 What comes to nursing school explain in this video on sickle cell anemia or sickle cell disease like it's sometimes called? Sickle cell is a little bit different from the other types of anemia because it is a hereditary disorder that is actually genetic and autosomal recessive Meaning that when two people who have the sickle cell trait get together and have offspring 25% of that offspring will be affected 50% will be a carrier and the other 25% will be unaffected and Patients at risk for developing sickle cell anemia a Descendant from tropical Africa and that's the majority of patients at risk But also people of Mediterranean and Middle Eastern descent, but a little bit to a lesser degree So what happens in sickle cell disease? So normally a red blood cell is kind of round in shape. It's very pliable It can kind of change shape as it goes through different Small blood vessels, but sickled red blood cells have exactly that shape that almost half moon shape That is that gives it the term sickle cell and so when these blood cells that are not only a different shape But they're also Stiffer and less pliable. So they can't really get easily through these small capillaries They tend to clump together. So a bunch of these sickle cells here have come together and they are Clumped now and if you imagine if they're in a very small blood vessel this clumping can lead to a thrombus and so then This can happen anywhere in the body So these sickle cells that come together can basically cause an obstruction in the blood flow and then the part that's Distal to that part of the blood vessel will be affected in terms of having hypoxia so These sickled red blood cells are elongated and stiff and they have this sickled appearance They break in the small capillaries and then cause clumping and thrombi and their lifespan is only 15 to 20 days Where a normal red blood cell has a lifespan of about 120 days And this then leads to anemia because these sickle cells have a very shorter much shorter duration than normal blood cells And so therefore the blood cell count will be down and then also There the spleen gets congested because the spleen tends to recycle these red blood cells and so it's kind of on on on hyperdrive because we're Cycling these cells out more frequently than usual signs and symptoms are similar to Those of any type of anemia where the patient has a low red blood cell count and so they will be fatigued dizzy probably pale and Then shortness of breath can also happen as that air hunger kicks in because we don't have that oxygen carrying capacity but in sickle cell disease there's a little bit of a More symptoms here that apply to this particular disorder and which is pain in the joints chest and the abdomen because when These clumped sickle cells stick together they can cause the significant amount of pain and Also for the extremities that can be delayed wound healing not only in the extremities But in general because first of all the patient is anemic then if we have a Clump of the sickle cell somewhere in a blood vessel again that distal part is not going to get perfused and Lack that oxygen care oxygen being carried to the side of the wound and then we have delayed wound healing and So sickle cell patients with sickle cell anemia don't necessarily always have symptoms, but Symptoms that are or things that exacerbate their symptoms are extremes in temperature So the very cold and they're very hot because They tend to make the patient more dehydrated or more ways are constricted in their extremities if it's cold dehydration because Now think about if we have a clump of these clusters here and then we're dehydrated so we don't have enough volume of intracellular fluid Flushing by here. There's less of a chance of that Clump of cells to be kind of carried away and and dissolved and then Infection because the spleen that plays a huge function in in the in but the immune system It can't function because it is congested and then in high altitude because we already know that we have lower oxygen in high altitudes so if we already have low oxygen carrying capacity because of the pathophysiology here with the Decreased lifespan and the decreased oxygen carrying capacity in high altitude It's even going to be more exacerbated and then pregnancy We know that pregnant patients are at risk for Blood clot formation and so people with sickle cell anemia are even more so at risk because of that clumping we talked about and So many times the patients won't really have symptoms all the time But then when they are in a crisis we have to distinguish between four different things that can happen So the crisis is first vaso occlusive So that's exactly that clumping I was talking about with the thrombus formation in any of the blood vessels So whatever blood vessel becomes occluded the distal part gets affected So if this is a small blood vessel of the heart the patient could have an MI if this is a small blood vessel That brings oxygen to the brain. It could be a stroke So think about anything that you can think that causes occlusion or decreased blood flow to a certain body part that thing can happen and Then we have the a plastic crisis which means that now there are no red blood cells Being produced from the bone marrow. So now in addition to sickle cell disease We're also having a plastic anemia, which is kind of like a different kind But sickle cell is the cause here rather than whatever else can cause a plastic anemia Then we have sequestration crisis Which means that now the spleen is so congested with these red blood cells that are being cycled out that it really can't function and all that blood volume just kind of is concentrated in the spleen it gets congested and the rest of the body Becomes dehydrated so the patient can become hyper volumic and also go into hyper volumic shock And then we have the hemolytic crisis where these red blood cells break And our lice so they kind of burst open then they get transported these fragments get transported to the liver For recycling but now the liver is on hyperdrive trying to keep up with this demand of recycling these blood cells and That'll increase the bilirubin So for diagnostic tests as with any type of anemia We're always going to check a CBC a complete blood count and the patient's red cells and H&H will be low as in any type of anemia But then for sickle cell disease there's a special protein We can check which is hemoglobin s and that will only be positive in somebody who has sickle cell disease and then also the Reticulous site count will be increased because remember that reticular sites are immature red blood cells and if we have red blood Cells that have a very shortened lifespan the bone marrow will try to produce more more blood cells More red blood cells and so these immature blood cells are going to be higher in count In the bloodstream and then the bilirubin will most likely be elevated if they're in a crisis and a hemolytic crisis like I discussed over here Many times we also check the patient for other types of anemia to kind of rule those out and those will be iron Pernicious and folate deficiency anemia So we're also going to check all these labs that pertain to those Those few different anemias and they will these lab valves We're most likely be normal because we're dealing with sickle cell anemia and not one of these other types and for red blood cell Indices sickle cell anemia will be a normal chromic and normal acidic anemia And I have a special video that goes over these RBC indices where you can learn a little bit more about the terminology and what that means Complications again with tissue hypoxia because of that clumping and the decreased blood flow There is a risk for necrosis. So again, if whether that's a wound in the extremities or Necrosis to the heart muscle being an MI or necrosis to the brain Cells which would be a cerebrovascular accident. So this can be really really severe And then patients are at higher risk for infection because of the problem with the spleen congestion and most likely That's going to be a pneumonia They are also at risk for a plastic anemia like I discussed over here with this a plastic crisis And then there's a special term that pertains To sickle cell anemia, which is acute chest syndrome And so the patient will present with chest pain, shortness of breath, fever, cough and infiltrates on a chest x-ray and that again is because of the tissue hypoxia and necrosis But the cause could be anything like an MI a PE or heart failure Or the patient could even go to renal failure and have a stroke as I discussed So what do how do we treat patients with sickle cell anemia? There is no cure because it is a genetic autosomal recessive disorder So it's very important to identify patients and educate them about the causes that can put them into these crises And then also teach them about the crisis and what to do Which is pretty much get medical attention because the treatment requires these three things Oxygen to help with the low oxygen carrying capacity and perfuse these tissues that are being hypoxic IV fluids and those are going to be Isotonic IV fluids to hydrate the patient to try and get these clumps of cells Kind of dissolved as that fluid kind of flushes through and hopefully dissolves these thrombi And then pain management because anything that causes hypoxia and necrosis is going to be painful Again, no matter what body part that is Also, we're going to treat the patient with antibiotics if there's an infection present And then there's a medication called hydroxyurea, which is a special treatment for sickle cell disease Which increases fetal hemoglobin, but unfortunately it can also cause myelosuppression and myelo referring to the bone marrow So now we have a patient in sickle cell crisis will give them this medication But now we're suppressing their bone marrow from producing more red blood cells and that is not a good thing So the the provider will have to very carefully discuss the risks and benefits with the patients here and then the other thing that is possible is a complete blood transfusion or complete transfusion where basically The patient sickle cells are exchanged for normal cells through the methods of a transfusion and This is something that can only occur so many times because the more transfusions a patient has The more likely they are to develop antibodies and the more likely they are to develop Transfusion reactions and I have a separate video that goes over blood transfusion reactions So as for nursing care as always I want to check vital signs and specifically keep an eye on their heart rate blood pressure respiratory rate because patients with anemia typically are Hypotensive and tachycardic and then check their extremity to see if we have any of this tissue hypoxia Present or maybe we see any delayed wound healing Check their skin for Jonas because of this bilirubin check their lungs for infection because we know they are high risk for pneumonia That's the most likely type of infection they'll get and then of course we're going to check their labs all these things We discussed here. We're going to administer that oxygen. That's the treatment. These IV fluids aggressively And then manage their pain and administer packed red blood cells as needed to help with transfusion to bring up their blood count we also Want to do a lot of education here and teach patients about all these causative factors I talked about here heat cold infection dehydration to prevent all those and Then also teach them not to wear any constrictive clothing or jewelry. So think about a A bracelet that could just Constrict those very small blood vessels and capillaries here of the risk and of the wrist and then These thrombi or the sickle cells that are already kind of trying to flow by there can become An occlusion in these small blood vessels and put them at higher risk We also want to teach patients about infection prevention and that includes everything from hand hygiene To vaccinations and staying on top of those to make sure that they don't get seriously ill and then certainly if we know The patient has sickle cell trait or is a carrier. We want to send them to genetic counseling if they are thinking about starting a family and So that they can get counseling the counseling that they need to make sure that they are aware of all the risks and benefits of Passing these genes on to their offspring Thanks so much for watching this video on sickle cell disease. I hope you have learned a little bit more about the topic Also, check out my other topics and the different types of anemia and I'll see you soon right here on nursing school explain Thanks for watching