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this is Sarah French Turner sorry and
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calm and in this video I'm going to be
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going over sickle cell anemia in this
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video is part of an Inc Lex review
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series over hematology and as always
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don't forget to access the free quiz at
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the end of this video to test you on
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this condition so let's get started what
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is sickle cell anemia this is where a
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patient has abnormal hemoglobin on their
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red blood cell and the type of
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hemoglobin that we're talking about is
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hemoglobin s now the normal type of
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hemoglobin is hemoglobin a patients who
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have hemoglobin a they don't have this
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condition only the ones who have
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hemoglobin s and the thing to remember
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about hemoglobin s is that it is very
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sensitive to low amounts of oxygen in
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the body so anything that causes the
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body to increase its demands for oxygen
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like going to a higher altitude
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infection dehydration things like that
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will cause these red blood cells to
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change their shape and when they change
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their shape they will become sickle
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shape like a seed they will become stiff
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and sticky and we don't want our red
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blood cells to stick together in our
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circulation because if they do that they
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will block blood flow to important
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organs and tissues and things like that
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and our patient can enter into sickle
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cell crisis and we don't want them there
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so that's where a lot of our nursing
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interventions and treatments things like
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that are going to come from so when we
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go over that part the lecture be sure to
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pay attention to that and another thing
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then nimi Aware's anemia part come from
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well because of these red blood cells
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unique shape of being sick a c-shape
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they are really weak so they don't last
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as long it has normal red blood cells
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normal red blood cells how long do they
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last
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about 120 days these tend to rupture and
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break down in about 20 days so the
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patient's going to suffer from anemia
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the bone marrow can't keep up with
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production and also the spleen is going
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to be affected as well the spleen helps
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recycle those old red blood cells it's
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going to become overworked blood flow
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can get blocked to the spleen because of
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all this sticking together of these sick
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old red blood cells which can overwork
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the spleen and the spleen
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also helps us fight infection and if our
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spleens not working because a lot of
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these patients have splenomegaly
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they're going to be at risk for
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infection as well now there are
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different types of sickle cell disease
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and sickle cell anemia is the most
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common form in the most severe form of
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all the types now what causes sickle
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cell anemia well a person is born with
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it they have inherited the abnormal
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hemoglobin s genes from each parent and
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it has caused them to receive both of
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them it's a homozygous disease so it
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means that they have hemoglobin s s on
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their red blood cell and a lot of times
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you're gonna see patients who are super
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young presenting with these signs and
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symptoms and I'm talking about 6 7 8
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months of age and what happens is that
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typically parent brings the child in
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they're like you know this child isn't
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the same child that I gave birth to
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they're extremely fussy I can't it's
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they're not a happy baby they're crying
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all the time it's literally like they're
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in pain they have a fever and they have
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swelling in their hands and in their
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feet dak Titus also Hannam fit syndrome
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and this is where these red blood cells
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have sickle they're sticking together
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they're blocking blood flow to those
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those extremities organs causing lots of
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pain and the swelling now why is this
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presenting now why didn't it present at
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birth to 3 months of age well in utero
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and during that first 6 months of life
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the babies had on board fetal hemoglobin
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which is the main transporter of oxygen
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but as that child has grown to six seven
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eight months of age that fetal
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hemoglobin role is diminished and what
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it received in his genetic code from mom
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and dad they received hemoglobin SS that
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starts to take over and that is very
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sensitive to low oxygen and these red
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blood cells start to sickle so that was
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why that patient is going to start
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having sickle-cell anemia
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so remember fetal hemoglobin cuz we're
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gonna be talking about that with one of
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the medication treatments for this
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condition so what type of genetic blood
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disorder is this and I would
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remember this for testing purposes it is
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an autosomal recessive disease which
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means that in order for a patient to
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have sickle cell disease they have to
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have written and learned genetic code
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for the red blood cell they have to be
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homozygous so they have to have
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hemoglobin SS I've got to receive that
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from each parent
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so father will give them hemoglobin s
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from his coat and mom will give
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hemoglobin s from her coat and whenever
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that happens there's a 25% chance that
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the child will have hemoglobin s s so
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that's how it occurs which is the
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opposite for like autosomal dominant
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where one parent just gives that I'm not
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I'm normal gene and the parent usually
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has signs and symptoms of that disease
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because here in this disease the parents
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usually are just carriers of it they
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have sickle cell trait so they don't
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normally have the signs and symptoms
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associated with sickle cell disease it's
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very very rare so let's look at scenario
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we have our Father and our mother they
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have sickle cell trait they carry the
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disease but they don't necessarily show
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signs and symptoms of it because they
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have hemoglobin a which is the normal
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hemoglobin in hemoglobin s so they have
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just enough of that normal hemoglobin to
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really prevent those red blood cells
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from changing their shape from sickling
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so you have father because from globin a
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yes and mother who has hemoglobin a yes
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when they get together have a child
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there's a 25% chance though they're
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going to pass each of those hemoglobin s
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genes to that child and give them
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sickle-cell disease
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now one patient population is that most
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risk for developing sickle cell anemia
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and I were to remember this for testing
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purposes according to the CDC Dhaka
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one in twelve African Americans contain
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the sickle cell trait so they may not
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necessarily know that they have this but
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they have hemoglobin ans and since it's
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one in twelve it's relatively high
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chance that when two african-americans
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get together they have a child there's a
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25% chance that they can each
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that hemoglobin s to their offspring and
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that offspring has sickle-cell disease
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it can also occur in Middle Eastern
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Asian Caribbean and eastern
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Mediterranean as well now let's talk
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about testing what's available to test
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the patient for sickle cell anemia
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because as a nurse you want to be
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familiar with these tests okay like I
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said this disease is really found early
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on because our pediatric patients about
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six months onward can start presenting
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with this so most hospitals have this as
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part of their newborn screening also
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babies still in the womb can be tested
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using amniotic fluid to see if the
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abnormal hemoglobin is present one of
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the most common relatively easy ways is
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through a test called the thionite test
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and it's also called sickle dex and this
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will the blood test it takes the blood
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and assesses for that abnormal
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hemoglobin but the thing about this test
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i want you to remember is that it cannot
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differentiate between if the person has
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the sickle-cell trait just that normal
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hemoglobin and then they also have the
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abnormal hemoglobin so hemoglobin ans or
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if they actually have full sickle-cell
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disease so in order to determine that
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they will need another test called a
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hemoglobin electrophoresis which will
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help determine that now let's talk about
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pathophysiology and we're gonna do this
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before we dive into sickle cell crisis
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and our nursing interventions okay we're
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gonna begin with what a normal red blood
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cell should look like because based on
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how it appears it has a normal function
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compared to the sick old red blood cell
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so a normal red blood cell should look
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similar to this it should be nice and
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round it should be smooth it should be
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concave with a flat Center on both sides
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and what's really cool about red blood
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cells is that they do not have a nucleus
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and this is actually really beneficial
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for the red blood cell which is why they
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were created like that and the benefits
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of this is that it allows them to easily
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squeeze through our vessels and carry
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oxygen so what we're really concerned
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about in this
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disease is the hemoglobin found within
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the red blood cell now what does normal
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hemoglobin do it helps transport oxygen
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throughout our body so it takes it that
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it received from the lungs and it takes
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it to all Vrbata organs and tissues and
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helps it work appropriately now we can
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only do this really well if it has a
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normal type of hemoglobin which is that
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again that hemoglobin a however people
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with sickle cell anemia and remember
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they don't have that type of hemoglobin
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they had hemoglobin s which again causes
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the red blood cells to be stiff and
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sticky and form that sickle type shape
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which isn't very vessel friendly they
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are going to start to stick together and
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they stick together so well that they
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can cause circulation problems and when
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does this happen when the body is
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experiencing low amounts of oxygen so as
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the nurse we have to educate our
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patients to avoid circumstances that
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cause that which we're going to talk
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about here in a moment so these patients
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will experience these sickling episodes
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and this is when those signs and
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symptoms are really going to manifest
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themselves so it's really important to
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remember that most patients with sickle
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cell anemia they're gonna have anemia
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and why are they having an anemia again
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it's because the lifespan of the red
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blood cell is short because that red
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blood cell is delicate and ruptures and
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they won't have major signs and symptoms
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until an actual crisis episode arises in
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crisis episodes can vary among patients
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and some can have them randomly while
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others can have them quite frequently so
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now let's talk about the factors that
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can cause a sickle cell crisis and I
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would remember these factors because
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tests love to ask you this along with
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education pieces that go along with it
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so to help us remember it let's remember
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the word sickle because what's happening
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is that these are factors that are
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causing low amounts of oxygen in the
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body it's either increasing the body's
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need for it or it affects how oxygen is
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being transported so s significant blood
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loss this can be from surgery a trauma
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and why is this well the body has just
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lost lots of blood which is going to end
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the demand of oxygen all of our red
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blood cells are gone that actually had
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some oxygen on it and this can lead to
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some sickling I for illness and like I
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said earlier because their spleen isn't
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going to be functioning properly just
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because of how much red blood cells are
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broken down the spleens blood flow can
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be diminished because of these cells
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sticking together causing it to swell
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they're at risk for illness so a lot of
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times these patients are going to be on
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prophylactic antibiotics like penicillin
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to prevent infection an infection can
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actually lead to one of these crisis's
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as well so you want to educate them
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about avoiding illness see climbing or
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flying to high altitudes and just going
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up and high altitudes increases the
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body's demand for oxygen so this can
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lead to an episode okay for keeping
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continued stress this can be mental
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stress this can be physical stress again
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that alters the way that the body is
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using oxygen l4 low fluid intake and
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this leads to dehydration it's why we so
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important we teach patients you need to
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watch your exercising you don't need to
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become dehydrated you need to drink
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regularly make sure you make it a
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priority that you're drinking fluids on
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a continual basis efore elevated
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temperature and this can arise from a
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fever or strenuous exercise so you want
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to teach your patient you know you can
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work out but don't overdo it because
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fever exercise increases the body's
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demands for oxygen which stresses those
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hemoglobin s cells out and can cause
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sickling also cold extreme temperature
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change going from relatively warm
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environment to just like a freezing
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environment may be like jumping into icy
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cold water because that increases the
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body's way of being able to keep itself
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warm which is going to increase the
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demands of oxygen so avoiding things
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like that where they're gonna immerse
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themselves into cold water we always
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talk about what can go on during a
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sickle cell crisis we just looked at the
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factors
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that can lead to one and we talked about
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what can send a patient into having an
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episode okay so you can have a vaso
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occlusive and it's just like the name
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says in the vessels they're becoming
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occluded so these sick old red blood
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cells are sticking together so well in
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the vessels that it's dropping the blood
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flow to our organs and tissues and this
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can lead to infarctions you can have
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strokes you have issues with the kidneys
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you can send them into renal failure
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things like that because all these major
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organs are being affected also you can
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have a hemolytic crisis where there's
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just the rapid death of these red blood
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cells because again they're super
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fragile they're not living very long
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only about 20 days compared to the
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lovely 120 days they normally live so
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they're breaking down now as they break
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down what do red blood cells release
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they released bilirubin and it's gonna
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be so much bilirubin that's being
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released that you're gonna see jaundice
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in these patients because they just
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break down so easily you can also have
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aplastic crisis and this is because the
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red blood cell production is literally
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coming to a halt because they're not
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living that long and your bone marrow
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can't keep up with trying to produce
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them every 20 days so you're gonna have
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some periods where you're literally not
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having any red blood cell production and
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the spleen can be involved with spleen
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sequestration and this is where the
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spleen is not working and again like I
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said earlier it's not working because
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you have those sickle cells in there
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dropping the blood flow it starts to
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swell with fluid and it gets congested
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so because it's trying to keep up with
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all these red blood cells breaking down
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because it helps recycle those and
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another thing it does is it helps filter
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our blood in a sense from those foreign
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invaders so we're a huge risk for
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infection as well now let's talk about
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those signs and symptoms that you can
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see in a patient who's having a sickle
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cell crisis and let's mesh that together
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with the nursing interventions what
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we're going to be doing for the patient
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okay so what's going on with a patient
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what's priority well they've entered
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into this crisis because they're
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probably dehydrated their body is
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experience
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in stress with low amounts of oxygen so
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we've got to help combat that and as
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you're going to see in a severe crisis
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this can affect every system of the body
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from the brain to the gallbladder to the
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kidneys to the lungs so as a nurse
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hydration is a huge focus for us we want
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to per physicians order have IV fluids
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on board making sure that they're
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consuming fluids because what's this
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going to do this is going to help dilute
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the blood because we have red blood
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cells that are sickle that are sticking
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together and if we dilute that that'll
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help alleviate that and also help with
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renal function preserving that blood
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flow to our kidney so that's one thing
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also oxygen these hemoglobin s on the
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red blood cell is already stressed from
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the oxygen that it's not really
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receiving so if we give them some more
00:16:09
that can help alleviate that pain
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control is a huge thing we want to do
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with these patients this is very very
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painful and we want to make sure that
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we're treating that appropriately
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they're going to be on bedrest and we
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want to monitor their respiratory status
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because they're at risk for acute chest
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syndrome with this condition so let's
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talk about those signs and symptoms
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dactyl itis we talked about that earlier
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you're gonna really see this in the
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infants rather than the older children
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that was like the swelling of the hands
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and the feet we want to elevate those
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hands and feed those extremities to help
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promote blood flow because what's
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happening is those red blood cells are
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sticking together decreasing perfusion
00:16:53
to those extremities pain like I said
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they can have this back joint throughout
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wherever these episodes are happening
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and that's because of that ischemia
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that's occurring because those sickle
00:17:04
cells decreasing blood flow and best way
00:17:08
to treat it is opioid medications
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around-the-clock not PR in as needed
00:17:14
when they request it but around the
00:17:16
clock to help control pain because it's
00:17:18
very very painful also warm compresses
00:17:21
on wherever the pains at not like cold
00:17:24
compresses why not cold well remember
00:17:27
with one of our factors cold can
00:17:29
actually increase the demand
00:17:30
of oxygen which can stress those cells
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out and cause worse sickling and we want
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to make sure that we're not leaving
00:17:37
restrictive items on them if they have a
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watch on or the don't leave by blood
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pressure cuff on their arm because
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that's gonna impede blood flow even more
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so make sure that they're not having
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anything restrictive on their
00:17:50
extremities another thing of course is
00:17:52
anemia and if they are going through a
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hyper hemolytic or an aplastic crisis
00:17:58
they have that breakdown of red blood
00:17:59
cells that's accelerated or they're just
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not really making any and you go to
00:18:04
watch out for increase heart rate with
00:18:07
this because the heart's trying to
00:18:08
compensate for those low oxygen levels
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infants can be really tired or fussy
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shortness of breath just where there's
00:18:16
not enough oxygen present whenever they
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just try to do a simple task it's like
00:18:20
wow I'm really short of breath of course
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low red blood cells making sure that
00:18:25
you're looking at those mucous membranes
00:18:27
to assess for pallor paleness because
00:18:31
most patients who have sickle cell
00:18:32
anemia they are African Americans so in
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order to tell if they're pale you need
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to look at the mucous membranes are they
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really lie instead of the dark red pink
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color and looking at the growth and
00:18:44
development because I can affect this in
00:18:46
children with the severe anemia so
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nursing standpoint blood transfusions
00:18:51
replacing them with fresh new red blood
00:18:55
cells increasing that number increasing
00:18:58
the oxygen available to the body also
00:19:01
folic acid why folic acid this helps
00:19:05
with red blood cell production so they
00:19:08
may be ordered to take a supplement in
00:19:09
bat rather than iron iron supplements
00:19:12
don't really help this type of anemia
00:19:14
like the other anemia as we've talked
00:19:16
about and in some patients with sickle
00:19:18
cell disease giving them iron
00:19:19
supplements can actually cause toxicity
00:19:21
where the iron will collect in the
00:19:23
organs so you want to avoid that now if
00:19:26
you aren't familiar with blood
00:19:28
transfusions from the nursing standpoint
00:19:30
I have a whole video on that that you
00:19:32
can watch and access to help you review
00:19:33
for NCLEX for that another thing is
00:19:35
infection we talked a lot about this the
00:19:38
spleens not working well or here
00:19:40
for infection because that helps filter
00:19:42
out or foreign invaders plays a role
00:19:44
with that and so one of these patients
00:19:47
can get pneumonia so you want to be
00:19:49
looking at respiratory status assessing
00:19:51
those breath sounds whether what's their
00:19:53
oxygen saturation how are they breathing
00:19:54
because a lot of patients can enter into
00:19:56
acute chest syndrome this can be
00:19:58
preceded by pneumonia or a pulmonary
00:20:01
embolism and a lot of times people with
00:20:03
sickle-cell disease have abnormal
00:20:05
clotting so they're at risk for throwing
00:20:08
off clots as well so monitor that and if
00:20:11
a patient has this they will have a
00:20:14
fever chest pain called low oxygen
00:20:18
saturation that'll probably tip the
00:20:20
physician off to order a chest x-ray
00:20:22
which is going to show you aliy a new
00:20:25
infiltrate on the chest x-ray so as a
00:20:28
nurse make sure you are being aware of
00:20:30
that and thinking of that as well
00:20:31
another thing is gall stones why in the
00:20:34
world gall stones well remember we have
00:20:37
the rupturing of a red blood cells
00:20:39
they're very fragile so if you are
00:20:42
breaking down lots of red blood cells
00:20:43
because you're in the severe crisis what
00:20:45
do red blood cells release Billy Reuben
00:20:47
well the body can't deal with all this
00:20:49
Billy Reuben so the gallbladder it
00:20:51
starts to make Stones out of it so
00:20:54
because the gallbladder you can't really
00:20:55
keep up so they will have that so watch
00:20:57
out for signs and symptoms of gall
00:20:59
stones like chest pain simular like
00:21:01
heartburn even some people have reported
00:21:03
it feels like they're having a heart
00:21:05
attack
00:21:05
lower pain in their shoulder where their
00:21:08
shoulder blade as well getting nausea
00:21:11
things like that next stroke this can
00:21:13
occur because if those red blood cells
00:21:16
are sticking together in important
00:21:19
vessels that feed our brain tissue it
00:21:21
can decrease blood supply and cause
00:21:24
brain death and what you want to be
00:21:27
doing is you want to make sure you're
00:21:28
monitoring their neuro status with that
00:21:30
as well and if you need an in clicks
00:21:32
review on stroke I have a whole one
00:21:34
right there that you can check out as
00:21:36
well
00:21:36
another thing is eye problems and it's
00:21:38
sort of the same concept all the sticky
00:21:41
red blood cells that feed the nerves so
00:21:43
the eyes can become blocked and they can
00:21:46
have vision changes so assessing their
00:21:48
vision making sure that they go and get
00:21:50
regular vision checkups and
00:21:53
leg ulcers this is typically in your
00:21:56
older children all your infant so the
00:21:58
doctor lightest the hannam fit syndrome
00:22:00
to main leader infants are leg ulcers
00:22:02
are going to be in our older children
00:22:03
and this is the decreased blood flow
00:22:05
that vaso occlusion to the leg so they
00:22:08
get ulcers that can be very painful and
00:22:10
slow healing now let's talk about the
00:22:12
prevention pieces on how to prevent a
00:22:16
sickle cell crisis okay well you want to
00:22:19
educate the patient or the parents is
00:22:21
number one they're at risk for infection
00:22:23
so you want to tell them they want to be
00:22:26
getting their vaccinations up-to-date
00:22:28
especially the pneumococcal influenza
00:22:31
and meningococcal because they're at
00:22:33
risk for developing this and you wanna
00:22:35
make sure they have those vaccines on
00:22:36
board avoiding high altitudes climbing
00:22:39
flying preventing infection hand hygiene
00:22:42
taking the prophylactic penicillin if
00:22:46
that's what the physician is ordered
00:22:48
staying away from sick people
00:22:50
hydration making sure that they are
00:22:53
staying on top of drinking fluids
00:22:56
consuming fluids especially on days when
00:22:59
it's hot that's usually last thing
00:23:01
you're thinking about that if you're
00:23:02
getting sweaty you're gonna be working
00:23:03
out you want to make sure you're keeping
00:23:05
your hydration because this continued
00:23:06
into one of these episodes keeping
00:23:08
stress levels low not smoking and not
00:23:11
overdoing exercise now let's quickly
00:23:14
talk about some medications used to
00:23:16
treat sickle-cell anemia
00:23:18
a common one is called hydroxyurea and
00:23:21
this medication is actually used to
00:23:24
treat some forms of cancer but they have
00:23:26
found that it actually can help some
00:23:28
sickle cell anemia patients now what
00:23:30
does it do now remember I told you to
00:23:32
remember that about the fetal hemoglobin
00:23:35
well this is what this medication does
00:23:37
it creates the fetal hemoglobin which is
00:23:40
going to decrease that sickling so
00:23:42
remember that fetal hemoglobin was what
00:23:44
was present in that baby that first six
00:23:47
months of life that helped them
00:23:49
transport oxygen then they get a little
00:23:50
bit older and their genes take over
00:23:53
producing hemoglobin SS and they get
00:23:55
sickle-cell disease so this we can throw
00:23:57
this on and it will help create that
00:23:59
fetal hemoglobin protein and it also
00:24:02
will help with anemia which will
00:24:03
decrease the need for so many
00:24:06
blood transfusions but one thing about
00:24:08
this medication that you want to watch
00:24:10
out for and educate the patient about is
00:24:11
that it lowers the white blood cell
00:24:13
count so that will need to be watched
00:24:15
and you'll need to teach the patient go
00:24:17
to hand hygiene avoiding people with
00:24:19
infection now is there a cure for
00:24:22
sickle-cell anemia
00:24:24
well a stem cell transplant can be
00:24:26
performed where the bone marrow will be
00:24:29
made to produce new healthy red blood
00:24:33
cells without the abnormal hemoglobin on
00:24:36
it
00:24:37
however it's rare because patients must
00:24:39
be matched with a donor to have a
00:24:42
successful stem cell transplant okay so
00:24:45
that wraps up this lecture over sickle
00:24:48
cell anemia thank you so much for
00:24:50
watching don't forget to take the free
00:24:52
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