Sickle Cell Disease and Fever

These instructions are for Children's Hospital of Philadelphia (CHOP) patients with sickle cell disease to learn what to do if they have a fever.

Important information about sickle cell disease and fever:

Children with sickle cell disease have a higher infection risk due to spleen damage caused by trapped sickle-shaped cells. The spleen helps fight infections by eliminating bacteria from the blood. If the spleen is damaged or removed due to serious injury, bacteria can remain in the blood and cause infections. Bacterial infections are particularly dangerous for children with sickle cell disease and fever is considered a medical emergency.

In the past, children with sickle cell disease faced a high risk of dying from a particular bacterial infection. This risk is reduced with the current practice of twice-daily penicillin and special vaccines. However, there is still a risk of infection, and fever may be the only sign of a serious bacterial infection.

Important information about blood cultures:

If your child has a fever, you must get medical care right away. They need to go to the hospital for a blood culture every time they have a fever. A blood culture is the only way to tell if there is a bacterial infection in the blood. Without treatment, the infection could make your child very sick or die.

Every time I bring my child in for fever the medical team tells me they couldn't find anything so why should I continue to bring them back for new fevers?

When the medical team says they couldn't find anything, it means that the blood culture did not show evidence of a bacterial infection. This is a good finding! Fevers can be caused by various infections, including viruses.

During these times, we may also discover other issues such as a large spleen, low blood counts, or acute chest syndrome, which are just as important to monitor. It's important to remember that each new fever could potentially be a bacterial infection, so we must check every time. There is no way to tell without coming to the hospital.

Important information about medicines that reduce fever:

Patients with sickle cell disease are not recommended to take medicines like acetaminophen (Tylenol®) and ibuprofen (Motrin®, Advil®) for fever. These medicines can bring a child's temperature down even when they have a serious bacterial infection. They relieve the symptom of the fever but do not treat the cause. Follow the fever rules below if your child has a fever or you need to give one of these medicines for pain.

Patient instructions:

Check your child's temperature when they do not feel well or if they have any of the following symptoms:

• Feels warm to the touch

• New fever greater than or equal to 101°Fahrenheit (38.3° Celsius) after 24 hours of no fever

• Fever that lasts more than four days

• Chills or ill appearance

• Irritability

• Decreased activity

• Weakness/exhaustion

• Difficult to wake up

• Drinking less than normal

• Vomiting and diarrhea

• Sore throat

• Rapid breathing

• Frequent coughing

• Runny nose or congestion

• Chest pain

• Ear pain

• Headache

• Neck pain

• Pain with urination

• Pale skin

Fever rules

• Take your child's temperature when they feel warm or seem to be sick.

• Always keep a thermometer handy. You should have a thermometer at home, at day care, at school or at relatives' homes where they stay. If you are traveling be sure to take your thermometer with you.

• If your child has a fever, please call the hematology team, and bring your child to the hospital immediately. A fever is:

  • 100.4° Fahrenheit (38° Celsius) or greater for children 2 months old and younger

  • 101° Fahrenheit (38.3° Celsius) or greater for children older than 2 months

• Do not give medicine like acetaminophen (Tylenol) or ibuprofen (Motrin or Advil) if your child feels warm or has a low-grade fever (temperature between 99-101°F).  Please check your child's temperature before giving these medicines to treat pain.

• If fever is present, then call your hematology team and bring your child to the hospital for blood work and antibiotics immediately.

Please contact your CHOP hematology team with questions or concerns.

Division of Hematology

215-590-3535

Sickle Cell Center

For non-urgent issues, send your team a message in the MyCHOP portal.

Reviewed on June 23, 2023, by Alexandra Kaspin, RN, MSN