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
 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

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
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
Infection
 Osteomyelitis
 Salmonella
 Staph aureus
 Presentation overlaps with VOC


Cannot differentiate on imaging
Diagnosis

Clinical assessment
 Fever
 Leukocytosis
 ESR
 Positive cultures
Infection
 Parvovirus B19
 Most common cause of transient red cell
aplasia
 Predilection for young erythroblasts


Presentation







Plentiful in children with hemolytic anemias
Fever
URI
Fatigue
Pallor
Absence of scleral icterus
Decrease from baseline Hb with
reticulocytopenia
 7-10d
Treatment

Transfusion
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
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
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
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)

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

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
VOC Pain Crises
 Treatment
 Multidisciplinary
 Aggressive pain management
Opioids
 NSAIDs




Hydration
PT
Ancillary therapy
 Avg hospital stay 4d
 If >10d, must wean opioids
Prognosis
 Life expectancy

SS
45y
 Was 14.3y just 30 years ago


SC

65y