acute chest syndrome of sickle cell anemia
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Acute Chest Syndrome of
Sickle Cell Anemia
Ahmed Al Gahtani, BSRC, RRT
Definition
• The acute chest syndrome is a vasoocclusive crisis of
the pulmonary vasculature commonly seen in patients
with sickle cell anemia. The crisis is often initiated by a
lung infection, and the resulting inflammation and loss of
oxygen tension leads to sickling of red cells and further
vasoocclusion.
• ACS is currently defined as a new infiltrate on chest
radiograph in conjunction with 1 other new symptom or
sign: chest pain, cough, wheezing, tachypnea, and/or
fever (> 38.5°C). Platt OS et al, N Engl J Med 2000
Epidemiology
• ACS is a common complication in children with SCD.
• The peak incidence for ACS was in children between two
and four years of age (25.3 per 100 patient-years) with a
higher prevalence during the winter months.
• For patients with SCD, ACS is the second most common
cause of hospitalization (second to vaso-occlusive pain)
with a reported rate of 12.8 hospitalizations per 100
patient /year.
1. Castro, O, Brambilla, DJ, Thorington, B, et al. The Acute Chest Syndrome in Sickle Cell Disease- incidence and
risk factors. Blood 1994.
2. Vichinsky, EP, Neumayr, LD, Earles, AN, Williams, R. Causes and outcomes of the acute chest syndrome in
sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000
Clinical Presentation of ACS
Possible symptoms
• Fever
• Cough
• Chest Pain
• SOB
• Pain in arms or legs
Physical examination
• Rales
• Normal examination
• Fever
• Tachypnea
• Wheezing
• Fever and cough are the most common presenting symptoms in
children, while chest pain, shortness of breath, and chills are common in
adults. Vichinsky EP, Styles LA, Colangelo LH, et al. CSSCD, Blood 1997
Diagnostic Testing in ACS
• Serial chest radiographs
• Consider ventilation and perfusion imaging
• Serial hematologic testing
- Complete blood count
- Reticulocyte count
• Secretory phospholipase A2 measurement if available***
• Arterial blood gas with co-oximetry
• Blood cultures
• Consider bronchoscopy
Aaron W. Bernard, Zahida Yasin, Arvind Venkat, Hospital Physician January 2007
Radiographic features
• New infiltrate most often
involving LL.
• Pleural effusion.
• Pulmonary edema.
• Pulmonary embolism.
• Pulmonary infarction.
CAN J ANESTH; 2003
Therapeutic Modalities for ACS
• Supportive measures: Oxygen for hypoxia, Appropriate
hydration, and Appropriate pain control
• Incentive spirometry
• Antibiotics: Third-generation cephalosporin, macrolides
• Transfusion therapy: Simple transfusion, or Exchange
transfusion
• Experimental therapy: Nitric oxide, or Corticosteroids
Aaron W. Bernard, Zahida Yasin, Arvind Venkat, Hospital Physician January 2007
Case Presentation
• On 22/01/09 @ 2100, a 13 Y/O patient
(318437), known case of SCA brought to ER
(NGH) complaining of lower back pain and left
elbow pain with the following vital signs:
• Body Weight: 36.7 Kg
Temp HR BP RR Sat
37.2 89 110/53 20 96% on RA
Patient History
• HPI:
Pt was well until one day ago when he started to have
lower back and left elbow pain, pain started suddenly.
With NO fever, respiratory symptoms, abdomen pain, or
any other symptoms.
• PMH:
The Pt is known case of Sickle Cell Anemia diagnosed at
the age of 5 years. With two previous hospitalization first
was on July, 2006 due to aplastic crisis and headache
(vaso-occlusive crisis) the second was on Jan, 2007 with
admitting diagnosis of SCA with Acute Chest Syndrome
versus pneumonia.
• Allergies: No Known Drug Allergy.
Patient History
• Past Surgical History: None
• Family Medical History: Mother and one of his sisters
is SC trait. Parents are related of the second degree.
• Psychosocial History: Immunization completed, Child,
Student.
• Developmental History: Appropriate to age.
• Nutritional History: The patient is on family diet with
good appetite.
Pain Assessment
• Current continuous pain for one day.
• Location: Lower back + Left elbow.
• Pain Score: 8/10
Physical Exam
• General Appearance: Look well, not under distress,
in pain, well hydrated.
• Heent: Clear and normal.
• Chest: Clear BS with equal E/A bilaterally.
• CVS: Normal Sinus Rhythm, Regular with normal heart
sounds S1+S2.
• Abdomen: Soft, Bowl Sound Present.
• Neurological: Grossly Intact.
• Musculoskeletal: Pain.
Labs
TYPE 22/01/2009 23/01/2009 NORMAL
WBC 13.9 17.5 (4000 – 11500)/mm3
RBC ----- 2.92 (4.35 -5.55)X10^6/mm3
Hgb 84 81 (125 -155) G/L
Hct ----- .229 (.360 - .460)
Plt 771 693 (150 – 400)X10^3/mm3
Lymph % 31 27 26% - 46%
Mono % 11 9 3% - 9%
Neut % 57 58 34% - 64%
Bands % ----- 1 0% - 6%
Labs
TYPE 23/01/2009 NORMAL
BUN 2.4 (3.0 – 7.5) MMOL/L
Sodium 135 (136 – 145) MMOL/L
Potassium 4.8 (3.5 – 5.1) MMOL/L
Chloride 101 (95 – 110) MMOL/L
CO2 20 (20 – 28) MMOL/L
Glu R 5.8 (2.9 – 7.8) MMOL/L
Crea 44 (44 – 88) MMOL/L
Assessment & Plan
• Assessment: 13 y/o boy with SCA came with
crisis.
• Plan: Admission, Hydration, Pain Control, and
start Antibiotic therapy if Pt develop fever.
Day 2, 24/Jan/2009
• Patient still in the ER.
• General: Patient looks under moderate to severe
respiratory distress.
• V/S:
• HEENT: Congested throat.
• CVS: Sinus Tachycardia, Regular with normal heart
sounds S1+S2.
• Abdomen: Soft, Bowl Sounds present.
• Labs: No significant changes, WBC 13.9, Hgb
electrophoresis Positive.
Temp HR BP RR Sat
39.0 124 126/73 35 91% on 10LPM via NRFM
Day 2, Cont.
• Respiratory: Tachypnic, with increased oxygen
requirement, and increased WOB.
- Chest: Subcostal and Suprasternal retractions.
- BS: Bilateral Insp. & Exp Crackles with decreased A/E.
- CXR: No previous films for comparison, airspace
consolidation involving apical and posterior segments of
LLL associated with mild left PE and minimal right PE.
Airspace infiltrate in the rests of LLL and at the right
base.
- ET Culture: Negative, no organisms seen.
Day 2, Cont.
• Assessment: ACS of SCA vs. Pneumonia
• Plan:
- Pt to be admitted to PICU as soon as possible.
- Broad Spectrum Antibiotics.
- Simple transfusion, if no good outcome then Exchange
transfusion.
- IV hydration with analgesics.
• Medications:
Morphine Sulfate, Ibuprofen, Ranitidine Hydrochloride,
and Ceftriaxone Sodium.
• @ 2000 of the same day (24/1) patient
was brought to PICU on 10 LPM via
NRFM. BS bilateral coarse crackles with
decreased A/E. CXR showed lower lobes
consolidation and infiltrates. With the
following V/S:
Temp HR BP RR Sat
38.5 94 115/65 32 90%
Day 3, 25/Jan/2009
• CNS: GCS 7/15, on Midazolam and Morphine infusion.
• CVS: Sinus Tachycardia, Regular with normal heart sounds
S1+S2. Central line inserted in right femoral.
• V/S:
• Labs:
Temp HR BP RR Sat
38.5 121 139/66 30 90% on 10 LPM via NRFM
Type 0533 1547 2200 Type 0533 1547 2200
WBC 28.9 23.0 32.6 Plt 214 220 262
RBC 3.81 3.47 3.16 Lymph% 12% 8% 14%
Hgb 109 98 89 Bands% ---- 14% 19%
Hct .306 .278 .254 Neut% 73% 61% 45%
Day 3, Cont.
• Respiratory: Increased WOB, Tachypnic, BS were
bilateral Insp. & Exp. Crackles with decreased A/E. Pt
continue to desat below 87% on NRFM. So he was
placed on BiPAP 15/8, FiO2 0.8 via face mask @ 0145.
- ABG on BiPAP:
pH PCO2 PO2 HCO3 BE Sat
7.32 51.6 71.2 26.3 - 0.1 94%
Day 3, Cont.
• Comparison was made to study dated
24/01/09. Worsening.
• On the right side, atelectasis of RML &
RLL with moderate PE.
• On the left side, mild PE.
• Patchy opacities are seen in both upper
lobes.
• The cardiac outline is obscured by
dense opacification of both
hemithoraces.
• No air leak is seen.
Day 3, Cont.
• Respiratory: Cont.
- @ 0630 Pt continue to desat on BiPAP 20/12 and FiO2
1.0 so Pt was intubated with cuffed ETT size 6.5 with no
procedural complications. Then he was placed on PCV
mode via Servo i on the following settings:
- ABG @ 1030 showed:
- So PC was increased to 24 and PEEP to 17.
Set Parameters Measured Parameters
PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2
22 12 22 1.0 1.1 35 23 240 91%
pH PCO2 PO2 HCO3 BE Sat
7.09 107 34.5 26.6 - 4.6 91%/76%
Day 3, Cont.
• Respiratory: Cont.
- Right Chest Tube was inserted.
- Multiple episodes of bagging & suctioning to maintain
acceptable saturation.
- Pt developed right pneumothorax . So the right chest tube
was repositioned and a left chest tube was also inserted.
- @ 1145, HFOV trail was failed due to hemodynamic
instability.
- @ 1240, Pt was placed on CMV (PCV) via Servo i:
Set Parameters Measured Parameters
PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2
35 19 22 1.0 1.1 54 32 384 83%
@ 0853 @ 2103
Day 3, Cont.
• Respiratory: Cont.
- ABG @ 1519:
so RR was increased to 25 and PC decreased to 24.
- iNO was started 25ppm by 0.2 LPM flow.
- ABG @ 2210:
so RR decreased to 22, PC decreased to 22, PEEP to 18,
and FiO2 t0 0.9
- Plan is to continue monitoring Pt closely, keep SpO2 > or =
92%.
pH PCO2 PO2 HCO3 BE Sat
7.21 56.5 34.4 22.1 - 5.8 84%/59%
pH PCO2 PO2 HCO3 BE Sat
7.39 37.4 67.4 22.6 - 2.8 94%/93%
Day 3, Cont.
• Medications:
- Midazolam.
- Fentanyl.
- Rocuronium.
- Morphine Sulfate
- Dopamine.
- Dobutamine.
- Ibuprofen.
- Ceftriaxone Sodium.
- Ranitidine Hydrochloride.
- Azithromycin.
- Vancomycin Hydrochloride.
- Folic Acid.
- D5 in NaCl 0.45%, 500 ml
with 10 mEq Potassium.
- Ventolin.
Day 4, 26/Jan/2009
• CNS: Pt on Morphine 30mg/Kg/h, Fehtanyl PRN, Midazolam
was stopped. Pt pupils react 2-3 mm and moving all limbs,
GCS 10/15.
• CVS: Normal Sinus Rhythm, Regular with normal heart sounds
S1+S2. Plan is to started weaning process from Inotropes
maintaining HR 85-110, BP 110-130/60-70s (80-95).
• V/S:
Temp HR BP RR SpO2
38.5 98 130/64 22 93%
Day 4, Cont.
• Respiratory:
- BS: bilateral fine crackles with diminished breath sounds
over both bases.
- CXR: airspace infiltrates in both upper lobes, PE, and
possible left lower lobe collapse.
- Bag/Suction: moderate, loose, and white ( done once)
- Oxygenation Management: FiO2 and PEEP were both
decreased gradually FiO2 0f 0.6 and PEEP of 10,
maintaining SpO2 above 92%.
- Third Chest Tube was inserted on the left.
Day 4, Cont.
• Respiratory: cont.
Ventilation Management:
- @ 0150 vent settings were as follow:
- iNO: no change.
- ABG @ 1624
Set Parameters Measured Parameters
PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2
22 18 22 0.8 1.1 40 26 234 94%
pH PCO2 PO2 HCO3 BE Sat
7.38 35 88.9 20.5 - 4.5 96%/97.5%
Day 4, Cont.
• Respiratory: cont.
Ventilation Management:
- @ 1630 vent settings were changed to the following:
- They continue to wean the PEEP to 14 then 12 based on
SpO2.
- ABG @ 2209:
Set Parameters Measured Parameters
PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2
22 16 20 0.6 1.1 38 24 278 95%
pH PCO2 PO2 HCO3 BE Sat
7.43 36.9 101 24.0 0.3 96%/98%
Day 4, Cont.
• Respiratory: cont.
Ventilation Management:
- @ 2320 vent settings were changed to the following:
The plan for RCP is maintain ventilation to keep SpO2 > or =
88 - 92%, Normal pH, Q8h ABG, Q4h Ventolin.
Set Parameters Measured Parameters
PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2
22 10 20 0.6 1.1 31 19 261 96%
Day 4, Cont.
• Medications:
- Epinephrine ( Vasopressin).
- Meropenem.
- Dexamethazone. 10mg IV q12h for 2 days
- Acetaminophen.
Day 7, 29/Jan/2009
• CNS: GCS 11/15, Pt on Morphine 30mg/Kg/h, Fehtanyl PRN.
• CVS: Pt is stable.
• V/S:
• Respiratory: bilateral fine crackles with fair A/E. CXR showed
no significant change. iNO was weaned to 8ppm by none but
was increased to 15ppm at 1600. FiO2 was 0.9 in the morning
then decreased to 0.65 at 1440 but at 1550 was increased to
0.9. Pt was bagged & suctioned twice giving small amount of
thick white secretion. Pt still has 3 chest tubes 2 on the left
and 1 on the right.
Temp HR BP RR SpO2
36.7 100 130/67 31 96%
Day 7, Cont.
• Respiratory: cont.
Ventilation Management: @ 0725
@ 1207
@ 1550
@ 1733
Set Parameters Measured Parameters
SPC PEEP PS FiO2 RR Ti Ppeak MAP VTe SpO2
26 14 12 0.9 20 1.1 27 28 180 96%
pH PCO2 PO2 HCO3 BE Sat
7.44 45.1 71.3 30.2 6.1 98%/95%
Set Parameters Measured Parameters
VCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2
270 10 1.0 30 0.75 40 19 231 93%
pH PCO2 PO2 HCO3 BE Sat
7.43 45.1 52.8 29.8 5.3 88%/85%
Day 10, 1/Feb/2009
• CNS: Pt is sedated, GCS 7/15.
• CVS: Pt is stable and off inotrops .
• V/S:
• Labs: WBC 30.6, Hgb 105, Plt 921, Na+ 144, K+ 4.7,
CO2 32, Cl- 103, Crea 42.
• Respiratory: BS were bilateral fine crackles with expiratory
wheezes. The chest tube #4 was reinserted at 1012, iNO was
weaned off, and Pt was bagged and suctioned for 6 times
during this day giving moderate amount of yellow thick
secretions. Plan is to keep SpO2 > or = 88 – 92% and blood
gas q8h.
Temp HR BP RR SpO2
37.4 94 117/72 33 93%
Day 10, Cont.
• Respiratory: cont.
Ventilation Management:
@ 1017
ABG @ 1129
@1200
Set Parameters Measured Parameters
PRVCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2
270 13 1.0 30 0.75 42 23 253 91%
pH PCO2 PO2 HCO3 BE Sat
7.42 50.6 71.9 32.3 7.8 90%/97%
Set Parameters Measured Parameters
PRVCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2
270 10 0.9 30 0.75 38 22 226 90%
Day 10, Cont.
Day 10, Cont.
• Medications:
- Folic Acid, 1mg tab, q am
- Potassium Chloride, 5 mEq via tube q12h
- Ranitidine, 30 mg IV/30 min, q8h
- Ibuprofen, 300 mg, q6h
- Acetaminophen, 500 mg IVP, q6h
- Furosemide, 10 mg IVP, q8h
- Ventolin, 6 puffs/ q4h
- Midazolam, 2 mcg/kg/hr
- Morphine, 55 mcg/kg/hr
Day 25, 16/Feb/2009
• General: Pt was placed on prone position during this day.
• CNS: Pt is sedated, GCS 3/15.
• CVS: Sinus Tachycardia, Regular with normal heart sounds
S1+S2. Inotrops were restarted.
• V/S:
• Labs: WBC 49, Hgb 91, Plt 1625, Na+ 141, K+ 4.4,
CO2 30, Cl- 102, Crea 33.
• Respiratory: Over the night Pt developed left pneumothorax
and it was difficult oxygenate and ventilate with PCO2
accumulation so Pt was shifted to HFOV. The fifth chest tube
was inserted on the left side. Endotracheal culture result
showed positive yeast growth.
Temp HR BP RR SpO2
38.1 148 100/90 35 89%
Day 25, Cont.
• Respiratory: cont.
Ventilation Management:
ABG
ABG
0030 Set Parameters Measured Parameters
Mode PC PEEP RR FiO2 Ti P peak MAP VTe SpO2
PC 39 13 30 1.0 0.75 49 30 200 85%
Time pH PCO2 PO2 HCO3 BE Sat
0058 7.17 107 61.1 38.5 10.0 81%/85%
0100 Set Parameters Measured Parameters
Mode PC PEEP RR FiO2 Ti P peak MAP VTe SpO2
PC 35 12 35 1.0 0.55 85%
Time pH PCO2 PO2 HCO3 BE Sat
0347 7.15 124 65.5 42.7 13.9 85%/89%
Time Set Parameters
0415Mode Hz ∆P MAP Ti% Flow FiO2
HFOV 8 66 32 0.33 20 1.0
Day 25, Cont.
ABG
ABG
ABG
ABG
Time pH PCO2 PO2 HCO3 BE Sat
0514 7.03 167 56.8 43.3 13.0 74%/81%
Time Set Parameters
0520Mode Hz ∆P MAP Ti% Flow FiO2
HFOV 6 68 35 0.33 20 1.0
Time pH PCO2 PO2 HCO3 BE Sat
0514 7.08 140.5 52.3 40.3 10.2 74%/81%
Time Set Parameters
0545Mode Hz ∆P MAP Ti% Flow FiO2
HFOV 5 70 34 0.33 20 1.0
Time pH PCO2 PO2 HCO3 BE Sat
0737 7.23 95.4 50.7 38.9 11.4 88%/80%
Time pH PCO2 PO2 HCO3 BE Sat
2215 7.27 88.9 67.2 40.0 13.1 90%/92%
Day 25, Cont.
Day 25, Cont.
• Medication:
- Medazolam, 5 mcg/kg/hr.
- Fentanyl, 8 mcg/kg/hr.
- Precedex, 0.8 mcg/kg/hr.
- Cisatracurium, 3 mg/kg/hr.
- Epinephrin, 0.1 mcg/kg/min.
- Dobamine, 20 mcg/kg/min.
- Dobutamine, 16 mcg/kg/min.
- Lasix, infusion.
Day 36, 2/March/2009
• General: Pt is on supine postion since the 20th of last month.
• CNS: Pt is sedated, GCS 6/15, on same sedatives as on day
25.
• CVS: Pt is tachycardic but stable, and off inotrops support.
• V/S:
• Labs: WBC 23.7, Hgb 100, Plt 651, Na+ 144, K+ 3.9,
CO2 37, Cl- 97, Crea 30
• Respiratory: Good wiggle, 6 chest tubes (3 on right, and 3 on
the left). ICU team inform patient’s family about his lungs
status and its poor prognosis, the team are planning to switch
the patient back to CMV. Endotracheal culture done on the
28/2/2009 and the result was negative
Temp HR BP RR SpO2
36.6 137 108/41 86%
Day 36, Cont.
• Respiratory: cont.
Ventilation Management:
VBG
No changes was done on the vent settings, only FiO2 was
titrated to keep SpO2 within the accepted range.
Time Set Parameters
0730Mode Hz ∆P MAP Ti% Flow FiO2
HFOV 5 60 25 0.33 20 1.0
Time pH PCO2 PO2 HCO3 BE Sat
1007 7.31 83.6 48.7 41.7 15.5 100%/81%
Day 36, Cont.
Day 36, Cont.
• Medication:
- Voriconazole.
- Metoclopramide.
THANK YOU
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