See Vaso-occlusive pain crisis
More debilitating and refractory pain than past episodes
Localized bone tenderness, elevated WBC
Fat embolism can be complication
Fat embolism can be complication
Tender, swollen hands/feet
May have low-grade fever
Occurs in <2yr old, extremely rare >5yr old
Radiographs will show soft tissue swelling in the acute phase. Recurrent episodes can lead to a mottled appearance of hand and feet small bones on radiography. 

Due to occlusion of blood vessel lumens, which causes microfractures, leading to collapse of cancellous bone and the articular surface
Occurs in 30% of patients by age 30yr, prevalence increases with age
Patients present with afebrile, inguinal pain with weight-bearing but can be asymptomatic
Decreased ROM and pain with ambulation or passive ROM
Other common sites of involvement are the humeral head and the area above the knee
Decreased ROM and pain with ambulation or passive ROM
Acute Chest Syndrome
Pneumonia
Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. flu
Only need blood culture in patients ill enough to require ventilator
Asthma
Common in patients with SCD
Increases likelihood of acute chest syndrome by 4-6x
Pulmonary Hypertension
Develops in 15-35% of children with SCD
Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. flu
Only need blood culture in patients ill enough to require ventilator
Common in patients with SCD
Increases likelihood of acute chest syndrome by 4-6x
Develops in 15-35% of children with SCD
Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
Pain crisis
3rd most common site of pain crisis
Sudden onset of poorly localized abdominal pain
May have tenderness, guarding; should not have rigidity/rebound
Gallbladder disease (stones) is common; may occur as early as 2-4yr old
RUQ pain, jaundice/bilirubin higher than baseline, anorexia, tender hepatomegaly, fever
Acute hepatic or splenic sequestration
Labs are variable
US or CT shows diffuse hepatomegaly or splenomegaly
Bacterial gastroenteritis
Increased risk for salmonella
Consider treatment with ciprofloxacin and Bactrim in ill-appearing
3rd most common site of pain crisis
Sudden onset of poorly localized abdominal pain
May have tenderness, guarding; should not have rigidity/rebound
May have tenderness, guarding; should not have rigidity/rebound
RUQ pain, jaundice/bilirubin higher than baseline, anorexia, tender hepatomegaly, fever
Labs are variable
US or CT shows diffuse hepatomegaly or splenomegaly
Increased risk for salmonella
Consider treatment with ciprofloxacin and Bactrim in ill-appearing
Across all ages, infection is leading cause of death
Increased prevalence of meningitis, pneumonia, septic arthritis, osteomyelitis
No spleen means more susceptible to encapsulated organisms
Children aged 6mo to 3yr at greatest risk for sepsis
Parvovirus B19
Can cause several different syndromes:
Erythema infectiosum ("slapped cheeks" rash)
Gloves and socks syndrome
Well-demarcated, painful, erythema of hands and feet
Evolves into petechiae, purpura, vesicles, skin sloughing
Arthropathy - symmetric or asymmetric, knees and ankles
Aplastic crisis
Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
Can cause serious anemia which lasts for 2wk
Increased prevalence of meningitis, pneumonia, septic arthritis, osteomyelitis
No spleen means more susceptible to encapsulated organisms
Can cause several different syndromes:
Erythema infectiosum ("slapped cheeks" rash)
Gloves and socks syndrome
Well-demarcated, painful, erythema of hands and feet
Evolves into petechiae, purpura, vesicles, skin sloughing
Arthropathy - symmetric or asymmetric, knees and ankles
Aplastic crisis
Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
Can cause serious anemia which lasts for 2wk
Erythema infectiosum ("slapped cheeks" rash)
Gloves and socks syndrome
Well-demarcated, painful, erythema of hands and feet
Evolves into petechiae, purpura, vesicles, skin sloughing
Arthropathy - symmetric or asymmetric, knees and ankles
Aplastic crisis
Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
Can cause serious anemia which lasts for 2wk
Well-demarcated, painful, erythema of hands and feet
Evolves into petechiae, purpura, vesicles, skin sloughing
Evolves into petechiae, purpura, vesicles, skin sloughing
Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
Can cause serious anemia which lasts for 2wk
Patients with SCD have increased rates of bone and joint infection
Difficult to distinguish from bony infarcts
High fever is more typical of infection
Limited range of motion is much more typical of infection
Left shift is unique to infection
ESR is unreliable
May require bone scan or MRI to definitely distinguish infection from infarct
Difficult to distinguish from bony infarcts
High fever is more typical of infection
Limited range of motion is much more typical of infection
Left shift is unique to infection
ESR is unreliable
May require bone scan or MRI to definitely distinguish infection from infarct
High fever is more typical of infection
Limited range of motion is much more typical of infection
Left shift is unique to infection
ESR is unreliable
May require bone scan or MRI to definitely distinguish infection from infarct
Major cause of mortality in <5yr old
Labs: hemoglobin drop, no change in bili, normal to increased retic count
2 types: major and minor
Major
Rapid drop of hb (>3pt)
Pallor, LUQ pain, splenomegaly
Can progress within hours to altered mental status, hypotension, cardiovascular collapse
Minor
More insidious, smaller drop in hemoglobin
2 types: major and minor
Major
Rapid drop of hb (>3pt)
Pallor, LUQ pain, splenomegaly
Can progress within hours to altered mental status, hypotension, cardiovascular collapse
Minor
More insidious, smaller drop in hemoglobin
Major
Rapid drop of hb (>3pt)
Pallor, LUQ pain, splenomegaly
Can progress within hours to altered mental status, hypotension, cardiovascular collapse
Minor
More insidious, smaller drop in hemoglobin
Rapid drop of hb (>3pt)
Pallor, LUQ pain, splenomegaly
Can progress within hours to altered mental status, hypotension, cardiovascular collapse
More insidious, smaller drop in hemoglobin
CVA is 250x more common in children with SCD
10% of children suffer clinically overt stroke
20% found to have silent CVA on imaging
Increased rate of cerebral aneurysm and ICH
10% of children suffer clinically overt stroke
20% found to have silent CVA on imaging
Priapism
Occurs in 25% by age 20
Papillary necrosis
Hematuria without casts or pyuria
Occurs in 25% by age 20
Hematuria without casts or pyuria
Sickle cell crisis
Vaso-occlusive pain crisis
Aplastic crisis
Bony infarction
Dialysis disequilibrium syndrome
Dactylitis
Hemolytic uremic syndrome
Avascular necrosis of femoral head