September is National Sickle Cell Month, and if you don’t know much
about the blood disorders known as sickle cell disease.
Latha Prasannan, MD, who specializes in pediatric hematology and oncology at
Covenant Children’s in Lubbock, Texas, tells you what you should know.
WHAT IS IT?
Sickle cell disease affects the body’s red blood cells. Normally,
the protein in these cells, called hemoglobin, gives them a round shape,
allowing them to pass easily through blood vessels to deliver oxygen throughout
the body. “However, with sickle cell disease, the hemoglobin is
abnormal and causes the red blood cell to take on a “C” shape
that looks like a sickle. Because of that shape—and because these
blood cells are rigid and sticky—they have a harder time doing the
job they need to do, and that can cause numerous problems,” Dr.
HOW DOES SOMEONE GET IT?
This is a genetic disease, Dr. Prasannan says. There are different types
of sickle cell disease, but generally a person inherits sickle cell genes
from both parents. If someone has one sickle cell gene and one normal
gene, he has sickle cell trait, and he can pass the gene for sickle cell
on to any children he may have.
WHO HAS IT?
An estimated 100,000 Americans have sickle cell disease, with millions
of cases worldwide. Among U.S. cases, about 1 of every 500 black or African-American
newborns has sickle cell disease, and 1 of every 12 has sickle cell trait.
People with Hispanic, Middle Eastern, Mediterranean or Asian Indian heritage
can also be at risk.
HOW IS IT DIAGNOSED?
Someone who has sickle cell disease is born with it. Testing can be done
prenatally (usually through amniocentesis or chorionic villus sampling)
or during newborn screening.
WHAT ARE THE EFFECTS OF SICKLE CELL DISEASE?
Symptoms usually start before a baby turns 1 year old. “The effect
sickle cell disease can have on someone’s health depends on the
individual, and the severity of symptoms or related health issues can
vary,” Dr. Prasannan says. “Many problems are caused by the
inability of the red blood cells to pass through the blood vessels. They
can stick to the vessel walls and cause blockages.” Those problems
include hand-foot syndrome, with swelling in the hands and feet; pain
episodes or crises; splenetic sequestration, which enlarges the spleen;
vision loss, if the blockages are in the blood vessels of the eye; deep-vein
thrombosis and stroke. “Patients with sickle cell disease are also
prone to infection and anemia, the latter because sickle cells die off
faster than normal blood cells, and symptoms include dizziness, fatigue
and increased heart rate,” Dr. Prasannan says.
WHAT IS THE TREATMENT?
The only cure now is stem-cell transplantation, which can be a difficult
process. Symptoms can have a range of treatments, which again, depends
on the individual patient. “For minor swelling, a patient may just
need to drink more fluids or take an over-the counter pain reliever,”
Dr. Prasannan says. “More severe cases could require a common sickle
cell medication called hydroxyurea, blood transfusions and prescription-strength
pain relief.” Dr. Prasannan adds that sickle cell patients should
undergo regular tests, such as blood, urine and vision exams, to monitor
their condition. Children will also be watched to make sure the disorder
isn’t impeding their growth and development. And patients should
also stay on top of vaccinations and flu shots to prevent infections.
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