|
Episode #1724
Sickle Cell Disease: Moving from
Education to Cure
Holloway: Jay Holloway,
host
Ware: Russell Ware, M.D., Professor of Pediatrics, Duke
Univ.
Wright: Marcia Wright, Executive Director, Sickle Cell
Disease Association
Barber Dominique Barber, Sickle Cell Disease Patient
Linden: Tom Linden, M.D., UNC-CH School of Journalism and
Mass Comm.
Redding: Rupa Redding-Lallinger, M.D., UNC-CH Pediatrician
Holloway: What does
singer T-Boz from the hip-hop music group TLC have in common
with thousands of young people? Sickle cell anemia, a deadly
disease that continues to take the lives of African American
youth and adults. Stay tuned next for Black Issues Forum
as we explore how this century-old disease continues to affect
the African American community.
[THEME MUSIC]
Voiceover: This program
was made possible by contributions to UNC-TV from viewers
like you. Thank you.
[THEME MUSIC]
Holloway: Hello and
welcome to Black Issues Forum, I'm Jay Holloway your
host. Many people may not have heard much about sickle cell
anemia because of the lack of attention the disease deserves,
or receives rather, from the media. Sickle cell anemia is
an inherited red blood cell disorder that affects the lives
of African American, Mediterranean, Middle Eastern and Indian
descendants. In North Carolina there are over 3000 African
Americans who have sickle cell disease, while over 12,000
carry the sickle cell trait. Despite the state's relentless
efforts to educate North Carolinians of the deadly blood disorder,
the number of babies born with the painful disease continues
to increase each year. Joining me on the program tonight to
discuss sickle cell disease is Dr. Russell Ware, professor
of pediatrics at Duke University. Also Dominique Barber; he's
a patient who has inherited the sickle cell disease. And Marcia
Wright, executive director of the Sickle Cell Disease Association
of America, Eastern North Carolina chapter, a community-based
organization located in Jacksonville, North Carolina. Thank
you all for joining us on tonight's program. Also before we
discuss the sickle cell disease, I would like to show you
how this painful and deadly blood disorder can affect life.
Voiceover: Meet 13-year
old twins, Vanessa and Valerie Sumner, their 15-year-old brother,
Victor, and their mother, Gloria Amarti. They all have sickle
cell disease.
F: Sickle cell is very
painful and it like slows you down two years late and you
can't do like regular stuff that other kids do.
Voiceover: The disease
is genetically determined. That means Vanessa, Valerie and
Victor inherited sickle cell disease from their parents. Gloria
is from West Africa where sickle cell evolved as protection
against malaria. In the U.S., about 1 in 800 African Americans
has sickle cell disease.
Linden: You can think
of a normal red blood cell as being a lot like a Cheerio.
The problem in sickle cell anemia is that the cell folds up
like a sickle, and that can lead to blockages in blood vessels,
even leading to a stroke.
Redding: Once a child
with sickle cell disease has had a stroke, then they need
monthly blood transfusions to prevent the recurrence of stroke,
another stroke which could leave them severely damaged or
could even kill them.
Voiceover: If, and
this is a big if, Victor's body could be told how to make
normal and not sickled blood cells, transfusions would be
unnecessary and the complications of sickle cell disease might
decrease or disappear. The key to this theoretical treatment
is finding a way to help his body make the correct form of
the protein that carries oxygen in red blood cells. That protein
is normal hemoglobin.
Redding: The genetic
research which may affect sickle cell disease patients in
the future which we are most excited about is the possibility
that a normal gene, a gene that codes for normal hemoglobin
could be inserted into the cells so that a person who was
born with sickle cell disease would be given the ability to
make normal hemoglobin.
Holloway: And we're
back with our guest now. Dr. Ware, let me start with you.
How many forms of this sickle cell disease are there and are
there more form-I should say, more forms that are more common
than others.
Ware: There are actually
six or eight different kinds of sickle cell disease, and yet
there are three types that are the most common. The type that
we call sickle cell anemia is when the child has inherited
the sickle gene from both parents, so we call that hemoglobin
SS.
Holloway: And these
graphics we have are what you're talking about?
Ware: And the second
most common is hemoglobin S with C and then sickle with thalassemia.
Holloway: Okay, and
these are what you see throughout-most often, I should say.
Ware: That's right.
Holloway: Marcia, what
about this disease, is it true that it's no longer just confined
to people of African descent and if so, what other groups
are affected?
Wright: That is very
true. That's one of the myths that we try to get rid of when
we're trying to do education for the public, to make people
aware that many populations can be affected. In our particular
community around Jacksonville, I have three major military
installations there, so we have a lot of ethnic diversity.
So we've seen sickle cell disease not only in African Americans
but also in people of Hispanic or Latino descent, Native Americans,
persons maybe from the Middle East, Mediterranean countries,
Pacific basin. So there are lots of different populations
that can be affected.
Holloway: Now, Dominique,
you actually have the disease, and how often in a typical
year do you get sick from this? Tell us what it feels like.
Barber: Well, I used
to get sick more often. And back like years ago I used to
get sick probably around like three or four times a year,
and but now since I'm getting older, my body is I guess maturing
I guess to where I don't get sick that often. I've probably
been in the hospital about twice this year and maybe about
twice last year. I'm not sure though.
Holloway: And you are
16 now right?
Barber: I'm going to
be 16, April 15th.
Holloway: Oh okay,
alright, just right around the corner. And so how does it
feel when you actually are sick from this disease?
Barber: It is very
painful and having to go into the hospital and get the medical
procedures and the needles and the medicine injected into
you, I mean, you have to go through the pain throughout certain
parts of your body. You never know where the pain is going
to strike. And once the pain does afflict you, you don't know,
maybe you might be able to stay home and just take pain medicine
that the doctor has prescribed or you might have to go into
the hospital and actually have to go with the medical procedures
and stuff.
Holloway: Dr. Ware,
we heard some of the other students in the video talk about
some of the same things Dominique said; is that pretty consistent
and does it vary with adults?
Ware: It varies quite
a bit, and one of the mysteries of sickle cell disease is
why some children and adults are affected more severely than
others. But the state of North Carolina identifies all babies
with sickle cell disease at birth so that we can begin to
educate the families and teach the children what to expect,
although we can't predict for any individual what will happen.
Holloway: And this
house bill that started testing folks makes a big difference
I guess, and there has been some recent legislation, I should
say, that affects sickle cell?
Wright: Well, I think
we're very fortunate in North Carolina to have had legislation
passed back in the early '70s and House Bill 32 was actually
the piece of legislation that created what's called the North
Carolina Governor's Council on Sickle Cell Syndrome. I believe
there are 12 members of that council; Dr. Ware and I are currently
members of the council. And people come from all walks of
life, education, and commerce and business and industry and
so on to sit on that council. And the council basically advises
the governor's office as to what should be done in the area
of sickle cell disease as far as funding and support and that
kind of thing from the state of North Carolina. That's been
in place since '72.
Holloway: Oh great.
Dr. Ware, what types of medicines are prescribed for this
kind of disease?
Ware: Well, after babies
are identified with sickle cell disease the general tendency
is to have that family be evaluated by one of the medical
centers, and I will say that from the west to the eastern
parts of North Carolina are very well represented with medical
institutions and providers that know a lot about sickle sell
disease. So persons in North Carolina are actually quite fortunate
to have good medical resources available to them. We first
talk to the families about the need for protection against
infections. Many serious infections can affect children with
sickle cell disease, even babies. And so we begin penicillin
treatment very early in life to prevent against serious, even
fatal bacterial illness. Often babies and older children are
prescribed folic acid as a vitamin to help with their red
blood cell production, and then just good health as with good
diet and good rest and that kind of thing.
Holloway: So babies
and adults pretty much are prescribed very similar medications?
Ware: Well, the risk
for infection is much higher in early childhood, so most older
children and adults do not take the penicillin prevention
that the young ones do.
Holloway: Dominique,
you are a spokesperson for this disease; who are some of the
people you talk to when you are talking about this disease;
what kind of things do you share with them?
Barber: When I'm talking
about the disease I guess I talk to a lot of people that I
know, because I go to a camp every year and that whole camp
is of sickle cell students. I talk to them on the phone and
I talk to my parents because to learn more about it I guess.
Holloway: And what
about people that don't have the disease, do you talk to them
about it, and what kind of questions do they ask you?
Barber: Yes, in school
I just tell people right up front that I have sickle cell
disease and they ask me about it and I let them know all the
things that I know about sickle cell disease. And some of
them may know family members or friends or other friends that
they have that have the same disease as well.
Wright: I think it's
very important for kids like Dominique to be as open as he
is. He probably is more of the exception than the rule as
far as being willing to talk about it openly and discuss it,
because we do feel that people who are not affected really
need to have this information. You know, kids go to school
with each other, people hire, employers and employees and
so on. And it is important for people who are not affected
by any illness to have more information about what's going
on with friends and neighbors and colleagues so that you can
deal with them better and everybody benefits from a situation
like that when you have more knowledge on both sides, both
with the person who has the disease and the person who's not
affected.
Holloway: Now speaking
of disease, I'm sure that many persons in our audience are
concerned about how this-well, we talked about it's acquired
through inheritance or it's a genetic disease, but is there
any way to prevent this and if so or if not, you all-I know
you talked about making an educated choice. Explain that.
Ware: Well, the sickle
gene, the abnormal gene, is present in about 8-10% of African
Americans, and so there are many persons who carry the sickle
cell trait without realizing it. That's an easy diagnosis
to make; a simple blood test can determine whether somebody
carries the sickle cell gene or not. Then if one person has
a child with another person who also carries the sickle cell
gene, that's the typical form of sickle cell disease that
we call sickle cell anemia. I think it's important to have
families have education about it to know what their risk is
of having a child with sickle cell anemia, because it's about
a 1 in 4 chance in most cases.
Holloway: Marcia, your
organization changes emphasis from years ago; do you want
to talk about that?
Wright: Yes, I was
mentioning in the conversation we had before that early on-I
have been with my program for 22 years, since 1980, and in
the beginning of the program there was a lot of emphasis on
prevention, trying to figure out a way to reduce or even eliminate
the incidence of sickle cell disease. And that was one of
several goals. We also talked about reducing the incidence
of morbidity and difficulties and so on, trying to do research,
that kind of thing. But we decided fairly early on-I remember
sitting on a committee back in 1983 when we really stopped
and looked at that whole issue of did we really want to be
about the business of trying to prevent people from being
born or did we really want to put our emphasis and our time
into education, and we made a decision then that we would
be better served by giving our families all the information
we could, explaining as Dr. Ware mentioned, their risks for
having a child with this disease. Some of the symptoms that
might occur, some of the consequences, and then leaving families
to make the appropriate choice for their life situation. And
I feel that's served us well, to take that approach, even
though the number of births has remained constant I think
that our medical care certainly in this state is superior
and I think a lot of that is due to the fact that we try to
educate families and give them the choice so they can make
the best decision for their own family situation.
Holloway: Dominique,
explain for the audience the difference between sickle cell
disease and the sickle cell trait.
Barber: Well, I believe
that the sickle cell-when you have the full sickle cell disease,
you get sick more or you're liable to get sick more and I
think you have to take more medicine than people with sickle
cell trait. The people with the trait have-I don't know.
Holloway: Help us out-
Ware: Well actually
Dominique's doing a good job. People with sickle cell trait
really don't get sick; it's a silent carrier state. And so
it's only when they have a child with another person with
an abnormal hemoglobin-like sickle that a child with disease
is born.
Wright: And again,
that's why education is so important because as Dr. Ware said
there's so many people who have trait, do not know it because
it does not affect their health in any way. So we do want
people to be aware that if you have not been tested or you
don't know or you've forgotten your results, contact someone
who can help you to have that knowledge so you can again make
the appropriate decisions for your family.
Holloway: Speaking
of your family, I know your mother has been very supportive
with your disease for quite a long time now. She had the trait
I believe, right?
Barber: Yes she does.
Holloway: Right. And
I imagine that it's fulfilling you to have a mother who's
so very supportive with you, with having this. Would you like
to comment on that?
Barber: Yes, she always
supports me and tries to lead me in the right way. She tries
to get me into everything that will make my life better and
so I won't have to feel like the outcast in any way, in doing
sports or anything in general; she tries to make me seem like
I'm better than all the rest.
Holloway: Let's talk
about that. When you have a crisis or pain as a result of
this disease, how often does it deter you from doing your
school work and how do you feel when that happens?
Barber: Whenever I
do get sick it does deter me from my-
Holloway: It does?
Barber: It does; and
I have to do a lot of make-up work and sometimes I try to
get the work and do it when I'm out, and when I'm out of school.
And I don't really like having to be out of school when I'm
sick because then I get left, like I don't feel like I'm with
the other classmates and I feel like they might know something
that I don't know and I have to make that up and it's just
hard for me to be able to balance school work and then makeup
work and homework and all that.
Holloway: Marcia, what
kinds of programs are set up for students like Dominique to
catch up and keep up?
Wright: Well, fortunately
in North Carolina we do have home-bound teaching programs
and because of certain, I guess, legislative or rule changes
or whatever, it's made it possible for students who have sickle
cell disease in most of our school systems to be able to get
homework, as he said, and home-bound teaching. Before I think
that most school systems require that a child be out of school
a certain number of days before the home-bound teaching program
begins, but our children can access that program early on,
because we don't want the children to fall behind. Sickle
cell disease does not affect intelligence, it's primarily
when you see children that are behind in school it's been
due to absenteeism, not to the fact that they can't keep up.
So we very much encourage families and students and also educators
to be aware of this disease to know that, send the homework
home, try your best to keep the child up and if you can do
that with the support of the family, most kids can stay on
track and graduate when they're supposed to and go on to a
higher education if that's their choice.
Holloway: Speaking
of higher education and an educator in the medical field,
how close are we, Dr. Ware, to finding a cure for this disease?
Ware: Well, I think
that we're making progress, but a cure is really not right
here. Dominique made a good point that not only does sickle
cell disease have painful events and medical complications,
but it also has a very unpredictable nature, and so you can
go along for a week or two or a month or two just fine and
then suddenly get sick. And it's to help prevent some of those
surprise or unpredictable complications that we are paying
so much attention to, so that even though we don't have cures,
we do have medicines now that can help prevent some of these
acute crises and these acute events that make the youngsters
and adults get so sick. One, for example, is called hydroxyurea.
This is a new medicine that has been around for maybe ten
years and only for younger people in the last couple of years.
What hydroxyurea does is to help that round red blood cell
stay round so that it never gets stretched out into that pointed
sickled shape. And by keeping the blood cells round, this
is a nice way to keep the blood flowing through the blood
vessels so they don't clog up. And we've now used hydroxyurea
in a number of children in North Carolina. In fact, several
medical centers around the state were involved in safety trials
for hydroxyurea in young patients and we've found that it
is every bit as effective as it is for adults and actually
may be a little bit more effective.
Holloway: What are
some of the side effects?
Ware: Well, it's a
medicine that has to be taken every day and so there is a
problem, if you will, just remembering to take medicine every
day. But beyond that it's actually quite easy to take in the
short term, that is, it doesn't make you sick on your stomach
usually, it's not a medicine that makes you feel different
one way or the other. Now the long-term side effects of hydroxyurea
really aren't known; no one has taken it for 30 or 40 years
but we've been very, very impressed with young people taking
hydroxyurea, how it helps them feel better, less anemic, helps
them grow better and helps them feel better about themselves.
Holloway: Dominique,
when you're sick from this, how supportive are your teachers
and classmates and friends for you?
Barber: They're supportive.
When I get back from being absent they'll make sure that I'm
okay, they'll ask me, was everything alright, and my teachers
will make sure that my make-up work is ready for me to take
home and people that I know but don't get to see that often,
once I do see them they'll make sure that I'm okay and everything
feels, you know, regular.
Holloway: In just the
last few minutes here, Marcia, let me ask you, financial support
is important not only for the research but for ongoing outreaching
things that you do. How supportive is the black community
in particular in financially supporting your efforts?
Wright: Well, in North
Carolina we have a North Carolina state sickle cell program
which is funded through the Department of Health and Human
Services, and that department has a contract with four community-based
programs: mine in Jacksonville, there's a community-based
program in Fayetteville, one in Greensboro and one in Charlotte.
There are other programs that operate in some of the other
counties that aren't part of that network but still provide
some services. We also have state sickle cell counselors who
work in some of the other counties. So every county in North
Carolina has someone who is providing services. As to financial
assistance, you're very right because this is, as any other
chronic disease can be, it can be a very expensive disease
to treat, particularly when you're talking about hospitalizations
and expensive medications, things like that. And we have a
purchase of medical care program that is funded by the General
Assembly and of course we're always battling, as other programs
are, for adequate funding for that program. And we've been
pretty successful so far, we'll be back to them again, this
session coming up to make sure our funding stays in place.
But we've had very good support from the community. Those
of us in the community-based centers do local fund-raising
activities and things of that nature to try to provide services.
Transportation is a big issue in North Carolina: it's a rural
state; even though we have excellent medical care in our medical
centers, trying to get there is a whole another issue. So
we're always working on issues again like transportation,
helping to pay medical bills, supporting students like Dominique-because
as he gets older he will I'm sure want to go to college one
day and so we have built in some programs on the community-based
level to try to support young people who want to go on to
school, because clearly if he can get a quality education
he can get a good job, you know, in this new century coming
up, support himself and again, pay taxes back into that system
that we're relying upon to take care of people who are not
as well as he is. So I think we've enjoyed very good support,
we certainly could use more, but we are very lucky I think
in this state to have the support we have.
Holloway: Dr. Ware,
what about strengthening the financial support and services
in North Carolina from your point of view?
Ware: Well, I think
each of the medical centers in the state has a commitment
to persons with sickle cell disease and we at the medical
centers also receive some support from the state sickle cell
program as Miss Wright described. There is a need to do further
research. I mentioned hydroxyurea; there's also the use of
transfusions of blood; there's bone marrow and stem cell transplants;
there's gene therapy that Dr. Redding-Lallinger mentioned
in the early video-
Holloway: Let me stop
you a minute, that stem cell was very controversial in the
last year or so, but that's a benefit for sickle cell?
Ware: Well, I think
there's a little bit of misconception about what the word
"stem cell" means in this context. It's clear that
in persons with sickle cell disease, the problem is in their
bone marrow, that is where they make blood, and it was proven
almost 20 years in an unfortunate child with sickle cell anemia
who also developed leukemia that if you had a bone marrow
transplant, that is if you replaced your bone marrow with
someone else's that it could cure sickle cell disease. So
there are persons who have a bone marrow donor, usually a
brother or a sister who matches them, who doesn't have sickle
cell disease that can be used for a cure for persons with
sickle cell disease. Unfortunately not many persons have that
type of donor who matches them. And for this reason, the search
is on for alternative sources of cells that could serve to
do a transplant, and that's what stem cells are. These are
not the same kind of fetal cells that are so controversial
in today's press.
Holloway: Dominique,
as we close, what would you say to parents and a kid who just
found out that they got the sickle cell disease?
Barber: I'd tell them
to not give up hope and just because they found out that they
have a disease, it doesn't mean that bad things are going
to happen to the child. And you should let the child be able
to do things that normal kids do but with precautions. And
you should try to make their life as normal as possible, because
if you keep them restricted from a lot of things, once they
grow older they might not, they might be afraid to do some
things or they might not feel comfortable with new atmospheres
or anything like that.
Holloway: Well best
wishes to you and thank you all so much.
Barber: Thank you very
much.
Holloway: Alrighty.
And thank you for watching. I'd like to thank our three guests
again for joining us on Black Issues Forum. If you'd
like more information on sickle cell disease, please go to
our web site, www.unctv.org, or contact us at 549-7167, that's
area code 919. You can fax us at the number or reach us by
email at the information on your screen. Thank you for joining
us again tonight and we invite you to join us back again every
Friday night at 9:30 right here on UNC-TV. You have a blessed
evening and good night.
|
