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Interesting Case Rounds

Interesting Case Rounds. Rebecca Burton-MacLeod R5 (yikes!), Emerg Med July 5 th , 2007. Case. 21M presents to ED c/o CP X ~4 hrs Retrosternal, radiated bilaterally Not pleuritic + SOB +nauseas; not dizzy, no palpitations

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Interesting Case Rounds

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  1. Interesting Case Rounds Rebecca Burton-MacLeod R5 (yikes!), Emerg Med July 5th, 2007

  2. Case • 21M presents to ED c/o CP • X ~4 hrs • Retrosternal, radiated bilaterally • Not pleuritic • + SOB +nauseas; not dizzy, no palpitations • Also c/o generalized abdo pain, and pain to bilateral thighs (present x2days)

  3. History cont’d • No previous similar episodes, but sometimes his legs “have pain” • Pt moved from India about 2mos prior, ++ language barrier ! • PMHx: “healthy” • Meds: none • Allergies: none

  4. Further hx • Questions ?

  5. Physical exam • HR 115, BP 128/72, RR 26, Temp 37.9, sats 92% r/a • Pt ++ uncomfortable, tearful • CNS—A+O • CVS—N heart sounds, PPP • Resp—lungs clear • Abdo—generalized mild tenderness, no guarding, no rebound, + BS • MSK—c/o ++ tenderness to palpation bilateral thighs; no erythema or swelling noted

  6. Investigations • ??

  7. Investigations • CXR • EKG • CBC, lytes, Cr

  8. EKG

  9. Bloodwork • Hgb 77 • Plt 185 • WBC 31 • Lytes, Cr N

  10. “Have you ever been told you have anemia before?”…

  11. Sickle cell disease • Autosomal recessive disease • Caused by substitution of valine for glutamine in Hgb beta-chain (Hgb S)

  12. Sickle cell distribution

  13. Sickle cell crisis • Vaso-occlusive crisis • Aplastic crisis • Splenic sequestration • Acute chest syndrome • Infections

  14. Case cont’d • Pt received: • IV N/S 1L bolus; 2x maintenance fluids • IV morphine 5mg boluses to total of 20mg • Oxygen by n.p.

  15. Case cont’d • Called back to see pt as noted to be ++SOB • Sats now 85% on r/a • Clinically—wheezes and crackles heard bilaterally on auscultation of lungs • Temp now 38.7C • ABG—on 8L O2: pH 7.24 /CO2 51 /O2 50; lactate 12

  16. Any thoughts ?

  17. Acute chest s/o • Defined as: • New infiltrate on CXR • Associated with new symptoms (fever, cough, sputum production, dyspnea, hypoxia) • Occurs in 50% of SCD pts; recurrent in 80% • Preceded by vaso-occlusive crisis in half of patients • Leading cause of death for pts with SCD

  18. Risk factors for ACS • Young • Homozygous sickle-cell genotype • Fever • Winter months • Surgery • Previous ACS events • AVN of bones • High Hgb levels • Low fetal Hgb levels • High steady-state leukocyte counts

  19. Pathophysiology • Infection • Fat embolism • Thromboembolism • Vaso-occlusive sickling erythrocytes • Hypoventilation due to rib/sternal infarction and pain • Hypoventilation due to narcotic administration • Pulmonary edema due to fluid overload

  20. Sign / symptoms ~25% of adults have other symptoms of pain (usually lower extremities)

  21. Mgmt • Identify and treat precipitating factors • Maintain/improve oxygenation • Prevent further alveolar collapse • Maintain adequate fluid volume • Control pain • Treat underlying infection

  22. Oxygenation • Administer O2 to keep PaO2 ~70-100 • Pts may require simple, or exchange transfusions (allow removal of Hgb S) if severe anemia • Incentive spirometry, CPAP, or intubation/ mechanical ventilation with PEEP

  23. Indications for exchange tx • Evidence of rapid clinical deterioration • SaO2 <80% despite aggressive ventilatory support • Serial decline in SaO2 • Worsening/unstable vital signs • Persistent RR >30/min

  24. Maintain fluid volume • Replenish dehydration with N/S • For further fluid volume, use D5W/0.25N/S • Hypotonic fluids preferred as allows water to enter RBC • Causes osmotic swelling and reduced tendency for sickling

  25. Treat underlying infection • 2nd-3rd gen cephalosporin +/- macrolide • Bacteremia in ~3.5% of cases of ACS • Strep pneumoniae • H. flu • Staph aureus • Salmonella • Enterobacter • Clostridia

  26. Other therapies • DBRCT of IV dex in peds (n=43) showed SS shorter hosp stays, dec blood transfusions, dec clinical deterioration • Multiple case reports and animal studies of nitric oxide improving pt symptoms (lack of NO bioavailability in SCD) • Poloxamer-188 (non-ionic surfactant) may shorten duration of ACS and length of hospitalization (n=43)

  27. Questions ?

  28. References • Rosens • Ballas SK, et al. Safety of purified poloxamer 188 in SCD. Hemoglobin. 2004. • Bernini JC, et al. Beneficial effects of IV dexamethasone in children with mild to mod severe ACS complicating SCD. Blood. 1998. • Gladwin MT, et al. The ACS in SCD. Am J Resp Crit Care Med. 1999. • Kararmaz, A et al. ACS in a patient with SC anemia successfully treated with erythrocytopheresis. Int J Emerg Inten Care Med. 2006. • Machado RF. Nitric oxide based therapies in SCD: the evidence continues to mount. Crit Care Med. 2007. • Vichinsky EP, et al. ACS in SCD: clinical presentation and course. Blood. 1997. • Yale SH, et al. ACS in SCD. Postgrad Med. 2000.

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