I am doctors Susmita Sarangi,
I am one of the pediatric hematologist oncologists
at the division of pediatric, adolescent and young adult hematology
oncology at MedStar Georgetown University Hospital.
Our team takes care of a variety of patients with various cancer diagnosis,
which includes leukemias, sarcomas and lymphomas.
We also take care of a lot of patients with blood disorders
like sickle cell anemia, hemophilia and bleeding disorders.
We also look at patients who have acquired blood problems,
like, for example, problem with their white blood cells, which fight
infection or problem with your red blood cells, which can cause anemia
or problem with your platelets, which can cause a tendency to bleed.
Our team is the only state of the art center
which focuses primarily on young adults. So basically,
we're taking care of patients right from birth to their 20s and 30s.
Dealing with cancer and blood disorders can be an extremely challenging time.
Our patients and their families are thrust in an alien and scary world
of numerous lab tests and surgeries
procedures and difficult to pronounce medications even.
What I want to do as their doctor is basically educate them
about their clinical condition, so they can feel empowered to ask
the right questions and find out what the best treatment option is for them.
We focus on Cura personalis, so we focus on the whole patient
and we want to partner with you and see what works best with you.
Our dedicated team of doctors, nurses, social workers,
nurse navigators, are all there to support you through this process.
So you don't have to face this by yourself alone.
We are living in very exciting times.
Innovations in the field of gene therapy are making previously crippling conditions
like sickle cell disease and hemophilia potentially curable.
Children with leukemia have over a 90 percent cure rate,
and even those children who were considered high risk
in their leukemia diagnosis have things like immunotherapy
offered to them, which also is offering really high cure rates.
So imagine using
your body's own immune system to fight your cancer cells,
and that is what promises of therapies like CAR T-cells
is offering to our patients. The best part of my job
is the relationship I form when I work with my patients.
I love getting to know about their lives and their different accomplishments
that they can do, even despite their challenging cancer diagnosis.
It makes me feel so proud to work with these heroes.
Sickle cell disease is an inherited disorder of the red blood cell.
The normal body's red blood cell is in the shape of a donut
or a disc shape in sickle cell disease.
The shape changes to a sickle shaped or crescent shaped.
This then causes the red blood cells to become
more sticky and inflexible.
So, then they start clogging the small blood vessels of your body,
and this is what we call the process of sickling.
When this happens, oxygen cannot reach areas of your body,
and this results in tremendous pain, and can also lead
to organ dysfunction, a process that we call a crisis.
Also, sickle cells
survive in your body for a shorter amount of time
so they can only circulate for 10 to 20 days as opposed to normal
red blood cells, which can survive for 90 to 120 days.
This breakdown of the red blood cells results in anemia,
and these patients are always feeling fatigued and tired.
The way to know
that your child has sickle cell disease is by a test that is commonly performed
in most states in the United States, including Washington, D.C.,
called the newborn screen, which can pick it up right at birth.
Sickle cell disease is an inherited disorder.
So, in other words, you can't catch it.
You need to get two sickle cell genes,
one from mom and one from dad in order to get sickle cell disease.
So, in other words, mom or dad are considered to have sickle cell trait.
So they have only one gene for it.
People who have sickle cell traits usually have no symptoms.
They can lead a long and healthy life.
However, knowing if you have the sickle cell
trait is really important because it helps you plan in the future
and to realize your risks of having a sickle cell disease child.
The most common and severe form of sickle cell disease is sickle cell anemia;
when you get two Hemoglobin S genes from either parent.
The other forms are Hemoglobin SC,
so you're getting one S gene from one parent and another C gene from another parent.
Similarly, if you get one thalassemia gene from one parent
and another sickle gene from the other parent, you can get
what is called sickle thalassemia syndrome.
The symptoms of these conditions
can vary with some patients having mild symptoms,
whereas other patients having severe symptoms.
The symptoms of sickle cell disease
can vary from person to person and change over time.
Most children, when they're born, they do not have any symptoms from sickle
cell disease for the first six months of their lives. As they grow older,
however, the most common symptom that we see is a pain or a vaso occlusive crisis,
where basically pain can strike them at any time in any area of the body.
It could be in the chest, in the back, in the abdomen
or even in the extremities. More seriously, if sickling occurs in the lungs.
This can abruptly cause decreased oxygen
delivery to your body and is called an acute chest crisis.
This can be life threatening,
and we want to see these patients as soon as possible.
Sickling in the brain, blood vessels can also lead to stroke.
So, you can see how sickle cell disease can really affect a child's life
and lead to repeated hospitalizations and emergency room visits.
But we want to see them in clinic
so we can prevent some of these complications that can happen.
About 100,000 Americans have sickle cell disease.
It's more common in people with African ancestry.
So, one in 13 babies born to African-American families
can have the sickle cell trait and one in 365 babies
born to African Americans can have the sickle cell disease.
Sickle cell disease is also common among Hispanics, in southern Europeans,
and Mediterranean ancestry, and also in south India.
It is very important for children who have sickle cell disease
follow up with the pediatric hematologist who has expertise in their care.
At MedStar Georgetown University Hospital,
We have a comprehensive sickle cell program
where we see these patients right from birth to young adulthood.
And this involves a multidisciplinary team of people,
including doctors, nurses, social workers,
child life specialists, blood bankers, and even integrative medicine
sometimes who helps take care of these patients and their families.
Over time, sickle cell disease
can cause a variety of long-term complications.
Because the healthy red blood cells are replaced by sickle cells,
This can affect the child's ability to grow and develop
and even delay puberty in the teenagers.
Due to constant cycling happening in various organs in your body.
The child can have dysfunction in the immune system
and be predisposed to certain infections.
Tiny blood vessels in the eye can be blocked due
to sickle cell disease and can lead to vision problems.
And over time, as
patients get older,
sickle cell can damage the bones and lead to chronic pain.
As a parent, it is very important to talk to your pediatric hematologist
about potential treatment options that are out there for your child.
It's also important to understand what can trigger some of these sickle-cell
crisis and do things accordingly to prevent some of these complications.
Always have your child have access to a water bottle at all times
to prevent dehydration, avoid stressors like extreme exercise,
also avoid extreme fluxes in temperature,
and keep your clothing on hand at all times.
In school, ask for a 504 plan, which can help schools
provide the accommodations that your child with sickle cell disease might need.
Over the past five years,
there has been tremendous strides in the field of sickle-cell care.
With a medicine called hydroxyurea,
we can actually decrease the number of sickle cells that the body produces,
and this prevents certain complications that can occur
and organ damage that occurs in these patients.
We start an antibiotic called penicillin right after birth, which prevents
some of the infection complications that can happen in these children.
The overall life span and the quality of life of children
having sickle cell disease is improving tremendously as well.
There are new therapies like L-glutamine, voxelotor, Crizanlizumab,
which are out there and work in different mechanisms
to prevent some of these complications from sickle cell disease.
Currently, a potential for curing sickle
cell disease is a stem cell or a bone marrow transplantation.
In this procedure, healthy stem cells from a donor who does not have sickle cell
disease is basically replaced in a patient who has sickle cell disease.
The only limitation of this is it's hard
to find a suitable donor for each and every of our patients,
and it does have some side effects.
Another emerging option on the horizon
is clinical trials looking at gene replacement therapy.
This will actually correct the genetic defect that can cause sickle
cell disease and offer a curative option for patients with sickle cell disease.