Other prescription drugs that may be used when adequate pain control is not possible are ketamine and gabapentin. Ketamine is a medication used for anesthesia; however, low doses of ketamine in conjunction with opioids can be used to treat acute pain in patients with sickle cell disease in acute settings, such as the emergency room and hospital. Gabapentin is an anti-seizure medication used to treat neuropathic or nerve pain. It also can be used to help treat chronic pain (pain lasting longer than 3 months) in patients with sickle cell disease. In the hospital, a patient-controlled analgesic (PCA) pump often is used to deliver pain medications (usually morphine or hydromorphine [Dilaudid]) as either “frequent” small infusions (on demand) controlled by the patient or as a small continuous infusion. The nurse programs and changes the settings of the PCA pump based on your child’s pain level and use of the pump. Pain medications may have side effects. The most common side effects are itching, upset stomach, constipation, and sleepiness. If your child is experiencing any of these side effects, speak with your child’s hematology team, and they will help your child with these side effects or even prescribe a different medication. There are also alternative treatments for sickle cell pain other than medications. Studies have shown that complementary alternative medicine (CAM) approaches have been useful alone and with medications for pain. These include heat application, distraction, acupuncture, aromatherapy, and healing touch. Other useful approaches are prayer, spiritual and energy healing, relaxation techniques, exercise, diet, and herbal medicines. Always consult your child’s hematology team to learn which approaches may be helpful for your child. Transfusion Occasionally, a transfusion of red blood cells may be needed to treat certain situations and complications of sickle cell disease. This transfusion will be given into a vein over the course of 2–4 hours. Transfusions may be planned in advance, as is the case before some surgeries; however, a transfusion also may be needed in an emergency situation. Individuals who have had strokes or an abnormal TCD likely will require chronic (every 2-4 weeks) transfusions for a period of time. If you have any questions or concerns about transfusions, talk to your child’s hematology team before an emergency happens. Hydroxyurea Before a child is born, their red blood cells contain fetal hemoglobin (hemoglobin F). If a child is born with sickle cell disease, this hemoglobin F is replaced by hemoglobin S in the first few months of life. Some people inherit the ability to keep making a small amount of hemoglobin F. As they get older, these people tend to have fewer complications of sickle cell disease. A medication, hydroxyurea, was found to stimulate the production of hemoglobin F. Hydroxyurea is a medication that has been found to turn on the body’s production of hemoglobin F. When taken correctly and monitored carefully, hydroxyurea does not have serious side effects. Hydroxyurea has been shown in research studies to decrease the number of pain episodes, the need for transfusions and hospitalizations, and incidences of acute chest syndrome. Hydroxyurea is approved by the U.S. Food and Drug Administration (FDA) and has been found to be safe to use even in very young children. Because it can harm a fetus, hydroxyurea should not be taken by people who are pregnant or plan to become pregnant.
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