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

Summary

In 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

00:00

🩸 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.

05:00

🧬 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.

10:02

🌡️ 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.

15:02

🩹 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.

20:03

💊 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.

Keywords

💡Sickle Cell Anemia

Sickle Cell Anemia is a genetic disorder characterized by abnormal hemoglobin, called hemoglobin S, in red blood cells. This abnormality causes red blood cells to become rigid, sticky, and sickle-shaped under low oxygen conditions, leading to various health complications. In the video, Sarah French Turner discusses how this condition can lead to crises, affecting organs and tissues due to blocked blood flow, and the importance of understanding this disease for nursing interventions.

💡Hemoglobin

Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. In the context of the video, normal hemoglobin is referred to as hemoglobin A, while the abnormal type is hemoglobin S, which is sensitive to low oxygen levels and can cause red blood cells to sickle. The video explains the significance of hemoglobin S in causing sickle cell anemia and the various complications that arise from its presence.

💡Crisis

In the video, a 'crisis' refers to a sudden worsening of symptoms in individuals with sickle cell anemia. This can include vaso-occlusive crises, where sickle-shaped red blood cells block blood vessels, leading to severe pain and organ damage. Crises can be triggered by various factors such as infection, dehydration, or stress, and are a critical aspect of managing the disease discussed in the script.

💡Anemia

Anemia is a condition where the body lacks a sufficient number of healthy red blood cells to carry adequate oxygen to tissues. In the video, Sarah French Turner explains that individuals with sickle cell anemia suffer from anemia because their red blood cells, which contain hemoglobin S, have a shortened lifespan and are prone to breaking down, leading to a reduced ability to transport oxygen.

💡Hemoglobin Electrophoresis

Hemoglobin electrophoresis is a laboratory test used to identify the types of hemoglobin present in a blood sample. As mentioned in the video, this test is crucial for diagnosing sickle cell anemia, as it can differentiate between individuals who have the sickle cell trait and those with full-blown sickle cell disease by analyzing the proportions of different hemoglobin types.

💡Autosomal Recessive

The term 'autosomal recessive' refers to a pattern of genetic inheritance where a person must inherit two copies of an abnormal gene (one from each parent) to exhibit the disease. In the video, Sarah French Turner explains that sickle cell anemia is an autosomal recessive disease, meaning that a child will only have the disease if both parents pass on the abnormal hemoglobin S gene.

💡Hydroxyurea

Hydroxyurea is a medication discussed in the video that has been found to be beneficial in treating some patients with sickle cell anemia. It works by increasing the production of fetal hemoglobin, which does not sickle, thereby reducing the severity of the disease. The video script highlights the importance of this medication in managing the condition and its role in reducing the need for blood transfusions.

💡Stem Cell Transplant

A stem cell transplant is a potential treatment for sickle cell anemia mentioned in the video. It involves replacing the patient's bone marrow with healthy bone marrow from a donor, which can produce normal red blood cells. The video explains that while this can be a curative treatment, it is rare due to the need for a matched donor and the complexities of the procedure.

💡Dactylitis

Dactylitis, also known as hand-foot syndrome, is a condition characterized by swelling of the hands and feet, often seen in young children with sickle cell anemia. In the video, Sarah French Turner describes it as a symptom where red blood cells sickle and block blood flow to the extremities, causing pain and swelling. It is an example of how sickling can affect different parts of the body.

💡Acute Chest Syndrome

Acute Chest Syndrome is a serious complication of sickle cell anemia where there is sudden inflammation and infection in the lungs, leading to chest pain and difficulty breathing. The video script mentions it as a potential outcome of sickle cell crises, highlighting the importance of monitoring respiratory status in patients with the disease.

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

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