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Pulmonary Complications of
Sickle Cell Disease(SCD)
Olufolake Adisa, MD
Assistant Professor of Pediatrics
Respiratory Care Update
10/18/2014
Objectives
• Brief introduction of Sickle Cell Disease
• Review the literature on pulmonary complications of SCD with
particular emphasis on pathophysiology
• Introduce the proposed mechanisms underlying Asthma in SCD
• Introduce the proposed mechanisms underlying acute chest
syndrome
• Review the current classification of ACS, merits and demerits
and the diagnostic dilemma
• Brief review of management strategies centered on proposed
mechanisms
Aflac Cancer and Blood Disorders Center | Emory University
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Sickle Cell Disease
•
•
•
•
•
SCD is a chronic hemolytic and inflammatory disease
1:400 African Americans
1:36 000 Hispanic-American births
90-100,000 patients in US
Median life expectancy in 2005
– 42 years in females, 38 years in males
• Improved pediatric survival
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Newborn screening
PCN prophylaxis
Pneumococcal and HIB vaccine
Parental and provider education
http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhoIsAtRisk.html
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Sickle Cell Disease Pathophysiology
4
Sickle Cell Disease
• Caused by a point mutation in the β-globin chain of
hemoglobin, causing the amino acid glutamic acid to be
replaced with the hydrophobic amino acid valine at the sixth
position
• The association of two wild-type α-globin subunits with two
mutant β-globin subunits forms hemoglobin S (HbS).
• HbS is less soluble than normal hemoglobin (HbA) when
deoxygenated
• Insolubility results in polymerization and aggregation of HbS
which distorts red blood cells into a sickle shape and decreases
their elasticity
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6
Sickle Cell Disease
•
Rigid, dense and sickled cells become entrapped in the
microcirculation (vaso-occlusion) producing ischemia and
reperfusion injury, propagating inflammatory, thrombotic
and oxidant stress
• Intracellular polymerization ultimately damages the
membrane and depletes erythrocyte energy stores, leading
to chronic and episodic extravascular and intravascular
hemolytic anemia
• These disease mechanisms could ultimately produce a
proliferative vasculopathy affecting the brain, kidney and
lung vasculature
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Complications of SCD
• Phenotypic manifestations of SCD vary broadly
• Vaso-Occlusion
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Vaso-occlusive crisis (VOC) or Events (VOE)
Strokes and cerebral vasculopathy
Acute Chest Syndrome
Avascular necrosis
• Hemolysis
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Priapism
Splenic sequestration crisis
Leg Ulcers
Pulmonary Hypertension
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Pulmonary Complications of SCD
• Acute
– Painful (VOC) Crisis with Atelectasis
– RAD/Asthma Exacerbations
– Acute Chest Syndrome
• Chronic
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–
–
–
Asthma
Pulmonary Hypertension
Cor Pulmonale
Sickle cell chronic lung disease (± fibrosis)
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Painful VOE with Atelectasis
Painful involvement of ribs, sternum, spine or abdomen
Splinting with reduced ventilation
Segmental or lobar atelectasis
Increased V/Q mismatch, alveolar hypoxia and intrapulmonary
shunting
• ? Prelude to Acute Chest Syndrome (ACS)
• Aggressive and effective pain management
• Inhaled bronchodilator therapy
•
•
•
•
Adapted from Leroy Graham, MD
Aflac Cancer and Blood Disorders Center | Emory University
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Asthma and Sickle Cell Disease
• A physician-diagnosis of asthma is associated with increased
rates of pain, ACS episodes and death in patients with SCD
• Lack of objective criteria for asthma diagnosis in SCD
• Significant overlap in clinical manifestations between an
asthma exacerbation and an ACS episode
• Patients with SCD have symptoms of wheezing, obstructive lung
disease and airway hyper-responsiveness
• ? Atopic Asthma or acute pulmonary manifestation of SCD
Field, DeBaun, Hematology 2009; 45-53
Sylvester et al, Pediatr Pulmonol. 2007;42:272-276
Bryant et al, J Pediatr Health Care.2005;19:157-162
Boyd JH et al, Haematologica.2007;92:1115-1118
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Asthma and Sickle Cell Disease
• 291 infants with HbSS followed for a mean of
11yrs (4062 patient years) in CSSCD
– 16.8% with asthma
– 2X greater rate of ACS episodes (0.39 vs.0.2,
p<0.001)
– Increased risk for ACS during VOC pain; 4 X
greater risk of ACS (95% CI, 1.7-9.5)
• The OR for respiratory symptoms within 96hrs
of a pain event was 4.9 (95% CI, 2.2-10.7)
for children with SCD +asthma+pain vs.
SCD-asthma+pain
• > Number of Children with ACS taking
asthma controllers compared with those
without ACS
Boyd JH et al, Pediatr Pulmonology.2004;38:229-232
Glassberg J et al, J Pediatr Hematol Oncol.2006;28:481-485
Sylvester et al, Pediatr Pulmonol. 2007;42:272-276
Boyd JH et al, Blood .2006;108:2923-2927
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Asthma and Sickle Cell Disease
• Prevalence of asthma in children with SCD is similar to that in
AA children in the general population (~20%)
• Asthma is inherited in a familial pattern in the families of
children with SCD
• Asthma and SCD are both chronic inflammatory diseases
• PFTs in SCD patients revealed obstructive changes in 35%
and restrictive changes in 8%.
• Positive response to bronchodilator in 78% in obstructive
group and 67% in restrictive group.
Field, DeBaun, Hematology 2009; 45-53
Boyd et al, Pediatr Pulmonol. 2004;38:229-232
Koumbourlis, J Ped 2001: 138:188-192
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Asthma and Sickle Cell Disease
• Chronic airway inflammation, potentiating vascular
inflammation
• “Hyperreactivity” noted with a significant fall in FEV1(78%
vs. 18%)
• V/Q mismatch may increase the rate of pain or ACS
• LT B4and CysLT levels are increased in patients with SCD at
steady state and during vaso-occlusive events and increased
CysLT levels correlate with asthma severity
• Altered nitric oxide homeostasis in SCD and Asthma
– Decreased plasma arginine
– Increased arginase activity
Holgate ST et al. J Allergy Clin Immunol. 2003;111:S18-34;
Morris CR et al. Am J Respir Crit Care Med.2004;170;148-153
Allen, J Ped 1997; 131:278-83
CDC
2002.MMWR.
2004;53:145-148
Aflac
Cancer and
Blood Disorders Center | Emory University
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Asthma in Adults with SCD
• Relationship between asthma and ACS in adults with SCD is
not established
– Improvement in asthma symptoms with age
– Recall bias and lack of rigorous evaluation for asthma
– Lower incidence of ACS in adults
• Obstructive pattern on PFTs common in SCD but restrictive
pattern > in adults
• Management is not established but patients with SCD with
signs or symptoms of asthma should be treated aggressively
Field, DeBaun, Hematology 2009; 45-53
Boyd et al, Pediatr Pulmonol. 2004;38:229-232
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15
Sickle Cell Chronic Lung Disease (SCCLD)
• Exact incidence, prevalence, natural history, and methods of
diagnosis of SCCLD are not established
• Prevalence of ~4% in patients with SCD
• Morbidity and mortality in SCD especially SS
• Begins in 2nd decade of life with death by the 4th decade
• Clinically presents with progressive dyspnea, exercise
intolerance, pleuritic chest pain and hypoxemia
• SCCLD is presumably related to recurring episodes of
infarction and infection
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Powars,D at al 1998. Medicine 1998;67:66-76
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Sickle Cell Chronic Lung Disease
• Abnormal PFT:
– Lower airway obstruction, restriction, abnormal DLCO and hypoxemia
– Restrictive abnormalities on PFT with increasing age
• Characterized by a decrease in radiolucency of the lungs and
moderate to severe impairment of pulmonary function
• CXR: diffuse interstitial fibrosis, pulmonary arterial
prominence and cardiomegaly
• Progresses to the development of pulmonary hypertension
and cor pulmonale in association with restrictive lung disease
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Powars,D at al 1998. Medicine 1998;67:66-76
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Sickle Cell Chronic Lung Disease
• The pulmonary arterial bed, which has low oxygen tension and
pressure in a slow-flow system, is ideally suited to facilitate the
polymerization of sickle hemoglobin, causing endothelial
damage and culminating in an obstructive arteriolar
vasculopathy
• Post mortem studies
– pulmonary vascular bed obliteration
– smooth muscle hypertrophy
– parenchymal fibrosis
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Powars,D at al 1998. Medicine 1998;67:66-76
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Pulmonary Hypertension
• 20-40% prevalence in SCD
• Prospective study of 195 patients with SCD from the community
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PFTs and transthoracic echocardiography
Pulmonary HTN: TR jet velocity >2.5m/sec
Pulm. artery sys pressure >30mmHg
Present in 32%
Predictors of high TR jet velocity included factors reflecting the presence
of hemolysis, chronic anemia and the need for frequent transfusions
• Another study of 60 patients with SCD
• Echocardiography evidence of Pulm HTN in 17 (28%)
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Aflac Cancer and Blood Disorders Center | Emory University
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Pulmonary Hypertension
• Etiology: hemolysis, impaired NO bioavailability, chronic
hypoxemia, thromboembolism, parenchymal and vascular
injury, chronic liver disease and asplenia
• High risk of death, resistant to hydroxyurea therapy
• Sildenafil therapy
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12 patients with SCD and Pulmonary HTN
6+/-1months
decrease of 9mmHg in pulmonary artery systolic pressure,(95% CI)
increase in 6min walk distance
diverse effects headaches 2, eye lid edema 4, Priapism in 9( 2 on CTX,
1 with ED)
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Aflac Cancer and Blood Disorders Center | Emory University
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Pulmonary Hypertension
• Recurrent vascular and parenchymal insults
• Increased hemolysis resulting in increased plasma levels of cellfree hemoglobin, heme and iron which rapidly depletes NO
• Increased arginase, limiting bioavailability of L-arginine, the
substrate for NO synthesis
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•
228 patients with SCD and 36 controls, increased plasma arginase
activity, highest activity in patients with secondary pulm HTN
Oxidant status of SCD patients
Vascular smooth muscle dysfunction
Chronic hypoxemia
Autosplenectomy
Gladwin, et al 2004. NEJM ,350,886-895
Machado and Gladwin,2005. BJH,129,449-464
Aflac Cancer and Blood Disorders Center | Emory University
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Case Review
• Pulmonary consult for patient M.K prior to elective orthopedic
surgery
• M.K is an18 year old AA male with HbSS disease
• Significant PMHx: – Multiple episodes of ACS preceded by VOC, about 3 episodes over
the last year
– ICU admission x 1, + BiPAP, no intubations
– AVN Rt. Hip
– Priapism
• Currently on Chronic monthly PRBC Transfusions x 1 year
• Body Plethysmography: Normal. ?Low FRC.
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Case Review
Day 1: CC: Pain in lower back and B/L LE
PE: 36.8°C HR 122, RR 32, SPO2 98%, BP 126/81.
Chest exam – Normal
Day 3 : Increasing respiratory distress, hypoxia
Day 6: PRBC Transfusion
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CXR on Day 1
CXR on Day 3
Acute Chest Syndrome (ACS)
The term ACS in patients with sickle cell disease (SCD)
was first proposed by Charache et al in 1979
Arch Intern Med 1979;139:67
“the combination of chest pain, fever, increased
leukocytosis, and appearance of a new shadow on chest
radiograph”
….difficulties in determining its pathogenesis
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Causes and Outcomes of the Acute Chest
Syndrome in Sickle Cell Disease
ELLIOTT P. VICHINSKY, M.D., LYNNE D. NEUMAYR, M.D., ANN N. EARLES, R.N., P.N.P., ROGER WILLIAMS, M.D.,EVELYNE T. LENNETTE, PH.D., DEBORAH DEAN, M.D.,
M.P.H., BRUCE NICKERSON, M.D., EUGENE ORRINGER, M.D.,VIRGIL MCKIE, M.D., RITA BELLEVUE, M.D., CHARLES DAESCHNER, M.D., AND ELIZABETH A. MANCI, M.D.,
FOR THE NATIONAL ACUTE CHEST SYNDROME STUDY GROUP
N Engl J Med 2000;342:1855-65
• ACS is the leading cause of death in SCD
• Etiology unknown, therefore therapy was supportive
• Aim was to determine causes, incidence, clinical
outcome and factors that predict prognosis
• 671 episodes of ACS in 538 patients
• Mean Age: 13.8 years
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The NACSSG: Definition of ACS
“The acute chest syndrome was defined …..
a new pulmonary infiltrate involving at least one
complete lung segment that was consistent with the
presence of alveolar consolidation, but excluding
atelectasis
In addition, the patients had to have chest pain, a
temperature of more than 38.5°C, tachypnea,
wheezing, or cough”
Vichinsky et al, N Engl J Med 2000;342:1855-65
Aflac Cancer and Blood Disorders Center | Emory University
The NACSSG: Incidence of ACS
~50% admitted for non ACS, 72% for VOC
ACS developed after 2.5d
Mean oxygen saturation was 92%
Decreasing hemoglobin values (mean 0.78g/dl from
baseline)
• Multilobar LL involvement common in all age groups
• 55% had pleural effusion
Symptoms at presentation varied with age!
•
•
•
•
Vichinsky et al, N Engl J Med 2000;342:1855-65
Aflac Cancer and Blood Disorders Center | Emory University
The NACSSG:
Symptoms at Presentation
Age at first episode of ACS (years)
All patients
0-9
10-19
≥20
p value
Vichinsky et al, N Engl J Med 2000;342:1855-65
Aflac Cancer and Blood Disorders Center | Emory University
The NACSSG:
Clinical Outcome of ACS
• All had antibiotics, fever resolved after 2d
• No difference in extent of hypoxia but younger patients
had <O2 requirement and mechanical ventilation (MV)
Mean FEV1 was 53% of predicted during episode
61% treated with BD, 20% with ≥15% increase in FEV1
13% required MV for mean of 4.6d; 81% recovered
72% received PRBC transfusions (68% simple) 
improved oxygenation
• Mean hospital stay 10.5d
• Mortality 18%
•
•
•
•
Vichinsky et al, N Engl J Med 2000;342:1855-65
Aflac Cancer and Blood Disorders Center | Emory University
The NACSSG:
Factors that predicted severe clinical course
•
•
•
•
•
•
•
•
Age >20 years
History of VOC
Pain in arms and legs at diagnosis
Pleural Effusion
Fever
PRBC transfusion requirement
Extensive CXR abnormalities ≥4 lobes
Platelet count 0-199k
Respiratory
failure 13%
- Neurologic events 11%
Vichinsky et al, N Engl J Med 2000;342:1855-65
Aflac Cancer and Blood Disorders Center | Emory University
The NACSSG: Causes of the ACS
•
•
•
•
Fat Emboli ± infection: 8.8% (n=59)
Infection: 29.4% (n=197)
Infarction: 16.1% (n=108)
Unknown: 45.7% (n=306)
Vichinsky et al, N Engl J Med 2000;342:1855-65
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The Acute Chest Syndrome in Sickle Cell
Disease: Incidence and Risk Factors
Oswaldo Castro, Donald J. Brambilla, Bruce Thorington, Carl A. Reindorf, Roland B. Scott, Peter Gillette, Juan C. Vera, Paul
S. Levy, and The Cooperative Study of Sickle Cell Disease
Blood, 1994. 84: pp 643-649
• ACS: new infiltrate on CXR and/or a perfusion defect on a
lung radioisotope scan
• 2 year prospective study of 3,751 patients with SCD
• 2100 ACS events in 1,085 patients
• Incidence of ACS:
o in HbSS, HbSβ0thal and HbSC was 12.8 , 9.4 and 5.2 per 100 patient
years respectively
o highest in children 2-4 years of age
o lower in pts with lower steady-state Hb levels and higher fetal Hb
o steady-state leukocyte count.
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Acute Chest Syndrome in Sickle Cell
Disease: Clinical Presentation and Course
Elliott P. Vichinsky, Lori A. Styles, Linda H. Colangelo, Elizabeth C. Wright, Oswaldo Castro, Bruce Nickerson,
and the Cooperative Study of Sickle Cell Disease
Blood 1997 89:1787-1792
• To examine whether specific presentations or risk factors are
predictive of clinical outcome
• Prospective study of 939 SCD patients with 1,722 ACS
episodes over 9 years
• Ages 0-66years
• ACS definition: new infiltrate on CXR and/or a perfusion defect
on a lung radioisotope scan
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Acute Chest Syndrome in Sickle Cell
Disease: Clinical Presentation and Course
Elliott P. Vichinsky, Lori A. Styles, Linda H. Colangelo, Elizabeth C. Wright, Oswaldo Castro, Bruce Nickerson,
and the Cooperative Study of Sickle Cell Disease
Blood 1997 89:1787-1792
Children
Adults
Fever and Cough
Often afebrile
Negative PE
SOB, chills
Pain rare
Severe pain
Upper lobe
Lower lobe and Multilobar
>Bacteremia
>Severe hypoxia
>Cases in Winter
Cases in Winter
< Transfusions but earlier
>Transfusions
LOS 5.4d
LOS 9d
Preceded by fever
Preceded by pain
<Mortality
>Mortality, X4
• Fatal cases generally developed rapid pulmonary failure and one third were
associated with bacteremia
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ACS
• What is consistent?
– Interpretation of CXR must be reliable: ACS is a NEW
radiodensity on chest radiograph
– Can occur with or without fever
– Is a spectrum of disease
– Symptoms at presentation vary with age
– Has a rapid rate of progression
– Higher rate of recurrence in certain patients
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Further studies on ACS…
Other clinical events associated with development of ACS:
- Intravascular sickling Davies et al, Lancet 1984;1(8367):36–38.
- Microvascular in situ thrombosis Gladwin MT et al,Am J Respir Crit Care
Med 1999;159(5 pt 1):1368–1376
- Acute drop in hemoglobin concentration preceding the
diagnosis of ACS
Gladwin MT et al,Am J Respir Crit Care Med 1999;159(5 pt 1):1368–1376
Vichinsky EP et al , Blood. 1997; 89(5):1787–1792.
van Agtmael MA et al , Arch Intern Med. 1994;154(5):557–561.
- Secretory phospholipase
A2 Styles LA et al, Blood 1996;
87(6): 2573-2578
- Over expression of endothelial VCAM-1 Stuart and Setty, Blood 1999;
94(5 ):1555-1560
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Heme Catabolism and ACS
• Hemin induced acute intravascular hemolysis in transgenic sickle
mice and autoamplification of extracellular hemin produced an
acute lung injury reminiscent of ACS
Ghosh S, et al. J Clin Invest. 2013doi:10.1172/JCI64578
• Higher baseline plasma free heme is associated with increased
odds of ACS
Adisa OA, et al. Br J Haematol. 2013;162(5):702–705
• Polymorphism in the HMOX-1 gene (enhances expression of
HO-1) was associated with lower rates of ACS incidence in
children with SCD
Bean CJ, et al. Blood. 2012;120(18):3822–3828
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Heme Catabolism in SCD
Intravascular Hemolysis. Haptoglobin halts hemoglobin’s havoc Gregory J. Kato, JCI 2009
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Extracellular Heme triggers ACS
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Hypothesis
Unscavenged circulating heme is a biomarker and
potential trigger for ACS
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Association between Heme and ACS
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Association of SCD complications with
biomarkers of hemolysis
Adisa OA, et al. Br J Haematol. 2013;162(5):702–705
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HO-1 in SCD
Aflac Cancer and Blood Disorders Center | Emory University
Plasma HO-1 in SCD patients
HO-1 Expression:
HbAA: 2.57ng/ml ± 0.8
HbSS:
± 8.2 (p<0.0001)
HbSC: 12.92ng/ml ± 1.35 (p<0.0001)
Adisa et al,2014. Unpublished
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HOP-ACS Study: Genetic Heterogeneity in Heme
Degradation and its Role in Acute Chest Syndrome
Hypothesis:
Unscavenged circulating heme is a biomarker and
potential trigger for ACS
Aflac Cancer and Blood Disorders Center | Emory University
Definitions of the phenotypic
manifestations of sickle cell disease:
Acute Chest Syndrome (ACS)
Samir K. Ballas, Susan Lieff, Lennette J. Benjamin, Carlton D. Dampier, Matthew M. Heeney, Carolyn Hoppe,
Cage S. Johnson, Zora R. Rogers, Kim Smith-Whitley, Winfred C. Wang, and Marilyn J. Telen on Behalf of the
Investigators at the Comprehensive Sickle Cell Centers
American Journal of Hematology 2009 (85) vol. 1
ACS was defined as an acute illness characterized
by fever and/or respiratory symptoms, accompanied
by a new pulmonary infiltrate on a chest X-ray
Diagnostic Criteria
A new* segmental (involving at least 1 complete segment)
radiographic pulmonary infiltrate AND at least one of the following:
1.
2.
3.
4.
5.
6.
7.
8.
9.
Temperature ≥38.5ºC
>2% decrease in SpO2 (O2 saturation) from a documented steady-state
value on room air (FiO2 = 0.21)
PaO2 <60 mmHg
Tachypnea (per age-adjusted normal)
Intercostal retractions, nasal flaring, or use of accessory muscles of
respiration
Chest pain
Cough
Wheezing
Rales
* Does not require a preceding radiograph or physical examination, but if
either was performed then the current findings must be new.
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Mild ACS
• Meets the diagnostic criteria above AND all of the
following:
1. Transcutaneous O2 saturation >90% in room air (FiO2 = 0.21)
2. Segmental or lobar infiltrates that involve no more than 1 lobe
by chest radiography
3. Responsive to simple transfusion of no more than 2 units of red
blood cells (or 15 cc/kg PRBC)
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Moderate ACS
• Meets the diagnostic criteria above AND all of the
following:
1. Transcutaneous O2 saturation ≥85% in room air (FiO2 = 0.21)
2. Segmental or lobar infiltrates that involve no more than 2 lobes
by chest radiography
3. Responsive to transfusion of ≥3 units of red blood cells (or
more than 20 cc/kg PRBCs )
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Severe ACS
• Meets the diagnostic criteria above AND 1 or more of
the following:
1. Respiratory failure (PaO2 <60 mmHg or PCO2 >50 mmHg)
2. MV support required
3. Transcutaneous O2 saturation <85% in room air or ≤90%
despite maximal supplementalO2
4. Segmental or lobar infiltrates that involve 3 or more lobes by
chest radiography
5. Requiring transfusion or exchange transfusion of red blood cells
to achieve hemoglobin A ≥70%
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Very Severe ACS
• Acute Respiratory Distress Syndrome (ARDS) or
sudden, life-threatening lung failure is the most severe
complication of ACS. ARDS as defined by the 3
criteria of the American-European Consensus
Conference includes:
1. Acute onset of bilateral infiltrates on chest radiograph
2. Pulmonary artery wedge pressure of <19 mmHg or the
absence of clinical evidence of left atrial hypertension
3. PaO2/FiO2 ≤200 regardless of positive end expiratory
pressure (PEEP) level
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ARDS
• Acute onset of tachypnea, hypoxemia, and loss of compliance
after a variety of stimuli; the syndrome that did not respond to
usual and ordinary methods of respiratory therapy
• Pathologic features were similar to infantile RDS
• Etiology was a combination of insults to the lungs: viral
infection, fat embolism, fluid overload,
• PEEP was most helpful in oxygenation, improves alveolar
ventilation
• Treat underlying process but grave prognosis
Ashbaugh et al.. Lancet,1967
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53
Case #1
• 6 yo M with HbSC and RAD
• 2-d hx of dry cough, sternal chest pain unresponsive to
lortab/ibuprofen @ home, and a 1-d of tactile fever, no
albuterol at home
• VS: BP 89/62 | Pulse 70 | Temp 36.9 °C (Temporal) |
Resp 22 | Wt 23.2 kg | SpO2 98% on room air
• Local urgent care, CXR:
Focal airspace disease is seen in the left lower lobe. Otherwise, the
lungs are clear. The cardiac and mediastinal contours are normal. No
pneumothorax or pleural effusion is seen. The bones and soft tissues are
normal.
IMPRESSION: Left lower lobe acute chest syndrome versus pneumonia.
• Bld Cx, ceftriaxone and azithromycin
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Case # 1
• 3/19= admit to SR. Pain is mild, pt wants to walk around. No
recent sick contacts. NO URI symptoms. claf/zithro, pulm toilet
• 3/20= miralax
•
….On the morning of discharge , Pt is OOB at sink with grandma. Per
grandma he had a good night. During the hospital course he has been
afebrile, counts have been stable, blood cultures are NGTD and he had no
oxygen requirement. Discharge instructions reviewed with grandma and
mom via phone.
• VS at Discharge: BP 97/68 | Pulse 73 | Temp 36.8 °C
(Axillary) | Resp 20 | Ht 107 cm | Wt 21.1 kg | BMI 18.43
kg/m2 | SpO2 99%
• Discharge Diagnosis: ?
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Case #2
•
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•
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•
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•
Presented with VOC in legs
On day 2, developed fever, tachypnea, hypoxia SPO2 90%
CXR on day 2: RUL infiltrate
Received PRBC transfusion 15ml/kg
BiPAP initiated
Transferred to PICU on day 3
Increasing respiratory distress
CXR on day 3: LLL and RUL consolidation
Repeat PRBC transfusion 10ml/kg
Diagnosis ?
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Case #3
•
•
•
•
•
•
•
•
Presented with VOC, generalized
Hypoxia SPO2 90% on admission, RR 20-34
Fever on day 2
BiPAP at night due to PMHx
CXR on day 3: B/L LL Consolidation with pleural effusion
Received PRBC transfusion 15ml/kg
Increasing respiratory distress on day 4, transferred to PICU
Intubated, Exchange PRBC transfusion
• Diagnosis ?
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Our Classifications………
• Early ACS
• ?ACS
• Developing ACS
• Mild ACS
• Severe ACS
?
Aflac Cancer and Blood Disorders Center | Emory University
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Acknowledgement
Solomon Ofori-Acquah, PhD
Clinton Joiner, MD, PhD
Michael DeBaun, MD
Aflac Cancer and Blood Disorders Center SCD Team
Emory University, Department of Pediatric Pulmonary,
Allergy/Immunology, Cystic Fibrosis, and Sleep
• Georgia Pediatric Pulmonary Associates ( Leroy
Graham, MD, Jon Popler, MD and LaTresa Lang, MD)
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Aflac Cancer and Blood Disorders Center | Emory University