eMedicine Specialties > Emergency Medicine > Pediatric

Pediatrics, Sickle Cell Disease: Follow-up

Author: Nedra R Dodds, MD, Medical Director, Opulence Aesthetic Medicine
Coauthor(s): Hosseinali Shahidi, MD, MPH, Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn
Contributor Information and Disclosures

Updated: Nov 21, 2007

Follow-up

Further Inpatient Care

• The primary care team should reevaluate the medication plan every day for patients admitted for pain control.
  • Taper IV drug doses when acute pain subsides, but do not change the dosing interval.
  • Once the patient tolerates a 50% decrease, consider oral analgesics and discontinue parenteral therapy.
• If IV antibiotics were started in the ED, continue therapy until appropriate culture results suggest a possible change in antibiotic choice.
• In patients who required emergency transfusion, hematocrits should be checked to ensure adequacy of the transfusion.
• The primary care team should review the patient's immunization schedule and prophylactic antibiotic therapy.

Further Outpatient Care

• The patient's pediatrician should examine the patient every 3-4 months to review their acute illnesses and possible precipitants and the frequency of crises, hospitalizations, and blood transfusions.
• The patient's CBC, reticulocyte count, liver function, and renal function should be monitored during these follow-up visits.
• In addition to the immunization schedule currently recommended by the American Academy of Pediatrics, pediatric patients with sickle cell disease require pneumococcal, meningococcal, and influenzal vaccines.
  • The pneumococcal vaccine should be administered at age 2 years with subsequent boosters determined by the patient's hematologist.
  • Meningococcal prophylaxis is administered as a single quadrivalent vaccine when the child is older than 2 years.
  • The influenza vaccine is administered annually.

Inpatient & Outpatient Medications

• Penicillin prophylaxis for encapsulated organisms is instituted as soon as the diagnosis of sickle cell disease is established, preferably by the age of 2 months.
  • An initial dose of penicillin V or G 125 mg twice daily (bid) is recommended.
  • The dose is increased to 250 mg bid by age 3 years.
  • If the patient is allergic to penicillin, erythromycin may be substituted.
• The pediatrician initiates folic acid therapy.
  • For patients younger than 6 months, the usual dose is 0.1 mg/d.
  • For infants aged 6 months to 1 year, the usual dose is 0.25 mg/d.
  • For children aged 1-2 years, the usual dose is 0.5 mg/d.
  • For patients older than 2 years, the dose is 1 mg/d.
• Hydroxyurea, which increases fetal hemoglobin production, reduces the incidence of pain episodes and acute chest syndromes in some patients who are severely affected. The patient's hematologist determines the doses by performing frequent laboratory determinations.

Complications

• The patient with sickle cell disease is susceptible to a multitude of complications during this life-long disease. Patients may experience any or all entities at some time.
• Susceptibility to infection, mainly those due to encapsulated organisms secondary to splenic dysfunction, begins in infants aged 4-6 months. Overwhelming sepsis can occur at any time.
• Osteomyelitis, particularly that due to Salmonella and Staphylococcus species, occurs more commonly in patients with sickle cell disease than in the general population.
• Avascular necrosis of the femoral head occurs in 10% of patients, who may require hip arthroplasty.
• The severity of anemia may induce high output failure, cardiomegaly, and flow murmurs.
• Retinopathy, secondary to sequestration of blood in the conjunctival vessels, is marked by dilated and tortuous retinal vessels, microaneurysms, and retinal hemorrhage.
• Patients who sustain trauma to the eye resulting in hyphema are at risk for glaucoma due to physical obstruction of the trabecular meshwork by sickled blood inside the anterior chamber.
• Cholelithiasis, particularly in patients older than 6 years, can occur due to chronic hemolysis.
• Irreversible renal damage causing hyposthenuria is present in almost all patients by the age of 3 years and may progress to renal failure requiring transplantation.
• Hematuria due to sickling in the vas recta or renal papillary necrosis is common. Patients with refractory hematuria may require hospitalization for bedrest, hydration, and a trial of epsilon aminocaproic acid (2-8 g/d by mouth). Patients with the sickle cell trait later may develop hyposthenuria or hematuria.

Prognosis

• At any time, the patient with sickle cell disease can be faced with a myriad of potentially life-threatening and unpredictable complications.
• Most patients and families with a good understanding of the disease process have good outcomes due to compliance with medication regimes and an overall healthy attitude that promotes well-being.

Patient Education

• Patient's families should have genetic counseling and education regarding clinical manifestations associated with the disorder and its complications.
• Reinforcement should occur incrementally during the course of ongoing care.
• Families should be educated on the importance of hydration, diet, outpatient medications, and immunization protocol.
• Patients should be instructed on proper splenic palpation and observation of pallor, jaundice, and fever.
• Families should be encouraged to contact community sickle cell agencies for follow-up information, new drug protocols, and psychosocial support. Families should also follow the advances of gene therapy, bone marrow transplantation, and the usage of cord blood stem cells.
• For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Sickle Cell Crisis.

• Search for CME/CE on This Topic »

• Sickle Cell Anemia (Pediatrics: General Medicine)
• Ellis-van Creveld Syndrome (Pediatrics: Genetics and Metabolic Disease)
• Ethical and Legal Considerations in Pediatric Surgery (Pediatrics: Surgery)
• Syndrome of Inappropriate Antidiuretic Hormone Secretion (Pediatrics: General Medicine)

• Sickle Cell Crisis Symptoms
• Sickle Cell Crisis Treatment
• Sickle Cell Crisis Overview
• Sickle Cell Crisis Causes
• Blood and Lymphatic System Center

• Severe H1N1 Infection in Children Linked to Asthma and Other Diseases
• American Academy of Pediatrics Updates Flu Recommendations
• PAS 2009: AAP Guidelines for Acute Otitis Media May Encourage Overtreatment

• 2009 H1N1 Influenza -- Just the Facts: Clinical Features and Epidemiology
• What's New in Newborn Screening?
• Ethics, Evidence, and Cost in Newborn Screening

• Pediatrics/Neonatal Care Nursing Resource Center
• Growth and Development