care and pain relief, NHS England is supporting seven NHS trusts to introduce a sickle cell disorder emergency department by-pass unit.
Stephanie
To find out more about these units, I’d like to welcome Chelsea and Carly from Manchester Royal Infirmary to talk about their unit and how they help children and young people prepare for adult services.
Chelsea Soka – Manchester University NHS Foundation Trust
Hello, my name is Chelsea Soka, I am the lead haemoglobinopathy nurse here at MRI.
Carly Honey – Manchester University NHS Foundation Trust
My name’s Carly Honey. And I’m the ward manager for Ward 10 at the Manchester Royal Infirmary, which is the new hyper acute sickle cell unit. You may start to come onto a unit like the sickle cell unit here, from the age of 16. We really support you with that transition from Children’s to adult services.
We are a very, very, friendly unit, but there’s lots in place to help manage that transition and make it as smooth as possible. You get direct admission as well (during a sickle cell crisis). So, we get you up here, or we can get your parent or guardian up here to support you. We do open visiting for parents, so you can have a parent [or guardian] with you at any time, during your entire stay. You get your own room and things like that, so there is lots and lots that we do.
We work really closely with the community team and specialist nurses, and there’s a really robust handover and a lot of the doctors are the same – so they’re friendly faces, you will have seen them before, just in a slightly different environment. You’ll work really closely with the senior nurse. So, like myself, the ward manager, I do a personal review three times a week just to make sure that you’ve got everything you need. I come and see you every day to check-in.
We facilitate engagement activities on the ward, so you don’t get to bored because being in hospital – it can be boring. We got funding for two iPads on the ward and that’s to support people doing their college work, continuing their university work. Can they log-in and do some work from here? And that just supports them with their day-to-day life. We support people completing their exams. So we give people, sort of, day-leave, make sure they’re safe to go. We’ve got on top of their pain relief so they can go and sit their exam, then return to the ward, because they’re not quite ready to go home. But sometimes, these things can’t be rearranged, so where safe to do so, we support it
Especially my team, they are really, really soft and supportive. They’re up doing cutting out, sticking and making bracelets, somebody had a TikTok dance on one of the iPads as all the staff were up and doing it, with one of the younger children.
Chelsea Soka
When Amanda Pritchard actually came to visit the ward, we had a young 17-year-old who had just transitioned from paediatrics. And our paediatrics unit at MRI is very lovely. You know, you get a lot of benefits. When he heard he was coming over to the adult side, it could be a little bit daunting because … we do try to prepare our younger patients, we say if you do end up in A&E this is potentially what you may see. It is a lot different, so knowing that he’s coming to the adult side and it’s pretty much a continuity of care of, I’m gonna by-pass A&E and I’m gonna be treated very well. It was really great for him, his mum was really impressed.
Dr Dianne Addei
Thank you Chelsea and Carly.
Stephanie George
I mentor patients in North Central London, and I know people who don’t have a unit, will be asking, what can be done to try and improve their care?
Isabel Adams
What we try and do, start, what we call the transition process, between sort of about 11 and 13. We’ll start talking to families around helping their child to achieve independence around their condition and their medication. I guess my first piece of advice is that it’s not necessarily age ready, but stage ready. Some children are ready to take full responsibility and know exactly what all of their medication does by the age of 10. And we have got children that can do that and conversely, other children really aren’t ready for that until later on.
The parents will have their own problems or issues around this. So, there is some reluctance to let go of your child because you’ve been very used to managing all of their medication, managing them when they’re poorly, bringing them to appointments. It can be really difficult for parents to let go.
So, the transition work is equally as much for the parents as it is for the young person. But then, alongside sort of helping the parents do that in a staged way, in a way that they can accept goes hand in hand with educating the young person. So, finding out what they know already about their condition and building on that slowly in a stage appropriate way. Providing them the kind of educational stuff that they need. Sign posting them to other people that can help. In Birmingham we are very lucky, we’ve got a local charity that do work with our young people. It’s kind of a multi-faceted process, looking at what they know already, and building on that slowly so that both parents and children can become confident in what they do. Trying to avoid, a young person going to an adult setting at 16, without any real knowledge of what their condition is about or what their medication is for, and suddenly being foisted into a much more adult world and feeling really out of their depth. And parents are really crucial in this. So they’ve got to feel confident that their child knows what they need to know, understands what their medication is for, will be honest about how they’re complying with it and taking it, which is massive, understands how to keep themselves well, knows what to do if they’re not well. Those kind of things will build confidence for the whole family.
Stephanie George
Thank you Amanda, Izzy and Chelsea and Carly for talking to us about sickle cell in children and highlighting the important role of the whole team. I mentor young adults, so from 10 to 24 [yeas of age]. If they suffer from sickle cell, they get allocated to a mentor, and that person will have to be someone who has sickle cell as well. And we meet them every month, for a minimum of six months, but it can be more, once a month.
We can do activities the young person likes, and we just talk about their goals, what they’d like to get out of the programme. So, we can talk about their daily lives. It’s just giving support to young children and young adults as well. Sickle cell can be so isolating, and it’s nice to talk to someone who’s been through what you’re going through now. [Who} can give you advice on certain things. I think its great, especially when they’re transitioning from paediatrics to adult, because a lot of the time you kind of get left by yourself. I remember when I was in paediatrics, you get all the support and then you get to 18 and it’s ‘Ok you just go to adult clinic’ and you don’t really know what’s happening, even though they did like one transition clinic, its not much.
So, I think [the peer mentoring] is gonna be really beneficial for young adults when they get to that point, and I guess going to uni [university] and some people are going outside London and maybe not having the support they would’ve had in paediatrics. So, I think that’s one of the main reasons why they [The Sickle Cell Society] do it.
I’m one of the mentors for North Central London, each other place in London has its own group of mentors as well. It’s just really supporting kids, and supporting young people doing the things they like, with people who understand what they’re going through because everyone, every mentor has to have sickle cell to be a mentor.
Yeah, so that’s why, that’s why, one of the reasons why I signed up, because I was like, it will be nice to help younger people. Cause I wish I had a mentor when I was growing up because a lot of the things you have to figure out yourself, and if we can help young kids, young adults get ready for that and tell them, “Okay, sickle cell is debilitating and very isolating but you know, you can always achieve what you want to achieve. You can got to work, you can go to uni, you just need things put in place or you need support for it.”
Some of the mentees I’ve got, their parents have referred them. So, if you go on The Sickle Cell Society website, and I think if you just put in mentoring, there is …a link to self-refer. Healthcare professionals can refer as well. So, nurses, doctors, GPs they can refer through the form on The Sickle Cell Society website. Then it all goes through to the lead mentors and gets disseminated.
I’m going to end this podcast with a question about education. Hello Dianne and Rossby.
Dr Rossby Awadzi – Heamatology Doctor
My name is Dr Rossby Awadzi, I’m one of the doctors in North-west London
I work in haematology on the medical day care unit. I’m also one of the clinical leads for NHS England’s campaigns on sickle cell and sickle cell trait and I’m also the founder of the Sickle Cell Education series, which is an educational platform designed to educate patients on their condition.
Stephanie George
Picking up on what Izzy and Amanda both said about parental support and also thinking about the support my parents gave me, how much do you think the sickle cell education series supports parents and families, or do you think we need something different?
Dianne Addei
I think It is a good question and I think we will only find out when we’ve made available educational material, and then people feedback to us.
But thinking as a parent, you know, being an expert patient. You cannot for example describe the degree of pain. You can only say, ‘I’m in a lot of pain’ as a patient and communicate that to a parent. Okay, so the parent would be advocating and say ‘My daughter is in a lot of pain’. But there’s a certain level of knowledge and experience that you as a patient goes through, that the advocate has never gone through.
So, I think, there are certain things that an expert patient will need to know which the advocate may not need or appreciate in that same depth. At the same time, the advocate also need it and then there’s also something I would say about repetition. So, as a patient you keep going to hospital, you keep hearing sometimes the same thing often and often, but when you go as an advocate you’re not hearing the same scientific language or medical language, so the patient understanding is very different from your understanding. I think there are aspects that the parent will need to know in very basic language, an emotive language. So, my view is that they need different levels … it needs to be pitched differently. So, that is where I would come from, but what do you think Rossby?
Rossby Awadzi
Since I started the education series, I’ve had a few interesting conversations with some parents, for example, hydroxycarbamide is a common drug used to treat sickle cell. So, essentially how it works is it increases a different type of haemoglobin which doesn’t cause problems within the red cell.
Hydroxycarbamide however, it was known commonly as a chemotherapy drug. So, already you can start to think as a parent, a lot of parents think, “Oh, I’m about to give a chemotherapy drug to my child”.
But after we did the education series, one thing I taught was that the concentration of hydroxycarbamide used in sickle cell is far less than what is used in chemotherapy. So then I had a lot of parents say, “Oh I didn’t know that, I didn’t know we were being monitored this much, I didn’t know it’s been given out in such as small dose, I didn’t understand how it worked”.
Of course, consultants don’t always have the time to explain this to every single parent. So, I feel like, this kind of off-loaded some of that understanding in a way that I just had more time to teach. We did almost a hour on it, so people began to really understand what it did, what it was about.
And the second benefit we saw was with clinical trials as well. So there’s not many drug options for sickle cell. There may be two compared to cystic fibrosis which has less patients and has almost 100 times more than what sickle cell does. And the reason is because of the research, and demystifying research is also part of what the education series aims to do. To understand that clinical trials are not there to make you be guinea pigs. The research is not there to abuse… yourselves or your body or your understanding, but actually to improve the quality of care as well. So breaking that down, we’ve had more uptake amongst people with sickle cell within research as well.
So I think there’s loads… The scope for what education can provide, along with mentoring as well, Stephanie you mentioned you do, I think the two together work really well to empower people more, while the healthcare system is trying to catch-up with… trying to improve the service as well. I think we can all do our bit and really improve the experience. And finally I’ve been a patient in hospital before, and just knowing what gonna happen next also helps the experience a lot. Being able to say “No, I should get this next, I should have this next” … it just makes the experience a lot easier to understand. So, those are the benefits I think I’ve seen so far.
Stephanie George
So Rossby, we spoke about patients being experts in our care, and I wanted to ask, how do people join the course?
Rossby Awadzi
We are remaking the website, so [when that] comes out and make it open but we will put it on Eventbright and then local organisations will also post it on their local channels as well. And we are also creating a social media for it as well, to try and get more bite-size information out to the younger population, so keep an eye keep an eye for that.
Dianne Addei
Thanks Stephanie and Rossby for an interesting discussion and for sharing how your personal experiences and how they have driven you both personally to improve health.
Don’t forget to listen to other podcasts in our sickle cell series. Goodbye.