The Management of Sickle Cell Disease
From the National Heart Lung and Blood Institute (NHLBI). The practice guidelines best supported by scientific evidence are:
- Penicillin prophylaxis prevents pneumococcal sepsis in children
- Pneumococcal vaccine prevents pneumococcal infection in children
- In surgical settings, simple transfusions to increase hemoglobin (Hb) levels to 10 g/dL are as good as or safer than aggressive transfusions to reduce sickle hemoglobin (Hb S) levels to below 30 percent
- Transfusions to maintain a hematocrit of more than 36 percent do not reduce complications of pregnancy
- Transfusions to reduce Hb S levels to below 30 percent prevent strokes in children with high central nervous system blood flow
- Hydroxyurea decreases crises in patients with severe sickle cell disease
- Stem cell transplant also called a bone marrow transplant
Sickle Cell Disease Emergency Guide:
It is very important that every person or family with a young child with sickle cell disease have a plan for how to get help immediately, at any hour, if there’s a problem. Be sure to find a place that will have access to your medical records or bring a copy.
Go to an emergency room or urgent care facility right away for:
- Fever above 101° F
- Difficulty breathing
- Chest pain
- Abdominal (belly) swelling
- Severe headache
- Sudden weakness or loss of feeling and movement
- Seizure
- Painful erection of the penis that lasts more than 4 hours
Call a doctor right away for:
- Pain anywhere in the body that will not go away with treatment at home
- Any sudden problem with vision
https://www.cdc.gov/ncbddd/sicklecell/documents/Sickle_Cell_Patients.pdf
https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease#treatment