Medical Management

• Transfusion Therapy
• Vaso-Occlusive Crisis (Pain)
• Infection/Fever
• Pneumonia/Acute Chest Syndrome
• Splenic Sequestration
• Aplastic Crisis
• Stroke

Transfusion Therapy

Transfusion therapy is generally used in the following circumstances:

• In patients with symptoms of
  • hypoxemia
  • severe anemia
  • acute chest syndrome

• In patients requiring a rapid decrease in Hemoglobin S (exchange transfusion) i.e.:
  • Severe infections
  • Stroke
  • Splenic sequestration
  • Severe Priapism
  • Acute Chest Syndrome
  • Refractory (unrelievable) pain crisis

• In preparation for surgery.

• In patients with certain chronic states every 2-4 weeks.
  • Minimum of 3-5 years after a stroke; may be lifelong
  • Leg ulcers
  • During a complicated pregnancy
  • In severe recurrent vaso-occlusive crises

Children on chronic transfusion therapy run the risk of iron overload. When the ferritin levels rise above 2000 ng/ml., the patient is started on Desferoxamine, a chelating agent. The kidneys excrete Desferoxamine. This may cause the urine to turn a reddish color in some patients. The medication is usually given subcutaneously (under the skin) at home and intravenously while in the hospital.

A child on a chronic transfusion program will miss at least one day of school every three to four weeks. He or she may also be on a program of iron chelation with the drug Desferoxamine, to remove the iron that accumulates from these transfusions.

Vaso-Occlusive Crisis (Pain)

Any condition that tends to cause dehydration (loss of water) may precipitate sickling in the microvasculature (small blood vessels).

• Analgesics Mild pain in younger children can often be successfully treated with acetaminophen at regular doses. With more severe pain, the usual oral regimen is a combination of codeine and acetaminophen. Most pain episodes can be successfully treated at home with oral analgesics and increased oral hydration. Severe pain may require parenteral narcotic analgesics and IV hydration. Some patients may be treated with drugs that might prevent pain crisis, such as hydroxyurea.

• Nonmedical pain management Self-hypnosis and relaxation exercises may help decrease the use of narcotic analgesics and also provide the child with a sense of control.

• Hydration Fluid intake should be increased during painful episodes. Caffeine-free soft drinks, juices, and bouillon are recommended.

Infection/Fever

Prevention or early aggressive treatment is very important. Infections will be treated with antibiotics.

• Penicillin prophylaxis (b.i.d. low dose penicillin) initiated by 3 months of age. Compliance is essential to prevent dangerous infection. Penicillin can be stopped in most patients by five years of age.

• With intercurrent infection, the physician may increase the dosage of penicillin and/or change the antibiotic.

Routine immunizations should be administered in accordance with The New Jersey State Sanitary Code, Chapter 14. It is also recommended that Hepatitis B be given in infancy as a routine part of these childhood immunizations. Administration of 23-valent pneumococcal polysaccharide vaccine should be given at 2 years of age, and repeated every three to five years until 10 years of age in all children with sickle cell disease. In a diagnosed child older than 10 years of age who has never received the vaccine, a one-time dose of 23-valent pneumococcal polysaccharide vaccine is recommended. This vaccine is given to children with sickle cell disease to help prevent infections caused by the pneumococcus.

Pneumonia/Acute Chest Syndrome

All patients with acute chest syndrome must be admitted to the hospital. Analgesics should be administered. Oxygen therapy is indicated for hypoxemia. Intravenous antibiotics and transfusions may also be necessary in treating this problem.

Splenic Sequestration

Treatment is directed toward prompt correction of hypovolemia with plasma expanders and particularly with blood transfusions. Splenectomy is considered if the child has had more than one episode.

Aplastic Crisis

Treatment is directed towards correcting the present problems. Transfusions should be given if needed. Hospitalization is not always required if the family continues follow-up visits.

Stroke

Management is aimed at early reversal of neurologic deficits and prevention of recurrence. Immediate treatment consists of intravenous hydration and transfusion to try to reverse or limit symptoms. Long-term management is aimed at prevention of a subsequent stroke.