hi I'm Kathy with level up RN in this video I'll be discussing sickle cell anemia and at the end of the video I'm going to provide a little quiz to test your understanding of some of the key facts I'll be discussing so definitely stay tuned for that and if you have our level up RN pediatric nursing flashcards go ahead and pull out your flashcards on CLE cell anemia so you can follow along with me and pay close attention to the bold red text on the back of the cards because those are the things that you are likely to get tested on in nursing school CLE cell anemia is an autosomal recessive genetic disorder that results in chronic anemia pain infection and organ damage in terms of the pathophysiology of this disorder with CLE cell anemia normal hemoglobin which is HBA is replaced with abnormal sickle hemoglobin which is HBS and this causes sickling of the red blood cells so normally red blood cells are nice and round but when they sickle they form a c-shape and this makes it such that these red blood cells get easily caught in the blood vessels which obstructs blood flow to the organs and can result in tissue hypoxia in terms of risk factors for this disorder a family history is definitely going to be a risk factor because this is a genetic disorder in addition African-Americans and individuals of Middle Eastern or Mediterranean descent are also going to be at higher risk for Cle cell anemia signs and symptoms of CLE cell anemia include pain fatigue swollen hands and feet as well as jaundice it's important to note that individuals with this disorder may experience crisises which can be brought on by an infection stress or dehydration so let's talk about about some of the key types of crisises that I would be familiar with the most important one to know is a vasoocclusive crisis so during this type of Crisis the patient will experience severe pain and they will need to be treated with opioid analgesics around the clock so nids and tylol are not going to address their pain another type of Crisis to be familiar with is a splenic sequestration crisis this this occurs when sickled red blood cells become trapped in the spleen which leads to enlargement of the spleen as well as severe anemia and then with an aplastic crisis the bone marrow stops producing red blood cells which leads to severe anemia this is usually caused by a viral infection most commonly fifth disease in terms of laps a patient with cell anemia will often have a decrease in hematocrit an increase in reticulocytes which are immature red blood cells an increase in white blood cells and an increase in Billy ruin because the abnormal red blood cells break apart more easily which releases Billy ribbon cyel anemia is diagnosed with hemoglobin electroforesis which is a blood test that measures hemoglobin levels and looks for abnormal types of hemoglobin in the blood and of note all newborns are screened for Cle cell anemia in terms of treatment as I mentioned before if a patient is experiencing a crisis they will need to be treated with opioid analgesics on a schedule antibiotics would be used to treat an infection and as the nurse you're going to be administering IV fluids blood products and oxygen therapy as ordered ongoing hydroxyurea is an oral medication that can reduce sickling of the red blood cells and help prevent complications such as a vasoocclusive crisis there is a lot of important family teaching that needs to be provided for a child with sickle cell anemia so first of all it is super important that the child gets adequate fluid intake in order to keep that blood flowing and to prevent the sickled red blood cells from sticking together which can lead to a crisis preventing infection is also incredibly important so Hand hygiene avoiding crowds and staying up to date on vaccinations is essential and then you want to advise families to seek immediate medical attention If the child has a fever or any other sign of infection all right it's quiz time and I have three questions for you question number one what blood test is used to identify abnormal hemoglobin in the patient's blood the answer is hemoglobin electroforesis question number two how should pain be treated in a patient with CLE cell anemia experiencing a vasoocclusive crisis the answer is with opioid analgesics given on a schedule and question number three which of the following lab findings would be expected for a patient with CLE cell anemia select all that apply a increased Billy Rubin B increased reticulocytes C increased hematocrit and D increased white blood cell count the answer is a b and d a decrease in hematocrit as opposed to an increase is expected with sickle cell anemia all right that's it for this video I hope it was helpful if so be sure to hit that like button take care and good luck with studying and then I would also be familiar forar for FR we invite you to subscribe to our Channel and share a link with your classmates and friends in nursing school and if you found value in this video be sure to hit that like button and let us know what you found to be particularly helpful