SICKLE CELL DISEASE Flashcards
Major Complications
Vasoocclusive Crises
Pain Crises
Acute Chest Syndrome
Stroke
Renal/Hepatic/Splenic/Bone infarction
A vascular necrosis
Priapism
Acute Sequestration
Aplastic Episode
Hemolytic Crises
Sepsis / Infection
Investigations
VBG
CRP
CBC
Glucose
Electrolytes
LFT’s
Lipase
Group & Screen
Reticulocytes
CXR
Approach to Pain Crises
Most commonly affects long bones and back
Consider septic arthritis / osteomyelitis if febrile, limited ROM, pain in different location from prior episodes
If abdominal, usually poorly localized, no tender and rigidity. If atypical, workup for other abdo etiology
BREATHING:
02 PRN - not required
CIRCULATION:
IF dehydrated
20 ml / kg IV bolus NS over 15-20 min
THEN
Maintenance
D5 NS + 20 mEq/L KCL if isotonic dehydration
D5 1/2 NS + 20 mEq KCL if hypernatremic dehydration
DISABILITY
Morphine 0.1 mg/kg/dose q2-4 hrs prn (initial max 5 mg)
OR
Hydromorphone 0.01-0.02 mg/ kg IV q 3 hr
AND
Toradol 0.5 mg/ kg PO/IM
OR
Ibuprofen 10 mg/kg PO
Incentive spirometry when receiving narcotics
Maintain or escalate IV narcotic dose by 25% every 15 to 30 min
Antihistamine PRN:
Reactine
Infants ≥6 months and Children <2 years: Oral: 2.5 mg once.
Children 2 to 5 years: Oral 2.5 to 5 mg once.
Children >5 years and Adolescents: Oral: 5 to 10 mg once.
pRBC NOT Recommended
Approach to Acute Chest Syndrome
Consider ACS in all with patients with SCA with chest pain a/w tachypnea, dyspnea, cough.
Beware rapid deterioration to respiratory failure
Obtain CXR: new infiltrates
BREATHING:
Maintain Sp02 > 95%
CIRCULATION:
IF dehydrated
20 ml / kg IV bolus NS over 15-20 min
THEN
Maintenance
D5 NS + 20 mEq/L KCL if isotonic dehydration
D5 1/2 NS + 20 mEq KCL if hypernatremic dehydration
PRBC if drop > 1 g/dL from baseline:
Abx:
Ceftriaxone 50-75 mg / kg IV q 24 hr (Step Pneumo)
AND
Azithromycin 10 mg / kg IV (chlamydia, mycoplasma) THEN 5 mg / kg q 24 x 4 day
DISABILITY
Morphine 0.1 mg/kg
OR
Hydromorphone 0.015 mg/ kg
AND
Toradol 0.5 mg/ kg PO/IM
OR
Ibuprofen 10 mg/kg PO
Admission to PICU
Approach to Stroke
Consider in SCA with sudden headache / neurological change
DDx: Acute chest sydrome, sudden severe anemia, hypoglycemia, meningitis
BREATHING:
02 PRN
CIRCULATION:
Urgent Exchange Transfusion
Goal: <30% HbS, do not raise Hgb above 10 g / dL
DISABILITY:
Monitor temp, glucose
Approach to Priapism
Painful sustained erection > 4 hrs
CIRCULATION:
Oral Rehydration Solution
Goal: replace deficits over 4-6 hrs and replace ongoing losses
Mild - 1 ml / kg / 5 min
Moderate - 2 ml / kg / 5 min
Pedialyte:
45 mEq/L Sodium
140 mEq/L Glucose
OR
20 ml / kg IV bolus NS over 15-20 min
Repeat until perfusion improves and urine output is adequate
THEN
Maintenance 4 cc / hr for 1st 10 kg, 2 cc / hr for 10-20 kg, 1 cc / hr per kg for every kg > 20 kg
D5 NS + 20 mEq/L KCL if isotonic dehydration
D5 1/2 NS + 20 mEq KCL if hypernatremic dehydration
DISABILITY
Morphine 0.1 mg/kg/dose q2-4 hrs prn (initial max 5 mg)
OR
Hydromorphone 0.01-0.02 mg/ kg IV q 3 hr
AND
Toradol 0.5 mg/ kg PO/IM
OR
Ibuprofen 10 mg/kg PO
Incentive spirometry when receiving narcotics
Consult Urology
Needle aspiration of corpora cavernosa and administration of vasoconstrictor (1:1,000,000 epinephrine)
Approach to Acute Sequestration Crises
Primarily < 5 yrs
Sudden onset LUQ pain, pallor, lethargy, tender splenomegaly; progression to hypotension, shock, death
Ask about Viral prodrome, previous episodes
Profound anemia (> 2 pts lower than baseline)
DDx: ASC, Vascoocclusive, abdominal pathology
CIRCULATION:
IF dehydrated
20 ml / kg IV bolus NS over 15-20 min
THEN
Maintenance
D5 NS + 20 mEq/L KCL if isotonic dehydration
D5 1/2 NS + 20 mEq KCL if hypernatremic dehydration
Gradual pPRBC transfusion:
5 ml / kg
Avoid release of sequestered RBC -> hyperviscosity
DISABILITY
Morphine 0.1 mg/kg
OR
Hydromorphone 0.015 mg/ kg
AND
Toradol 0.5 mg/ kg PO/IM
OR
Ibuprofen 10 mg/kg PO
Approach to Aplastic Episodes
Gradual onset pallor, dyspnea, fatigue
Ask about recent viral prodrome (MC Parvovirus B19)
CBC: low Hgb, retic count, normal WBC and plt
CIRCULATION:
Consider pRBC transfusion:
10 -15 mL/kg
2.5 mL/kg/hour (10 mL/kg over four hours)
Expected increase 2-3 g/dL
Close outpatient follow up in 7-10 days for repeat labs
OR
Admission
Approach to Infections
Impaired splenic function increases bacteremia to 0.3%-1.1% (immunized) vs. up to 10% (unimmunized)
Beware sepsis in T > 40 C, WBC > 30,000 or <5,000, toxic appearance
CBC, blood clx, retic count, U/A / U Clx
+/- CXR
CIRCULATION:
20 ml / kg IV bolus NS over 15-20 min
Repeat until perfusion improves and urine output is adequate
THEN
Maintenance 4 cc / hr for 1st 10 kg, 2 cc / hr for 10-20 kg, 1 cc / hr per kg for every kg > 20 kg
D5 NS + 20 mEq/L KCL if isotonic dehydration
D5 1/2 NS + 20 mEq KCL if hypernatremic dehydration
Abx:
Coverage for S. Pneumo and H. Influenza
Ceftriaxone 50 mg / kg IV or IM q 24 hr
+/- Vancomycin
History & Physical
Pain
Chest Pain
Fever, chills, cough
Priapism
Previous Attacks
Spleen
Baseline Hgb
