Sickle Cell Anemia Nursing | Symptoms, Pathophysiology, Sickle Cell Crisis & Trait

this is Sarah French Turner sorry and calm and in this video I'm going to be going over sickle cell anemia in this video is part of an Inc Lex review series over hematology and as always don't forget to access the free quiz at the end of this video to test you on this condition so let's get started what is sickle cell anemia this is where a patient has abnormal hemoglobin on their red blood cell and the type of hemoglobin that we're talking about is hemoglobin s now the normal type of hemoglobin is hemoglobin a patients who have hemoglobin a they don't have this condition only the ones who have hemoglobin s and the thing to remember about hemoglobin s is that it is very sensitive to low amounts of oxygen in the body so anything that causes the body to increase its demands for oxygen like going to a higher altitude infection dehydration things like that will cause these red blood cells to change their shape and when they change their shape they will become sickle shape like a seed they will become stiff and sticky and we don't want our red blood cells to stick together in our circulation because if they do that they will block blood flow to important organs and tissues and things like that and our patient can enter into sickle cell crisis and we don't want them there so that's where a lot of our nursing interventions and treatments things like that are going to come from so when we go over that part the lecture be sure to pay attention to that and another thing then nimi Aware's anemia part come from well because of these red blood cells unique shape of being sick a c-shape they are really weak so they don't last as long it has normal red blood cells normal red blood cells how long do they last about 120 days these tend to rupture and break down in about 20 days so the patient's going to suffer from anemia the bone marrow can't keep up with production and also the spleen is going to be affected as well the spleen helps recycle those old red blood cells it's going to become overworked blood flow can get blocked to the spleen because of all this sticking together of these sick old red blood cells which can overwork the spleen and the spleen also helps us fight infection and if our spleens not working because a lot of these patients have splenomegaly they're going to be at risk for infection as well now there are different types of sickle cell disease and sickle cell anemia is the most common form in the most severe form of all the types now what causes sickle cell anemia well a person is born with it they have inherited the abnormal hemoglobin s genes from each parent and it has caused them to receive both of them it's a homozygous disease so it means that they have hemoglobin s s on their red blood cell and a lot of times you're gonna see patients who are super young presenting with these signs and symptoms and I'm talking about 6 7 8 months of age and what happens is that typically parent brings the child in they're like you know this child isn't the same child that I gave birth to they're extremely fussy I can't it's they're not a happy baby they're crying all the time it's literally like they're in pain they have a fever and they have swelling in their hands and in their feet dak Titus also Hannam fit syndrome and this is where these red blood cells have sickle they're sticking together they're blocking blood flow to those those extremities organs causing lots of pain and the swelling now why is this presenting now why didn't it present at birth to 3 months of age well in utero and during that first 6 months of life the babies had on board fetal hemoglobin which is the main transporter of oxygen but as that child has grown to six seven eight months of age that fetal hemoglobin role is diminished and what it received in his genetic code from mom and dad they received hemoglobin SS that starts to take over and that is very sensitive to low oxygen and these red blood cells start to sickle so that was why that patient is going to start having sickle-cell anemia so remember fetal hemoglobin cuz we're gonna be talking about that with one of the medication treatments for this condition so what type of genetic blood disorder is this and I would remember this for testing purposes it is an autosomal recessive disease which means that in order for a patient to have sickle cell disease they have to have written and learned genetic code for the red blood cell they have to be homozygous so they have to have hemoglobin SS I've got to receive that from each parent so father will give them hemoglobin s from his coat and mom will give hemoglobin s from her coat and whenever that happens there's a 25% chance that the child will have hemoglobin s s so that's how it occurs which is the opposite for like autosomal dominant where one parent just gives that I'm not I'm normal gene and the parent usually has signs and symptoms of that disease because here in this disease the parents usually are just carriers of it they have sickle cell trait so they don't normally have the signs and symptoms associated with sickle cell disease it's very very rare so let's look at scenario we have our Father and our mother they have sickle cell trait they carry the disease but they don't necessarily show signs and symptoms of it because they have hemoglobin a which is the normal hemoglobin in hemoglobin s so they have just enough of that normal hemoglobin to really prevent those red blood cells from changing their shape from sickling so you have father because from globin a yes and mother who has hemoglobin a yes when they get together have a child there's a 25% chance though they're going to pass each of those hemoglobin s genes to that child and give them sickle-cell disease now one patient population is that most risk for developing sickle cell anemia and I were to remember this for testing purposes according to the CDC Dhaka one in twelve African Americans contain the sickle cell trait so they may not necessarily know that they have this but they have hemoglobin ans and since it's one in twelve it's relatively high chance that when two african-americans get together they have a child there's a 25% chance that they can each that hemoglobin s to their offspring and that offspring has sickle-cell disease it can also occur in Middle Eastern Asian Caribbean and eastern Mediterranean as well now let's talk about testing what's available to test the patient for sickle cell anemia because as a nurse you want to be familiar with these tests okay like I said this disease is really found early on because our pediatric patients about six months onward can start presenting with this so most hospitals have this as part of their newborn screening also babies still in the womb can be tested using amniotic fluid to see if the abnormal hemoglobin is present one of the most common relatively easy ways is through a test called the thionite test and it's also called sickle dex and this will the blood test it takes the blood and assesses for that abnormal hemoglobin but the thing about this test i want you to remember is that it cannot differentiate between if the person has the sickle-cell trait just that normal hemoglobin and then they also have the abnormal hemoglobin so hemoglobin ans or if they actually have full sickle-cell disease so in order to determine that they will need another test called a hemoglobin electrophoresis which will help determine that now let's talk about pathophysiology and we're gonna do this before we dive into sickle cell crisis and our nursing interventions okay we're gonna begin with what a normal red blood cell should look like because based on how it appears it has a normal function compared to the sick old red blood cell so a normal red blood cell should look similar to this it should be nice and round it should be smooth it should be concave with a flat Center on both sides and what's really cool about red blood cells is that they do not have a nucleus and this is actually really beneficial for the red blood cell which is why they were created like that and the benefits of this is that it allows them to easily squeeze through our vessels and carry oxygen so what we're really concerned about in this disease is the hemoglobin found within the red blood cell now what does normal hemoglobin do it helps transport oxygen throughout our body so it takes it that it received from the lungs and it takes it to all Vrbata organs and tissues and helps it work appropriately now we can only do this really well if it has a normal type of hemoglobin which is that again that hemoglobin a however people with sickle cell anemia and remember they don't have that type of hemoglobin they had hemoglobin s which again causes the red blood cells to be stiff and sticky and form that sickle type shape which isn't very vessel friendly they are going to start to stick together and they stick together so well that they can cause circulation problems and when does this happen when the body is experiencing low amounts of oxygen so as the nurse we have to educate our patients to avoid circumstances that cause that which we're going to talk about here in a moment so these patients will experience these sickling episodes and this is when those signs and symptoms are really going to manifest themselves so it's really important to remember that most patients with sickle cell anemia they're gonna have anemia and why are they having an anemia again it's because the lifespan of the red blood cell is short because that red blood cell is delicate and ruptures and they won't have major signs and symptoms until an actual crisis episode arises in crisis episodes can vary among patients and some can have them randomly while others can have them quite frequently so now let's talk about the factors that can cause a sickle cell crisis and I would remember these factors because tests love to ask you this along with education pieces that go along with it so to help us remember it let's remember the word sickle because what's happening is that these are factors that are causing low amounts of oxygen in the body it's either increasing the body's need for it or it affects how oxygen is being transported so s significant blood loss this can be from surgery a trauma and why is this well the body has just lost lots of blood which is going to end the demand of oxygen all of our red blood cells are gone that actually had some oxygen on it and this can lead to some sickling I for illness and like I said earlier because their spleen isn't going to be functioning properly just because of how much red blood cells are broken down the spleens blood flow can be diminished because of these cells sticking together causing it to swell they're at risk for illness so a lot of times these patients are going to be on prophylactic antibiotics like penicillin to prevent infection an infection can actually lead to one of these crisis's as well so you want to educate them about avoiding illness see climbing or flying to high altitudes and just going up and high altitudes increases the body's demand for oxygen so this can lead to an episode okay for keeping continued stress this can be mental stress this can be physical stress again that alters the way that the body is using oxygen l4 low fluid intake and this leads to dehydration it's why we so important we teach patients you need to watch your exercising you don't need to become dehydrated you need to drink regularly make sure you make it a priority that you're drinking fluids on a continual basis efore elevated temperature and this can arise from a fever or strenuous exercise so you want to teach your patient you know you can work out but don't overdo it because fever exercise increases the body's demands for oxygen which stresses those hemoglobin s cells out and can cause sickling also cold extreme temperature change going from relatively warm environment to just like a freezing environment may be like jumping into icy cold water because that increases the body's way of being able to keep itself warm which is going to increase the demands of oxygen so avoiding things like that where they're gonna immerse themselves into cold water we always talk about what can go on during a sickle cell crisis we just looked at the factors that can lead to one and we talked about what can send a patient into having an episode okay so you can have a vaso occlusive and it's just like the name says in the vessels they're becoming occluded so these sick old red blood cells are sticking together so well in the vessels that it's dropping the blood flow to our organs and tissues and this can lead to infarctions you can have strokes you have issues with the kidneys you can send them into renal failure things like that because all these major organs are being affected also you can have a hemolytic crisis where there's just the rapid death of these red blood cells because again they're super fragile they're not living very long only about 20 days compared to the lovely 120 days they normally live so they're breaking down now as they break down what do red blood cells release they released bilirubin and it's gonna be so much bilirubin that's being released that you're gonna see jaundice in these patients because they just break down so easily you can also have aplastic crisis and this is because the red blood cell production is literally coming to a halt because they're not living that long and your bone marrow can't keep up with trying to produce them every 20 days so you're gonna have some periods where you're literally not having any red blood cell production and the spleen can be involved with spleen sequestration and this is where the spleen is not working and again like I said earlier it's not working because you have those sickle cells in there dropping the blood flow it starts to swell with fluid and it gets congested so because it's trying to keep up with all these red blood cells breaking down because it helps recycle those and another thing it does is it helps filter our blood in a sense from those foreign invaders so we're a huge risk for infection as well now let's talk about those signs and symptoms that you can see in a patient who's having a sickle cell crisis and let's mesh that together with the nursing interventions what we're going to be doing for the patient okay so what's going on with a patient what's priority well they've entered into this crisis because they're probably dehydrated their body is experience in stress with low amounts of oxygen so we've got to help combat that and as you're going to see in a severe crisis this can affect every system of the body from the brain to the gallbladder to the kidneys to the lungs so as a nurse hydration is a huge focus for us we want to per physicians order have IV fluids on board making sure that they're consuming fluids because what's this going to do this is going to help dilute the blood because we have red blood cells that are sickle that are sticking together and if we dilute that that'll help alleviate that and also help with renal function preserving that blood flow to our kidney so that's one thing also oxygen these hemoglobin s on the red blood cell is already stressed from the oxygen that it's not really receiving so if we give them some more that can help alleviate that pain control is a huge thing we want to do with these patients this is very very painful and we want to make sure that we're treating that appropriately they're going to be on bedrest and we want to monitor their respiratory status because they're at risk for acute chest syndrome with this condition so let's talk about those signs and symptoms dactyl itis we talked about that earlier you're gonna really see this in the infants rather than the older children that was like the swelling of the hands and the feet we want to elevate those hands and feed those extremities to help promote blood flow because what's happening is those red blood cells are sticking together decreasing perfusion to those extremities pain like I said they can have this back joint throughout wherever these episodes are happening and that's because of that ischemia that's occurring because those sickle cells decreasing blood flow and best way to treat it is opioid medications around-the-clock not PR in as needed when they request it but around the clock to help control pain because it's very very painful also warm compresses on wherever the pains at not like cold compresses why not cold well remember with one of our factors cold can actually increase the demand of oxygen which can stress those cells out and cause worse sickling and we want to make sure that we're not leaving restrictive items on them if they have a watch on or the don't leave by blood pressure cuff on their arm because that's gonna impede blood flow even more so make sure that they're not having anything restrictive on their extremities another thing of course is anemia and if they are going through a hyper hemolytic or an aplastic crisis they have that breakdown of red blood cells that's accelerated or they're just not really making any and you go to watch out for increase heart rate with this because the heart's trying to compensate for those low oxygen levels infants can be really tired or fussy shortness of breath just where there's not enough oxygen present whenever they just try to do a simple task it's like wow I'm really short of breath of course low red blood cells making sure that you're looking at those mucous membranes to assess for pallor paleness because most patients who have sickle cell anemia they are African Americans so in order to tell if they're pale you need to look at the mucous membranes are they really lie instead of the dark red pink color and looking at the growth and development because I can affect this in children with the severe anemia so nursing standpoint blood transfusions replacing them with fresh new red blood cells increasing that number increasing the oxygen available to the body also folic acid why folic acid this helps with red blood cell production so they may be ordered to take a supplement in bat rather than iron iron supplements don't really help this type of anemia like the other anemia as we've talked about and in some patients with sickle cell disease giving them iron supplements can actually cause toxicity where the iron will collect in the organs so you want to avoid that now if you aren't familiar with blood transfusions from the nursing standpoint I have a whole video on that that you can watch and access to help you review for NCLEX for that another thing is infection we talked a lot about this the spleens not working well or here for infection because that helps filter out or foreign invaders plays a role with that and so one of these patients can get pneumonia so you want to be looking at respiratory status assessing those breath sounds whether what's their oxygen saturation how are they breathing because a lot of patients can enter into acute chest syndrome this can be preceded by pneumonia or a pulmonary embolism and a lot of times people with sickle-cell disease have abnormal clotting so they're at risk for throwing off clots as well so monitor that and if a patient has this they will have a fever chest pain called low oxygen saturation that'll probably tip the physician off to order a chest x-ray which is going to show you aliy a new infiltrate on the chest x-ray so as a nurse make sure you are being aware of that and thinking of that as well another thing is gall stones why in the world gall stones well remember we have the rupturing of a red blood cells they're very fragile so if you are breaking down lots of red blood cells because you're in the severe crisis what do red blood cells release Billy Reuben well the body can't deal with all this Billy Reuben so the gallbladder it starts to make Stones out of it so because the gallbladder you can't really keep up so they will have that so watch out for signs and symptoms of gall stones like chest pain simular like heartburn even some people have reported it feels like they're having a heart attack lower pain in their shoulder where their shoulder blade as well getting nausea things like that next stroke this can occur because if those red blood cells are sticking together in important vessels that feed our brain tissue it can decrease blood supply and cause brain death and what you want to be doing is you want to make sure you're monitoring their neuro status with that as well and if you need an in clicks review on stroke I have a whole one right there that you can check out as well another thing is eye problems and it's sort of the same concept all the sticky red blood cells that feed the nerves so the eyes can become blocked and they can have vision changes so assessing their vision making sure that they go and get regular vision checkups and leg ulcers this is typically in your older children all your infant so the doctor lightest the hannam fit syndrome to main leader infants are leg ulcers are going to be in our older children and this is the decreased blood flow that vaso occlusion to the leg so they get ulcers that can be very painful and slow healing now let's talk about the prevention pieces on how to prevent a sickle cell crisis okay well you want to educate the patient or the parents is number one they're at risk for infection so you want to tell them they want to be getting their vaccinations up-to-date especially the pneumococcal influenza and meningococcal because they're at risk for developing this and you wanna make sure they have those vaccines on board avoiding high altitudes climbing flying preventing infection hand hygiene taking the prophylactic penicillin if that's what the physician is ordered staying away from sick people hydration making sure that they are staying on top of drinking fluids consuming fluids especially on days when it's hot that's usually last thing you're thinking about that if you're getting sweaty you're gonna be working out you want to make sure you're keeping your hydration because this continued into one of these episodes keeping stress levels low not smoking and not overdoing exercise now let's quickly talk about some medications used to treat sickle-cell anemia a common one is called hydroxyurea and this medication is actually used to treat some forms of cancer but they have found that it actually can help some sickle cell anemia patients now what does it do now remember I told you to remember that about the fetal hemoglobin well this is what this medication does it creates the fetal hemoglobin which is going to decrease that sickling so remember that fetal hemoglobin was what was present in that baby that first six months of life that helped them transport oxygen then they get a little bit older and their genes take over producing hemoglobin SS and they get sickle-cell disease so this we can throw this on and it will help create that fetal hemoglobin protein and it also will help with anemia which will decrease the need for so many blood transfusions but one thing about this medication that you want to watch out for and educate the patient about is that it lowers the white blood cell count so that will need to be watched and you'll need to teach the patient go to hand hygiene avoiding people with infection now is there a cure for sickle-cell anemia well a stem cell transplant can be performed where the bone marrow will be made to produce new healthy red blood cells without the abnormal hemoglobin on it however it's rare because patients must be matched with a donor to have a successful stem cell transplant okay so that wraps up this lecture over sickle cell anemia thank you so much for watching don't forget to take the free quiz and to subscribe to our channel for more videos

Channel: RegisteredNurseRN

Views: 652,182

Keywords: sickle cell anemia, sickle cell anemia nursing sickle cell anemia crisis, sickle cell anemia registered nurse rn sickle cell anemia symptoms, sickle cell crisis, sickle cell trait, sickle cell disease, sickle cell disease nursing, sickle cell disease pathophysiology, hydroxyurea sickle cell. anemia nursing, hematology nursing, hematology lecture, acute chest syndrome in sickle cell disease

Id: YGSjJHnCsAY

Length: 24min 56sec (1496 seconds)

Published: Sat May 12 2018