Health Maintenance for Sickle Cell Disease

At Hassenfeld Children’s Hospital at NYU Langone, doctors prescribe medications, vaccines, and use other strategies to reduce the risk of infections, increase levels of normal hemoglobin, and relieve pain in children with sickle cell disease. Some medications can also improve quality of life for children with sickle cell disease.

Preventing Infection

Our doctors prescribe antibiotics and immunizations for children with sickle cell disease to prevent infections that can result in a serious complication known as sepsis. This is a life-threatening blood infection that causes a dangerous drop in blood pressure.

Treatment with penicillin, an antibiotic given twice daily by mouth, usually begins as soon as your baby is diagnosed with sickle cell disease. It may be continued until age six or older.

Vaccines are also administered to protect against bacterial infections, including Haemophilus influenzae type b, also known as Hib (not the flu virus), and Streptococcus pneumoniae, a bacterium that can cause pneumonia, sinus infections, and ear infections. These vaccines can also prevent respiratory infections and complications, such as sepsis. Preventing infections also helps to reduce the risk of painful episodes in children with sickle cell disease that are due to reduced blood flow to the limbs and organs.

Our specialists can help you to ensure that your child’s vaccination schedule is up to date. They can also offer information about signs and symptoms—such as a fever of 101 degrees Fahrenheit or higher—that may prompt you to take your child to the emergency room, where he or she can be evaluated for sepsis.

Hydroxyurea

Your doctor may prescribe a chemotherapy drug called hydroxyurea to boost production of hemoglobin F—the main type of hemoglobin in newborn babies—and reduce the frequency and severity of painful episodes. Increasing the amount of hemoglobin F in children with sickle cell disease dilutes the amount of hemoglobin S, reducing the risk of sickle cell-related symptoms and complications.

Treatment may be offered starting when the child is nine months old. It can help to reduce the risk of complications of sickle cell disease, even in babies and children who have not had several painful episodes. Hydroxyurea is taken once a day by mouth.

Because this medication can reduce the number of white blood cells, children who are treated with hydroxyurea have an increased risk of infections and are monitored every four to six months with blood tests to measure blood cell levels.

Pain Medication

Doctors commonly prescribe over-the-counter and prescription pain medications to relieve sudden or chronic pain in children with sickle cell disease. Medications such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) can relieve mild-to-moderate pain. Acetaminophen is generally well tolerated by children, but taking NSAIDs for several days or longer can cause an upset stomach.

If your child has a severe painful episode caused by a blockage in a blood vessel, our doctors may recommend an opioid medication, such as morphine, given by mouth or through an intravenous (IV) catheter, a thin tube inserted through a vein in the arm or leg. Opioids slow down breathing, which can trigger acute chest syndrome. This leads to a reduction in oxygen, causing coughing, chest pain, and shortness of breath.

Vitamin Supplements

Certain vitamin pills, taken by mouth, can help to prevent symptoms and complications in children with sickle cell disease. Our doctors, for example, prescribe high doses of folic acid, a B vitamin, to help in the production of red blood cells, which die faster than they can be replaced in children with sickle cell disease.

A Vitamin D supplement may help to maintain bone health and ensure normal growth.

Fluids

Fluids can help to prevent dehydration, which can trigger painful episodes and acute chest syndrome. Fluids may be given by mouth or with an IV infusion.