This is Airing Pain, a programme brought to you by Pain Concern, the UK charity providing information and support for those of us living with pain and for healthcare professionals. I'm Paul Evans, and this edition's been funded by donations from Pain Concern's friends and supporters. There was one time I went to North Wales and wanted to climb Snowdonia, and then I told them that I couldn't. And then they said, why? Why can't you climb the snow down your mountain? Come on, it's just 1,800 and something metres. And they said I couldn't, that I have a condition called sickle cell disease. Sickle cell disease is an inherited disorder, so it's a disease or a disorder that people are born with. Basically, your haemoglobin structure is different to what it should be from when you're born. This means that your red blood cells don't work in the same way. They don't live as long, and so you're often very anemic. I don't think there's a condition that quite creates such suffering from cradle to grave as sickle cell disease.
It's difficult to assess the number of people who live with sickle cell disease in the UK, but based on available data, the Sickle Cell Society estimate that there are around 15,000 people living with it in the UK and at least 250,000 carriers. Sickle cell disease is a disorder of the blood. Dr Elizabeth Rhodes is a consultant haematologist at St George's Hospital in London, and she specialises in looking after people with sickle cell disease. Within your blood, you've got white blood cells that fight infection, platelets that are the small sticky cells that... make sure you don't bleed or clot too much if you've got the right number. And then red blood cells carry the oxygen and your iron around, so they take the oxygen to your tissues that need oxygen. So if they're not functioning correctly, what happens? So what happens in sickle cell is that actually they don't live for very long, so they get destroyed very early. So a normal red blood cell will live for about 120 days, so sort of three months. And in sickle cell disease, it can be as short as two weeks. And so you get all sort of the broken down bits of red blood cells in your blood. And also the red blood cells are a funny shape, and so they don't work as well and they get a bit stuck and your blood flow is quite sticky. So what happens is your bone marrow, which is the factory that makes blood.
It's working many, many times harder than it might do otherwise. So you're always producing red blood cells to carry enough oxygen around. And then your body also then has to sort of process and dispose of the bits that are broken down as well. So you pass that out through your kidneys and your feces as well. And what does it mean to the patient? What they often notice is they're anemic, so can feel quite tired or short of breath at certain times. But the main problem that patients with sickle cell disease get is they get problems with pain. And that's probably due to because the blood flow is so disrupted and those blood cells don't work. The blood is stickier. You get less oxygen delivered to the tissues that need it, particularly in bones. And so patients can experience.
very, very severe at times, episodes of pain that's sometimes required them to come into hospital for treatment. So who gets it? You inherit it, so you will need to have inherited two genes that don't work properly, and you've taken one in from your mother and one from your father. And we see it mostly in patients that have got a family background from Western Africa or the Caribbean, and it's the geographical areas actually where malaria is most prevalent. In the UK, our patients tend to be centred in the big cities, so London and Birmingham. But as people move and jobs move and universities move and lives change, we're seeing it more and more all around the UK. Consultant haematologist Dr Elizabeth Rhodes. Now, one of those areas in West Africa where malaria is prevalent is Nigeria. That's where Khadija Jose is from. She's currently a PhD student at Cardiff University and she has sickle cell disease. I got the sickle genes from my parents who are carriers. I fall sick quite a lot because of that. It causes pain in my bones. Maybe if I don't have good flow.
Because sickle cell, basically, you probably would have good flow of your cells because your cells are kind of like the letter C and they don't flow properly in your veins. So sometimes it gets stuck and that causes episodes of pain. So for me, that happens when I'm stressed out or I'm cold or I have malaria, which happens to me quite a lot when I'm in Nigeria. So I fall sick. I'm in a lot of pain for days or weeks. I was going to ask how long you've had this. You've had this from birth.
When did it become an issue? According to my mum, I fell sick the first time when I was about three months old. And while I was growing up, as far as I remember, I used to go to the hospital a lot. I was always in pain from when I was age four to 13 or something. Then it got a bit better. When I was a child, it was more intense for me. I'm interested in that you said that maybe it showed itself when you were three months old. Now, you won't remember this. But what would your mother remember of that?
They thought that my dad was AA, that's a genotype, and my mom was a carrier of sickle cell, meaning that they were not going to have a child who had a sickle cell disease. As far as it would go, they would only have a child that was a carrier. But the genetic tests were done wrong for my father. He was a carrier as well. So what my mom said was when he found out that I had sickle cell, they were really shocked about it because they weren't expecting.
something like that to happen so she just said I used to fall sick quite a lot and well in school I know I couldn't do much of you know very physical activities if you're a carrier you probably would need to take a few medicines maybe folic acid but you can be a carrier and you don't really need any medical care because the sickle gene itself was basically as a way to be resistant to malaria so it was actually a good thing because the genotypes we have AA we have AS the S was kind of like the genetic mutation of the body in Africa to fight malaria So if you have AS, genotype, then you're fine. You're basically resistant to malaria. But if you are AA, which is when you don't have any sickle gene, you're very healthy, but sometimes you fall sick when mosquitoes bite you, but otherwise you're fine. But then if you are sickle cell, you have no protection from malaria and you fall sick all the time. So it's kind of being AS is like the ideal thing, but not being sickle cell. Let me get that right. So if you're a carrier, you have some sort of immunity against malaria. Yes, you do.
For people who have the serious version of this disease, there is a gradation. It's quite heterogeneic in its make-up. There are people who have it really, really badly and there are people who don't have it so badly. Even if you don't have it badly, it can really interrupt your school life and your work life. But if you have it badly, you are in agony is not too bad a word, on a daily basis, from noon till night.
And so a serious situation that needs addressing. People need to realise that when they meet somebody with sickle cell disease, not only are they holding down their job, bringing up their children, interacting with society in different ways, they're living with this on a day-to-day basis. And I don't know about you, but when I get a toothache, I become pretty useless. But my toothache has an end, either with antibiotics or the dentist. Sickle cell disease is incurable. It doesn't have an end. And managing it as best we can and enabling people to function is our drive. The pain feels like someone taking a nail and hitting you with a hammer.
with a nail and a hammer like you know how they crucify someone basically that's how I feel I'm not sure if that's the general thought about it but that's how I feel when I'm having a pain it feels like someone's hitting me with a hammer so it's a sharp it's a sharp pain yes and that's continuous it's continuous yes it continues for days but once I have pain relief then that helps to take it away and sometimes it comes back but I need to completely clear the flow in my veins before I can say I'm in no pain at all so it's not just about pain relief for me so when you have an episode how long does that last my worst episode It was about three weeks, but on average, one week or a few days. If I go to the hospital when I'm having a crisis, then I am connected to intravenous fluids, so that helps to hydrate my body, which helps proper flow of the cells in the veins that are probably stuck. You go into hospital for every episode? No, I don't go to the hospital for every episode. There are some episodes that I can manage by myself at home. It depends on how bad the pain is for me, then I decide to go to the hospital. Do you get any warning when these episodes are going to come? Sometimes I...
get a warning but sometimes I don't know that anything is going to happen. I just wake up in the morning and I'm in pain. Sickle cell pain comes in all sorts of formats. The common ones, as we mentioned, are the bones. And when a patient feels sickle cell pain, they feel it in their lower back, their arms, their legs, their chestnut and their abdomen. The fact that it affects their lungs doesn't seem to be painful, but it can cause all sorts of disability. Pulmonary hypertension, high blood pressure in the blood circulation of the lungs is something that runs into you. That's not typically pain, but it has serious equally on how people can go and live their lives. They get short of breath, up half a flight of stairs. And when you're a young person, that's very limiting. Some people actually never get any pain or get very, very rare episodes of pain. Some people manage their pain.
if they're moderate pains at home and we try and educate patients as to when that's a good thing to do and how to manage it and to support them doing that but also making sure they know when they should be coming into hospital if there's you know the pain is too severe or there's any other concerns if they're sort of sickling affecting lungs and things like that or infection so some people are fine some people have everyday or chronic pain that doesn't need an inpatient admission so it's trying to identify what the pain is what the triggers are and what make it worse as well so we know for example that people who are very stressed at various times, you might have other things going on in your life, that makes pain difficult to manage. When it's difficult to deal with pain, that can make the pain worse. If you've got a busy job or if you're looking after children, you know, Wednesdays are always a busy job. So trying to identify things like that, trying to identify if mood plays a role, how we can help with that, you know, pain makes mood difficult, mood makes pain difficult. And looking at things like relaxation techniques and distraction and all those other things that can work together and making sure that the medication that you're taking is the right one for that situation as well. So we're trying to get sort of the best pain relief with...
the least amount of medicine, really. So self-management is important as in any chronic pain condition. Absolutely. They talk about, you talk about, we talk about the pain cycle where the condition makes you depressed or anxious, this, that, the other, that feeds back into the pain, so it's a spiral that just keeps going and going. And that's definitely one role, but also...
The different thing probably with sickle cell disease is because there are these crises going on, so making sure that also you're safe and that actually it's not affecting you in a way that does need to see a doctor in a medicine as well and hospital. Part of the syndrome of sickle cell is what's called a vascular necrosis, and that's where these sickle cell blood shapes cause blockage and inflammation to the blood vessels that travel to the bone, and it causes the bone to collapse. It does occur most commonly in the hips. Apart from the spleen, which is an organ in the tummy, the hips are the next most commonly destroyed organ by sickle cell disease. The knees are partially affected, but to a much lesser extent, and the shoulders perhaps more than the knees, but less than the hips. The vertebrae, the bones that make up the back, they also have a...
pretty poor blood supply, and they can collapse. And you can imagine if a bone collapses, it can have profound effects on function and pain. Can that sort of damage be prevented? From the bone pain point of view, again, it affects different types of the disease in a different way. We know that 50% of people with the homozygous variant, which means they've got the serious hemoglobin SS, at 35 years of age, 50% of people will have their hips severely affected by a vascular necrosis. Can it be preventable? Yes. If spotted and diagnosed early, there is a small place for prophylaxis. Some surgical operations are available to prevent the head of the femur, the hip, from breaking down. Otherwise, you're looking at good physiotherapy, bed rest if needed, to allow perhaps some of the bone to regenerate a little bit. But no, prophylaxis is largely absent.
Dr Oliver Seyfried of St George's Hospital in London's multidisciplinary red cell pain management team and the term prophylaxis means prevention. Two other members of the team are clinical psychologist Dr Jenna Love and specialist physiotherapist Rebecca McLaughlin. Rebecca first. Within a really good multidisciplinary team, there's a huge amount of overlap with roles, but then there are aspects of the roles that each profession will have more expertise and more knowledge about. So a physiotherapist working in pain management might mainly focus on the impact of pain on movement and activity and physical function and understanding of pain mechanisms, the way that pain influences our body and our movement and our activity. Where the psychologist in the team tends to focus more on the impact that pain will have on moods and emotion, might be working with people exploring particularly unhelpful thoughts that they have about pain or their condition, thinking about communication.
and the impact of pain on relationships and families. And I think what's really nice about Rebecca and I is that we're able to be in the same room at the same time a lot of the time with the people that we work with. And so you'll hear that the things dovetail together really nicely. So Rebecca, for example, might be talking to somebody about increasing activity or movement, but what she'll also be doing is helping them to explore what thoughts and beliefs are getting in the way of them doing that activity. So we're very much always talking about physical and psychological in a very integrative way. So if we're able to identify what the beliefs are that stop them,
making that change, then we can help them to change those beliefs to more helpful ideas. So it really is a very integrated way of working. So for instance, just telling somebody that they need to do more exercise. is ignoring the fact that it's not just the condition that might stop them doing the exercise, but it's the thought processes. What happens if? Why? Yeah, absolutely. And those things are always unique to the individual. So a population of people with the same condition may have very different beliefs about what that condition means in terms of their ability to move or the consequence of movement or the consequence of activity. So part of our role is to really unpick for the individual what does having this condition mean and what impact does that have on the choices you feel able to make, on the beliefs that you have, on the thoughts you have about, say, movement and activity. We focus quite a lot on people with chronic pain, so thinking about not necessarily...
the type of pain that might bring them into hospital with a crisis but the kind of everyday or regular pain that is there a lot of the time and often that's the sort of pain that people just struggle with at home and there's quite an expectation well you have sickle you will have pain often if people do a lot of movement they might find that that flares up their pain significantly so they get worried about doing movers and think I better not do that because I've got to be able to pick the children up later so they often limit movement and then of course the impact of that is that they become you know, sort of lose a bit of fitness and become a little bit deconditioned so that if they then do more activity again, it can increase pain. So people can get into really unhelpful patterns with activity, very understandably, where it actually feels quite difficult to do activity and movement. You call that the cycle of pain, don't you? Yeah, quite often. Or thinking, people talk about different words, so kind of boom and bust of activity or over and under activity. And I think all of those patterns, as Jenna said, are very understandable. It makes sense in the short term that if something feels painful and if something has a really difficult consequence, it makes sense to avoid it. But if we...
have a condition where lots of things aggravate pain and lots of things have an unpleasant consequence and we start avoiding them all, quality of life gets significantly reduced and then we start to hit more and more problems. The boom and bust that you mentioned, that's when you're feeling good, when you're feeling well, you try and do everything and you bust. Yeah, you pay for it. And you go further back even sometimes. Absolutely. And one of the things that we've noticed is sometimes because people with sickle cell have that history of...
regular crises that what can happen is again their thoughts get in the way and they think ah pain's increasing does this mean a crisis is coming I better rush around get everything done because who knows what's going to happen might I end up in hospital so I think the boom and bust can even be exacerbated in sickle cell because there is that history of perhaps needing to be hospitalised with pain that can mean that actually the pressure to get things done is quite significant. How do people feel when they're told that a psychologist is going to manage your pain? There's a psychologist also based in our service called Dr Penelope Cream and she's been working with the sickle cell patients for about four months. five years one of the very helpful things she's done is help to normalize the sense of seeing a psychologist and actually we've not come across as much resistance to psychology as we might have anticipated there's always the people we don't see of course but I think we're very very careful to explain that we're never referring to a psychologist because we don't believe the person's pain because we think that they're it's all in their mind we are very much of the idea that
psychology is important because pain affects our thoughts and our emotions and it can lead to a whole host of really difficult psychological conditions it's very understandable why people with very significant ongoing pain can start to feel anxious can start to feel worried about doing things like activity that they can start to feel low in mood they start to feel angry and there's a lot about sickle cell about Why me? And the injustice of the condition as well. So I think we help to give a clear rationale for why psychology might be involved in that it's about helping you cope with the emotional impact of the condition and nothing to do with being seen as in any way that they're not coping in a helpful way. I suppose a lot of that is down to education as well. I mean, getting people to understand that pain is a biopsychosocial thing. Yeah, and I think that's one of the really important reasons why we work within a multidisciplinary team and that we work mainly in...
the outpatient setting but that our team spreads across inpatient and outpatient settings and that some of that education comes kind of filters down from all sources and it's really about trying to help people to see that a very physical condition like sickle cell that's often traditionally been treated very medically isn't just going to have a medical physical impact it is going to have that broader impact and our consultants like Dr Rhodes do a huge amount in terms of helping to educate patients about the broader impact of pain and hooking them in to more biopsychosocial founded interventions. That's clinical psychologist Dr Jenna Love. Haematologist Dr Elizabeth Rhodes again. What we should be doing in the UK is actually identifying all children born with sickle cell disease or sickle cell anemia before they're born actually. So we've managed to educate the parents who are at risk and then making sure we make that diagnosis at birth. So from the very beginning, you know, that you get to meet your sickle cell team and you get to see your paediatric haematologist or the haematology doctor that's going to be looking after your child, you know, at sort of three months of age. And so we start that education process right at the very beginning. And then it's really key to remember to do that as that child gets older, so as they take on, you know, their own condition and as they come into adulthood that we don't just assume.
that it's all remembered from when their parents were told. We run workshops, so we educate patients on their disease, but also on their pain and different types of self-management and hospital management as well. For somebody who's just found that they have sickle cell disease, what advice would you give? One important thing is to make sure that you are known to a specialist centre that looks after, so either via the GP or something like that, is to get yourself referred to the hospital. That doesn't mean you can spend all your time in hospital, but it's important that we see you as an outpatient as well, so we try and see patients at least once a year. Some patients need more, and then we can make sure that all the complications are being looked for and managed, and therefore you also get...
into the system where we've got the nursing support and the psychology support and everything else that you need it if you need it. So trying to make sure that you're in with the hospital team that's looking after sickle cell disease is really important because they'll have the access to the other support systems that you'll need as well. So I guess in that it's a genetic illness, there's a good start for you that you know who will get or who might get it. And getting that help into the family in one generation might pass all the way through. Yes, so it's picking up who's a carrier because people who are carriers usually...
have no symptoms so it's trying to make sure we identify those people who are carriers so that and we usually do that in pregnancy so then they know that they're at risk of having a baby who might be affected and then once we know that that child's affected throughout their life we'll talk to them about partner testing and genetics as well. For me being Not just a carrier, but I have sickle cell disease. It affects my relationships a lot because it's not just about meeting someone and liking someone. I have to ask, what's your genotype? Because you can't be a carrier. You have to be someone who doesn't have the sickle genotype at all. I mentioned at the start of this programme that exact figures for the instance of sickle cell disease in the UK were hard to come by. Now, does this mean that there's been a lack of interest or awareness by the medical profession or indeed by society itself? Dr Oliver Seyfried.
The life expectancy of sickle cell disease in the early part of last century was teens. If you survived infancy, you were lucky to make young adulthood. We now, through medical and social advances, longevity is almost normalising. So we can now concentrate on how we manage this. There's another side of it, I think, that people expect sickle cell disease to come with pain. And so you say to your patient, you've got sickle cell disease, are you in pain? They say yes. You go, right, you are.
It's almost expected that people live with it. And I think maybe that's the attitude that's changing. Don't also forget that, rightly or wrongly, people in this country, in the United Kingdom with sickle cell disease, either black or from the Indian subcontinent, are part of an ethnic minority. And with that comes all sorts of social issues that I hope are starting to dispel, but may have suffered at the hands of being an ethnic minority and all that comes with that. It's education to the communities themselves, to the potential patients, to the carriers. Absolutely. I meet many patients who could be carriers of the disease and you ask them, do you have sickle cell disease or sickle cell trait? And they look at you blankly. It's got to be spread. People have to know, just I think on a general level, especially in the black and Indian subcontinent community, about the thalassemias as well. This is another type of haemoglobin disorder that's important and that can create pain. They've got to be aware because it does affect them and their family if they're unlucky. If two people with sickle cell trait meet, have a child, one in four of the children will have the serious version of sickle cell disease. And NHS England are running the sickle cell and beta thalassemia programme with a view to getting people prior to...
through pregnancy and then to get early diagnosis in there because early diagnosis might need to earlier intervention and a healthier person throughout their life. Dr Oliver Seyfried, he mentioned the National Sickle Cell Screening Program in the UK. Dr Elizabeth Rhodes again. If you're in an area of high prevalence, so for example if you're pregnant in central London, we'll test all women. And then if you're affected, we'll offer to test the father of the baby. If you're in an area of low prevalence, so perhaps where sickle cell isn't as very common, we do a family origin questionnaire and we look at your full blood count, so to look to see if you're anemic. And if there's some suggestion that you might need screening, we'll screen you then as well. So we should be trying to pick up everyone in pregnancy. Are there specialist centres throughout the UK? Yep, throughout the UK, but not in sort of absolutely every area. So again, the areas of low prevalence may not have a specialist centre, but they should be able to link to a specialist centre. There's several in London, Manchester and Birmingham.
Dr Elizabeth Rhodes, consultant haematologist at St George's in London. And you can find more information about sickle cell disease at the Sickle Cell Society website. And the address for that is sicklecellsociety.org. I'll just remind you that whilst we in Pain Concern believe the information and opinions on airing pain are accurate and sound, based on the best judgements available, you should always consult your health professional on any matter relating to your health and wellbeing. He or she is the only person who knows you, your circumstances, and therefore the appropriate action to take on your behalf. Don't forget that you can download all editions and transcripts of airing pain from Pain Concern's website, which is painconcern, once again, no gaps, painconcern.org.uk.
There you'll find information and support for those of us with chronic pain, our families and supporters, and for healthcare professionals. There's also information on how to order Pain Concerns magazine Pain Matters. Last words to Khadija Chose. You don't want to meet someone and then, you know, you're hitting it over and the next thing you're asking, what's your genotype? It's kind of weird, but it's something that has to be done. And, pardon me if I'm prying, have you done that with people? Oh my God, several times. All my boyfriends, I've asked.
What's your genotype? And to be honest, I think that has been a good thing for me because I have a son now. And maybe if I didn't ask or we didn't do tests, then I would have a son who is having a sickle cell disease just like me. Because if I marry someone who's a carrier, then my child has a 75% probability of being someone with a sickle cell disease. And I don't want to help with my child through what I'm going through. I want a child to be healthy and happy to do whatever he wants to do, whenever he wants to do everything. So yeah, it's quite important to ask those questions. So is your son a carrier? My son is a carrier. All my children are going to be carriers.
But he doesn't have sickle cell disease. But he doesn't have sickle cell disease. So he's just a carrier. So you'll be passing that information on to him. Yes. And that affects the whole of his life to come as well. Yes, that does. It's quite important that we all educate ourselves on this issue. If you have sickle cell disease or you're just a carrier, it's important that they know and when they're dealing with their life issues, relationships, that they also ask those questions because it'd be nice if a lot more people knew about this condition and then that way maybe there'll be a little more empathy as well. Empathy, not sympathy. Empathy, yeah.