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Shortness of breath in a woman with sickle cell anemia

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Pt was in her USOH until 1 month PTA when she developed mild, persistent ... CT angiogram: No pulmonary embolism. Diffuse bilateral ground glass opacity ... – PowerPoint PPT presentation

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Title: Shortness of breath in a woman with sickle cell anemia


1
Shortness of breath in a woman with sickle cell
anemia
  • Douglas B. White, MD
  • Chest Conference
  • April 1, 2003

2
  • ID/CC 36 year-old woman with SS anemia and
    recurrent DVTs who presents with acute-onset SOB.
  • HPI
  • Pt was in her USOH until 1 month PTA when she
    developed mild, persistent pleuritic chest pain
    without SOB.
  • 2 weeks PTA, she developed severe long bone pain
    and was effectively treated as an outpatient with
    Oxycontin and hydration.

3
  • The bone pain resolved and the patient was well
    until the day of admission when she developed
    progressive SOB over about 2 hours.
  • She was SOB at rest and unable to walk 1 block
    due to SOB and dizziness.
  • She denies CP, fever, cough, sputum production or
    LE swelling.
  • She states that she has been taking her coumadin
    regularly.

4
  • Past Medical History
  • Sickle Cell Anemia infrequent bony crises, no
    chest crises
  • Recurrent DVTs last DVT 5/00
  • Schizophrenia
  • Pyelonephritis
  • Medications
  • Coumadin
  • Depo-Provera IM
  • Wellbutrin
  • Risperdal
  • Folic Acid
  • Hydroxyurea

5
  • Social History
  • Active tobacco use 1ppd x 20years
  • No IVDU
  • Rare EtOH
  • HIV status unknown
  • 2 children, ages 5 and 15
  • Family History
  • No FH of sickle cell anemia

6
  • Physical Exam
  • 39.7? 37.0 110/60 HR110 RR24 88RA
  • Gen thin AA woman, mildly tachypneic
  • CV lift no S3 or S4 no murmurs
  • Resp No wheezes or rales frequent coughing
    during exam.
  • Abd benign
  • Ext no clubbing no edema

7
  • Labs
  • WBC 10.5 (nl diff)
  • Hct 31
  • Plt 331
  • Electrolytes normal
  • INR 3.7
  • ABG 7.45/30/47 RA
  • EKG ST S1Q3T3
  • Baseline Hct 28
  • Recent INRs
  • 2 months PTA 2.0
  • 1 month PTA 1.9
  • 1 week PTA 2.6

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9
Imaging Studies
  • V/Q scan
  • Severe perfusion defects in post segment RUL and
    sup segment RLL
  • Smaller subsegmental defects throughout.
  • High probability for acute PE
  • Echocardiogram
  • Normal LV size and function
  • No valvular abnormalities
  • RV dilatation, decreased RV function.
  • PAsp 40

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11
  • Overnight Clinical Course
  • Treated with heparin, ceftriaxone, doxycycline,
    MSO4 and 3 L NS IV overnight.
  • Unchanged hypoxia overnight and intermittent
    temp? 38.5 C.

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14
  • CT angiogram
  • No pulmonary embolism
  • Diffuse bilateral ground glass opacity
  • Enlarged PA, RV and RA.
  • Scattered cystic spaces

15
Clinical Course
  • Heparin was discontinued.
  • The pt was treated with antibiotics and over 4
    days her hypoxia and fever resolved.
  • She was discharged on HD5.
  • HRCT performed on the day of discharge.

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18
Pulmonary Manifestations of Sickle Cell Disease
19
Acute Pulmonary Complications of Sickle Cell
Disease
  • Pneumonia
  • Infarction due to In-situ thrombosis
  • Embolic Phenomena due to bone marrow infarction
  • Pulmonary edema
  • Acute chest syndrome (ACS)

20
Acute Chest Syndrome
  • The most common pulmonary complication of sickle
    cell disease.
  • Working definition
  • New segmental infiltrate
  • Chest pain
  • Temp gt 38.5 C
  • Tachypnea, wheezing or coughing

21
Clinical Manifestions of Acute Chest Syndrome
  • Fever 80
  • Cough 62
  • Tachypnea 45
  • Chest pain 44
  • SOB 41
  • Bony pain 37
  • Abdom pain 35
  • Rib pain 21
  • Wheezing 13

22
Acute Chest Syndrome
  • Etiology in 671 episodes in 538 patients
  • Unknown 46
  • Pulmonary infarction 16
  • Fat embolism 9
  • Chlamydia PNA 7
  • Mycoplasma PNA 7
  • Viral infection 4

23
Management of the Acute Chest Syndrome
  • Antibiotics (with atypical coverage)
  • Supplemental 02 (pA02 70-100mm Hg)
  • Narcotics to prevent splinting? atelectasis
  • Gentle volume repletion
  • Transfusions
  • Exchange transfusions if progressive resp failure
  • No empiric anticoagulation given the risk for
    renal and intracranial hemorrhage.

24
Pulmonary Edema in Sickle Cell Disease
  • 4/51 admissions for SS pain crisis in 1 year were
    complicated by pulmonary edema.
  • Pulmonary edema occurred in the setting of
    aggressive fluid resuscitation (200cc/hr) with ½
    NS and parenteral narcotics.
  • Normal LV function by echo, normal PCWP by RHC.
  • Hypothesis excessive narcotics increase the
    permeability of an already abnormal pulmonary
    circulation and hypotonic fluid decrease plasma
    oncotic pressure ? pulmonary edema.
  • Conclusion Overly aggressive IVF use during SS
    pain crisis may cause pulmonary edema.

25
Prevention of Acute Chest SyndromeEffect of
hydroxyurea on the frequency of painful crises in
sickle cell anemia
  • Double-blind RCT of hydroxyurea vs placebo in 299
    patients with SCD and painful crises.
  • Hydroxyurea group
  • fewer painful crises (2.5 vs 4.5 crises/yr
    plt0.001).
  • Fewer episodes of acute chest syndrome (25 vs 51
    plt0.001).
  • Conclusion Hydroxyurea can ameliorate the
    clinical course of sickle cell disease.
    Questions effect on chronic lung disease?
    Toxicity?

26
Diagnosing Pulmonary Embolism in Sickle Cell
Disease
  • Autopsy data show large and small vessel
    pulmonary thrombi in patients with SCD.
  • Due to thromboembolism vs.microvascular occlusion
    and subsequent in-situ thrombosis.
  • 16 asymptomatic patients with SCD had V/Q scans.
  • 2/16 read as high probability for acute PE
  • 6/16 were abnormal
  • 4/16 Subsequent admissions with SOB showed
    changes from the baseline V/Q scan?
    anticoagulation for presumed PE.

27
Diagnosing PE in Sickle Cell DiseaseScintigraphi
c evidence of vascular occlusion in SCD
  • 27 yo man with SCD and acute onset SOB.
  • V/Q scan demonstrated multiple large, peripheral,
    wedge-shaped defects.
  • LE doppler US normal
  • Treated for ACS with IVF and analgesics pt was
    not anticoagulated.
  • Pt clinically improved in 2 days and repeat V/Q
    scan at 7 days showed resolution of defects.

28
Chronic Pulmonary Complications of Sickle Cell
Disease
  • Restrictive lung disease with fibrosis
  • Decreased diffusing capacity
  • Airflow obstruction
  • Obstructive sleep apnea
  • Pulmonary Hypertension

29
Pulmonary Hypertension in Sickle Cell Disease
  • Possible mechanisms chronic hypoxemia, in-situ
    thrombosis, parenchymal and vascuar injury due to
    fat embolization and sequestration of sickled
    cells.
  • Echocardiograms retrospectively analyzed in 60
    consecutive patients with SCD referred for
    cardiac symptoms.
  • 12/60 had evidence of pulm HTN (PAsp 53 mmHg)

30
Pulmonary Hypertension in Sickle Cell Disease
  • Sutton et al noted 40 vs 8 mortality rate at 22
    months in SCD patients with pulm HTN vs SCD
    without pulm HTN (OR 7.86).
  • 2 year mortality 30-50 in 3 retrospective
    studies of patients with SCD and pulm HTN.

31
Summary
  • Sickle cell disease is complicated by both acute
    and chronic pulmonary complications.
  • Acute chest syndrome is the most common pulmonary
    complication of SCD.
  • The frequency of acute chest syndrome can be
    reduced with hydroxyurea.
  • The finding of pulmonary hypertension in SCD is
    associated with increased mortality.

32
References
  • Platt et al. Mortality in SCD. NEJM. 1994
    3301639.
  • Vichinsky et al. Causes and outcomes of ACS in
    SCD. NEJM. 20003421855.
  • Walker et al. Diagnosis of PE in SCD. Am J
    Hematol. 19797219.
  • Sutton et al. Pulmonary HTN in SCD. Am J Cardiol.
    199474626.
  • Haynes et al. Pulmonary edema in SCD. Am J Med.
    198680833.
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