Sickle cell crisis: Difference between revisions
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==Background== | ==Background== | ||
[[File:Sickle cell 01.jpg|thumb|(A) normal red blood cells flowing freely through a blood vessel. (B) abnormal, sickled red blood cells sticking at the branching point in a blood vessel. Cross-section shows long polymerized sickle haemoglobin (HbS) strands stretching and distorting the cell.]] | |||
===Precipitating Factors=== | ===Precipitating Factors=== | ||
*For vaso- | *For [[vaso-occlusive crisis]]: | ||
**Stress | **Stress | ||
**Cold weather | **Cold weather | ||
**Dehydration | **[[Dehydration]] | ||
**Hypoxia | **[[Hypoxia]] | ||
**Infection | **[[Infection]] | ||
**Acidosis | **[[Acidosis]] | ||
**Alcohol intoxication | **[[Alcohol intoxication]] | ||
**Pregnancy | **[[Pregnancy]] | ||
**Exertional stress | **Exertional stress | ||
*For aplastic crisis: | *For [[aplastic crisis]]: | ||
**Parvovirus B19 infection | **[[Parvovirus B19]] infection | ||
** | **[[Folate deficiency]] | ||
==Clinical Features== | ==Clinical Features== | ||
===Vaso-Occlusive Crisis<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref>=== | ===Vaso-Occlusive Crisis<ref>Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47</ref>=== | ||
''See [[Vaso-occlusive pain crisis]] | |||
===Respiratory | ===[[Avascular necrosis|Bony infarction]]=== | ||
*More debilitating and refractory pain than past episodes | |||
*Localized bone tenderness, [[leukocytosis|elevated WBC]] | |||
**[[Fat embolism]] can be complication | |||
===[[Dactylitis]]=== | |||
*Tender, swollen hands/feet | |||
*May have low-grade [[fever]] | |||
*Occurs in <2yr old, extremely rare >5yr old | |||
*Radiographs will show soft tissue swelling in the acute phase. Recurrent episodes can lead to a mottled appearance of hand and feet small bones on radiography. <ref>George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link</ref> | |||
===[[Avascular necrosis]] of femoral head=== | |||
<ref>George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link</ref> | |||
*Due to occlusion of blood vessel lumens, which causes microfractures, leading to collapse of cancellous bone and the articular surface | |||
*Occurs in 30% of patients by age 30yr, prevalence increases with age | |||
*Patients present with afebrile, inguinal pain with weight-bearing but can be asymptomatic | |||
**Decreased ROM and pain with ambulation or passive ROM | |||
*Other common sites of involvement are the humeral head and the area above the knee | |||
===[[Respiratory distress]] and [[chest pain]]=== | |||
*[[Acute Chest Syndrome]] | *[[Acute Chest Syndrome]] | ||
*[[Pneumonia]] | *[[Pneumonia]] | ||
**Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. | **Caused by [[chlamydia]], [[mycoplasma]], [[viruses|viral]], [[strep pneumo]], [[staph]], [[H. flu]] | ||
**Only need blood culture in patients ill enough to require ventilator | **Only need blood culture in patients ill enough to require ventilator | ||
*[[Asthma]] | *[[Asthma]] | ||
Line 43: | Line 50: | ||
*[[Pulmonary Hypertension]] | *[[Pulmonary Hypertension]] | ||
**Develops in 15-35% of children with SCD | **Develops in 15-35% of children with SCD | ||
***Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE | ***[[Chest pain]], [[dyspnea|DOE]], [[hypoxia]], right-sided [[heart failure]], [[syncope]], [[PE]] | ||
===Abdominal | ===[[Abdominal pain]]=== | ||
*Pain crisis | *[[vaso-occlusive crisis|Pain crisis]] | ||
**3rd most common site of pain crisis | **3rd most common site of pain crisis | ||
**Sudden onset of poorly localized abdominal pain | **Sudden onset of poorly localized abdominal pain | ||
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*[[Gallbladder disease]] (stones) is common; may occur as early as 2-4yr old | *[[Gallbladder disease]] (stones) is common; may occur as early as 2-4yr old | ||
**[[RUQ pain]], [[jaundice]]/bilirubin higher than baseline, anorexia, tender hepatomegaly, fever | **[[RUQ pain]], [[jaundice]]/bilirubin higher than baseline, anorexia, tender hepatomegaly, fever | ||
*Acute hepatic sequestration | *Acute hepatic or [[splenic sequestration]] | ||
**Labs are variable | **Labs are variable | ||
**US or CT shows diffuse hepatomegaly | **[[ultrasound: Abdomen|US]] or CT shows diffuse hepatomegaly or splenomegaly | ||
*Bacterial [[gastroenteritis]] | *Bacterial [[gastroenteritis]] | ||
**Increased risk for salmonella | **Increased risk for [[salmonella]] | ||
**Consider treatment with [[ciprofloxacin]] and [[Bactrim]] in ill-appearing | **Consider treatment with [[ciprofloxacin]] and [[Bactrim]] in ill-appearing | ||
===Infection=== | ===[[Infection]]=== | ||
*Across all ages, infection is leading cause of death | *Across all ages, infection is leading cause of death | ||
**Increased prevalence of [[meningitis]], pneumonia, [[septic arthritis]], [[osteomyelitis]] | **Increased prevalence of [[meningitis]], [[pneumonia]], [[septic arthritis]], [[osteomyelitis]] | ||
**[[Asplenic|No spleen]] means more susceptible to encapsulated organisms | |||
*Children aged 6mo to 3yr at greatest risk for sepsis | *Children aged 6mo to 3yr at greatest risk for sepsis | ||
*[[Parvovirus B19]] | *[[Parvovirus B19]] | ||
**Can cause several different syndromes: | **Can cause several different syndromes: | ||
*** | ***Erythema infectiosum ("slapped cheeks" rash) | ||
*** | ***Gloves and socks syndrome | ||
****Well-demarcated, painful, erythema of hands and feet | ****Well-demarcated, painful, erythema of hands and feet | ||
*****Evolves into petechiae, purpura, vesicles, skin sloughing | *****Evolves into petechiae, purpura, vesicles, skin sloughing | ||
*** | ***[[arthralgia|Arthropathy]] - symmetric or asymmetric, knees and ankles | ||
*** | ***[[Aplastic crisis]] | ||
****Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop | ****Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop | ||
****Can cause serious anemia which lasts for 2wk | ****Can cause serious anemia which lasts for 2wk | ||
===Musculoskeletal | ===Musculoskeletal infection=== | ||
*Patients with SCD have increased rates of bone and joint infection | *Patients with SCD have increased rates of [[osteomyelitis|bone]] and [[septic arthritis|joint infection]] | ||
**Difficult to distinguish from bony infarcts | **Difficult to distinguish from bony infarcts | ||
***High fever is more typical of infection | ***High [[fever]] is more typical of infection | ||
***Limited range of motion is much more typical of infection | ***Limited range of motion is much more typical of infection | ||
***Left shift is unique to infection | ***Left shift is unique to infection | ||
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***May require bone scan or MRI to definitely distinguish infection from infarct | ***May require bone scan or MRI to definitely distinguish infection from infarct | ||
===Splenic | ===[[Splenic sequestration]]=== | ||
*Major cause of mortality in <5yr old | *Major cause of mortality in <5yr old | ||
*Labs: hemoglobin drop, no change in bili, normal to increased retic count | *Labs: hemoglobin drop, no change in bili, normal to increased retic count | ||
Line 89: | Line 97: | ||
***Major | ***Major | ||
****Rapid drop of hb (>3pt) | ****Rapid drop of hb (>3pt) | ||
****Pallor, LUQ pain, splenomegaly | ****Pallor, [[LUQ pain]], splenomegaly | ||
****Can progress within hours to altered mental status, hypotension, | ****Can progress within hours to [[altered mental status]], [[hypotension]], [[pediatric shock|cardiovascular collapse]] | ||
***Minor | ***Minor | ||
****More insidious, smaller drop in hemoglobin | ****More insidious, smaller drop in hemoglobin | ||
===Neurologic | ===Neurologic disease=== | ||
*CVA is 250x more common in children with SCD | *[[CVA]] is 250x more common in children with SCD | ||
**10% of children suffer clinically overt stroke | **10% of children suffer clinically overt stroke | ||
**20% found to have silent CVA on imaging | **20% found to have silent CVA on imaging | ||
*Increased rate of cerebral aneurysm and ICH | *Increased rate of cerebral aneurysm and [[ICH]] | ||
===GU=== | ===GU=== | ||
Line 110: | Line 118: | ||
==Evaluation== | ==Evaluation== | ||
[[File:Sickle Cell.png|thumb|Algorithm for the Evaluation and Management of Sickle Cell Crises]] | |||
===Work-up=== | ===Work-up=== | ||
''Based on clinical presentation, but may include:'' | ''Based on clinical presentation, but may include:'' | ||
*CBC (assess for significant anemia) | *CBC (assess for significant anemia) | ||
*Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults) | *Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults), calculating reticulocyte index | ||
**Laboratory value is a percentage, and absolute count corrects for level of anemia | |||
**Absolute retic count = % retics x (pt Hct / normal Hct) | |||
**Retic index = absolute retic count / maturation factor<ref>Poorana PP and Subhashree AR. Role of Absolute Reticulocyte Count in Evaluation of Pancytopenia-A Hospital Based Study. J Clin Diagn Res. 2014 Aug; 8(8): FC01–FC03.</ref> | |||
***≥35%, maturation factor 1.0 | |||
***25 to <35%, maturation factor 1.5 | |||
***20 to <25%, maturation factor 2.0 | |||
***<20%, maturation factor 2.5 | |||
**Reticulocyte index ≥2 means adequate response | |||
**Reticulocyte index <2 suggests hypoproliferation | |||
*Metabolic panel, lipase (if abdominal pain) | *Metabolic panel, lipase (if abdominal pain) | ||
*[[CXR]] (if cough, shortness of breath, or fever) | *[[CXR]] (if cough, shortness of breath, or fever) | ||
*[[ECG]] | *[[ECG]] | ||
*VBG | *[[VBG]] | ||
* | *[[Urinalysis]] | ||
*Pregnancy test | *Pregnancy test | ||
*CT | *[[CT brain]] (if symptoms of CVA) | ||
**Note that if patient is anemic, CT non-contrast is unreliable in ruling out hemorrhagic stroke, [[subarachnoid hemorrhage]]<ref>Lyon M et al. Approach to the diagnosis and treatment of acute subarachnoid hemorrhage in a patient with sickle cell disease. Am J Emerg Med. 2015 Mar;33(3):481.e3-4.</ref> | |||
**Will need CTA, MRI, and/or MRA | |||
===Evaluation=== | ===Evaluation=== | ||
Line 127: | Line 147: | ||
==Management== | ==Management== | ||
===Anemia=== | *Only use supplemental [[oxygen]] for patients who are hypoxic (<92%) | ||
* | *Reserve [[IVF]] bolus for patients who are hypovolemic | ||
**Over hydration may cause atelectasis which may precipitate [[Acute Chest Syndrome]] and hyperCl acidosis which could lead to further sickling \ | |||
**Make sure to use hypotonic fluids: 1/2NS, D5-1/2NS | |||
===[[Anemia]]=== | |||
*[[Packed red blood cells]] (transfuse 10 mL/kg over 2hr period) | |||
**Indications: | **Indications: | ||
***[[Aplastic crisis]] | ***[[Aplastic crisis]] | ||
***Sequestration crisis | ***[[splenic sequestration|Sequestration crisis]] | ||
*** | ***Hemoglobin <6 with inappropriately low retic count | ||
*** | ***Hemoglobin <10 with acute crisis (e.g. symptomatic dizziness, [[SOB]], [[chest pain]]) | ||
===Vaso- | ===[[Vaso-occlusive pain crisis]]=== | ||
*Analgesia | *[[Analgesia]] | ||
**IV | **IV [[opioids]] ([[morphine]] or [[hydromorphone]]) | ||
***Redose in 30min if inadequate | ***Redose in 30min if inadequate | ||
**[[ketorolac]], | **Non-opioid analgesia | ||
***[[NSAIDs]] (e.g. [[ketorolac]] 15mg q6h) | |||
***[[Acetaminophen]] | |||
***IV/IM [[haloperidol]] or [[droperidol]] 5-10mg<ref>*Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics</ref> | |||
***[[Ketamine]] (IN 1mg/kg 1ml per nostril or IV 0.3mg/kg)<ref>*Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics</ref> | |||
**Nonpharmacologic measures (e.g. heating packs, distraction) | |||
*Hydration | *Hydration | ||
** | **Controversial | ||
** | ***Dehydration promotes sickling | ||
***[[IVF]] bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. <ref> Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20</ref> <ref> Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1) </ref> <ref> Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019. </ref> | |||
*O2 | ***There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.<ref>Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4</ref> | ||
*O2 if [[hypoxia]]; otherwise may inhibit erythropoiesis | |||
===Acute Chest Syndrome=== | ===[[Acute Chest Syndrome]]=== | ||
* | *[[O2]]; titrate to pulse oximetry >92% | ||
*Incentive Spirometer | |||
*Hydration: [[oral rehydration therapy|Oral hydration]] preferred, [[IV hydration]] with hypotonic fluid if patient unable to tolerate PO | |||
*[[Analgesia]]: pulmonary toilet is important but avoid excessive sedation | |||
*[[Bronchodilators]] | |||
*[[Antibiotics]]: 3rd generation [[cephalosporin]] + [[macrolide]] | |||
*[[Transfusion]] (leucocyte depleted); consider [[pRBCs|transfusion]] to goal of hemoglobin 11 / hematocrit 30 for: | |||
**O2 Sat <92% on room air | |||
**hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit | |||
*[[Exchange transfusion]]; consider for: | |||
**Progression of acute chest syndrome despite simple transfusion | |||
**Severe [[hypoxemia]] | |||
**Multi-lobar disease | |||
**Previous history of severe acute chest syndrome or cardiopulmonary disease | |||
===[[Priapism]]=== | ===[[Priapism]]=== | ||
*Hydration | *[[IVF|Hydration]] | ||
*Transfusion and/or exchange transfusion | *[[pRBCs|Transfusion]] and/or [[exchange transfusion]] | ||
*Urology consult | *Urology consult | ||
*If persists for >4-6hr: | *If persists for >4-6hr: | ||
Line 158: | Line 201: | ||
**Irrigate and infuse 1:1,000,000 epinephrine solution | **Irrigate and infuse 1:1,000,000 epinephrine solution | ||
===Neurologic | ===Neurologic disease=== | ||
* | *[[tPA]] is ''not'' recommended | ||
*Exchange transfusion urgently (within 8 hours) to decrease hemoglobinS below 30% | *[[Exchange transfusion]] urgently (within 8 hours) to decrease hemoglobinS below 30% | ||
*Hydration | *[[IVF|Hydration]] | ||
===[[Splenic sequestration]]=== | |||
*[[Volume resuscitation]] | |||
*Simple [[pRBCs|transfusion]] vs [[exchange transfusion]] | |||
=== | ===[[Fever]]=== | ||
* | *Any fever of ≥38.5C requires empiric [[antibiotics]] (regardless of well appearance or any localized infection found) | ||
* | *[[Ceftriaxone]] 50mg/kg (maximum single dose 2g) | ||
==Disposition== | ==Disposition== | ||
===Consider admission to the hospital=== | |||
**Acute chest syndrome is suspected | *[[Fever]] | ||
* | *[[Acute chest syndrome]] is suspected | ||
* | *[[Sepsis]], [[osteomyelitis]], or other serious infection is suspected | ||
* | *[[Priapism]], [[aplastic crisis]], [[hypoxia]] | ||
* | *[[leukocytosis|WBC >30K]] | ||
*[[thrombocytopenia|Platelet <100K]] | |||
*Pain is not under control after 2-3 rounds of analgesics in ED | |||
*<1yr old | |||
===Consider discharge=== | |||
*Pain is under control and patient can take oral fluids and medications | |||
*Ensure appropriate oral analgesics are available | |||
*Provide home care instructions | |||
*Ensure resource for follow-up | |||
==See Also== | ==See Also== |
Latest revision as of 11:03, 23 April 2022
Background
Precipitating Factors
- For vaso-occlusive crisis:
- Stress
- Cold weather
- Dehydration
- Hypoxia
- Infection
- Acidosis
- Alcohol intoxication
- Pregnancy
- Exertional stress
- For aplastic crisis:
- Parvovirus B19 infection
- Folate deficiency
Clinical Features
Vaso-Occlusive Crisis[1]
See Vaso-occlusive pain crisis
Bony infarction
- More debilitating and refractory pain than past episodes
- Localized bone tenderness, elevated WBC
- Fat embolism can be complication
Dactylitis
- Tender, swollen hands/feet
- May have low-grade fever
- Occurs in <2yr old, extremely rare >5yr old
- Radiographs will show soft tissue swelling in the acute phase. Recurrent episodes can lead to a mottled appearance of hand and feet small bones on radiography. [2]
Avascular necrosis of femoral head
- Due to occlusion of blood vessel lumens, which causes microfractures, leading to collapse of cancellous bone and the articular surface
- Occurs in 30% of patients by age 30yr, prevalence increases with age
- Patients present with afebrile, inguinal pain with weight-bearing but can be asymptomatic
- Decreased ROM and pain with ambulation or passive ROM
- Other common sites of involvement are the humeral head and the area above the knee
Respiratory distress and chest pain
- Acute Chest Syndrome
- Pneumonia
- Caused by chlamydia, mycoplasma, viral, strep pneumo, staph, H. flu
- Only need blood culture in patients ill enough to require ventilator
- Asthma
- Common in patients with SCD
- Increases likelihood of acute chest syndrome by 4-6x
- Pulmonary Hypertension
- Develops in 15-35% of children with SCD
- Chest pain, DOE, hypoxia, right-sided heart failure, syncope, PE
- Develops in 15-35% of children with SCD
Abdominal pain
- Pain crisis
- 3rd most common site of pain crisis
- Sudden onset of poorly localized abdominal pain
- May have tenderness, guarding; should not have rigidity/rebound
- Gallbladder disease (stones) is common; may occur as early as 2-4yr old
- Acute hepatic or splenic sequestration
- Labs are variable
- US or CT shows diffuse hepatomegaly or splenomegaly
- Bacterial gastroenteritis
- Increased risk for salmonella
- Consider treatment with ciprofloxacin and Bactrim in ill-appearing
Infection
- Across all ages, infection is leading cause of death
- Increased prevalence of meningitis, pneumonia, septic arthritis, osteomyelitis
- No spleen means more susceptible to encapsulated organisms
- Children aged 6mo to 3yr at greatest risk for sepsis
- Parvovirus B19
- Can cause several different syndromes:
- Erythema infectiosum ("slapped cheeks" rash)
- Gloves and socks syndrome
- Well-demarcated, painful, erythema of hands and feet
- Evolves into petechiae, purpura, vesicles, skin sloughing
- Well-demarcated, painful, erythema of hands and feet
- Arthropathy - symmetric or asymmetric, knees and ankles
- Aplastic crisis
- Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
- Can cause serious anemia which lasts for 2wk
- Can cause several different syndromes:
Musculoskeletal infection
- Patients with SCD have increased rates of bone and joint infection
- Difficult to distinguish from bony infarcts
- High fever is more typical of infection
- Limited range of motion is much more typical of infection
- Left shift is unique to infection
- ESR is unreliable
- May require bone scan or MRI to definitely distinguish infection from infarct
- Difficult to distinguish from bony infarcts
Splenic sequestration
- Major cause of mortality in <5yr old
- Labs: hemoglobin drop, no change in bili, normal to increased retic count
- 2 types: major and minor
- Major
- Rapid drop of hb (>3pt)
- Pallor, LUQ pain, splenomegaly
- Can progress within hours to altered mental status, hypotension, cardiovascular collapse
- Minor
- More insidious, smaller drop in hemoglobin
- Major
- 2 types: major and minor
Neurologic disease
- CVA is 250x more common in children with SCD
- 10% of children suffer clinically overt stroke
- 20% found to have silent CVA on imaging
- Increased rate of cerebral aneurysm and ICH
GU
Differential Diagnosis
Sickle cell crisis
- Vaso-occlusive pain crisis
- Bony infarction
- Dactylitis
- Avascular necrosis of femoral head
- Acute chest syndrome
- Asthma
- Pulmonary hypertension
- Gallbladder disease
- Acute hepatic sequestration
- Infection
- Parvovirus B19
- Splenic sequestration
- CVA
- Cerebral aneurysm and ICH
- Priapism
- Papillary necrosis
Evaluation
Work-up
Based on clinical presentation, but may include:
- CBC (assess for significant anemia)
- Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults), calculating reticulocyte index
- Laboratory value is a percentage, and absolute count corrects for level of anemia
- Absolute retic count = % retics x (pt Hct / normal Hct)
- Retic index = absolute retic count / maturation factor[4]
- ≥35%, maturation factor 1.0
- 25 to <35%, maturation factor 1.5
- 20 to <25%, maturation factor 2.0
- <20%, maturation factor 2.5
- Reticulocyte index ≥2 means adequate response
- Reticulocyte index <2 suggests hypoproliferation
- Metabolic panel, lipase (if abdominal pain)
- CXR (if cough, shortness of breath, or fever)
- ECG
- VBG
- Urinalysis
- Pregnancy test
- CT brain (if symptoms of CVA)
- Note that if patient is anemic, CT non-contrast is unreliable in ruling out hemorrhagic stroke, subarachnoid hemorrhage[5]
- Will need CTA, MRI, and/or MRA
Evaluation
- Generally a clinical diagnosis
- Certain syndromes require imaging/labs for confirmation (see below)
Management
- Only use supplemental oxygen for patients who are hypoxic (<92%)
- Reserve IVF bolus for patients who are hypovolemic
- Over hydration may cause atelectasis which may precipitate Acute Chest Syndrome and hyperCl acidosis which could lead to further sickling \
- Make sure to use hypotonic fluids: 1/2NS, D5-1/2NS
Anemia
- Packed red blood cells (transfuse 10 mL/kg over 2hr period)
- Indications:
- Aplastic crisis
- Sequestration crisis
- Hemoglobin <6 with inappropriately low retic count
- Hemoglobin <10 with acute crisis (e.g. symptomatic dizziness, SOB, chest pain)
- Indications:
Vaso-occlusive pain crisis
- Analgesia
- IV opioids (morphine or hydromorphone)
- Redose in 30min if inadequate
- Non-opioid analgesia
- NSAIDs (e.g. ketorolac 15mg q6h)
- Acetaminophen
- IV/IM haloperidol or droperidol 5-10mg[6]
- Ketamine (IN 1mg/kg 1ml per nostril or IV 0.3mg/kg)[7]
- Nonpharmacologic measures (e.g. heating packs, distraction)
- IV opioids (morphine or hydromorphone)
- Hydration
- Controversial
- Dehydration promotes sickling
- IVF bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. [8] [9] [10]
- There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[11]
- Controversial
- O2 if hypoxia; otherwise may inhibit erythropoiesis
Acute Chest Syndrome
- O2; titrate to pulse oximetry >92%
- Incentive Spirometer
- Hydration: Oral hydration preferred, IV hydration with hypotonic fluid if patient unable to tolerate PO
- Analgesia: pulmonary toilet is important but avoid excessive sedation
- Bronchodilators
- Antibiotics: 3rd generation cephalosporin + macrolide
- Transfusion (leucocyte depleted); consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
- O2 Sat <92% on room air
- hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
- Exchange transfusion; consider for:
- Progression of acute chest syndrome despite simple transfusion
- Severe hypoxemia
- Multi-lobar disease
- Previous history of severe acute chest syndrome or cardiopulmonary disease
Priapism
- Hydration
- Transfusion and/or exchange transfusion
- Urology consult
- If persists for >4-6hr:
- Aspiration of corpora
- Irrigate and infuse 1:1,000,000 epinephrine solution
Neurologic disease
- tPA is not recommended
- Exchange transfusion urgently (within 8 hours) to decrease hemoglobinS below 30%
- Hydration
Splenic sequestration
Fever
- Any fever of ≥38.5C requires empiric antibiotics (regardless of well appearance or any localized infection found)
- Ceftriaxone 50mg/kg (maximum single dose 2g)
Disposition
Consider admission to the hospital
- Fever
- Acute chest syndrome is suspected
- Sepsis, osteomyelitis, or other serious infection is suspected
- Priapism, aplastic crisis, hypoxia
- WBC >30K
- Platelet <100K
- Pain is not under control after 2-3 rounds of analgesics in ED
- <1yr old
Consider discharge
- Pain is under control and patient can take oral fluids and medications
- Ensure appropriate oral analgesics are available
- Provide home care instructions
- Ensure resource for follow-up
See Also
References
- ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
- ↑ George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link
- ↑ George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link
- ↑ Poorana PP and Subhashree AR. Role of Absolute Reticulocyte Count in Evaluation of Pancytopenia-A Hospital Based Study. J Clin Diagn Res. 2014 Aug; 8(8): FC01–FC03.
- ↑ Lyon M et al. Approach to the diagnosis and treatment of acute subarachnoid hemorrhage in a patient with sickle cell disease. Am J Emerg Med. 2015 Mar;33(3):481.e3-4.
- ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
- ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
- ↑ Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
- ↑ Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1)
- ↑ Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019.
- ↑ Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4