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Hydroxyurea Sickle Cell

hydroxyurea sickle cell

Hydroxyurea, a myelosuppressive agent, is the only effective drug proven to reduce the frequency of painful episodes. It raises the level of HbF and the haemoglobin level. It usually decreases the rate of painful episodes by 50 %. It was first tested in sickle cell disease in 1984.24 May 2013.

What is hydroxyurea?

Hydroxyurea is a medicine that can help children and adults with sickle cell disease.

Research studies show that hydroxyurea lowers the following:

• The numbers of acute chest syndrome (pneumonia) events

• The number of pain crises

• The need for blood transfusions

• The number of trips to the hospital

Hydroxyurea also might prevent damage to the spleen, kidneys, lungs, and brain.

Hydroxyurea is given by mouth one (1) time each day. It comes in liquid or capsule form.

The U.S.

Which children should take hydroxyurea?

We consider hydroxyurea for children with sickle cell disease who have had:

• Many painful events,

• Several cases of acute chest syndrome (pneumonia),

• Severe anemia, or

• Other special problems with their internal organ

How does hydroxyurea work?

Hydroxyurea works in two ways. The first way is in the red blood cells. Red blood cells contain hemoglobin that carries oxygen to allparts of the body. People who have sickle cell disease make an abnormal hemoglobin called sickle hemoglobin. Sickle hemoglobincauses the red blood cells to become stiff and sticky. The sickle cell can then cause a block in the flow of blood to important organs.Hydroxyurea helps the body make another hemoglobin called fetal hemoglobin or hemoglobin F which is usually only producedby babies in the fetal stages of life (before birth). With higher levels of hemoglobin F, red blood cells are less likely to sickle andcause problems.

How will I know that hydroxyurea is working?

You will not feel better right away, because it takes a few months for hydroxyurea to start working. If you take it once a day everyday, then you will eventually notice that you feel better and have fewer problems like pain. Your doctors can also see changes inyour lab work that shows the medication is working. There are a few people who won’t respond, so the medication doesn’t help.But, you should try the hydroxyurea for at least six months before stopping It.

What tests are done to monitor hydroxyureatreatment?

Patients taking hydroxyurea usually have physical exams and have their blood counts checked

every month. They will be seen more often if needed. If your child is part of a research study,

then more tests may be performed. These tests help to determine the long-term risks and benefi ts

of hydroxyurea for people with sickle cell disease. Areas of the body tested can include the blood,

brain, kidneys, heart, and other internal organs.

side effects of hydroxyurea:

Lowers white blood cells (WBCs) - increased risk of infection if WBCs are too low

  • Lowers platelets - increased risk of bleeding if platelets are too low
  • Discolored nails
  • Weight gain or weight loss
  • Skin rash
  • Hair thinning
  • Nausea
  • Diarrhea or constipation

Conclusions

The evidence shows that hydroxyurea is likely to be effective in the short term at decreasing the frequency of painful episodes and raising fetal haemoglobin levels in the blood in people with SCD. Hydroxyurea is also likely to be effective in preventing first strokes for those at an increased risk of stroke and does not seem to be associated with an increase in any side effects (including serious and life-threatening side effects).

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