Sickle Cell Anemia Nursing | Symptoms, Pathophysiology, Sickle Cell Crisis & Trait
Summary
TLDRIn this educational video, Sarah French Turner discusses sickle cell anemia, a genetic blood disorder characterized by abnormal hemoglobin S, which causes red blood cells to become rigid and sickle-shaped under low oxygen conditions. This leads to various complications, including anemia and increased risk of infections. The video covers the pathophysiology, types of crises, and nursing interventions for management. It also highlights the importance of hydration, avoiding triggers, and the potential use of hydroxyurea to increase fetal hemoglobin. Although stem cell transplants offer a cure, they are rare due to donor matching requirements.
Takeaways
- 😷 Sickle cell anemia is a genetic condition characterized by abnormal hemoglobin (hemoglobin S) in red blood cells, leading to a sickle shape and reduced oxygen transport.
- 🩸 Hemoglobin S is sensitive to low oxygen levels, causing red blood cells to become rigid, sticky, and sickle-shaped, which can obstruct blood flow and lead to crises.
- 🌡️ Triggers for sickle cell crises include high altitude, infection, dehydration, and extreme temperatures, all of which can increase the body's demand for oxygen.
- 🧬 Sickle cell anemia is an autosomal recessive disease, requiring inheritance of two abnormal hemoglobin S genes, one from each parent.
- 👶 The condition often presents in infancy, typically around 6-8 months when fetal hemoglobin levels decrease, and hemoglobin S starts to dominate.
- 🏥 Newborn screening and prenatal testing, such as amniotic fluid testing, are available to detect sickle cell anemia early.
- 🧪 The thionite test and hemoglobin electrophoresis are common diagnostic tests to identify and differentiate sickle cell anemia from the sickle cell trait.
- 🩹 Symptoms of a sickle cell crisis include pain, dactylitis (swelling of hands and feet), anemia, and potential organ damage due to blocked blood flow.
- 💊 Hydroxyurea is a medication that can increase fetal hemoglobin and reduce the severity of sickle cell anemia, but it requires monitoring due to its potential to lower white blood cell count.
- 🏥 Management of sickle cell anemia includes hydration, pain control, blood transfusions, and avoiding triggers to prevent crises, with a stem cell transplant being a potential cure in specific cases.
Q & A
What is sickle cell anemia?
-Sickle cell anemia is a condition where a patient has abnormal hemoglobin, specifically hemoglobin S, on their red blood cells. This type of hemoglobin is sensitive to low oxygen levels, causing red blood cells to change shape into a sickle or C-shape, becoming stiff and sticky, which can block blood flow to organs and tissues.
Why are red blood cells with hemoglobin S problematic?
-Red blood cells with hemoglobin S are problematic because they become stiff and sticky under low oxygen conditions, leading to a sickle shape. This shape causes them to clump together and block blood flow, potentially leading to organ damage and pain.
What is the difference between hemoglobin A and hemoglobin S?
-Hemoglobin A is the normal type of hemoglobin that allows red blood cells to function properly, carrying oxygen throughout the body. Hemoglobin S, on the other hand, is abnormal and causes red blood cells to become sickle-shaped under low oxygen conditions, leading to complications.
How does the spleen become affected in sickle cell anemia?
-The spleen becomes affected in sickle cell anemia because it is overworked trying to recycle the rapidly breaking down red blood cells. Additionally, blood flow to the spleen can be blocked by the clumping of sickle-shaped red blood cells, potentially leading to splenomegaly and an increased risk of infection.
What causes sickle cell anemia?
-Sickle cell anemia is caused by inheriting abnormal hemoglobin S genes from both parents, resulting in a homozygous condition where the individual has hemoglobin SS on their red blood cells.
Why do symptoms of sickle cell anemia often present around 6 to 8 months of age?
-Symptoms of sickle cell anemia often present around 6 to 8 months of age because that is when the fetal hemoglobin, which is less prone to sickling, is replaced by hemoglobin S as the child grows. This switch makes the red blood cells more sensitive to low oxygen levels, leading to the onset of symptoms.
What is the significance of the thionite test in diagnosing sickle cell anemia?
-The thionite test, also known as sickle solubility test, is significant in diagnosing sickle cell anemia because it can detect the presence of abnormal hemoglobin. However, it cannot differentiate between sickle cell trait and full sickle cell disease, requiring a follow-up hemoglobin electrophoresis test for confirmation.
How does hydroxyurea help in treating sickle cell anemia?
-Hydroxyurea helps in treating sickle cell anemia by increasing the production of fetal hemoglobin, which is less likely to sickle and thus reduces the severity of the disease. It also helps with anemia by decreasing the need for blood transfusions.
What is the role of the thionite test in newborn screening for sickle cell anemia?
-The thionite test is often used as part of newborn screening for sickle cell anemia to identify the presence of abnormal hemoglobin early. Early detection allows for prompt intervention and management of the condition.
What is the potential cure for sickle cell anemia mentioned in the script?
-A stem cell transplant is mentioned as a potential cure for sickle cell anemia. This procedure involves replacing the bone marrow to produce new, healthy red blood cells without the abnormal hemoglobin. However, it requires a matched donor and is not commonly performed.
Outlines
🩸 Understanding Sickle Cell Anemia
Sarah French Turner introduces sickle cell anemia, a condition characterized by abnormal hemoglobin (hemoglobin S) in red blood cells. This abnormality makes the cells sensitive to low oxygen levels, leading to a sickle shape that can obstruct blood flow and cause crises. The video is part of a hematology review series, and viewers are encouraged to take a quiz to test their knowledge. The discussion highlights the importance of recognizing the onset of the disease, often around 6-8 months of age when fetal hemoglobin, which protects against sickling, decreases.
🧬 Genetic and Testing Aspects of Sickle Cell Anemia
The paragraph delves into the genetic basis of sickle cell anemia, explaining it as an autosomal recessive disease requiring inheritance of abnormal hemoglobin genes from both parents. It discusses the difference between carriers with sickle cell trait and those with the disease, emphasizing the rarity of symptoms in carriers. The risk is particularly high among African Americans, with one in twelve carrying the trait. Newborn screening and prenatal testing via amniotic fluid are mentioned as methods for early detection. The thionite test and hemoglobin electrophoresis are highlighted as diagnostic tools, with the latter able to differentiate between the sickle cell trait and full-blown disease.
🌡️ Triggers and Types of Sickle Cell Crises
This section outlines the factors that can precipitate a sickle cell crisis, using the acronym 'SICKLE' to remember elements that lead to low oxygen levels in the body. These include significant blood loss, illness, climbing to high altitudes, stress, low fluid intake, and extreme temperatures. The paragraph discusses the different types of crises, such as vaso-occlusive, hemolytic, aplastic, and spleen sequestration crises, each with its own set of symptoms and complications. The focus is on understanding the body's response to these triggers and the importance of patient education to prevent crises.
🩹 Managing Sickle Cell Crises and Symptoms
The discussion shifts to managing sickle cell crises, with a focus on nursing interventions. Key strategies include maintaining hydration, administering oxygen, ensuring pain control, and monitoring respiratory status. The importance of blood transfusions and folic acid supplementation is highlighted, along with the avoidance of iron supplements due to potential toxicity. The paragraph also addresses the signs and symptoms to watch for, such as dactylitis, pain, anemia, and infection risks, with an emphasis on the role of the spleen and the potential for acute chest syndrome and stroke.
💊 Treatment and Prevention of Sickle Cell Anemia
The final paragraph covers the treatment and prevention of sickle cell anemia. It mentions hydroxyurea, a medication that increases fetal hemoglobin and reduces sickling, but also lowers white blood cell count, necessitating careful monitoring and infection prevention. The video concludes with a discussion on the potential cure through stem cell transplants, which requires a matched donor. Prevention strategies include vaccination, avoiding high altitudes and infections, maintaining hydration, managing stress, and avoiding smoking and excessive exercise. The lecture wraps up with a reminder to take the quiz and subscribe for more educational content.
Mindmap
Keywords
💡Sickle Cell Anemia
💡Hemoglobin
💡Crisis
💡Anemia
💡Hemoglobin Electrophoresis
💡Autosomal Recessive
💡Hydroxyurea
💡Stem Cell Transplant
💡Dactylitis
💡Acute Chest Syndrome
Highlights
Sickle cell anemia is caused by abnormal hemoglobin S in red blood cells.
Hemoglobin S is sensitive to low oxygen levels, causing red blood cells to change shape and become sickle-shaped.
Sickle-shaped red blood cells can block blood flow to organs, leading to sickle cell crisis.
Normal red blood cells last about 120 days, while sickle cells last only about 20 days, causing anemia.
The spleen is often affected in sickle cell anemia, becoming overworked and at risk for infection.
Sickle cell anemia is the most common and severe form of sickle cell disease.
The condition is inherited when both parents pass on the abnormal hemoglobin S gene.
Symptoms often present between 6 to 8 months of age when fetal hemoglobin is replaced by hemoglobin S.
Sickle cell anemia is an autosomal recessive disease, requiring two copies of the abnormal gene for manifestation.
African Americans have a higher risk of carrying the sickle cell trait, increasing the likelihood of the disease.
Newborn screening and amniotic fluid testing can detect sickle cell anemia in early stages.
The thionite test and hemoglobin electrophoresis are used to diagnose sickle cell anemia.
Normal red blood cells are round and smooth, without a nucleus, allowing for efficient oxygen transport.
Sickle cell crisis can be triggered by factors that decrease oxygen levels or increase the body's demand for oxygen.
Vaso-occlusive crisis is a type of sickle cell crisis where blood vessels become blocked by sickle cells.
Hydration and oxygen therapy are crucial in managing sickle cell crisis to prevent complications.
Pain management with opioids is essential for patients experiencing a sickle cell crisis.
Blood transfusions and folic acid supplements can help manage anemia in sickle cell anemia.
Hydroxyurea is a medication that increases fetal hemoglobin, reducing sickling in red blood cells.
Stem cell transplants offer a potential cure for sickle cell anemia but require a matched donor.
Transcripts
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
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