Teegan Conroy

Sickle Cell disease

What is Sickle Cell disease?
Sickle cell disease (sometimes called sickle cell anemia) is a
hereditary disease that affects the shape of red blood cells. In a
healthy body red blood cells are round and elastic, which
allows them to pass through narrow capillaries without getting
stuck; sickle cells are deformed red blood cells whose
hemoglobin has formed strands instead of free-floating lumps
inside of the cell. Cell sickling damages the membrane of the
cell which causes them to lose their elasticity, and because of
their rigidity it is difficult for them to pass through the body. In
addition to inhibiting the red blood cells ability to carry oxygen,
sickle cells are also much more fragile than healthy blood cells
(while a healthy blood cell may live for 90-120 days, a sickle
cell will last only 10-20). This can lead to severe anemia, as not
only would blood cells not be delivering oxygen fast enough,
but the cells themselves would also be quickly disappearing.
Sickle cell disease is passed hereditarily from parent to child,
with several factors determining its severity (or if it will even be
passed on). Parents can unknowingly carry a benign gene that,
when combined with another carrier gene can potentially
cause sickle cell disease in their children. This gene defect is
found mostly in Africa and certain parts of Europe and India,
generally places where people malaria is common. People with
SCD are generally immune to malaria, so it is believed that this
genetic defect exists to protect people in these regions.
Research being done
Researchers have recently discovered that sickle cell disease causes a component of
haemoglobin called haem to form in the blood, which seems to be what prevents people with sickle
cell to contract malaria. Researchers injected mice with haem before injecting them with malaria,
and those that were injected with haem first did not contract the disease. The protective qualities of
haem seem to stem from the fact that it produces small amounts of carbon monoxide as it breaks
down, which (though highly toxic in high amounts) is already used therapeutically in different
treatments.
Support groups
Several support groups for sickle cell exist all across the world including The Sickle Cell
Foundation Support Group in Atlanta, the Sickle Cell Society in London, and the Sickle Cell
Awareness Group of Ontario.

http://www.sicklecellanemia.ca/
http://www.sicklecellsupportgroup.org
http://www.sicklecellsociety.org/

These groups provide information about sickle cell to those who have it and provide a safe (and
informed) place for them to talk about problems related to their illness. Support groups also raise
awareness and hold fundraisers for sickle cell research, in addition to being a good source of
information to those who do not have sickle cell.
Treatments available
People with sickle cell are at risk for severe infections because their spleen does not work properly.
Children with sickle cell can take two tablets of penicillin daily to reduce the risk of serious
infection, but penicillin isnt usually prescribed for sickle cell past the age of five (except for those
who have had their spleens surgically removed).

Another medicine called hydroxyurea can be used to help with many of the more fatal symptoms of
sickle cell (including severe anemia, artery/blood vessel blockages, and painful episodes), and can
be prescribed to children over nine months old. It is dangerous to use during pregnancy, however,
and can cause the users white blood cell count or platelet count to decrease which can be very
dangerous when paired with extreme anemia.

Barring the risk of transmitted disease, blood transfusions are considered the most stable method
of treating SCD (and anemia). This treatment can also be used for strokes, chest crises, and
multi-organ failure. Stem cells can also be taken from a donors blood or bone marrow (the donor is
usually a close relative, like a brother or sister) to replace the diseased cells in the persons body.
The new cells created from the donors stem cells will not sickle, but this treatment has also
caused severe complications in 15% of the children that it was given to.
Frequency of occurrence
Approximately 1 in every 13 black or
African-American babies are born with sickle
cell trait, which can lead to sickle cell disease in
their children. Of their 318.9 million person
population, an estimated 100 000 americans
have SCD with 60-80% being black or
African-American. With the discovery of new
treatments and vaccinations, infant mortality as
a result of SCD has gone down by 42% from
1999 to 2002. Though no new screenings have
been done since, medicine in 2017 is much
more advanced that it was nearly 15 years ago,
and more treatments exist now for SCD than
ever before.
Alternative approaches
Most of the treatments available for sickle cell disease are a result of years of
research and advanced medicine, so there isnt a way yet to treat SCD without
seeing a doctor. Home remedies can be used to treat some of its painful
symptoms, but unfortunately the only treatment close to a cure that is available
today involves a blood transfusion (or at least the use of drugs).
Sources
https://www.nhlbi.nih.gov/health/health-topics/topics/sca/treatment

https://en.wikipedia.org/wiki/Sickle-cell_disease

https://www.cdc.gov/ncbddd/sicklecell/data.html

https://www.nhlbi.nih.gov/health/health-topics/topics/sca

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/home/ovc-20303
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