n HOW IS SICKLE CELL DISEASE TREATED? Penicillin
A major research study conducted in the 1980s found that penicillin taken twice a day until the age of 5 decreases the risk of death from infection by 84% in infants and toddlers living with sickle cell disease. Because of this study, many states began screening newborns for sickle cell disease so penicillin could be started right away. These newborn-screening programs have improved the survival rate in early childhood. Penicillin (taken twice a day), caregiver education, and immunization with the pneumococcal vaccine polyvalent (Pneumovax 23) have made a huge improvement in the health of babies born with sickle cell disease. Folic Acid Folic acid is a vitamin needed to make hemoglobin. Because children with sickle cell disease need to make more hemoglobin, they need more folic acid. Often, the hematologist will prescribe a folic acid pill to take once a day.
Pain Medication Pain crisis or vaso-occlusive crisis is the number one reason children with sickle cell disease seek medical attention or come to the hospital. However, some pain can be managed at home with medications prescribed by your child’s hematology team. It is important to know that pain can be reduced and managed but not always eliminated. The goal of pain management is to decrease pain to allow for improved patient function and mobility and to allow the ability to cough and take deep breaths to avoid complications like acute chest syndrome. There are two main types of pain medications prescribed. The first is nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Motrin®, Advil®), ketorolac (Toradol®), and naproxen sodium
(Aleve®, Naprosyn®). These medications are helpful in reducing some of the swelling and inflammation associated with pain crisis as well as relieving the pain. The second group of medications is opioid pain relievers such as morphine, hydromorphone (Dilaudid®), and oxycodone (included in Percocet®, OxyContin®). Acetaminophen (Tylenol®) with codeine rarely is used because of how it is absorbed by the body, not relieving pain for some patients and lasting longer than is safe for others. In cases when pain crises are not well controlled, adding opioids like morphine, oxycodone, and hydromorphone to a Tylenol or Motrin regimen may be more effective in relieving the pain. Although adding these medications has the potential for patients to become tolerant and dependent, but rarely addicted, they are safe when used as prescribed. Tolerance means the body gets use to a medication and more is needed to treat the pain or a different medication is needed. Dependence refers to a state of needing a medication to function normally and the patient having distressing physical symptoms when the medication is taken away. Your child’s hematology team can address these issues in more detail.
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