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- [Prakash Chandran] Today, we're talking about
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living with sickle cell disease
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with two experts from Prisma Health.
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I'm excited to be joined by Dr. Alan Anderson.
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He's a director of
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the Comprehensive Lifespan Sickle Cell Disease Program
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at Prisma Health.
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And, Dr. Carla Roberts,
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director of the Pediatric Sickle Cell Disease Program
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at Prisma Health as well.
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(uplifting music)
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This is "Flourish",
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the podcast brought to you by Prisma Health.
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My name is Prakash Chandran.
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So, Dr. Roberts,
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I'd love to start with you
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and hopefully you can just tell us a little bit
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about what sickle cell disease is
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and how it's different from sickle cell anemia.
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- [Dr. Roberts] Great, I'm glad you asked that.
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So, I actually tell people that sickle cell disease
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is kind of like the...
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It's like a family name
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and they're different members of the family.
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There are four main types that we see in the United States.
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There's the SS type
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where somebody's inherited a sickle hemoglobin gene
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from each parent.
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The second most common type is the SC type,
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where someone's inherited the sickle gene from one parent
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and hemoglobin C from the other parent.
1:03
And, then the third and fourth most common types
1:05
are a sickle beta plus thalassemia
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or a sickle beta zero thalassemia,
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where they've inherited the sickle gene from one parent
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and beta-thalassemia from the other parent.
1:15
And, people with beta plus type,
1:17
they can make a little bit of normal hemoglobin,
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usually have a milder type of sickle cell disease,
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whereas people with the beta zeroth thalassemia type
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don't make any normal hemoglobin
1:26
and have a...
1:27
May have a more severe form of sickle cell disease.
1:29
So, technically the term sickle cell anemia,
1:33
that refers to the most severe types of sickle cell disease,
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which are the, in general,
1:38
the SS type and the sickle beta zeroth thalassemia type.
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So, it's...
1:42
I think people...
1:43
A lot of people,
1:44
they kind of use these terms loosely,
1:45
but it's really important to know
1:46
what type of sickle cell disease someone has,
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'cause that can...
1:50
You can kind of predict how they may behave.
1:52
So, again,
1:53
sickle cell disease is kind of like the family.
1:54
They're different members of the family.
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And, technically,
1:57
sickle cell anemia is referring to the SS
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or sickle beta zero thalassemia type of sickle cell disease.
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- [Dr. Anderson] And, I was just going to add as well,
2:05
sickle cell disease is the most common inherited
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red blood cell disorder seen in the United States
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with over 100,000 individuals affected across the US.
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And, here in South Carolina, we see,
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or it's estimated approximately 2,500 individuals
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affected by sickle cell disease.
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And, specifically here in the upstate,
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we take care of close to 500
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and I know even greater numbers there in the Midlands
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with Dr. Roberts' group as well.
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So, certainly, it's a disease that affects people
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that were around at work, and at school,
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and has a significant number of individuals affected
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across our state and across the country.
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- [Prakash Chandran] Yeah, Dr. Anderson,
2:45
I was just going to ask you about that,
2:46
and maybe you can expand a little bit more
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on who exactly is at risk for sickle cell disease.
2:52
In doing a little research,
2:53
I'm seeing that some demographics
2:55
are affected more than others.
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So, maybe talk broadly about that.
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- [Dr. Anderson] Yes, so the genetic change
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that cause sickle cell disease
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actually comes from areas where malaria is endemic.
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Malaria's a parasite that gets into the red blood cell,
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and causes damage to the red blood cell,
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and is seen in places that are along the equator.
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And, so that is...
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It...
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Going to be areas in central sub-Saharan Africa,
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parts of India,
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parts of Central and South America.
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So, anyone who has genetics coming from those regions
3:24
can be affected by sickle cell disease.
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As Dr. Roberts already mentioned,
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if you get one copy of that gene that causes sickle cell,
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we call that sickle cell trait,
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you're a carrier.
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And, in those places where malaria's a problem,
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that is somewhat protective
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against the malaria parasite getting
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into the red blood cell.
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And, so there are more individuals from those areas
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who will have both parents have sickle cell trait,
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or be carriers,
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and ultimately those two parents
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can pass their gene down to a child
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and the child has an increased chance
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of being affected by sickle cell disease.
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So, within the United States,
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we see a disproportionate effect of sickle cell disease
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on the African American community
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and the majority of patients that we see
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are African American.
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That being said,
4:12
we have high numbers of individuals
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from the Hispanic American community that are affected.
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One in, I think, around 13,000 Hispanic Americans,
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one in 365 African Americans
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are affected by sickle cell disease
4:27
within the United States.
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But, we don't just test babies
4:31
who are of African American descent or other ethnicities.
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The newborn screening that we do for sickle cell disease
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tests all babies,
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regardless of their genetic background,
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their ethnicity, their race, et cetera,
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because we know that it's important
4:46
to diagnose sickle cell disease very early in childhood,
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so that we can do something about it
4:51
to help protect the child that's affected.
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- [Prakash Chandran] Now, Dr. Roberts,
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can you talk about the symptoms
4:56
that someone might experience
4:57
if they have sickle cell disease?
4:59
- Sure, the hallmark of sickle cell disease is pain.
5:03
And, so the red cell that's affected
5:06
with sickle cell disease
5:08
and the red cells should be round and healthy.
5:11
And, if you have sickle cell disease,
5:13
under certain conditions, those shells...
5:15
Those cells can become misshapen like a crescent moon
5:18
or a sickle shape,
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but hence the name sickle cell disease.
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And, those, those sickle cells,
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aren't as healthy
5:25
and they sort of irritate the vessels throughout the body,
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and it blood flows everywhere throughout your body.
5:29
And, so if they...
5:30
If you have an unhealthy vessel with unhealthy blood flows,
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you can't get...
5:34
Deliver any oxygen to whatever that blood vessel
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is trying to supply,
5:38
then that person could experience pain
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and it can be just a little bit of pain
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they're treating at home
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or very severe where they're having to get treatment
5:46
in our clinics, or in the emergency room,
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or in the hospital with IV pain medication,
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and including the opioids,
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and as well as some non anti-inflammatory medicines
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like ibuprofen or an IV form called Ketorolac.
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But, I think a lot of people
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don't realize how much pain
6:05
these people are having to deal with on a daily basis
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or a near daily basis at home
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that they are living with,
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trying to go to school and work.
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And, so we only see a minority of the pain
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coming into the clinic, or the ER,
6:16
the hospital.
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But, a lot of them live with this day after day
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and are quite remarkable and and stoic in their...
6:22
With their sickle cell disease.
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The other problems with sickle cell disease is, people,
6:27
that they can be more susceptible to certain infections,
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because the spleen,
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which I kind of tell people,
6:32
it's kind of like a garbage disposal for your body.
6:33
It helps you get rid of certain infection, and old cells,
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and things.
6:37
It doesn't work like it's supposed to.
6:38
So, these children and adults can be susceptible
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to very severe life-threatening infection.
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And, so that's what Dr. Anderson was saying.
6:45
We do newborn screening so these patients
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can be started on Penicillin
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to try to prevent one certain germ
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and then they are treated with that for several years
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as well as get multiple vaccines.
6:56
And, we react quickly though to any fever.
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These patients can also be at risk
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for basically things from head to toe,
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at risk for stroke even in children,
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though fortunately we have this ultrasound capability now,
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so we can detect in children
7:09
who might be at risk for having a stroke,
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and so we've seen a significant decrease
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with the interventions that we use
7:15
whenever we find someone at risk.
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These patients, they can be at risk for...
7:18
They have...
7:19
Can have eye problems,
7:20
they have to go to start going to the eye doctor
7:21
when they're around 10 years old and older,
7:23
they can have more problems with sleep apnea,
7:26
they can have heart problems, kidney problems,
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liver problems, skin ulcers.
7:32
So, kind of basically really from head to toe
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and they're dealing with this on a daily basis,
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some of them.
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- [Prakash Chandran] Dr. Anderson,
7:38
can you talk to us a little bit about the effects
7:41
of sickle cell disease,
7:42
especially as you age?
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- [Dr. Anderson] Yes, so sickle cell disease
7:46
affects the entire body
7:48
as Dr. Roberts has already mentioned.
7:50
And, I like to talk to my patients and families
7:53
about the fact that it's a chronic illness
7:56
where the damage to any organ system in the body
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that needs oxygen,
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which is every place in our body,
8:03
starts in childhood and then can progress over time.
8:07
And, so we talk about
8:09
more severe types of sickle cell disease
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or less severe types,
8:13
but really when we look at the various types,
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what we're looking at is a difference in time
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at which it takes for the damage to become severe enough
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that then the organ just can't function the way it should.
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And, so we will see, in childhood,
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significant increased risk of stroke
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compared to other children of the same age
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who don't have sickle cell disease.
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We'll see some problems start to occur within the bones
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where you get damage to the bones from lack of oxygen
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that's leading to bone pain and chronic bone damage.
8:46
We get some children starting to show signs
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of early irritation of their kidneys
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or irritation of their eyes, et cetera.
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And, what we see over time is that disease progresses
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or the damage progresses within those organs
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to the point that,
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once they reach young adulthood and then older adulthood,
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we see that patients start showing signs
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of overt organ failure.
9:12
And, so we will have patients dealing
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with severe and chronic damage to their lungs
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that can make them need chronic oxygen therapy.
9:20
We can see patient's hearts starting to fail,
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because they're having a pump against tight blood vessels
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within the lungs,
9:29
due to the damage of the sickle cells
9:31
as they move through some of those blood vessels.
9:33
We can see problems in the brain
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where adults who've had those early signs of damage
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start when there were children,
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then progressed to having aneurysms
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and risk of bleeding in the brain
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associated with problems there.
9:48
Of course, chronic kidney disease that can lead to dialysis
9:52
and other major complications as well.
9:56
And, so really,
9:57
even though it's not well understood
9:59
out in the medical community right now,
10:01
and even in the communities where patients
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are disproportionately seen,
10:06
this is a chronic illness that is progressive
10:09
in the same way that diabetes, and hypertension,
10:12
and other diseases are progressive.
10:14
The issue is that we haven't, in the past,
10:16
had great treatments to try to prevent that progression,
10:19
but now the fun part is that we're starting to see
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more and more treatments coming on into the landscape
10:24
that can potentially have an impact
10:26
on this disease progression over time.
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- [Prakash Chandran] Yeah, so Dr. Roberts,
10:30
let's dive into some of those new treatment options
10:32
that are coming onto the landscape
10:34
and just talk broadly
10:36
about how sickle cell disease is treated
10:38
and also if it can be cured.
10:40
- [Dr. Roberts] Yes, so yes,
10:41
like Dr. Anderson said,
10:42
thankfully we have some more medications
10:44
and treatments that are coming out now
10:46
for sickle cell disease.
10:47
We currently have four FDA-approved medications
10:51
and they are aimed at...
10:53
They have different roles with the disease.
10:55
The one that's been around the longest
10:57
is called Hydroxyurea.
10:59
And, that medicine is what...
11:01
Something you take by mouth and every day.
11:03
And, we start that in our infants
11:05
and toddlers with sickle cell disease
11:07
in an effort to try
11:08
to prevent some of these problems that...
11:10
Or delay the onset of some of these problems.
11:13
And, that medication helps your body
11:16
to make some more baby blood that you're born with.
11:18
And, we know that the more baby bloods you make
11:19
that keep that red cell rounder and healthier
11:22
and can't go to the sickle shape.
11:24
It also has some other beneficial effects
11:26
on reducing inflammation
11:27
and other benefits that will help the blood vessel itself
11:30
stay healthier.
11:32
And, then,
11:33
fortunately,
11:34
we had in over the past three years or so,
11:35
three new medicines that have been introduced
11:37
and FDA approved for people with sickle cell disease.
11:40
One of those is called Adakveo
11:42
which is an...
11:44
Actually, an IV infusion that people get once a month
11:46
and that is used to help reduce pain, and acute chest,
11:50
and other acute sickling problems.
11:52
And, so those patients are coming to...
11:55
Into the...
11:55
Into our clinic once a month for this medication.
11:56
Another one is called Oxbryta.
11:59
and that medicine's taken by mouth.
12:02
and it was created specifically for sickle cell disease.
12:05
Dr. Anderson's done lots of work
12:06
with that particular medication.
12:08
Very exciting that these drug companies
12:10
have gotten on board
12:11
and are now making medications
12:13
specifically for people with sickle cell disease.
12:15
But, that medicine is taken by mouth every day,
12:18
and that's currently approved
12:19
for people that are four years and older,
12:22
and it helps you not be as anemic
12:23
with your sickle cell disease.
12:25
And, then that has some very beneficial downstream effects.
12:27
And, then the fourth medicines that the FDA approved
12:30
is called ENDARI,
12:32
which is approved
12:33
for young children with sickle cell disease and older
12:35
taken twice a day at home,
12:37
again by mouth.
12:38
And, that's helped to reduce pain.
12:41
Now, as far as a current cure for sickle cell disease,
12:43
we do have a cure,
12:44
but it's just not widely available,
12:46
and that's with a bone marrow or a stem cell transplant.
12:50
and the transplant process is...
12:52
It can be a bit rigorous to go through.
12:54
So, we've recommended that for our patients
12:56
who have had more severe sickle cell problems
12:59
and when their bodies are relatively healthy
13:00
that they've not had some of those issues
13:03
that Dr. Anderson was referring to,
13:04
with the effects on the the kidney and things,
13:07
because you wouldn't be able
13:08
to handle the transplant as well
13:10
if your organs were too damaged.
13:13
But, the problem is it's best if you get a transplant
13:17
from a brother or sister who's a full match
13:19
and that's not widely available.
13:21
Only about 15, 20% of our patients have a sibling
13:24
who would be a good match for the transplant.
13:26
But, what's exciting though,
13:27
that you don't have to depend on other people for,
13:30
are...
13:31
Is gene therapy.
13:32
And, the trials, they started off in Europe
13:33
before the United States,
13:34
but we now have the trials ongoing in the United States
13:37
and have the first report of success for gene therapy
13:40
was published last year.
13:44
And, so for the gene therapy,
13:46
you rely on it, again,
13:48
on your own body to make it work.
13:50
And, so I think we're waiting for some longer term data
13:53
to make sure it's going to be safe and efficacious.
13:55
But, I've been telling my patients,
13:56
let's work on all these...
13:58
With these other medicines that we do have available now,
13:59
try to keep you as healthy as possible,
14:01
because maybe gene therapy's going to become
14:04
a more widely available treatment in the future.
14:07
We...
14:07
And, we need...
14:08
Let's work on keeping you healthy now
14:10
so that you'd be able to get that.
14:11
And, in the gene therapy trials,
14:14
it's kind of making people basically
14:15
like they have sickle cell trait
14:16
and don't have the clinical problems
14:17
that we've been talking about.
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- [Prakash Chandran] Yeah, we're starting to get into this,
14:20
but, Dr. Anderson,
14:21
I'd love for you to talk broadly
14:23
about how people can manage living with sickle cell disease,
14:27
and prevent a sickle cell crisis,
14:30
and also maybe address if there are triggers
14:32
that can bring on a crisis.
14:33
so they can be aware of that as well.
14:36
- [Dr. Anderson] That's a great question.
14:37
So, what we typically tell our patients and families
14:39
is that any stress to the body
14:42
could trigger the change in the shape of the red blood cell
14:45
that happens in individuals
14:47
who are affected by sickle cell disease.
14:49
And, not a lot a lot of folks in the community
14:51
really understand that fact.
14:53
But, some of the hallmark types of stress
14:56
that we know can trigger sickling
14:59
that change in the shape of the red blood cells
15:01
that can block flow of blood and oxygen
15:03
could include things like temperature change.
15:05
And, so we encourage our patients
15:08
to make sure they keep an extra layer with them
15:10
if they go into the movie theater or restaurant
15:13
where they may go from hot temperature during the summer
15:16
into an environment that's very cold,
15:19
just that change in temperature by itself
15:21
could stress the red blood cells enough
15:23
to cause the change in shape
15:25
and then ultimately cause some complications like pain.
15:29
That can happen even in the midst of the summer in a pool,
15:32
if a child or an adult swims in a pool,
15:35
drops their core body temperature down
15:37
to the ambient temperature of the pool,
15:39
even though that may be 85 degrees,
15:42
that's below their body temperature
15:44
and that can trigger sickling.
15:45
And, we know lack of oxygen.
15:47
So, in our affected patients,
15:50
many times they will try to keep up with peers
15:52
in playing sports or doing other things
15:54
and they may get overly winded,
15:56
drop their oxygen level,
15:57
and that can trigger sickling.
15:59
Infection can do that,
16:00
certainly COVID 19,
16:02
other viruses,
16:03
other bacteria in our patients can trigger sickling.
16:06
Other causes of drop in oxygen,
16:08
like going from sea level to altitude,
16:12
we're talking about altitudes above eight to 9,000 feet,
16:17
where patients can have a lower oxygen level,
16:19
that can trigger sickling.
16:22
One of the things that we haven't understood as well,
16:25
but we're now starting to have a better feel for
16:26
is that anxiety and depression
16:28
can also trigger complications with sickle cell disease.
16:32
And, we think that's at the basic level
16:34
of just triggering that shape change in the blood cell.
16:36
We know at...
16:37
With COVID 19 we're seeing much higher rates
16:40
of anxiety and depression
16:41
and, of course,
16:43
that is having some effect
16:44
on the amount of complications our patients are seeing
16:47
with sickle cell disease as well.
16:49
And, so we're trying somewhat normalize
16:49
depression, and anxiety,
16:51
and chronic illness like sickle cell,
16:53
so that we can encourage patients
16:55
to open up about their feelings
16:57
and that we can plug them into treatments.
17:00
In terms of how do you prevent some of those stressors,
17:03
as I've talked about that list of things,
17:05
some of those,
17:06
our patients can't necessarily prevent.
17:08
They can try,
17:09
but they can't 100% stay away from some of those triggers.
17:12
But, we do talk about a couple things.
17:14
One is the idea of wellness in general
17:16
is very important for those with sickle cell disease
17:19
in a similar way that it would be
17:21
to others with chronic illness.
17:22
And, so we talk about proper diet,
17:24
looking towards what we would expect
17:26
of a good diet to be for others,
17:28
it would be lean meats, fruits, and vegetables.
17:32
That is, of course, important.
17:34
Lots of antioxidants in the fruits and vegetables
17:37
that we can get in our bodies,
17:38
and that's going to be in important.
17:39
Sickle cell disease
17:41
is an inflammatory condition at baseline,
17:43
because the red blood cells are breaking down,
17:45
releasing all these inflammatory products into the body.
17:49
We talk to our patients about trying to use mild
17:52
and moderate activity levels
17:54
and not to push themselves past the point
17:57
of being able to carry on a conversation
17:58
with those around them.
18:00
So, when I'm discussing exercise with my patients,
18:03
I usually say start off slow and build up,
18:06
build out foundation.
18:07
Make sure that when you're doing any activity,
18:09
you can carry on a conversation very easily
18:12
with someone with you,
18:14
without getting overly winded.
18:15
We talk to our patients all the time
18:18
about making sure they're staying well hydrated.
18:20
So, dehydration in the body can lead to sickling
18:22
and so we want to make sure that our patients
18:24
are drinking extra amount of water.
18:27
They're needing to drink in our young adult
18:30
and adult populations
18:31
as much as three 32 ounce water bottles in a day.
18:35
And, then,
18:36
above that,
18:37
monitoring their urine
18:38
to make sure their urine's staying clear
18:40
and using the urine color as a good marker
18:42
of what their hydration status is.
18:45
So, hydration is...
18:46
Can have a significant impact on the disease process.
18:49
And, then I think just as I mentioned already,
18:51
management of anxiety and depression I think
18:53
is a huge part of this as well
18:56
that can help patients to be...
18:59
To have less severe disease complications
19:02
when their mental health is under better control.
19:06
- [Prakash Chandran] So, Dr. Roberts,
19:07
we've just learned about some tools and tips
19:09
to potentially prevent a crisis,
19:12
but what should one do
19:14
if they're having a sickle cell crisis
19:16
and at what point should they go to the ER?
19:19
- [Dr. Roberts] Sure.
19:20
So, we want to make sure
19:23
that they have been educated about the pain crises
19:26
and make sure that they have tools at home
19:27
to try to treat that,
19:28
because, like we've mentioned earlier,
19:30
most painful crises are actually treated at home.
19:33
And, so when a person starts having pain,
19:35
we would recommend they start maybe
19:37
with just some plain Tylenol and some ibuprofen.
19:42
And, if that pain is more severe,
19:44
then they need to add a narcotic in to their pain regimen.
19:48
And, unfortunately, with the opioid crisis
19:50
that has happened in the United States,
19:52
that has had a negative impact
19:54
on some of our patients being able
19:55
to get medication or being stigmatized.
19:58
But, we have...
19:59
We work hard on a daily basis
20:01
to try to minimize that stigma for them.
20:04
And, as Dr. Anderson said too,
20:06
as in addition to the medication treatment,
20:08
hydration is super important for these patients
20:11
to try to prevent pain,
20:12
to stay hydrated all the time.
20:14
But, then when they're having a painful episode at home,
20:17
to try to drink plenty of water.
20:18
And, we like to try to stay away
20:19
from a lot of the salty drinks such as Gatorade,
20:22
a little bit Gatorade's okay,
20:24
but stay away from too much Gatorade,
20:25
because could that salt actually cause further dehydration
20:28
of that red cell.
20:30
And, then when the pain though is unbearable
20:32
or if they have other things with their pain crisis,
20:35
like if they have fever along with it
20:36
or if they have difficulty breathing,
20:38
then it's time to go to the emergency room.
20:41
And, in the emergency room,
20:43
we want them to be treated promptly,
20:45
because there could be a...
20:46
They could have a serious underlying infection going on
20:48
that could actually be life-threatening.
20:51
So, we want to make sure that they get seen
20:52
and get treated quickly,
20:54
one, for their comfort, for their pain,
20:55
but also, two,
20:56
to make sure there's nothing,
20:57
not a life-threatening problem
20:57
that's going on along with their pain.
21:00
And, in the emergency room,
21:01
they'll usually start an IV
21:03
and give those same types of medication,
21:05
so the ibuprofen,
21:06
the anti-inflammatory,
21:08
but give an IV form,
21:09
and then give an IV form of an opioid narcotic,
21:13
such as morphine or Dilaudid.
21:15
And, then they tend to get some fluids
21:16
in the emergency room as well if they're dehydrated.
21:19
- [Prakash Chandran] So, Dr. Anderson,
21:20
if someone is listening to this and they have a loved one
21:23
or a friend with sickle cell disease,
21:25
what is the best thing that they can do to support them?
21:28
- [Dr. Anderson] Yes,
21:28
so I think that when we look
21:29
at any patients with chronic illness
21:31
like sickle cell disease,
21:33
they needed support from their caregivers and loved ones.
21:37
And, so if you know someone
21:38
who's affected by sickle cell disease,
21:40
I think the biggest thing that you can do
21:41
is just be in their court.
21:43
Be, ready to support them
21:45
when there are those times
21:46
when they're dealing with a painful episode at home,
21:49
they may need someone who can run errands for them,
21:53
who can do some laundry for them.
21:56
They may also need someone who's going to be in their court
21:59
if they've experienced stigma,
22:01
if they've been in situations in the community
22:03
where people don't know about sickle cell disease.
22:05
They can have a loved one, a friend,
22:07
another caregiver who can start to put out better education
22:11
about sickle cell disease within the community.
22:15
Many of our patients have difficulty with transportation
22:18
or they may be dealing with pain,
22:20
they may be taking narcotic pain medications
22:22
that could impair their ability to drive.
22:24
And, so if you know someone with sickle cell disease,
22:27
certainly you could offer to drive them to appointments,
22:30
you could offer to drive them for other needs
22:34
that they may have in the community where they live
22:37
and work.
22:37
And, so that can certainly be helpful as well.
22:41
And, I think that one of the things
22:43
that caregivers, friends,
22:44
can do as well
22:46
is just become as educated about the disease process
22:49
as they can
22:50
and to help spread that education around to the community.
22:53
What we know about sickle cell disease
22:55
is that it was the first molecular based,
22:58
genetic based disorder
23:01
that was discovered affecting the red blood cell
23:02
in about 1910.
23:04
And, yet there's been very little understanding
23:06
in the community
23:07
about the disease process.
23:10
This is not only in our medical communities,
23:10
but out in the communities where patients live and work.
23:14
And, so I think sort of dispelling rumors
23:17
about sickle cell disease,
23:18
helping to get rid of the stigma
23:21
that exist in various locations where patients are seen
23:24
who are dealing with sickle cell disease.
23:26
These are all things that our champions within the community
23:29
can help us to sort of improve education about the disease
23:34
and make sure that people are aware
23:35
of what sickle cell disease is,
23:37
and what it can do,
23:39
and its debilitating nature to those who deal with it
23:41
on a day-to-day basis.
23:43
- [Dr. Roberts] And, then I would also add
23:45
to advocate for blood donation.
23:47
Many of our patients with sickle cell,
23:49
they require blood either on an ongoing basis
23:51
or periodically for stroke prevention,
23:54
if they have a...
23:55
Something called acute chest syndrome,
23:57
they may require a transfusion.
23:59
If they get more anemic from infection,
24:01
they may require a transfusion.
24:03
And, these,
24:04
the blood that they need is what...
24:05
It has to be especially matched.
24:07
not just for your main blood type,
24:09
such as your A, B, or O blood type,
24:11
but their other identifiers that we want to match up for.
24:13
And, most of our patients, not all,
24:15
but many of them are African American
24:17
and would have the best chance
24:19
of having a better matched blood
24:21
from an African...
24:23
Some people in the African American community.
24:25
And, so increasing blood donations
24:27
in the African American community
24:28
would be very beneficial
24:30
and helpful for our patients.
24:31
And, we have some families that actually do...
24:33
One of our patients, for his birthday every year,
24:36
they host a blood drive to give back,
24:38
and of course it's...
24:40
He's required blood whenever he was younger
24:41
and they're trying to raise awareness and raise donations.
24:45
- [Prakash Chandran] Yeah,
24:46
that's wonderful pieces of advice.
24:47
Thank you so much for that.
24:48
Just as we close here today,
24:49
Dr. Anderson,
24:50
I'd love for you to just talk broadly
24:51
about the support that Prisma Health offers
24:54
for sickle cell disease patients.
24:56
- [Dr. Anderson] Yes.
24:56
So, we have treatment centers for sickle cell disease
25:00
both here in the upstate of South Carolina
25:02
and in the Midlands of South Carolina.
25:04
Here in the upstate, we have an all ages program,
25:07
so that means that when we say the word lifespan,
25:09
that means we take care of babies
25:11
all the way up through adulthood.
25:14
My oldest patients are in their upper 70s,
25:17
and so we see all ages under the same...
25:20
In the same clinic, under the same roof,
25:21
with the same dedicated team.
25:23
So, that's a little different
25:25
in terms of a model across the southeast,
25:27
but that is what we have here
25:30
to be able to take care of the needs
25:32
of individuals affected by sickle cell disease.
25:34
In the Midlands, we have both the pediatric program,
25:37
that Dr. Roberts runs,
25:39
and then there's an adult sickle cell disease medical home,
25:42
as well as infusion center
25:43
for patients here in the Midlands.
25:45
Both of our comprehensive programs
25:48
have capabilities of transfusion, pain medicine infusion,
25:52
antibiotic infusion,
25:54
apheresis,
25:55
which is...
25:56
Just means taking away blood
25:58
and giving back blood at the same time
26:00
in order to decrease the amount of sickle cells
26:03
that are circulating in the patient at any given time.
26:06
So, all those treatment capabilities
26:08
are available for patients
26:10
across both areas of Prisma Health
26:14
that we have for our patients right now.
26:16
We also have capability to screen for sickle cell trait
26:19
to provide counseling for sickle cell trait
26:21
at our centers here
26:23
in the upstate and in the Midlands as well.
26:25
- [Dr. Roberts] And, I'd like to add that too
26:27
that Prisma help support programs
26:29
outside of the four walls of the hospital.
26:31
We've not been able to have camp recently due to COVID,
26:34
hopefully next summer,
26:35
but we have...
26:36
We started a camp
26:37
specifically for children with sickle cell disease
26:39
as well as other blood disorders.
26:41
And, it's been been wildly successful.
26:44
And, one...
26:45
One, these...
26:46
It's the first time that a lot of parents
26:47
have allowed their children to spend the night elsewhere
26:50
and they felt safe with all the medical staff being there
26:53
that they know.
26:55
But, at camp, we focus on how to...
26:56
How we, I think, live responsibly, play responsibly,
26:59
many life lessons are learned at camp.
27:02
Prisma also helps to support,
27:03
we have a teen group
27:04
as well as some family support groups as well.
27:07
And, so we try to...
27:09
We worked within our clinics,
27:11
but also try to take care of the whole family.
27:14
So, a whole team approach and be inclusive of the family,
27:18
including outside of the four walls of the hospital.
27:21
- [Prakash Chandran] Well, Dr. Anderson and Dr. Roberts,
27:23
thank you so much for your time today.
27:25
This was a truly informative conversation.
27:27
- [Dr. Anderson] Thanks for having us.
27:28
- [Dr. Roberts] Thank you.
27:29
- [Prakash Chandran] That was Dr. Alan Anderson,
27:30
director of
27:32
the Comprehensive Lifespan Sickle Cell Disease Program
27:33
at Prisma Health,
27:34
and Dr. Carla Roberts,
27:36
director of the Pediatric Sickle Cell Disease Program,
27:39
also at Prisma Health.
27:41
For more information,
27:41
you can visit our website at prismahealth.org.
27:45
And, to listen to other podcasts just like this one,
27:47
you can head over to prismahealth.org/flourish.
27:51
This has been "Flourish",
27:53
a podcast brought to you by Prisma Health.
27:55
My name's Prakash Chandran.
27:56
Thanks again and be well.
27:57
(uplifting music)
