Title: Shortness of breath in a woman with sickle cell anemia
1Shortness 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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9Imaging 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
15Clinical 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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18Pulmonary Manifestations of Sickle Cell Disease
19Acute 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)
20Acute 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
21Clinical 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
22Acute 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
23Management 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.
24Pulmonary 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.
25Prevention 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?
26Diagnosing 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.
27Diagnosing 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.
28Chronic Pulmonary Complications of Sickle Cell
Disease
- Restrictive lung disease with fibrosis
- Decreased diffusing capacity
- Airflow obstruction
- Obstructive sleep apnea
- Pulmonary Hypertension
29Pulmonary 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)
30Pulmonary 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.
31Summary
- 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.
32References
- 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.