Hydroxyurea treatment for sickle cell anemia
Sickle-cell disease is one of the
most common severe monogenic disorders in the world. It effects nearly 100,000
individuals in the United States and is associated with many acute and
chronic complications that require immediate medical attention. It occurs
mainly in people of African descent, but is also carried by some persons of Mediterranean
descent. Sickle cell disease is a multisystem disease, associated with episodes
of acute illness and progressive organ damage. Sickle cells contain an abnormal
hemoglobin termed hemoglobin S. There crescent shape makes it difficult for
them to pass through capillaries, causing a pileup of cells in small vesicles. The
can lead to severe pain from ischemia and may cause infarcts. This article talks
about the effects of hydroxyurea in the management of the disease in children
and adults. Hydroxyurea is a type of chemotherapy that is used for treatment
sickle cell because it lowers the number red blood cells. Hydroxyurea mainly
works by reducing the amount HbS made and increasing the amount HbF in the
blood. HbF is a natural is a protection against sickle problems. It is very
effective as HbF actually carries oxygen around the body releasing it to any
sickle blood under stress so preventing the process that causes almost all of
the problems in sickle cell disease. According to this evidence based article; Hydroxyurea
therapy was consistently associated with overall increases in HbF. In adults
and children, decreased rates of hospitalization were reported for patient
reciving hydroxyurea therapy. Also Hydroxyurea treatment was associated with
decreased mortality in symptomatic patients with SCA compared with patients
receiving shorter-term hydroxyurea or no hydroxyurea. The strength of the
evidence and serious consequences of frequent VOC provide the basis for strong
recommendations for hydroxyurea use in treating adults and children with Sickle
cell anemia.
That is interesting that they found a chemotherapy drug can treat sickle cell anemia. It sounds beneficial to the patient as a form of treatment, and maybe it would help prevent a sickle crisis as well as prevent ischemia and infarcts. It would be interesting to know how often the patients would have to receive the drug and if they would have problems sticking with the treatment due to the brutal side effects chemo drugs can have. My concern with this treatment is that patients will not want stick to the treatment. Sickle cell can be very painful and drains the person of energy, but chemo drug side effects like nausea, vomiting, loss of appetite, and possible hair loss may turn the patient away from this choice of treatment. This treatment sounds very promising, but it would be interesting to know how many patients who have received this therapy continued it and stuck with it.
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