Managing Sickle Cell Disease with Hydroxyurea
What is Hydroxyurea?
Hydroxyurea is a drug that until recently had been used to treat conditions such as leukemia, a form of cancer, and polycythemia vera, a rare blood disease which causes the body to produce excessive quantities of red blood cells.
Hydroxyurea has the ability to stimulate the production of fetal hemoglobin (Hgb F) a type of hemoglobin that does not sickle. Individuals with higher levels of fetal hemoglobin tend to have a milder course of the disease. This usually makes for longer intervals between painful crises and if they occur, they are less severe.Some Benefits of Hydroxyurea
Hydroxyurea is not a cure, but can prevent some pain attackes and other problems from occurring. Individuals who take Hydroxyurea tend to have fewer pain episodes (about 1/2 less), fewer attacks of pneumonia and do not require as many transfusions or hospitalizations as often as those not taking Hydroxyurea. Studies have shown that patients with higher levels of fetal hemoglobin, as a result of taking Hydroxyurea live longer than patients not taking Hydroxyurea.Hydroxyurea can be taken safely!
Close monitoring of your blood count is mandatory!
It is not a treatment for pain once a pain episode has started!
Concerns of Hydroxyurea
Hydroxyurea can suppress the bone marrow's production of red blood cells, white blood cells, that fight infection and platelets that are necessary for clotting. Therefore, taking this drug under the direction of a physician experienced in the use of Hydroxyurea is mandatory! There could be a risk of leukemia or cancer from long-term therapy (>5 years); however, this risk is considered to be very low. No sickle cell patients have developed leukemia or cancer after 10 years of monitoring the drug!
As Hydroxyurea is a chemotherapy agent, there might be a risk to a fetus conceived by a mother or father who is taking Hydroxyurea at the time of conception. Therefore, an acceptable method of birth control is necessary while actively taking Hydroxyurea.
WOMEN WHO ARE OR WANT TO BECOME PREGNANT SHOULD NOT TAKE HYDROXYUREA!
What will I need to do?
Before starting Hydroxyurea the following tests are needed:
- Complete blood count (CBC), including platelet count, MCV and reticulocyte count
- Liver and renal function tests
- Fetal hemoglobin level