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Morning Report

Morning Report. March 17, 2011. Sickle Cell Disease. Chronic hemolytic anemia Multiple hemoglobin variants SS SC S-beta thal One of the most common genetic diseases worldwide Geographic distribution corresponds to distribution of malaria Carrier state is protective. Sickle Cell Disease.

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Morning Report

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  1. Morning Report March 17, 2011

  2. Sickle Cell Disease • Chronic hemolytic anemia • Multiple hemoglobin variants • SS • SC • S-beta thal • One of the most common genetic diseases worldwide • Geographic distribution corresponds to distribution of malaria • Carrier state is protective

  3. Sickle Cell Disease • Pathogenesis • Substitution of valine for glutamic acid • Chromosome 11 • Polymerization of deoxygenated Hb • Classic crescent shape • Less deformable cells in the microcirculation • Leads to complications

  4. Sickle Cell Disease • Diagnosis • NBS • Decreased deaths from pneumococcal infection • Isoelectric focusing • Hb electrophoresis • HPLC • DNA analysis • If diagnosed, refer by 3 months to hematology • If not available, see chart

  5. Infection • Spleen • Functional asplenia • 30% at 1y • 90% at 6y • Risk for encapsulated organisms • Strep pneumo • Prevention • PCN • Immunization • Treatment • Parenteral antibiotics for all children with fever • Ceftriaxone • Outpatient or inpatient • Resistance and incomplete protection

  6. Infection • Osteomyelitis • Salmonella • Staph aureus • Presentation overlaps with VOC • Cannot differentiate on imaging • Diagnosis • Clinical assessment • Fever • Leukocytosis • ESR • Positive cultures

  7. Infection • Parvovirus B19 • Most common cause of transient red cell aplasia • Predilection for young erythroblasts • Plentiful in children with hemolytic anemias • Presentation • Fever • URI • Fatigue • Pallor • Absence of scleralicterus • Decrease from baseline Hb with reticulocytopenia • 7-10d • Treatment • Transfusion

  8. Acute Splenic Sequestration • Diagnosis • Sudden enlargement of spleen • >2g/dL decrease in Hb from baseline • Reticulocytosis • May also have thrombocytopenia • Very rapid • <3y of age in HbSS • All other ages of HbS variants • Treatment • Volume expanders • Blood transfusions • Prevention • Parental education • Clinical signs • Palpating the spleen • Splenectomy • ≥2 events

  9. Acute Chest Syndrome • 2nd leading cause of hospital admissions • 50% of SCD patients • Age • More common in children • More severe in adults • Definition • Radiologic appearance of a new pulmonary infiltrate • Fever • Hypoxia may be present

  10. Acute Chest Syndrome • Causes • Infection • Bacteria, viruses, mycoplasma and chlamydia • Fat embolism • VOC • May be due to hypoventilation associated with opioids • Treatment • Broad-spectrum antibiotics • Cephalosporin • Macrolide • Oxygen • Hydration • Incentive spirometry • Early transfusion • Exchange if severe

  11. Cerebrovascular Disease • Stroke • 11% of SS patients <20y • 22% silent infarcts • Peak incidence • 2-10 • Path • Large-arteries • Internal carotid • Anterior and middle cerebral • Ischemic or thrombotic in 75% (predominately children)

  12. Cerebrovascular Disease • Prevention • TCD • Detecting children at risk • Flow velocity >200cm/sec • Screening recommended in all children between 2-16y • Chronic transfusion therapy • HbS <30% • Prevents second stroke in 80% • Reduces stroke risk 10 fold for patients with at risk TCDs

  13. VOC Pain Crises • Most distressing symptom • Spectrum is wide • 34% ≤ 1/year • 5% multiple events • Frequent admissions <6y is a known RF for death • Triggers • Infection • Temperature extremes • Dehydration • Emotional stress

  14. VOC Pain Crises • Treatment • Multidisciplinary • Aggressive pain management • Opioids • NSAIDs • Hydration • PT • Ancillary therapy • Avg hospital stay 4d • If >10d, must wean opioids

  15. Prognosis • Life expectancy • SS • 45y • Was 14.3y just 30 years ago • SC • 65y

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