Sickle Cell Pain Crisis

Sickle cell anemia requires regular medical attention by your healthcare provider and awareness about when to seek medical care. Pain is a common problem in children with sickle cell disease. This usually starts at less than 1 year of age. Pain can occur nearly anywhere in the body but most commonly occurs in the extremities, back, chest, or belly (abdomen). Pain episodes can start suddenly or may follow an illness. These attacks can appear as decreased activity, loss of appetite, change in behavior, or simply complaints of pain.

DIAGNOSIS

  • Specialized blood and gene testing can help make this diagnosis early in the disease. Blood tests may then be done to watch blood levels.

  • Specialized brain scans are done when there are problems in the brain during a crisis.

  • Lung testing may be done later in the disease.

HOME CARE INSTRUCTIONS

  • Maintain good hydration. Increase you or your child's fluid intake in hot weather and during exercise.

  • Avoid smoking. Smoking lowers the oxygen in the blood and can cause the production of sickle-shaped cells (sickling).

  • Control pain. Only take over-the-counter or prescription medicines for pain, discomfort, or fever as directed by your caregiver. Do not give aspirin to children because of the association with Reye's syndrome.

  • Keep regular health care checks to keep a proper red blood cell (hemoglobin) level. A moderate anemia level protects against sickling crises.

  • You and your child should receive all the same immunizations and care as the people around you.

  • Mothers should breastfeed their babies if possible. Use formulas with iron added if breastfeeding is not possible. Additional iron should not be given unless there is a lack of it. People with sickle cell disease (SCD) build up iron faster than normal. Give folic acid and additional vitamins as directed.

  • If you or your child has been prescribed antibiotics or other medications to prevent problems, take them as directed.

  • Summer camps are available for children with SCD. They may help young people deal with their disease. The camps introduce them to other children with the same problem.

  • Young people with SCD may become frustrated or angry at their disease. This can cause rebellion and refusal to follow medical care. Help groups or counseling may help with these problems.

  • Wear a medical alert bracelet. When traveling, keep medical information, caregiver's names, and the medications you or your child takes with you at all times.

SEEK IMMEDIATE MEDICAL CARE IF:

  • You or your child develops dizziness or fainting, numbness in or difficulty with movement of arms and legs, difficulty with speech, or is acting abnormally. This could be early signs of a stroke. Immediate treatment is necessary.

  • You or your child has an oral temperature above 102° F (38.9° C), not controlled by medicine.

  • You or your child has other signs of infection (chills, lethargy, irritability, poor eating, vomiting). The younger the child, the more you should be concerned.

  • With fevers, do not give medicine to lower the fever right away. This could cover up a problem that is developing. Notify your caregiver.

  • You or your child develops pain that is not helped with medicine.

  • You or your child develops shortness of breath or is coughing up pus-like or bloody sputum.

  • You or your child develops any problems that are new and are causing you to worry.

  • You or your child develops a persistent, often uncomfortable and painful penile erection. This is called priapism. Always check young boys for this. It is often embarrassing for them and they may not bring it to your attention. This is a medical emergency and needs immediate treatment. If this is not treated it will lead to impotence.

  • You or your child develops a new onset of abdominal pain, especially on the left side near the stomach area.

  • You or your child has any questions or has problems that are not getting better. Return immediately if you feel your child is getting worse, even if your child was seen only a short while ago.