Thank you. Welcome back, everybody. This is Food
for Thought Thoughts If It Were You by Neogentrics.
Now, I'm just going to continue this whole thing
that I'm talking about with the sickle cell anemia.
It is very important. It's a very volatile topic.
A lot of people don't know about it, or if they
know it's there, they don't know anything about
it. Now, last time we covered a couple of the
different types of sickle cell that exist. There
is still two remaining. They're much like the
last couple, if you've listened to the last podcast.
Those would be sickle cell SS and sickle cell
SE hemoglobin types. In the SE format, they have
one that has the S trait and one that has the
E trait. In the SS format... Both parents have
the sickle cell trait type S and the child inherits
both. This being one of the more severe cases,
not as bad as beta zero and beta plus, but of
the same anomaly. The conditions for this though
is an abnormal protein mutation in the genes.
Now I'm not going to go into these two too in
depth. But just know that they do a lot of screening
and checking for these specific ones as well.
Pushing on these factors. Now, like I mentioned
before, there are a lot of risk factors involved
with this. And in the United States, most people
with this disease are African American of ancestry.
And they identify themselves as black more or
less. About 1 in 13 black Americans are born
with this trait. And one out of every 365 black
American babies are born with a sickle cell disease
in the first place. But there are some people
who come from the Hispanic, Southern, European,
Middle Eastern, or Asian Indian backgrounds as
well. Summing it up to about 100 ,000 Americans
who even have this disease to start with. Now...
There's things for screening and prevention in
place to allow for early detection of this. Okay,
and people who do not know whether or not they
carry the abnormal gene can ask their doctors
to have blood tests to check to see if they do.
Couples who are planning to have children and
know that they are at risk of the child having
the disease may want to meet with a genetic counselor.
The genetic counselor can answer any questions
about the risk and explain the choices that are
available. Okay? Now, the thing is, what to look
for, I mean, They're going to diagnose, diagnosis
will discuss tests, procedures that your doctor
may want to use to diagnose a sickle cell disease
for you and your baby. Living with is one of
the things that they want to talk to you about.
They'll discuss with you a recommended way to
manage your complications. There's a lot of research
in health regarding this. They'll also discuss
with you about their current research and advancing
research to treat and cure the said diseases.
So some people might want to look into that as
well. And then if you want to, you can participate
in clinical trials where they openly discuss
and enroll clinical studies on people and their,
well, mostly their blood, not so much the people,
and investigate treatments and approaches to
deal with these sickle cell diseases. Okay? Let's
go into signs, symptoms, complications, things
of that nature. If a person has the disease,
it is present at birth. But most infants don't
have any problems from the disease until they're
about five or six months of age anyway. Every
state in the United States, especially the District
of Columbia and every U .S. territory, it is
required to have newborn babies receive screening
for this disease. When a child has the disease,
the parents are notified immediately. Some children
with the sickle cell disease will start having
problems early on and some later. Early symptoms
of the disease may include a yellowish color
of the skin, known as jaundice, or whites of
the eyes, known as icterus. That occurs when
a large number of red blood cells undergo hemolysis.
It's not pretty. I'm just going to say that right
now. Fatigue or fussiness from the anemia. Painful
swallowing of the hands and feet known as dactylitis.
The signs and symptoms of sickle cell will vary
from person to person and can change over time.
Most of the signs and symptoms of a sickle cell
disease are related to complications of the disease.
A lot of the complications include acute pain.
Sickle cell or vaso -occlusive crisis is one
word for it. The pain episodes or crises may
occur without warning when sickle cell block...
blood flow, and decreased oxygen delivery. People
describe this pain as sharp, intense, stabbing,
and throbbing, and or throbbing. It could be
either or or both. Severe cries can even be even
more uncomfortable than post -surgical pains
or childbirth. Surprisingly enough, a person
screaming in pain of this is more ear -piercing
than a lady in labor, believe it or not. Pains
can strike almost anywhere in the body and in
more than one spot at a time But the pain often
occurs in like several different places I mean
it could be in your abdomen your arms your chest
your lower back your legs I mean a crisis can
be brought on by various conditions high altitudes
dehydration not drinking enough fluids illness
stress temperature changes I mean, but often
a person doesn't know the triggers or the causes
of the crisis. It just happens at random I mean
many adolescents and adults with sickle cells
suffered from chronic pain. I mean, this kind
of pain can be hard for people to describe, but
it is usually different from crisis pains and
the pain that results from organ damage. Chronic
pain can be severe and can even be, it makes
your life difficult. Let me just say it that
way. Its cause is not well understood, for one.
How do I put this? Severe anemia. Let me go with
that. People with the single cell disease usually
have mild to moderate anemia. It's usually not
that bad in some people's cases. Minus the SS,
SE, and SEB. I mean, minus those situations,
okay? Those can be minor too, but I'm just saying.
At times, however, they can have severe anemia.
Severe anemia can be life -threatening, okay?
Especially in an infant or a child. Many different
things can be caused by this. A plastic crisis.
It's usually caused by a parvovirus B19 infection.
It's also known as the fifth disease or slapping
cheek syndrome. It's a very common infection,
but in sickle cell disease, it can cause the
bone marrow to stop producing new red blood cells
for a while. And this is dangerous, okay? Leading
to some very, very severe anemia, okay? Another
one would be blood getting trapped in the spleen.
Now, I know I've mentioned this before, okay?
And I've played it off like it's not as bad,
but I mean, honestly, it is. The spleen is an
organ that is located in the upper left side
of your body, okay? And it filters your blood
and destroys old blood cells. And people who
have this disease, the red blood cells get trapped
in the spleen, making it enlarge, okay? With
red blood cells trapped in the spleen, fewer
are available to circulate through the body,
and this can offset anemia, and the large spleen
may cause pains in your left side and in the
belly, and a parent can usually feel the enlarged
spleen in the belly of his or her child. With
this being said, if your spleen should ever explode,
you're in actually a lot of danger, because all
that's going to start spreading throughout your
body, and it can actually be as poison to you,
so this is just a heads up to you. Splenic sequestration.
Man, these words are hard to pronounce. I wrote
them down, okay? That's the reason I wrote them
down because I can't remember them because they're
so hard to say. Splenic sequestration. Okay.
Crisis and a plastic crisis most commonly occur
in infants and children though who have the disease
anyway But adults who have this the the cell
the sickle cell disease may also experience episodes
of severe anemia still from time to time Okay,
but these usually have other causes no matter
the cause those severe meaning anemia can lead
to symptoms that include fatigue dizziness pale
skin shortness of breath I don't know why I said
that so sluggishly But babies and newborns with
severe anemia may not want to eat and may seem
very sluggish. With that being said, there are
infections to also be aware of. The spleen is
important for protection against certain kinds
of infections. So there's its function. The sickle
cells can damage the spleen and weaken and or
destroy its functions even if... Even in young
patients. And I mean, people who have the disease,
who have damaged spleens, are at risk of severe
bacterial infections in the blood, lungs, brain,
bones. I mean, it's life -threatening. Let's
just put it that way. A lot of the bacterias
that you can end up with, one of them being chlamydia,
ironically enough, it's not just an STD. You
can get that from this. Hemophilius, influenza
type B, meningococcus, mycoplasma, Uh, pneumonia,
uh, pneumococcus, salmonella can be resulted
as this, uh, cephalococcus. I hate that word.
Also is one of the, uh, one of the different
bacteria you can get infected with. And keep
in mind, each one of these is worse going down.
Okay. I listed them in that order on purpose.
Chlamydia is not as bad as it sounds, believe
it or not. That's why it's at the top. It's bad,
but it's not that bad. Then there's the other
one, acute chest syndrome. Sickling in blood
cells of the lungs can deprive a person's lungs
of oxygen, obviously. And when this happens,
areas of the lung tissue can get damaged and
cannot exchange oxygen properly. And this is
known as acute chest syndrome. At least one segment
of the lung is damaged as a result of this. And
the condition is very serious and should be treated
immediately if it should ever happen. It usually
often starts a few days after a painful crisis
begins. A lung infection may accompany acute
chest syndrome at any given time. And with that,
the symptoms may include chest pains, coughing,
fever, rapid breathing, shortness of breath.
Now we're getting into the more serious stuff
here. I had to take a break. Everyone needs a
break. We're going to move on to the next thing
here. I stopped with the last topic. I was talking
about acute chest syndrome. Let me go into another
more complicated matter. It also affects your
brain, okay? This disease, it's very, very important.
It's one of the main fields that, you know, aside
from cancer, is... very detrimental to a lot
of humans now while it's not as common a thing
it's still you know it we lose a lot of people
as a result of it okay so with the brain complications
okay there's something called a clinical stroke
okay a stroke occurs when blood flow is blocked
to a part of your brain okay and when this happens
the brain cells can be damaged or they can die
which i know how bad that sounds but i mean it's
basically the case in sickle cell disease a clinical
stroke means that a person shows Outward signs
that something is definitely wrong with them.
The symptoms depend upon whether a part of the
brain is affected. Symptoms of a stroke may include
loss of balance, severe headache, trouble speaking,
walking and understanding, weakness of an arm
or leg on one side of the body. As many as 24
% of people with hemoglobin SS, out of all of
them, 10 % of people with hemoglobin SC may suffer
a clinical stroke by the age of 45. Now, in children,
clinical strokes occur most commonly between
the ages of 2 to 9, but recent prevention strategies
have lowered that risk. Now, when a person who
has these symptoms, or rather, shows symptoms
of a stroke with the sickle cell disease, their
family or friends are required to dial 911 immediately
because a solid stroke, or even a stroke in general,
can be detrimental. The other one to worry about
is silent stroke and thinking problems. Brain
imaging and tests of thinking cognitive studies
have shown that children and adults with hemoglobin
SS and hemoglobin sigma beta 0 often have signs
of silent brain injury, also called silent strokes.
Silent brain injury is damage to the brain without
showing outward signs of a stroke at all. And
this injury is common. Silent brain injury can
lead to difficulty in hearing, making decisions,
or holding down a job. Believe it or not, that's
a cause. Another one would be eye problems. Like
I mentioned before, it can injure blood vessels
in the eyes. A common site of damage is the retina.
Where blood vessels can overgrow, get blocked,
or bleed. And the retina is the light, sensitive
layer of tissue that lines the inside of the
eye and sends visual messages through the optic
nerve to your brain. Detachment of the retina
can occur from this. And when the retina detaches,
it is lifted and pulled from its normal position.
These problems can cause visual impairment or
vision loss. So yeah, going blind is a big deal.
Another one would be heart disease. And this
one should be pretty much understood. But, you
know, I'm going to cover it anyway. People who
have this problem with blood vessels in the heart
cause, you know, heart problems and heart function
issues. The heart can become larger than normal
because they're building up inside it. People
who have sickle cell can also develop pulmonary
hypertension. And, you know, basically to block
the blood vessels to the point where the heart
can't pump. And it just stops. It's similar to
cardiac arrest. Except the heart is basically
inflamed, like it's enlarged. People who have
the sickle cell disease who have received frequent
blood transfusions may also have heart damage
from iron overload as well. The pulmonary hypertension.
In adolescents and adults, injury to blood vessels
in the lungs may make it hard for the heart to
pump blood through those blood vessels because
they're damaged. For one, this causes the pressure
in the lung blood vessels to rise. High pressure
through these vessels is called pulmonary hypertension.
Symptoms may include shortness of breath, fatigue.
When this condition is severe, it is also associated
with a higher risk of death. For one, you can't
breathe. Another thing to keep in mind is kidney
problems. The kidneys are sensitive to the effect
of blood, especially the red blood cell cells.
Sickle cell disease causes the kidneys to have
trouble making the urine as concentrated as it
should be. This may lead to the need to urinate
often, and I mean often, to the point where you're
bedwetting and you have uncontrollable urine
during the night, like you'll wake up and your
bed's just freaking soaked. I mean, this often
starts in childhood and other problems may occur
as well, such as blood in the urine, decreased
kidney function, kidney failure, in some cases
kidney disease, protein loss in the urine as
well. Uh, no, another one that's not as common
would be, uh, pre -epism. Males who have the
sickle cell disease can have unwanted, sometimes
prolonged, painful erections. Okay. And this
condition is called pre -epism, uh, pre -epism.
I hate this word. Anyway, when blood flows out
of the erected, uh, penis, it is, it blocked
it. How do I say this? When it flows out of the
penis, it's blocked. bicycle cell okay and if
it goes on for a long period of time it can cause
permanent damage to your uh package and lead
to uh impotence impotence i hate that word there's
a lot of words i don't like but specifically
talking about a man's junk in this regard it's
just painful to even say these words and the
thing is a pre -opism can last for more than
four hours if it does you need to see medical
attention immediately i take that back if it
occurs at all for more than 20 minutes seek medical
attention just the thought of this as another
guy is just awful moving right along here Next
is gallstones. I don't think I need to even explain
what this is. But basically, when the red cells
undergo hemolysis, they release hemoglobin. This
is understood. Hemoglobin gets broken down into
a substance called bilirubin. It can form stones
that get stuck in the gallbladder. The gallbladder
is a small, sack -shaped organ beneath the liver
that helps with digestion. And the gallbladder
is... You know, gallstones are a common problem
with sickle cell disease, okay? They may be formed
early, but may not produce symptoms for years,
okay? And when symptoms do develop, just a thought
of this, it could cause nausea, vomiting, right
side upper belly pain. I mean, if problems continue
to occur, you're going to need surgery to remove
them. Just put it this way. And by the way, if
you ever have to pass a gallstone, God help you.
That crap is painful. You think it's bad enough
when you hear someone scream bloody murder, just
wait till this happens to you. Or rather yet,
make sure you drink lots of fluids so this doesn't
happen to you. Another is a liver complication.
There are a number of ways in which the liver
may be injured by the sickle cell disease. Sickle
cell intraheptic colostasis. It is an uncommon
but severe type of liver damage that occurs when
sickle red cell blocks blood vessels in the liver.
This blockage prevents enough oxygen from reaching
liver tissue. These episodes are usually sudden
and may reoccur. Children often recover, but
some adults may have chronic pain that leads
to liver failure later. People who have sickle
cell disease who have received frequent blood
transfusions may also develop liver damage from
iron overload. um another one would be leg ulcers
sickle cell ulcers are sores that usually start
small and they get larger and larger the number
of ulcers can vary from one to many some ulcers
will heal quickly but others may not heal and
may last for long periods of time and some ulcers
come back after healing so this is another problem
to keep be aware of People who have sickle cell
disease usually don't get ulcers until after
the age of 10, but the fact remains that they
still are there. Another one would be joint complications,
sickling in the bones of the hip, which is less
common. The shoulder joints, knees, ankles can
decrease oxygen flow and result in severe damage
in those areas as well. The damage is a condition
called... a vascular or a septic necrosis. The
disease is usually found in adolescents and adults,
not so much kids because their bodies haven't
developed yet. I mean, but the symptoms include
pain and problems with walking and joint movement.
A person may need pain medicines, surgery, or
joint replacement if the symptoms even persist.
Another one would be delayed growth and puberty.
Children who have the sickle cell disease may
grow and develop more slowly than their peers
because of the anemia. They will reach full sexual
maturity, but this may also be delayed. Another
one would be pregnancy. Pregnancy in women who
have this disease can be risky, both for the
mother and for the child. Mothers have the medical
complication that includes blood clots, high
blood pressure, increased pain episodes, infections.
Oh, and on that note, they also have a higher
risk of miscarriages, premature births, small,
for -date, or underweight babies as another problem.
And while this sounds sad, this is the god -awful
truth of the situation. Another thing to be aware
of is mental health. In another chronic... As
in other chronic diseases, people who have sickle
cell disease may feel sad, frustrated in times.
I mean, the limitations that sickle cell disease
can impose on a person's daily activities may
cause them to feel isolated from others at times,
sometimes even depressed. People who have the
disease may also have trouble coping with pain,
fatigue, as well as the frequent medical visits
and hospitalizations that they have to deal with.
You know, there are things to look for that would
help with this, like treatments. When you go
to the doctor, they discuss different treatments
to help with complications or side effects. And
then they help you to live with these different
things that happen to you. I will say this. As
tragic as this disease sounds, in some cases,
it's a lot worse than in other scenarios. But
the fact of the matter remains that they can
at least still do something for you. Okay? Now,
that's it for now with this specific topic. Next
time, we'll be covering a little bit more in
this field. Enjoy the rest of your day. Again,
this is Food for Thought, Thoughts If It Were
You by Neogentrics. Please look forward to part
three.