Sickle Cell Disease Program
Sickle cell Transition Education Project (STEP)

Pain

NO. While studying is important, sleep is critical for your health. Teenagers should probably aim to sleep about 9 hours every night.

YES. Exercise is good for you. However, do not push yourself very hard because too much stress will trigger a crisis. When exercising, always drink lots of fluids and take breaks to rest. Individuals with sickle cell disease should not exercise as strenuously as people without sickle cell disease, so you may not be able to participate in certain competitive sports. However, you should still be able to play most sports for fun as long as you know your body’s limits.

NO. The air in very high altitudes like Mount Everest contains little oxygen and can trigger a crisis. If you travel to a higher altitude, rest and allow your body to acclimate (get used to the lower oxygen level) for a few days.

YES. People with sickle cell disease can fly on airplanes but may need to take extra precautions. The air on airplanes contains less oxygen which can cause problems for some people with sickle cell disease. Talk to your doctor if he or she thinks that you may need to arrange for supplemental oxygen when flying. When flying, also drink extra fluids because the air on airplanes is dry. When traveling don’t forget to bring all your medications and look up a treatment center at your destination in case of an emergency during your trip..

YES. People with sickle cell can become both mothers and fathers. Pregnant women with sickle cell disease do, however, need special medical care. It is important to remember that being a parent involves exhausting work—feeding, changing diapers—that is hard on the body. To best take care of your children and yourself, it is important to have people to help support you. Also remember that you can pass along the sickle cell gene to your children (link to genetics section). You and your mate should both know your hemoglobin genes and discuss if it is possible for your baby to also have sickle cell disease.

False, hydroxyurea is not a cure. Hydroxyurea can sometimes dramatically help a person with sickle cell disease like a cure, however, people taking hydroxyurea still have sickle cell disease and have health problems related to their sickle cell.

True, hydroxyurea has been shown to decrease pain crisis and acute chest syndrome episodes. In a group of studies in children with sickle cell disease, hydroxyurea cut the rate of hospitalizations by half or more (56% to 87%).

True, Hydroxyurea reduces the chances of death from sickle cell lung problems. Studies show that adults with sickle cell disease and taking hydroxyurea are living several years longer than those not taking hydroxyurea.

True, one way hydroxyurea helps people with sickle cell disease is by increasing the percentage of fetal (baby) hemoglobin in red blood cells. Red blood cells with fetal hemoglobin are more flexible and less sticky, and so are less likely to cause problems when traveling throughout the body’s blood vessels. Unfortunately, hydroxyurea does not significantly increase the percentage of fetal hemoglobin in some people who take the medicine.

False, hydroxyurea does have a few common side effects including upset stomach, darker fingernail beds, and mild weight gain.

True, the dosage of hydroxyurea is tailored to your body. Hydroxyurea can significantly decrease white blood cell and platelet counts, so a person taking hydroxyurea needs to have their blood counts checked. When starting hydroxyurea or increasing the dose, your doctor will check your blood counts to make sure you have a safe number of white blood cells and platelets. If your counts become too low then hydroxyurea is often stopped and the counts can return to normal.

False, hydroxyurea is not currently recommended for all people with sickle cell disease. In general, hydroxyurea is recommended more for people who have had significant problems from their sickle cell disease. Some people may have to stop hydroxyurea because of side effects; for example, hydroxyurea can sometimes decrease white blood cell counts so low as to increase the risk of serious infections. While many people may respond very well to taking hydroxyurea, others may not experience as significant benefits. Hydroxyurea is especially helpful in people with sickle cell who after taking the medicine have a good rise in the amount of fetal hemoglobin.

False, hydroxyurea is not a pain medication. Hydroxyurea is a medicine that causes the body to make “healthier” red blood cells in people with sickle cell. While hydroxyurea can help prevent pain episodes, it will not help decrease pain immediately like opiate pain medications (morphine, dilaudid).

False, hydroxyurea often takes several months before it helps decrease complications from sickle cell disease.

True, hydroxyurea only works if a person takes it daily.

False. In the past it was worried by some that hydroxyurea may increase the risk of cancer, however, it now appears that people taking hydroxyurea have the same risk of cancer as those people not taking the medication. Hydroxyurea (at a higher dose) was actually first used as an treatment for cancer. It is not yet known if there is any long term increased risk of cancer from taking hydroxyurea for many years. Medical experts believe this potential risk is low compared to the real risk of untreated sickle cell disease.

True. It is recommend that women should not get pregnant and men should not father children while taking hydroxyurea. Hydroxyurea might also decrease sperm counts and quality of sperm (decreasing the chance a man will get a woman pregnant). While there are concerns about hydroxyurea causing birth defects, a study of babies whose mother or father was taking hydroxyurea for sickle cell disease, did not actually show an increase in birth defects in these babies.