Over time, because of sickling of the red blood cells,
patients can also develop chronic organ damage to almost any
organ. Bone, kidney, lung and spleen are the most common sites
of chronic damage. Currently, there is no cure for sickle cell
disease except for a bone marrow transplant.
About 100,000 people in the United States are diagnosed with
sickle cell disease. One in 13 African Americans carry the
sickle cell trait, and many do not know they have it.
Despite it being fairly common, many myths surround sickle
Myth 1: I am going to catch sickle cell disease from a
This is simply not true. Sickle cell disease is not a virus
or infection. You will not catch it from another person. People
with sickle cell disease have it because they inherited two
sickle cell genes, one from each parent.
Myth 2: The sickle cell trait can turn into sickle cell
Sickle cell disease and the sickle cell trait are two
separate things. If a baby only inherits a single sickle cell
gene, she or he has the sickle cell trait, but will not develop
sickle cell disease. A child must inherit two sickle cell genes
to develop the disease. Children of parents who have sickle cell
disease are obligate carriers of the gene, and have a 25% chance
of having a child with sickle cell disease if he or she has
children with someone who has the trait.
Myth 3: The patient with sickle cell disease is drug-seeking.
This is likely the most common myth. Sickle cell disease pain
is real, and just because patients have learned to distract
themselves with their phone or video games or TV, doesn’t make
their pain any less real. Individuals with Sickle cell disease
should choose providers who are educated about the nature of the
pain associated with the disease, so that they can get the
relief they need.
Myth 4: Sickle cell disease patients are just addicted to
their pain medication.
Again, sickle cell disease patients often develop chronic
recurrent pain related to their disease, and unfortunately,
there are no good studies showing the benefit of alternative
therapies for their pain other than narcotics. Their pain needs
to be treated adequately. Most patients with sickle cell disease
have a far shortened lifespan compared to the general
population, living on average to only their mid-forties.
However, most patients who can maintain themselves on some
degree of disease-modifying antisickling agent tend to do better
When babies are little and identified as having sickle cell
disease early, providers should take the following course of
1. Family education and counseling, making sure the family
understands the type of disease the patient has and any
potential complication risk factors .
2. Preventive therapy such as penicillin prophylaxis,
immunizations, and appropriate screening (for stroke and other
conditions) at the appropriate time.
3. Disease-modifying therapy including hydroxyurea, blood
transfusion, L-glutamine (5 and over), voxelotor (12 and over),
and crizanlizumab (16 and over), all of which provide some
degree of relief from acute complications and potentially slows
chronic organ damage that occurs in patients with sickle cell
While Sickle cell disease cannot be cured at this
time,symptoms can usually be managed with a range of
medications, including those that address ongoing chronic pain.
These treatments are incredibly important to help