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Okay. Hi, everyone. I'm sorry for this. Like, delay. We were just getting the slides sorted. Um, can use Can you see us now? Okay. Eso today we've got on with Stop, declare Anderson, who's very kindly agreed to do this talk. She's a hematology registrar. Great Ormond Street Hospital presenting on sickle cell disease. I just wanted to say just now that we will have, like, a feedback form at the end. If you complete that, then you'll be up to get a sticker and everything. So I'll post a link, um, at the ends, the access that easily. But I think it also be down to you. I think so. Um, I'm going to stick around because I got control of the slide to think. But I'm gonna hand over to you, Claire. Teo, get to talk. Thank you very much, sir. For instruction on good evening, Everybody, really. Apologies for the slight technical delay that's delayed r start icing time, but hopefully she get through everything. So I said are saying I'm clamor. There's a world that hemoglobin. I should start that together on one of the hematology registrars that great on the street. And I'm just going to talk to you about sickle cell disease. Um, my next slide. Um, really sickle cell is that I don't have any disclosures to disclose to you on. Really? My main objection objectives are to give a broad overview off. Sickle cell disease is a massive topic, but I thought it's important if we just touch of problems, really common scenarios that you were seeing clinical practice and just go through the complications will. I will describe the pathophysiology that underpin sickle cell on then you ST Some of the clinical management aspect switch. Some case studies go through tip called acute and chronic complications, but also childhood specific aspects and pregnancy considerations. So sickle cell disease it's the most common genetic disease worldwide and effects persons of African heritage estimated now that between it. But 12.5 1000 to 15,000 people in the UK live with sickle cell disease. But that's likely an underestimate with the diaspora of people from Africa all over the globe and addition, more people immigrating from countries where superstars more prevalent, will see an increase off people with sickle cell in the United Kingdom. So the genetics off sickle cell is that in order to have a sickle cell disease, you inherit the sickle hemoglobin with another, sickling him, agree with war, bit of thalassaemia and mutations of that could be the delusion or a non transcription. Allow a fallacy. MC hemoglobin So sick of cell disease there's appointment a shin at position seven with glue to make it replaced by Valin, just to note that they've changed in the make sure of naming the amino acids. So all the textbooks say Position six is whether you mean also changes. But after renaming to include the methionine residue, it's now position seven. The most other common hemoglobin 16 hemoglobin that you encounter is hemoglobin C. Or actually, it's the same position in the beach gene. But instead of a glutamate being replaced by failing and HB A C is replaced by lysine on, then if you've got a sickle hemoglobin with a bit of a fallacy. Me er so that's either beat a plus of meter +01 of the delta beat on the poor pharmacy. Make Kim Global's You get the sickle cell disease and then other more common in Asian and Middle East and populations deporting job. Oh, our and he so much just the genetics. So in summary, you need a hemoglobin s with another 16 hemoglobin to have sickle cell disease. Sickle cell anemia is when you've got to s is that's just a little blanket. So in terms off the pathophysiology, So the cardinal features six old disease is that this amino acid change means there's a non Apoula pocket that form. So when you have the oxygenation off the hemoglobin molecule, the polymers kind of shift to hide that pocket, so it's not to expose. It was very hydrophobic. But while that process happening that leads to the site of skeleton change in the sickling shape off the sickle cells it on. That's why you then end up with phase occlusion because the usual red cell shape is a biconcave shape, and that's to allow it to pass easily three small capillaries, or when it's got sick laying happening within it. That leads to rigidness off that structure so it can easily pass through. That's where you get the phase of constriction. Unfortunately, once the face of constriction sets in, that then cascades into India feel damage, so you can imagine that you've got Stasis here because of the basic constriction that leads to a sterile inflammation with a non infected infiltrate of white cells. Really eating, leasing cytokine is we should then, um, attract small white cells and platelets the area on then because the sickle cell with when it's caught that sicking process. It's very fragile and liable to hemolysis on when you have polycystic free hemoglobin is usually scavenged, but when you've got hemolysis, you got more free Humalog, which is actually quite toxic, and you get scavenging off nitric oxide on what happens is that leads to even more a basic constriction. It dysregulated the endothelium. So in somewhere, you have phase a constriction. You have sterile information. You have endothelial damage and hemolysis, and those are the into play of the pathophysiology that happens in sickle cell. Really, it's hard to predict how the patients behave because some maybe have more of these occlusive phenotype. Others may have more hemolysis on. It's not well understood what we know that modulators, such as how much hemoglobin F can affect this, and if you really cold hair it a G six PD deficiency or an alpha trait, they play into it but a lot of it's poorly understood, but there's quite a complex into play in the Pathophysiology. So just to really reiterate that sickle cell disease, although it causes a DVD name, is just the tip of the iceberg, it really is a multi system disorder. I mean, if you think about any organ in the body, that will be a a factor in that system that is affected by sickle cell. So you're cognitive dysfunction is a major complication on that could be more acute stroke. It could be ischemic or hemorrhagic in the truth setting, but also just cooperative decline, predisposed to retinopathy and written for teachers and also hemoglobinopathy we worry about in the cute setting acute chest syndrome, where you actually stickling in the long parenchyma and that's life threatening. But in the long term, lung damage complete to pull my hypertension. And then once these patients that they have nocturnal hypoxia through other diseases or disorders that can compound this effect come in in the part you know, that chronic anemia itself for six straight of the heart and then there are also prone to diastolic heart function. In fact, call me hypertension. This also to place with cardiomegaly and cardiac dysfunction. Because of hemolysis, there is a tendency to develop pigmented. Goldstone's You can get they can present to the surgeon with acute Kurdish estate is because closest titers or symptoms of biliary colic, not to mention the liver itself, be prone to be affected and sickle cell. So really every system renal dysfunction, anemia infection because of the fact that they have functionally selenia, Not to mention there's also thought to be some tea. So related dysfunction all, Sir Onda. It affects the G I system in the GI you system serve. Women may have difficulties with conception or pregnancy related complications, which we'll get to in case on in men. Priapism is that be concerned? But after that subsides submental left with erectile dysfunction. So it really is a chronic disease with lots of complications. So has this slight early straits. You will counter patient sickle cell in every specialty, so it's important to have a wellness how to manage them, what to look out for. So now I'm just gonna go here some case studies to just explain how we manage patients with sickle cell in acute settings in outpatient and then in the pediatric clinic and then when they present ups and dining. So the first patient we're going to talk about these are theoretical cases. So Kofi's a 21 year old male with hemoglobin s s who's on her dropsy carbon mind, or known as I dropped a year outside the UK Is that university studying computer sides? But he presents to any with severe back pain. So already in this context, we're alerted by Scott Sickle Cell and me wondering probably has basically sit crisis. So these are my thoughts after we get his triage. So looking at the triage, he's a separate heart rate, slightly elevated, but that could be due to pay. Even I space for is 10. Although BP SATs are are acceptable, he's very distressed, so we need to manage him. So these are the sorts of things that I think about when I'm going to see a patient with sickle cell. So really, it isn't a B c. D E approach, and you have to summon help quickly. You sure the next slide, please. Um, it's important to remember that these patients you have to monitor very closely they have reduced visual article reserve and can just decompensate. So it's important to make sure that they're on your radio. You need to treat pain is a medical emergency because it snowballs for these patients. If they're in pain, they're not taking deep breaths. And then what can happen is that can then lead to acute tests syndrome, which could be life threatening. You need to be very vigilant looking out for complications and critical warning signs, and it's really important. Intervene early and aggressively on some expertise with the hematology team. We want to know about these patients early so that we can arrange action escalation toe itchy if necessary. And it's important that we have a much notice is possible because I cannot always. It's always that straightforward that managing these patients, for example, some of our patients develop antibodies, so it may take quite a bit of time to get blood ready if they need a transfusion. So it's just these things to bear in mind. Why, to give us much nurse that notices possible and to really intervene at an early stage to avoid decompensation, mix like please. So in an assessment, it's good to look at the patient's key record and clinic letters. You're really looking at three B six or complications the pertinent things to pick out. Really, What complications have they had? Do they have any other comorbid Iti's, for example, asthma or PE's or anything else that could be affecting or compounding the sickle cell crisis? You want to know drug history? A lot of our patients with sickle cell in this country of trouble to Africa's. You need to still think about other general medical conditions, and malaria in particular, is important to know that baseline hemoglobin renal function and SATs, um, move air. And I cannot stress that it's really important to look at the vital signs. And then you really peruse the chart and scrutinized the critical warning signs in the cath a specific complications. So just to go through the red flag, things that should have your alarm bells ringing. So if the patients just seem general, well, they are, um, well, on any abnormal chest findings. Clinically, you cannot distinguish between a bone. You're in acute chest, so we just treat them the same. On de saturation is a cardinal worrisome sign, Really? So the sax, a less that 94 or if it's a 4% change from baseline, that some patients may have chronic a proxy. But it's really that change or any focal signs crackles signs of pleural effusion. For example, any cardiovascular instability and if they have an anemia that's 2 20 grounds below their baseline, mean the's patients tend to be anemic. That's why we cannot rely on the lab normal reference ranges and interpret the hemoglobin in the context of the patient's hemoglobin. On more more levels, any acute neurologic court sign is worrying. Fear, pain. Any fever, especially as they have functional explain. You do, too quarter spoon it. To me. That happens fairly early on due to the cycling of sickling. Any social abdomen they can get Goldstone's but also taking get sick, laying in the gut in cash, developed you ileus or any scheme it guts. So that's also pouring, and then an enlarged liver or spleen because these are symptoms of Sechrest rations, and that could be life threatening because you're pulling off blood in the liver and spleen and they can just have a a shock and collect right in front of you very, very quickly. So the management of basic lucid crisis. This is probably the most common presentation that you will encounter in your clinical practice. It's really important to get pain relief within 30 minutes, and your goal is to try and get the pain controllable within two hours. A lot of sickle cell patients have an individual pain plan on D is important. That may seem that they're on high doses off opioids, but that's because of it in chronic pain, and they may need that to actually get the pain under control. So I think the first step is to look at the individual pain plan and follow that because it's quite frustrating for these patients. When people don't know the moderate know the pain plan and start them off, you know, 20% of the dose that they need in it there in agony. Failing that, someone who doesn't come very off down or is knew you would look at the who ladder and your local policies most like to include opioids, nonsteroidals and paracetamol with obviously check for any hepatic dysfunctional renal dysfunction before just reflexively prescribing these on. It's very early on that we'll need a PCA. That's another reason like escalate early cause. Certain wards may not do PCs. You may need to evolve the anesthetic seem to set that are just to know that we never give these patient's past the dean because there's increased risk of seizures with that, Yeah, it's really important when you're prescribing opioids to remember the supported medications that should form a part of the order set. So lax tears do not want patients getting constipated because that will just exacerbate the pain. You want them to not feel nauseous. That makes you prescribe anti emetics and also antipruritic so because on high just the opioids, they can get quite itchy. I think it's great if you have non former logical pain measures, so tens machine or warm packs can be quite hopeful. I did work in one place where they had a hydro pore, but doesn't that so easily available? But that can certainly be very helpful. I'd like to stress, but incentives for it trees very important. So I want to see when she prescribed the drug chart that you find in sentence for it, and that's that patient's bed. It's a little bit of lung disease machine and they have to take deep breaths in, and it's helps to recruit the lower airways. And this is to prevent hopefully, did the venom of acute chest crisis, which happens in the setting of a neck acute pain crisis. Because these patients, when they're in pain, they hyperventilate shallow breathing that liter. They're not really expanding the basis of their lungs, and that leads to the bottom. All along is being hypoxic at least a six thing, and then it starts that cycle of acute chest. The other thing is, I want to make sure you maintain normal volume. Yeah, so I would say previously, people put patients with sickle cell on 1.5 points maintenance. But that's actually falling out of favor because if they got colic dysfunction start good to flood thumb. Sometimes patients may have renal complications, so they can have very dilute urine because of the sickling and the kidneys. There is not that concentrating mechanism, so they may be full uric and that way they do need to have more fluid. However, on the other end of the spectrum, they had severe real dysfunction and renal failure. They may not B polio can actually maybe all of your uric and you don't want to flood these. So it's important to monitor the fluid balance and in for normal Polina keep patients warm. You want to avoid cold water because that least movies a constriction I must must stress. Make sure everyone's on VT prophylaxis. Low medical heparin. If there's no contraindications and 10 stockings and close monitoring, she wants them somewhere where they can monitor it close to the nurse's station. So that's just summarizes face occlusive banishment. Sort of the investigations you will send you wanted to for blood. Count on reticulocyte count looking to see the ridiculous, that corner size with the severity of malicious, although it's quite done specific, saying with ldh looking at hemolysis and just reaching or chemistry, particularly paying attention to caressed mean if there's worsening jaundice, looking at if it's on conjugated, which goes with her mal asses or if it's conjugated, which goes with her patio dysfunction and CRP is infection, scooch to tender hemoglobin s level. A group and screed on please, with your blood bank. Barely because if a patient has any antibodies on previous screening, they need is bunch notice is possible to get a Procrit. Blood in it. Necessary patients a febrile just do routine cultures, but you're and copin cove it the trouble. History determines that their risk of my layer. That's something you should screen for on patients who have been on transfusions for a long time. Get by and overloaded on May need. Kelation and kelation is a risk factor for you senior. So any patient has fever, diarrhea on my ankle, a shin. You need to think of the organism and similarly amylase because these patients get pancreatitis. So the clinical assessment is concerning foreign acute abdomen. I would send amylase and then chest X ray and A. C G. Looking for any longer Filtries tested you for any tachycardia and so forth. So despite doing everything appropriate on the ward's poor, Kofi now is febrile his tachycardia. BP is still okay, but up from before, and he's very tack it near. Can his SATs a dropped from 97 to 18% in room air? He looks terrible. He's very diaphoretic, and when you listen, there's reduced Aaron trial on the right compared to the left. So this we see this scenario quite commonly where patients develop acute chest crisis following pain on, it's hard to predict that will happen. You try to mitigate that by doing the fluid management antibiotics. It's a sorry the PT Prophylaxis, making sure they do this Intersport you. But this could happen. And the managed for this is that you have to liaise with your eye to you start because they often need or spiritually support at this patient. In this case, just needed some number belongs gym, but as she did require bipap and a red cell exchange transfusion, which is the management. So you're taking out the sickle cells, replacing it with a two volume exchange to bring down the sickle percentage right down to less than 30. Me often get it less than 20 on because it's indistinguishable from pneumonia. Treat them with visit back relied such a zit for my said and a third generation Catholics pouring, such as kept track so or catheter axon depending on your local antimicrobial policy. So that kind of summarizes face occlusive crisis and to chest syndrome, which you're the most common presentations on the acute medical unit. Okay, so I will go now to the outpatient setting and the sort of things you need to consider in that case. So I'm glad that coffee we followed him. Our peace, thankfully recovered from his pain crisis, an acute chest. Now he's back at university doing well, feeling much better and his comfort on your review. So I'm just gonna go through how you review someone. I love you because I suspect if you are in court, medical training and happy to rotate through hematology or in general practitioner message patient, sickle cell. It's a good idea to just go through how you have elevated patients on what sort of things they need to maintain a healthy life. So kind of the theme of the days that sickle cell disease is more than just a Neemia and there's lots of different factors to consider. So at the genetic level, the type of sickle cell disease you have can effect the phenotype. So HVS s and P H bsp to zero tend to be more severe than your beater. Pluses. We've got a bit of normal adult hemoglobin and Kim Global S C tends to be milder with exception of retinopathy symptoms. So generally hates me s s or hate BSP to zero are the worst in terms of clinical symptoms. You also assess the disease burdens are all they having Lots of crisis. So they're having ultimate humanistic anemia and the complications. This is a big one. So people are having recurrent strokes or acute tested drums, real impairment and so forth. They're obviously going to be need to seem by other members of the multiple every team, and that's gonna have a significant impact on their social wife on their ability to go to work and school. Onda. I cannot stress how it's so important to have a care network in a support network with any chronic disease, something that our patients, even when they're feeling better there never fully well on just having a loving supporting environment really helps them. And then, sadly, is just about access to care. There was a post card lottery unfortunate there. If you already area that hasn't him going with center access to trials, you may have a better experience that you're somewhere in the country that doesn't have that set up. So these are some things to consider when caring physical cell patients on. I just want to talk about general considerations so with any quantity. Just patients. You're having a therapeutic allies with your patients. It's really important to engage thumb and shared decision making because an engaged patient is going to be better for them, for them and also for you, because you'll be working together. It's really important to be empathetic because some of our six or patients have had terrible experience is they've lost trust in healthcare system. Sometimes we have been treated badly on, I think, just listening on being empathetic, realizing it, you know it is communion. People are really suffering when they're in pain or they've had a stroke while they're having difficulties with employment. I'd like to just put a put it out there that I really to test the term Sickler. And I'm not alone in this because patients shouldn't be defined by the disease. So please avoid using the term Sickler. And it's really important to manage these patients in a specialist Hemoglobinopathy team, where they can access to a hematologist, the psychologist renal cardiac, your spirit, treat and surf and have that one point of access. Usually they have a special, similar monopoly center who works and no in tandem and supports the local hospital. But it's really important that these patients are seen in a specialist center. So my next Clyde is just some of the things that you have to cover. It's not. It's not exhaustive, but just gives you a flavor for the things that I would covering at a new review. So neuro you be asking them about pain stroke prevention. So that's mainly medication if they having. And then maybe on hydroxycodones mind, um, for stroke prevention or transfusions. Those are the modifying treatments. Currently, it's very important to just haven't opened dialogue about how their coping with the quantity. Listen, if indeed the psychological support make sure they've been to rest up with the screening, quite a few patients may have struck Tiddly partners is important to specifically ask about structure sleep apnea symptoms because these patients you want to manage that aggressively because nocturnal hypoxia associate it with worsening clinical symptoms upon the hypertension and pain crisis and acute chest prices. Because that high pox era that predisposes more of a seclusion and then they will see the quality of cold really just have her call you in echocardiograms for screening. If they're on, I n kelation all they have been on a transfusion program. They need to be screened for quality iron that's in intervals and support to dress other cardiovascular risk factors because with excellent supportive care the life expectancy of patient that's super cells improving. And that's wonderful. But as with all aging people in the population, the increased risk of cardiovascular risk factors, especially with the Western diet off too much sugar, too much fat. So just about dressing, smoking, diabetes, obesity because they've already caught sickling in the blood vessels, which leading to a vascular pathic process and then adding more vasculopathies no good combination. Then you're just with the G. I got all stones that they need to be screened for any eye and overload in the liver, dealing sensitively with the gene you renal system so enquiring about private visit. Um, any fertility family planning in nocturia because they impaired kidney concentrating ability. That's a problem that can sometimes be something that people made a volunteer have to ask to simply. They're going up multiple times a night on, then screening the year around by doing a dipstick looking specifically at 14 urea because 40 some of our patients to end up developing, uh, even the 40 range, uh, protein urea and need to be started on a sin hip. It'll a managed in conjunction with the nephrology. You look a leg ulcers because with chronic sickling and poor venous sufficiency, you get leg ulcers. And then I don't quite understand the mechanism, but they're at risk of somebody or osteomyelitis. Anyone with a fever and a bone pain. Make sure you think about after myelitis and, unfortunately, a vascular necrosis off the family hedges on fairly common complication that needs to be picked up because you don't want to avoid missing. That's actually two more in younger Children. But Legler this discrepancy on morbidity from that going forward and then the therapies hydroxy carbon mind people having frequent pain crises. Three more three per year. You think about starting Contrave Carla Mind Certainly that had acute chest injury. Um, um probably casted the Shipping Pen V, ideally for life but compliance where that does Wayne so but it's really essential in the first five years of life, and if they want a transfusion program, they may need to have, I think elation, if they're 13, is rising off they had over 10. Transfusions has mentioned patient. The sickle cell can be prone to renal failure on they may be on a sin. Hip itches patients for black and supporting to make sure we talk about bone health in general so that there are vitamin D. They should be on vitamin D supplementation, and if they want to travel to up going abroad, it should have antimalarials. And then I think it's really important to bring up clinical trials and transportation because I think that technology, particularly with gene editing, it's really exciting. And I think it's important to make sure you have a conversation with patients. And if they seem interested in actually passages in the possible clinical trials or curative inventions, we should definitely support that with that. And then you want to make sure that our state, with their vaccines, tick you in the adult population. Make sure that had the pneumococcal vaccine coverted hepatitis, be faxing it made it a booster. Any patient that's going to be a regular transfusions with the screen for hepatitis and make sure they've had the hepatitis B vaccines and make sure that they get boosters. It's immunities were eating and figuring the broad base really have trouble. Vaccines. An annual flu vaccine Just general things about inquire about symptoms of anemia, making sure reiterate the size and symptoms of a plastic crisis. So, typically, Paul Virus B 19 in patients with red cell disorders convey to a red cell a place here and really severe anemia on that's Typically they have made that that flu, like in a bit ill and then suddenly crush their counts. And that's usually the history and more in kids. It will be seeing run through families because it all together in one simply gets another one. That's just one thing to always talk about that and then restraint that vice, that they have an infection or fever to go to hospital for antibiotics because of their head immunity due to poor Spanich function, I was like to talk about health motion, and it's important with all patient about make sure they get enough diet exercise hydration on. Just inquire about the feeling supported socially employment education issues that they have an issue with housing that help him with because we're not just looking after the super cell we're looking at simplistically, there's a complex into play. These factors, it's important that we do follow a bicycle social framework to swallow patients on. Then, for annually, we we tend to send the following blood. So full blood count take film. It's not always the film, but initially the initial visit. We do film by chemistries, uh, human cynics and ferritin at the initial visit. So that's a patient hasn't been seen in a sickle cell center or a child. We do set of G six PD level because a lot of patients from Africa who have super so we'll start current hair it G six PD deficiency on there. That combination of the sickle cell on G six PD deficiency could lead to more Hemolysis is, but also that has management implications, because you're gonna avoid certain drugs of the got six PD deficiency. So it's important to know that we also at the first we will tend to do hemoglobinopathy screen tract, the heat Bs percentage do before humans women operative screen. At the initial visit, we send virology hep B and C and HIV because these patients may have transfusions on, then we always do a blood group and then extended finished fight at the initial visit. What that means is they look for a specific panel of antibodies and try to make sure that patients have blood that's match to all these minor group antibodies. The reason for that is patients with sickle cell you get transfused are more like developed a low antibodies. And that's probably because the altruistic donors in the UK tend to be quite a genetically different, letting to be more white Caucasian. So this light antibody difference between black African on occasion during this. So that's what we do. See more rates of a low antibodies in patients with sickle cell who require transfusions and say, transfusions in patients who work location for, say, cancer, for example. Okay, so the next uh, scenario we're going to cover is the pediatric clinic, and it's pretty similar to elbow there. A few specific difference is that I want to cover all of which are go through. So we're gonna meet the goal of Solar Family s Oh, Florence and African. They're coming to the pediatric clinic with their four year old son, Nathaniel, for his routine visit, and we're just gonna go through how we come to his parents. So just with the pediatric considerations, think it's really important to educate the parents and care gives because they're going to be the Africa for their child. Um, I cannot stress that key workers really invaluable in this. You should roll over CNS, and also this is the same as well. For the adult population. It's really important to have a C and S length, or particularly in Children's that is really invaluable. Actually, we want to involve key stakeholders like the nursery and school, making sure they're aware of sickle cell. They need any education. Commonly, one of the desks practitioners sometimes visit the school or arrange a consultation with the school nurse just to explain. What is sickle cell? Why, when Children go to school, they mean to maybe excuse to go to the toilet because they need to. Possibly more frequently. They can't run around too long and get dehydrated and so forth. Just have those discussions Onda. It's quite helpful if they have a pediatric passport, and what I mean by this is just a little handheld. Documents say I have sickle cell on that and they're local still allows him to bypass a knee and get seen directly saw soft. Um, works quite well. So just go for assistance review. So it is similar you're gonna go from head to toe, basically, but a few differences. So in Children, in terms of pain, particularly under a year of one they get that'll itis, which is the hand foot syndrome that basically get swelling of the digits on. That's a sign off phase occlusion in the small vessels in the fingers and toes and actually that kind of Harold that they may be quite a severe phenotype sickle cell on patients that I've presented with that till itis I might be having a conversation of the family start hydroxy carbonamide when they're nine months of age, so that's usually paid in a child. But then, as they get older from verbalized, you talk about pain if they complained with our painting of school and so forth stroke prevention. So they're gonna have cranial Dopplers, which we'll talk a bit more about in a moment and then just in development is really important that they're retaining their milestone to the growing well into your required by an instructor. Sleep up here because hypertrophic adenoids are quite common, and again that could need two more eye pox ear and more worsening pain. So that's important that we ask about that NFS A really getting seen by ent for intervention on 40 sickling consultant early to hearing impairment to make you sure caster have any concerns about hearing asthma's common? Uh huh, curb ability with sickle cell. And that needs to be banished because anything that promotes hypoxia worsen sickle cell and again acute syndrome. Usually in later child, would you be doing Eckerd? And if there are I in transfusions Sorry, blood transfusions, then you need to look for iron overloading that after a period off transfusion and then in Children, they're more like to get Spanish. Sechrest rations, because they're spleen is still normal. Size is only drug charge that or just the next. So it's usually in the first five years that they're high risk of spirits. Orchestration on. They can just literally crash. So that's what it was. Teach errands how to fill the spleen and to seek urgent metal attention. They no suspicion is bigger, and same with the liver or the liver. Sechrest rations know, quite common. You can get gallstones and then we talk about any resist, usually from the age of six, intrusion of called C West in the bed. It's really important to have a talk about that and warm the parents because we need an empathetic and understanding way to manage this problem, because kids can feel really offset and you don't want to feel punished because then that just really affect some psychologically. And then talk to parents about young boys of priapism and held. This is a medical emergency. As with adults, a vascular necrosis is a problem, and it's 0.2. Detect that and intervene to avoid leg length discrepancy and Children so they may need, if it quite bad that they have to pick replacement. Although that's know, ideally, try the further as late as possible because hip prosthesis is have a limited finite period. Time that that works. A senator adults you because they're the same treatments, immunizations and just again with general talking about since the anemia, particularly plastic crisis. So anyone slapped cheek beware, um, avoid pregnant women and so forth and with the social 0.2, and for the families about the support groups, the sickle cell summer comes think it's really helpful with Children and particular, particularly Advil, is adolescence. When they have that piece of pork and can interact with other Children, they're going through the same thing. Just something when you see it and you know that you're not alone, that's really empowering. And nowadays, with the use of technology in the teenagers young adults, we can incorporate that to help thumb particular time of transition, to take more ownership of their own care. So those are sort of the pediatric aspects that we address just in the first year of life. I There's so much to discuss with the families, and it's important to try and give information in manageable chunks. And we discuss. But I think in the first year, the priorities to make sure you teach them spending help patient and counseling about the risk of stroke, Sechrest rations and signs of a plastic crisis on dactylitis. Those are the most common complications in the first year really stressed to them that the penicillin prophylaxis is vital because the life expectancy off six l patients is reading creased. One reason for that is penicillin, because that's reduced the rates off overwhelming pneumococcal sepsis which is the highest incidents, but it's in the first five years of life. That's why it's crucial that the first five years Please, please please give the penicillin the prophylaxis and just impractical situations. They have to have it refilled with the GP because liquid is has a shorter half life on the tablets. So when they get older, it's not better with the tablet because it's a much longer shelf life. Make sure that they see their pediatrician or GP for the vaccines because they will need the meninges, cocoa vaccines and the A mechanical vaccines. Um, in addition to the normal schedule and from nine months of age. I talked to them about eyedrops a cold by because the Baby Hulk study one of the major studies looking at the ficus E of addressing cup lining Children sure that from nine months of age it was effective in reducing pain. And there's some other organ preserving functions, so it has been safe to use from nine months of age. So just give this information and revisit hydroxy carbon, right? So the other thing, but pertinent for the pediatric care. But patient sickle cell is strict prevention, so they have transcranial Doppler ultrasounds on this has done annually from the ages of 2 to 16 because this is the period where stroke risk is highest. Just to put it in context, a patient with sickle cell disease is 300 times more like a stroke than some with no who does not have sickle cell. So it's really, really a major risk. So this is why we do the screening and a screening test go. This is quite noninvasive, not painful. So it's perfect. Yeah, so they're two things look up for the report, if it's conditional. So that's when the velocity between 172 199 is support. Another child has a repeat in three months, but if it's abnormal like very fast, more than 200 that's when you would refer them for a chronic transfusion program. Because transfusion reduces the risk off stroke. So this is about for me prevention. So which is ideal because anything that could stop the catastrophe happening. That's what we would like to do. So just a few considerations with chronic transfusions. So things we think about top up first is exchange. We prefer exchange because you're taking out blood, you're not just topping up, so there's less iron overload, but the risks all that you have to get IV access. That's like a vast calf on that to be big enough for that to accommodate. That also takes a longer time. So trying to find availability because one of the problems is capacity. It's not gone everywhere. The exchange transfusions that he's being a special team of going off of 15 send her. That has availability. So that is one of the rate limiting steps. Use a lot more blood when you do an exchange transfusion than a simple top up. So that has issues. And, as I discussed before, patients with sickle cell disease to have a risk of developing alloantibody. So sometimes we get a point where it becomes increasingly difficult to find compatible blood thing. Good news is that there was a twitch study, a a major study looking at after having transfusions for a year with normalization to switch over to, I drops a car mind. So in patients that have a least a year of transfusions and their doctors have improved on that had a normal scan, we could possibly add that stage, consider switching to drop the carbs white, but obviously in patients that have had a stroke or those doctors are normalizing the haven't MRI abnormality, although no frank stroke with students, continue with transfusions. So those are just the things to consider. And that's why logistically, although we'd like to exchange everybody, it's not always possible. Okay? And then just a finish off all. Just go through six cell disease in the obstetric clinic and the approach and things to consider in that setting. So we're gonna meet Mary. So she's a first year old sister with sickle cell disease. She's been very compliant, hydroxy carbonamide, and actually has been quite healthy. She got married on. We'll start a family, so we're just going to go through the counseling off. We're with sickle cell disease. What they want to start a family. Quite a few things to consider. Um, so, unfortunately, there's a higher mortality and morbidity in months with six cell disease, so this could be related to six cell itself, so they're more like to get a face occlusive crisis in pregnancy and acute chest and infection on that make sense because pregnancy in itself is a stressful physiological state on people with six already have a stress to physiological state because they're anemic. They're always they're working harder, so they're gonna be more like to get complication. Sickle cell also pregnancy itself. You liable to get three clumps here, and that's higher in black women than gotten with. Sickle cell is even higher because they already have a vascular dysfunction, and then in the feet. Or complications also increased because to center function may be affected. Same maker Ideal are you. G are free term labor and still worth slightly so those are the risks. But we highlight two mothers well, potentially expected most or sickle cell. So pre pregnancy you do you want to do a bit of groundwork, screen the partner and do prenatal diagnosis. If the partner has also hemoglobinopathy trait or it has hemoglobinopathy to see that patient, see if the babies at risk off a sickle cell disease. Um, sometimes we can off Korea within its sampling. Well, I'm near city suspect. This is really important. That patient has sickle cell. Get a referral to a person going to need very early on in order to facilitate this should they wish to go down that line of having prenatal diagnosis. It's a couple with cycles. Have a child already with sickle cell disease. They can have free implantation diagnosis, but that is limited and has to be done in defense of these things to discuss as we discussing this patient's on hydroxy called myd, so any woman that wants to get pregnant three. Surprised to trying to conceive simple, to stop her dressing, cup mind and any other to write a genetic medications which may be needed. Such a state inhibitors or in Kelation, make sure they're taking that formally casted. So Jerry Pregnancy. You want to refer these ladies early to our high risk prince unit with expertise and sickle cell disease? Because of the risk of pre attempts here and just General vascular classic process. Ease there on aspirin. Once that pregnant, we start them on aspirin, which one of I avoid non steroidals later in pregnancy and also really improvement? See just you to that the risks of that on it's really important because pregnant women are in free scripts off having from birth embolism. But if you had sickle cell is a chronic medical condition that increases the risk. So usually these women's from 28 extra station or mom lemon actually help friend on. Some patients will need to be transfused regularly in pregnancy. So if they're ready on a transfusion program, well, patient that people being hydroxy card might so for Mary and refer her to have transfusions. Because in our dressing, carbon mind and then even if patients have been on quite transfusion programs, they're very knee. Mikel have acute chest or a stroke than they get a transfusion. Anyone with cardiac disease, you're going to transfuse thumb to avoid that physiological strain, really, And then same again with multiple pregnancy. Because that is more of a physiological string. You want to make sure support them with transfusions. Through them all, they're always see the caveats. If it's difficult to get large, that will affect the clinical practice, and then they need enhancement a ring, so they will need viability scan, then different scans. So, just in summary from around off the enormous ground at 20 weeks, they have monkey scans into 34 weeks. Where there, Um, sorry. After 24 weeks is going to be every two weeks on and then weekly thereafter. And you also want to check the BP and your visit after these up These visits, particularly after 34 weeks, because the risk of preeclampsia and then delivery and post partum the mode delivery is based on obstruction indications. So another way to put that is that you wouldn't automatically do it. This is Aaron section. They can have a normal delivery, but if they're in distress, you do it. A cesarean section offer induction of labor 30 weeks. You don't want these ladies going pro states. Really? If you can avoid it, um, we would prefer reach honest, easier if they're having cesarean so as to not put the wonder anesthetic on recommend in fetal heart monitoring and pregnant in livery on their increased risk or face occlusive crisis is just being a pay attention to make sure there hydrated that you've got blood ready in advance to get antibodies that were having the prophylaxis for with low molecular heparin on. Then just off uh, they finished breastfeeding. That's the point. We read this start I dropped C carbon mind. So I realized that's quite a lot to cover and six l disease. But These are my key points that just remember, sickle cell disease is more than anemia. The clinical, uh, finish up is very hair teratogenic. And it does require a multi disciplinary approach. Empathetic communication and patients, Really all the experts in their own disease because they know their individual pattern of cycles are how it affects thumb. Really French. His best. Think you're on just knowing that since our patients can deteriorate very rapidly, so it's important to be proactive and treat complications early and aggressively on these areas. Some of resource is that you can find online freely. If you want to do any further reading on cycle. So care in the UK my next life. Uh huh. That's it. Thank you very much for attention, especially in this hot weather and any questions at all. Yeah. Okay. Oh, you're welcome. I just want to say thank you so much for uh, doing is talking really, really brilliant. I just leave sometime. Just see if any questions come through and I'm just gonna put me, um, linked to the feedback form in the chart as well. Okay, this, uh, do so just to say we do have enough hematology hematology Webinar next week on the 26th Tuesday on anemia as well. Just increasing. Lantus, are you today? Um, See. And any questions? Anyone you can just take it for one about. Okay, well, maybe no one instead. Thank you. Thank you. I'm glad it was useful on. Yeah, thank you so much for attention and thank you again. Plateau around for my pressure was just Oh, okay. Thank you. Thank you. Have anything by Oh, oh, oh, that Just hold on that This is 11 question yet? Yes. So off to surgery. So because of the, um, physiological stress of surgery, they're more like to get paying crises on acute, chest injured and actually, prior to surgery. We do tend to transfuse patients to really reduce. The 60% is, you know, wait a mitigate that wrist. So, yes. So paid and chest crises. What? We worry about post operatively. Okay. Um, yeah, I think that's the movie. It once a day. Thank you.