Introduction

Sickle cell is a life-long debilitating condition that predominantly affects people of black African and black African Caribbean background.

In episode 1 of our podcast series Dr Dianne Addei, a consultant in public health medicine and NHS England’s lead for sickle-cell services, is joined by patient, Stephanie George, and Dr Rossby Awadzi, a North-west London haematology doctor to help clinicians, patients and the public to get a better understanding of sickle cell. Stephanie explains what it is like to live with sickle cell, while Dianne and Rossby discuss the benefits of genetic blood testing ahead of blood transfusions, the sickle cell patient education programme, role of staff education and Dianne provides an update on the new NHS sickle cell disorder emergency department by-pass units.

Transcript

Dianne Addei – NHS England [00.00 – 00.15]

Welcome to NHS England health inequalities improvement program’s first sickle-cell podcast
series. We created the series to coincide with national sickle-cell month and black history month

Stephanie George – patient [00.15 – 00.27]
Throughout out this series we are talking to people like me who have sickle cell, as we learn more about the disorder and explore what is happening in the NHS to improve the care adults and children receive in a crisis

Stephanie George – patient [00.27 – 00.55]
Hi everyone, my name is Stephanie George I am a sickle cell warrior. So, a lot of the stuff that I do, is raising awareness around sickle cell.

I’m also one of the patients on the NHS sickle-cell patient advisory group. I joined that, I think, I thought like two years ago, after sharing my experience of the care that I received.

I also work for the society where I do peer-to-peer mentoring for children and young people with sickle cell.

Dr Rossby Awadzi [00:56]
My name is Dr Rossby Awadzi, I’m one of the doctors in North-west London. I work in haematology on a medical daycare unit where I manage and treat patients who come with sickle cell crisis. I’m also the clinical lead for NHS England educational campaign 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

Dianne Addei – NHS England [01:24 – 01.39]

Hi 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.

Dr Rossby Awadzi [01.39 – 1.52]
Hi Stephanie, thank you for being here today.  So, as we know sickle cell is a lifelong condition that can be unpredictable, but what kind of impact does the condition have on your life?

Stephanie George – patient [01.52 – 04.07]
As you said it is unpredictable. You know I have times, I’ve woken up and I’ve been felt fine and then you know before you know it I am in A&E because I’m having pain or there’s times I’ve gone out with my friends and I’ve had to leave the event early to go to hospital all day you know because I don’t feel well and so it is like it’s so unpredictable night days are the same and it has had a significant impact on my life, because I had, so I had a stroke as a child which is one of the complications I can happen with sickle cell, I’ve had regular blood transition since I was  about 10 years old, but even with the transfusions, it’s still you know… there are times I have been admitted into hospital,  I still do have regular A&E attendance and I think the last one I had was like, maybe two weeks ago.

so it’s always … it’s just that things are constantly changing. I’m having to make sure I wrap up warm as much as I can to minimise the risk of getting sick – but obviously I don’t have that much control over it. So, yeah wrapping-up warm, making sure that I’ve got my medicines with me if I’m going out just in case the pain starts, just been in a central location as well sometimes, because if I do need to go hospital, I don’t want it to be too far away from me for whenever I’m going out with my friends.

My friends know about it, so they also kind of be that prepared if anything does go wrong, and so, yeah, it’s just day-to-day life is challenging. And when those episodes of pain do come! It can start off mild and then you know I got like a regimen in my head of what medication I should take, but sometimes it can just start off like really intense pain and it can come on so quickly that no matter preparation you’ve done… that kind of goes out the window, and you just have to always be constantly adapting to the situation that you’re in.

So it affects me every day and it’s weird. It affects me every day, but I don’t know a life without it. I’ve never not had sickle-cell.

Dr Rossby Awadzi [04.07 – 04.12]
Can you tell me about particular crisis you’ve had that really sticks in your mind?

Stephanie George – patient [04.12]
Two-and-a-half years ago, and I was in A&E I was really, really, unwell. I’d been there all day and when the haematology team were there it was… you know my medication was being given on time and I was being looked after really well.

Then when haematology had gone off, even though they are on call, you know they weren’t being utilised on call. It was like, ‘Oh they’ve gone now’.

The nurse that was looking after me all day he gave me, like, the last medication at the end of his shift, this was like 7 o’clock when I got that.

And then by the time handover had come, the two nurses that were there, put me in the corner in A&E. So you can’t see me, I’m behind the curtain and I pressed the call button. I could hear them talking, and at this point I am like screaming, I’m crying, and when they did [come] I think it would’ve been maybe two and a half hours after my last medication. Because morphine is a controlled drug, so two nurses need to check it and sign it.

And I thought, and I really thought, I was gonna die that night because I just knew it was getting worse. It wasn’t being controlled. And I think it was just a really scary feeling, knowing that if anything happens to me, what would they tell my parents? What would they tell my sibling? And I thought about Evan and then I thought about what he must’ve experienced. Knowing that you know your body and that something is wrong, but staff aren’t taking you seriously.

And I also called my sickle cell matron. This is about now 10 o’clock, 11 o’clock at night. She shouldn’t answer. She has finished at like, you know, five pm, but she answered because she knows that if I’m calling that late something is wrong, but she answered.

And I told her ‘Oh my God I think they’re gonna kill me this is what’s happening’ and you can hear at this point … I’m just like this … you could just hear my breathing was changing. And she, from her house, was trying to get things moved around.

The nurses were upset but I was just like I can’t die here, not like [this] this isn’t, this shouldn’t be the way for me to go. And the fear, the fear that I felt, (Sorry) the fear that I felt, that guys is just too scary, because you know that something is wrong and you know your body starts telling you that you know. ‘You’re fine with that, there’s nothing to help you’.

Eventually I got moved, and that I felt so sorry, because that nurse, she was the only person on that unit and you could see that she was trying to help. She’s trying to do everything she could help me. Eventually I got moved to resuscitation, and then they called the anaesthetist and started my PCA [patient controlled analgesia].

But just not been listening to, and feeling so unsafe in an environment that’s hard. (I’m sorry) (I’m sorry) and people are dying in the UK. Yeah and that’s my, difficult crisis, I won’t ever forget.

I have heard of people with sickle cell who, just didn’t have … I think you’re so ill you just don’t even have the strength to do that. But the fact that, you know, I have to constantly stand up for myself, even when I’m seriously sick. And, just, you know, going on social media because if anything happened, so people saw me and they knew that I did everything I physically can. Those things that scare me.

Dr Rossby Awadzi [08.39]
Thank you, sorry to hear about your experience.

Dianne Addei – NHS England [08.42 – 09.45]

Stephanie, I’ve heard you talk about this before and it never seems, you know, it’s always so moving and it’s a very difficult experience. You know I said earlier about sickle cell pain, and it’s one of those things that has to be felt. You know it is felt, that there is no amount where … you know we’ve all been through pain but you cannot understand the pain that a sickle cell patient goes through.

You know you and we we’ve got to be sensitive about it as healthcare practitioners, as members of the public, we’ve got to understand that this there is pain and there is pain and sickle cell pain is the latter. You know it’s significant pain.

And I’m very sorry we let you down.

Stephanie George – patient [09.45 – 10.10]
So moving on. Thank you…
As stated before I have regular blood transfusions, and I did have, I think I was the first to have it this year, the genetic blood matching test on the NHS. Can you tell me more about this service and do you think patients know enough about it?

Dianne Addei – NHS England [10.10]

So, we all know, well we know very little about our blood groups. Very few people know what their blood group is, but even the people who know, only know about the main blood grouping system which is what we call the A,B,O system. So someone will say, blood group A, I’m blood group B. I’m blood group O but apart from those blood groups, there are over 300 blood groups that we call the minor blood groups. And each of these minor blood groups, you have what we call it the antigen. It’s the main thing, so if you take the capsule as the antigen, it has something that we call an antibody. That causes the body to react when we see something. Or when the body sees something that it doesn’t know, it develops antibodies to it.

So when you have the blood group A and it comes in contact with blood group O it’s saying this is a strange blood group so I have to make sure it doesn’t destroy me, so it’s develops an antibody to it.

If you think about it, we know our ABO blood group system we don’t know about the 300 other blood groups and any time the test your blood group when you go in for a transfusion they will test to match it. She’s blood group A but they generally not tested for the 300 unknown blood groups.

So you’ll get, because you are getting repeated transfusions and they are not so significant when you come in contact immediately, but as you keep getting it, your body starts reacting to it. And so, you could get to a point in time where they say you’ve had a transfusion reaction. What that reaction is basically let’s say I’m blood group A and their giving me so much other blood group, so blood group has spotted this one and spotted this one and spotted that one and so when you give it to me that I just destroy it. And that’s what happens normally.

NHS England and NHS blood transfusion service has now made [this test] available to all patients with sickle cell and other trans-red cell conditions, that we will test you, not just for your ABO but we will test you for the 300 other minor blood groups which antibodies are present, so that when you come in and you need a transfusion, we are checking the blood that has been donated. We will get to the point where that will also be tested. The whole idea is to make sure that we don’t give you something that you will react to.

Patients know about it [testing] yes, we put out some information, but I don’t think everybody knows about it because not everybody has been tested. The initial program, it will take some time and as people go in for their annual reviews they will get offered testing, and we will encourage people to get the testing done.

So, I think from an NHS England and NHS blood and transfusion perspective, we would like more people to get to know about it we would like to communicate that this testing is available and encourage patients to go to opt-in when they go in for the reviews or when their at hospital for any other reason. It will improve the safety of transfusions and that’s quite an important thing for people who, especially, have repeated transfusions.

Stephanie George – patient [14.56]
Thank you that’s good

Dianne Addei – NHS England [14.58]

And I should add quickly, the information [about the test] is on the NHS blood and transfusion site, and sickle cell society and other advocates are putting information out to patient and there will definitely be information on social media.

Stephanie George – patient [15.17]
I’m very happy that it’s out there. And I do think it’s something that is needed, because I said my sister she has antibodies, so she can’t have blood transitions as regularly. But I wish that, if this test was you know…years ago before she developed the antibodies it would’ve been a great test, at least then we know we’re trying to get as close a match as possible.

People that are listening, they might wonder why we concerned about people potentially developing antibodies after blood transitions?

Dr Rossby Awadzi [16.00 – 18.34]
It goes off from what Dianne was saying. So, typically we have a A B and O blood group, so
universally tested, then you have the rhesus factors positive or negative so that’s typical. Now I said when your screen for a blood transfusion, you initially screen for those blood group types. But every time you introduce something foreign into the body, the body will pick up the less likely to be seen proteins, so rare proteins will start to pick up as well. It will say ‘I don’t like that one either’ and it will create an immune response against that. So you need to send those bloods off to be further matched for the person to make sure the blood they get, is more closely in line with what their blood is like. The difficulty with that is that blood is rarer to obtain, and sometimes these rare matches can be due to the ethnicity of the person requiring blood. This is something that blood and transplant talk about [ethnically matched blood.] and that’s the reason why. Because some of these proteins that are seen, are seen within communities, so you need more of those communities to try and donate. Because it [blood donation] doesn’t happen as much [in these communities] shortage of these kind of blood groups and for some patients who have sickle cell, they go through some red cell exchange which removes the red cells from the person with sickle cell and replaces it with blood from donation, and I typically prescribed anywhere between 8 to 10 Bags of blood. If you’ve got this rare [blood type], or if this person is developing antibodies and you only accept a rare form of blood the shortage is much more prevalent and sometimes we can’t adequately transfuse some patients safely. So, if we continually transfuse using blood that isn’t appropriately matched up, these problems and the shortages become more obvious and some people will go without transfusion and that’s the main concern with developing antibodies.

Stephanie George – patient [18.34 – 18.44]
Can you tell me more about the patient education series that you run and how that came about?

Dr Rossby Awadzi [18.44 – 22.00]
The series is a free platform that we created to educate people with the condition about sickle cell and everything you didn’t know about sickle cell. So I tried to provide in a medical school setting, completely impartial, the same way I would’ve been taught in medical school. We try and build it from the cells all the way through to treatment.

It started because, so I used to teach in secondary schools, so I used to merge GCSE, A’levels with healthcare teaching because I found that you can’t be led astray if you know one plus one equals two. That’s the best way I could describe it. Because I’m from north London, and there are people who are quite against general Western healthcare practices, because there’s a lot of mistrust within the community. So I thought I’ll break down this mistrust, so I thought as you were learning about science, we throw the healthcare in there, and allow people to make informed decisions based on fact.

And during my time on the medical daycare unit, because I manage crisis, of course people are with me the entire day, based with me the entire day and I’d often say ‘your blood results show this, because of this’ and then get more questions. So, I remember there were two people with sickle cell on the unit, in the bed sitting next to each other talking, cause I just spoken to one about the condition and they said ‘that was quite interesting can you explain more?’ So, I made a very quick presentation, and while they were in the bed I showed the presentation to them both, ‘oh this is quite good, why don’t you do more of it?’ I said okay I’ll make a little [platform] link and you guys can watch it on a random Thursday evening if you feel like it. I gave it [the link] to 4 people and then we had 50 people attend, on the first ever one. This is, this is very, quite overwhelming, so that was just about understanding what the blood test mean part one. So I’m gonna do part two in four weeks’ time. And then we had 150 at part two, I didn’t, I didn’t send the link to more people, I just put in on [platform] and it started to fly.

And then, a few people in NHS England had attended and said this is something that it seems like it’s doing really well, we’re happy to support, and they did. And my friend who at the time had just started working for Vogue doing videography, I said let’s make this even more interesting, I want it to be a case where you can sit at home, press play and watch it , and like, just have a great time while you’re learning. And that’s when we had 350 people attend and then we had our subsequent ones had more than 500 people attend each one.

So, I believe, we are the first global largest workshop online for sickle cell. We may be the only one, but I claim that we were the largest as well! So it’s been it’s been going really, really, well. And that’s how we’ve moved onto workshops and developing the kind of website to allow people to tap in I don’t have a good time learning about it.

Dr Dianne Addei [22.00 – 22.10]
In terms of a sickle cell crisis or blood transfusion, Rossby, what do you cover in your education series?

Dr Rossby Awadzi [22.10 – 23.32]
What we’ve covered the education series is the basis of crisis in terms of sickle cell or crisis, so what are the triggers, what happens and understanding the process of sickle cell crisis and how to break the cycle of continual crisis. The science to talk about and what research is currently undergoing to try and find preventative ways.

In terms of transfusion we do talk about transfusion science, so all the stuff I have discussed – going a lot deeper into the understanding of having appropriately matched blood, and the reason is some of our listeners back home in Africa tend to discuss with me, mismatch testing of their blood groups and some centres and we, you do educate on the need for appropriate testing and why there’s quite a robust process here in the UK and we invite you for screening. That’s not to say that abroad care is substandard ,but there are some centres that don’t have as much rigorous quality assurance and testing as we do here, so that is the stuff that we cover, We are starting to do some live workshops as well, when we play games for people to understand what the blood groups actually mean and what antigen and antibodies are, as you discussed.

Stephanie George – patient [23.32]

That’s really good, I’m glad that at least, you know, I think, the more correct information being out there, different ways for people to accept it. Can I just ask, how do people join the course?

Dr Rossby Awadzi [23.50 – 24.16]
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 around London 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.

Stephanie George – patient [24.16 – 24.19]

Later we are talking to Carly about Manchester sickle-cell disorder emergency emergency bypass unit –  that is quite a long name – Dianne, who has a unit and how will they improve care?

Dianne Addei – NHS England [24.19 – 24.38]

These units have been set up and in some places they’re called ambulatory care and basically. The whole idea is that you get access to care and pain relief when you’re in a crisis without having long to wait in an emergency unit. That’s why it’s called an emergency unit bypass. You know your bypassing A&E basically.

So, some of these units already existed in the country, what we’ve done is that in places like London, Manchester, where it didn’t exist, we’re setting up these units. In London you can go to Hammersmith, where there’s such a unit has been running for maybe four or five years – and they are expanding their capacity. In the south-west you’ve got St George’s Hospital, which has also set up a unit. In North London you’ve got North Middlesex Hospital which has a unit. In South London, Southeast London you’ve got Lewisham General Hospital.

So, in these places the patient can go into almost a purpose built units, where the people who staff these units, understand what it is to have a sickle cell crisis. They’ve been trained so you would call in, go in, and there isn’t going to be that wait to get access to pain relief because one they know you are coming in, two they understand it and then they make provisions to care for these patients.

I should add that, places for example like Hammersmith already had this unit, and there was also a place in Nottingham. It was a patient from these areas who had utilised these facilities who flagged that, actually, when we get a crisis and we go into our units, they know exactly what we need, they are expecting us and we don’t experience the delays and the difficulties that other patients are experiencing and that is why NHS England has increased the number of units providing this type of care. And the idea is to improve care for all patients faster. They’re going to be open 24/7 and every day, not just during the day because some places provide care during the day and then at night you have to go through A&E or it’s not available over the weekend, but this this is definitely something that improves access to care rapidly and I’ve seen Carl report on the difference it has made to his care in terms of the time he stays in hospital, the care he receives, the relationship even with the medical staff, the way that has changed and the way the experience of a crisis, you know significantly improves the quality of care, and the impact on his life.

We also looked at the evidence in terms of what has been published in places that have done this and there is some to show the patient stays in hospital shorter, there’s fewer admissions and there’s general better quality of life. Significantly improves people’s quality of life. So I hope that we will be able to bring the service to a lot more patients.

Stephanie George – patient [29.00]

I was very excited I think when it was coming out. I was excited to hear about it.

I’m gonna ask you both, that we’re gonna also hear about the ACT NOW approach for sickle cell crisis care that has been referenced in guidance from the royal College of emergency medicine, that any team or professional can use in a crisis. Educating staff about sickle cell is an important element of crisis care, why do you think that more education is needed?

Dr Rossby Awadzi [29.36]
When staff see those with sickle cell, you have those who are very familiar ie your haematologist, and you have those who are not so familiar. Now the reason why it’s important for staff to continue to be educated, is guidance changes all the time. that’s the first thing, so how you would treat sickle cell 5, 10 years ago, is very different to what the guidance should be for now, because the research changes, the work around it changes a lot. If you went to med school 10 years ago and we talk how to treat sickle cell, you shouldn’t be surprised that it’s gonna change down the line, not just with the painkillers given but also with things like the antibiotics recommended as that changes quite frequently as well. And also, some people may not see those with sickle cell for many many years, so it’s important to always refresh your knowledge as a medical professional I think, because you never know who’s gonna come next through the door. So it’s very important, especially as a condition that’s as unpredictable as sickle cell, Its very important to be aware of the red flags the key symptoms, management and what needs to be, and what needs to be done, hence why the care plan is such a good thing as well, because it minimises human error. And also educate staff not just to treat patients but also to understand the condition, and what it does to the person in front of you sometimes as well, because having an empathy is, is a big thing in regards to treating pain as well. If you empathise improperly with the pain, you’re not going to treat it as adequately as you should, if you don’t believe in it. So all these things tied into education change your behaviours, changing attitudes, keeping up-to-date the guidance and I think only education can really provide this for healthcare staff.

What has the best way to provide it to healthcare staff and it’s really nice to know that people with sickle cell are involved in pushing the education towards staff, as well, what you do Stephanie, it’s really nice to see, that you can see things changing because of the patient voice actually and it’s really nice, really nice to see yeah.

Dianne Addei – NHS England [31.58]

Yeah, I think the only thing to add is that, a lot of people think that when you go to hospital you should only see a haematologist and they are the only people who will care for you. But the practical issue that we’ve got to realise is that there are in the UK for example, there are over 200 sites in which you can access care when you have a sickle cell crisis. We don’t have enough haematologist to staff each of these sites. It will not be realistic to expect that every site would have a sickle cell specialist.

That’s not realistic so we’ve got to train everybody. They’ve got to have a basic knowledge so that they can manage patients in the first run, and then know enough to also refer when specialist care is required. So, training healthcare practitioners is very important. I mean I cannot downplay it, it’s very important and that’s why NHS England is actually working with an education team from North Central London to develop and make available training material that all trusts can access and use in training all their healthcare practitioners and I think that’s very important. Once that is done you know, we’ll do our best we make it available and let people know the training is available and to encourage their staff to use it.

Dr Rossby [33.51]

It’s very important, that we shouldn’t take lightly, I think not just clinically but I think as a whole is what it is to live with such unpredictable and quite a very serious condition. What that does not just physically, but psychologically. Things things that we take you for granted as people who don’t have the condition, people with sickle vell will have to fight for someone of the basic things that we take for granted, such as air-conditioning was mentioned, opening windows. You know, just taking things for granted.

If you don’t understand what the person next to you, know what they’re going through, you never … you subconsciously begin to isolate people, you know. Think about five year-old on the playground who suddenly stopped playing because they’re in pain. What the other kids think, ‘they don’t want to play with me’. Or in the workplace, where people have to go off sick from work and the other staff thinking ‘ahh they’re just skiving off, they were fine yesterday’. You know, not understanding can have such a profound impact, not just in hospital but outside as well.  

That’s why I think education is really important across-the-board. I think if we can all be able to understand each other, work together as healthcare professionals, carers, friends, family, all those who have no relation at all, I think we’re making really big strides to support those with the condition. One thing I’m happy, really happy about, is that I’m seeing  – I’ve only been a doctor maybe six years now – and I can already see the shifts in attitudes towards sickle cell. I can start to see really big, you know improvements and what’s nice to see is that those with sickle cell at the centre of driving all of this work here, they help lead, we create the research. They say this is working this is not working. And as Dianne was saying with the hyper acute units [Sickle cell emergency department bypass units] it was patients who they said took that idea and brought it across, and now we’re seeing such a big change so the patient voice is very, very, very, very powerful one and it’s very brave to share your experiences in order to make change so, you know, commending to Stephanie and all those who the condition who you know shout out loud and we do our best on our side to try and support as well.

Dianne Addei – NHS England [36.13]

NHS England takes responsibility to provide good quality care to which every patient should have access. When they come in for care we want them to have an excellent experience and ultimately we want them to also have optimal outcomes of care, that’s the aim of NHS England for every NHS organisation delivering care.

From an organisation perspective we seemed to have some challenges with sickle cell and a lot of work has been done to ensure patients have an excellent experience and experience, you know, an excellent experience and experience optimal outcomes, and we will keep pushing to ensure our patients get that.

Stephanie George – patient [37.09]

Just looking over my life, from childhood into adulthood, I know there’s so much work that still needs to be done but things are changing, and what has been done. But because a lot of us have had experiences of bad, those experiences that kind of stick out in your mind a lot, but you know having the education series is so important, doing stuff with NHS England and the amount of work NHS England have put out since that No one’s listening report is, you know, it is important as well.

And I just think for the community, I’ve seen a lot of us who advocate for ourselves and for others as well. And that is something that I’m really, really proud of to be part of. Because at the end of the day, we don’t want to see people 10, 15, 20 years down the line, to still be going through what we’re going through now, and what people have gone through before.

Thank you Stephanie and Rossby for an interesting conversation, and how you have used your personal experiences to improve health.

Thanks Dianne and Rossby [38.17]

Dr Rossby

Thank you very much