Kacie Gray-Caruthers
Sickle cell anemia can be defined as a genetic blood disease caused by the presence of an
abnormal form of hemoglobin. This abnormal form of hemoglobin is called hemoglobin s. sickle
cell anemia is an autosomal recessive disorder. This means that in order for a child to inherit this
disease both parents must have the defected gene and it on to the child. If only one parent passes
the defective gene on the child will have the sickle cell anemia trait. People with the trait produce
normal and abnormal hemoglobin. The people with just the sickle cell trait normally do not
experience any symptoms of sickle cell. However those who have full blown sickle cell
experience many symptoms of the disease. These symptoms include anemia, frequent sickness or
infection, delayed growth, vision problems, swelling in the hands and feet, pale skin, fever,
abdominal swelling, and severe pain. One main symptom of sickle cell anemia is called crisis.
Specifically this paper will focus on explaining what is meant by sickle cell crisis and also
provide vivid details of what happens during a crisis along with preventative measures and daily
coping information.
The main symptom of sickle cell is crisis. A crisis is when the crescent shaped cells block
blood flow in the vessels causing severe pain and damage to integral organs. Even though sickle
cell crisis causes pain there are many different types of crises that do more than cause pain. For
example Vaso-occlusive Crisis is caused because sickle shaped blood cells block blood flow to
organs and impede capillaries which causes pain and death of tissues. Splenic Sequestration
Crisis is when the spleen is painfully enlarged. The purpose of the spleen is to get rid of
defective red blood cells but because of sickle cell anemia sometimes red blood cells pool in the
spleen stopping it from doing its job and making it enlarged. .This in the end causes
hypovolemia. Another form of crisis is called aplastic crisis. This crisis is caused by the
parvovirus B19. This virus affects the productions of red blood cells. This virus makes
duplicates of itself and destroys the red blood cells. Then end result is a more severe form of
anemia in the patient. Another form is hemolytic crisis which occurs when hemoglobin levels
drop which causes destruction to the red blood cells.
Many of those forms of crisis are so difficult to manage that patients seek treatment
options. Such treatment options include blood transfusions, bone marrow transfusions,
medications such as pain killers and antibiotics, immunizations, supplemental oxygen, and gene
therapy. During a blood transfusion red blood cells are replaced with those of red blood cells
from a supply of donated blood. This procedure has its pros and cons. This decreases the risk of
stroke however because normal blood has a regular amount of iron , the increase in iron builds
up in the body of the person with sickle cell causing damage to the heart ,liver and other vital
organs. Bone marrow transplants are the only true cure to sickle cell anemia however the
problem with this procedure is finding a person willing to donate their marrow. Medication such
as painkillers are used to treat pain cries. Antibiotics and immunizations are given to many
children and some adults who have sickle cell in order to prevent life threating infections such
pneumonia. Supplemental oxygen is just when you are given a breathing mask which allows
oxygen into the body helping the patient breath during a crisis. Gene therapy is still being
researched however the science behind it is that doctors believe that since sickle cell is a genetic
disorder/disease if they can insert a normal gene into the bone marrow of a person with sickle
cell anemia the normal gene will force the body to begin to produce normal hemoglobin. Also
under research is the idea of simply stopping the defective gene from working all while they are
starting the gene that is normal and forcing it to produce normal hemoglobin which will allow
scientist and doctors to change the blood fetuses receive.
Some people may wonder how people can continue to have children with sickle cell
when they know how its caused. But many people don’t even know that they have the sickle cell
trait until their child is born with sickle cell. A way to prevent the continuation of sickle cell is to
get genetic counseling. During genetic counseling parents are told about the risk factors of their
procreating , family trees are made to show each parent what each bloodline has ,as well as
discuss any disorders or disease that are found. Parents can then decide whether or not the risks
are too high or not.
Even though it is too late to prevent sickle cell for those who have it some ways to
prevent a crisis include maintaining normal oxygen levels , staying hydrated , avoiding strenuous
activities , stress, smoking , no pressurized flights , and high altitudes. Too much sun exposure
can also cause a person to go into crisis. It is very important for a person with sickle cell to
always have fluids near them. Seeing a doctor regularly meaning every 3 to 6 months just to
make sure everything is going well as well as getting your blood tested frequently. Regular eye
exams are also recommended because those with sickle cell tend to have vision problems. For
infants and children infections and sicknesses can be life ending therefor preventing these
infections can save their lives.
In order to live a healthy life with sickle cell anemia it is very important to maintain your
health. By eating health , introducing vegetable s and fruit into your diet as well low sodium
foods , poultry such a chicken that has no skin and that is baked or roasted, low fat foods are
recommended as well as low fat dairy products are encouraged. Nuts seeds and beans are also
recommended for daily consumption. Regular physical activity is also encouraged such as
walking and bike riding , making sure to avoid activities that make be considered strenuous that
would could a loss of proper oxygen levels and breathing. Making sure to get enough sleep is
very important for a person sickle cell because they really need their immune systems to be work
properly. Therefor people with sickle cell anemia who also have sleep apnea or that snore
frequently should contact their doctors about that. Also it is very important to make sure that
people with sickle cell know exactly what causes a person’s crises can help them make sure these
things don’t occur.
Sickle cell anemia is an autosomal recessive disorder. It is a genetic disorder that is
called buy the presence of a defective gene that causes abnormal hemoglobin to be produced.
This abnormal hemoglobin is called hemoglobin s. Sickle cell anemia is an inherited
disease/disorder. People with sickle cell anemia have many difficulties that come along with this
disorder. One main symptom of sickle cell is crisis. There are many different forms of crises
however they all are the result of sickle shaped red blood cells blocking and restricting normal
blood flow. This causes pain and destruction to many vital organs.