“You were concerned today because [his] eyes were darker
than normal?” Dr. David Goo asks Schenequa Norfleet, “And
you said that he had a low-grade fever of 100.4?” A fever
for her son, two-year-old Quest Norfleet, could mean trouble. He
has sickle cell anemia.
The doctor explains, “they get a minor infection, something
that’s minor in you or me, their body doesn’t fight
it off well and they can get an overwhelming infection.” And
that, he says, could lead to pneumonia.
X-rays are taken of his chest but, “looking at his lungs,
they look normal,” says Goo, “I don’t see any
evidence of pneumonia.”
As for the discoloration in his eyes, “when the eyes get
dark, it means the blood is undergoing what we call hemolysis,
or breakdown,” the doctor explains to Schenequa. In other
words, in a sickle cell crisis, blood cells die when they don’t
get enough oxygen.
A blood test is performed but, “we were able to look at
his biliruben, and the biliruben is the substance that I said when
your blood breaks down, makes your blood turn yellow and your skin
turn yellow,” explains Goo, “and his was less than
.2 which is very low, meaning that it’s not related to the
sickle cell.”
“And that made me think he might have what we call muddy
sclera,” says the doctor, “Muddy sclera is just the
terminology [used] when the whites of the eyes turn really brown,
muddy.”
Among Asians and African-Americans especially, time in the sun
can darken the whites of the eyes. It’s as harmless as a
suntan.
Still, “we’re very happy that you came in today, because
the temperature was low-grade, and any child with sickle cell and
fever, you know, you’re supposed to come in and we’re
supposed to do the work-up,” says the doctor. |
By Larry Eldridge
CWK Network, Inc.
Sickle cell anemia is an inherited disease of the red blood cells
that can cause attacks of pain. It can also damage vital organs, cause
serious infections and lead to early death.
Why is it called “sickle?” Unlike normal blood cells,
which are round like doughnuts, sickle cells become hard, sticky and
shaped like sickles used to cut wheat. When these hard and pointed
red cells pass through small blood tubes, they clog the flow and break
apart. This can cause pain, damage and a low blood count or anemia.
Most cases of sickle cell disease occur among African-Americans and
Hispanics of Caribbean ancestry. About one in every 400 African-Americans
has sickle cell disease. It also affects people of Arabian, Greek,
Maltese, Italian, Sardinian, Turkish and Indian ancestry. There are
several forms of sickle cell disease. According to the March of Dimes,
the most common forms include the following:
- SS – The child inherits two sickle cell genes.
- SC – The child inherits one sickle cell gene and one gene
for another abnormal type of hemoglobin celled “C.”
- S
beta thalassemia – The child inherits one sickle cell gene
and one gene for a type of thalassemia, another inherited anemia.
|
By Larry Eldridge
CWK Network, Inc.
The symptoms of sickle cell disease
vary greatly from one person to the next. Some affected people have
few symptoms, while others are frequently hospitalized. One of the
most serious complications of sickle cell anemia is stroke (bleeding
or blockage of blood vessels in the brain). The March of Dimes says 10 percent
of children with sickle cell anemia have a stroke, which can lead to lasting
disabilities by age 20. The risk is highest between the ages of 4 and 6. Recent
research has found that regular blood transfusions greatly reduce the risk
of a first stroke in children with sickle cell disease. Unfortunately, regular
transfusions pose some major risks, including a potentially fatal buildup of
iron in the body, infections and other problems. Other treatments to prevent
sickle-cell anemia are being tested and studied, with various results.
The Georgia Comprehensive Sickle Cell Center (GCSCC) reports that
most states now perform the sickle cell test when babies are born.
The simple blood test will detect sickle cell disease or sickle sell
trait. Other types of traits that may be discovered include Hemoglobin
C trait, Hemoglobin E trait and Hemoglobin Barts, which indicates
an alpha thalassemia trait.
The GCSCC recommends the following general guidelines to help keep
a sickle cell patient healthy:
- Take the vitamin folic acid (folate) daily to help make new red
cells.
- Take daily penicillin until age 6 to prevent serious infection.
- Drink plenty of water daily (eight to 10 glasses for adults).
- Avoid too hot or too cold temperatures.
- Avoid overexertion and stress.
- Get plenty of rest.
- Get regular checkups from knowledgeable health-care providers.
Parents of sickle cell patients should watch for the following conditions
that need an urgent medical evaluation:
- Fever
- Chest pain
- Shortness of breath
- Increased tiredness
- Abdominal swelling
- Unusual headache
- Any sudden weakness or loss of feeling
- Pain that will not go away with home treatment
- Priapism (painful erection that will not go down)
- Sudden vision change
In addition to the conditions listed above, the GCSCC suggests
familiarizing yourself with the following complications from which
your child may suffer:
- Pain episodes
- Strokes
- Increased infections
- Leg ulcers
- Bone damage
- Yellow eyes or jaundice
- Early gallstones
- Lung blockage
- Kidney damage and loss of body water in urine
- Blood blockage in the spleen or liver (sequestration)
- Eye damage
- Low red blood cell counts (anemia)
- Delayed growth
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