Pain crisis is the most common type of vasoocclusive event.The pain usually localizes to the long bones of the arms or legsbut may occur in smaller bones of the hands or feet in infancy(dactylitis) or in the abdomen. Painful crises usually last 2 to7 days. Vasoocclusive crises within the femur may lead to avascular necrosis of the femoral head and chronic hip disease.Treatment of pain crises includes administration of fluids, analgesia (usually narcotics and nonsteroidal anti-inflammatorydrugs), and oxygen if the patient is hypoxic. Although pain isoften impossible to quantitate, the risk for drug dependency ishighly overrated, and appropriate use of analgesics is necessary.
Priapism occurs in boys usually between 6 and 20 years old. The child experiences a sudden, painful onset of a
Anemia Chronic, onset 3–4 mo of age; may require folate therapy for chronic hemolysis; hemoglobin usually 6-10 g/dL
Aplastic crisis Parvovirus infection, reticulocytopenia; acute and reversible; may need transfusion
Sequestration Massive splenomegaly (may involve liver), crisis shock; treat with transfusion
Hemolytic crisis May be associated with G6PD deficiency
Painful crisis Microvascular painful vasoocclusive infarcts of muscle, bone, bone marrow, lung, intestines
Acute chest Infection, asthma, atelectasis, infarction, fat syndrome emboli, severe hypoxemia, infiltrate, dyspnea, absent breath sounds
Chronic lung Pulmonary fibrosis, restrictive lung disease, disease cor pulmonale, pulmonary hypertension
Priapism Causes eventual impotence; treated with transfusion, oxygen, or corpora cavernosa-tospongiosa shunt
Renal Hematuria, papillary necrosis, renal concentrating defect; nephropathy
Skeletal Osteonecrosis (avascular) of femoral or humeral head
Infections Functional asplenia, defects in properdin system; pneumococcal bacteremia, meningitis, and arthritis; deafness from meningitis; Salmonella and Staphylococcus aureus osteomyelitis; severe Mycoplasma pneumonia
Growth failure, May respond to nutritional supplements delayed puberty
Psychological Narcotic addiction (rare), dependence problems unusual; chronic illness, chronic pain syndrome
G6PD, Glucose-6-phosphate dehydrogenase.
*Clinical manifestations with sickle cell trait are unusual but include renal papillary necrosis (hematuria), sudden death on exertion, intraocular hyphema extension, and sickling in unpressurized airplanes.
tumescent penis that will not relax. Therapeutic steps for priapism include the administration of oxygen, fluids, analgesia, and transfusion when appropriate to achieve a hemoglobin S less than 30% (often by partial exchange transfusion). Fluid management requires recognition that renal medullary infarction results in loss of the ability to concentrate urine.
Overt stroke occurs in approximately 8% to 10% of patients with SS disease. These events may present as the sudden onset of an altered state of consciousness, seizures, or focal paralysis. Silent stroke, which is defined as evidence of cerebral infarction on imaging studies but a normal neurologic examination, is more common and occurs in approximately 20% of patients with SS disease. A significant change in school performance or behavior has been associated with silent stroke. Children with Hg SS disease over 3 years of age should be screened for increased risk of stroke using transcranial Doppler (TCD).