Sickle Cell Complications: Management in Pediatric Patients
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Managing Acute Complications of Sickle Cell Disease in Pediatric Patients (Pharmacology CME)

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Table of Contents
 
Table of Contents
  1. Abstract
  2. Case Presentations
  3. Introduction And Epidemiology
  4. Critical Appraisal of the Literature
  5. Etiology And Pathophysiology
  6. Differential Diagnosis
    1. Vaso-Occlusive Crisis
    2. Acute Chest Syndrome
    3. Febrile Illness
    4. Splenic Sequestration
    5. Stroke
  7. Prehospital Care
  8. Emergency Department Evaluation
    1. History And Physical Examination
      1. Vaso-Occlusive Crisis
      2. Acute Chest Syndrome
      3. Febrile Illness
      4. Splenic Sequestration
      5. Stroke
      6. Priapism
  9. Diagnostic Studies
    1. Vaso-Occlusive Crisis
    2. Acute Chest Syndrome
    3. Febrile Illness
    4. Splenic Sequestration
    5. Stroke
    6. Priapism
  10. Management
    1. Vaso-Occlusive Crisis
      1. Analgesia
      2. Hydration
      3. Nonpharmacologic Therapies
    2. Acute Chest Syndrome
      1. Supplemental Oxygen
      2. Blood Transfusions
      3. Corticosteroids
    3. Fever In Sickle Cell Disease
    4. Splenic Sequestration
    5. Stroke
    6. Priapism
      1. Aspiration Of Corpora Cavernosa
  11. Special Populations And Circumstances
    1. Patients With Sickle Cell Trait
      1. Exertional Collapse Associated With Sickle Cell Trait
      2. Hematuria
      3. Traumatic Hyphema
    2. Pregnancy And Sickle Cell Disease
    3. Unvaccinated Children
    4. Infections With Human Parvovirus B19
    5. High Emergency Department Utilizers With Sickle Cell Disease Pain
  12. Controversies And Cutting Edge
    1. Magnesium Sulfate Use In Vaso-Occlusive Crisis
    2. Corticosteroid Use In Vaso-Occlusive Crisis
    3. Nitric Oxide Use In Vaso-Occlusive Crisis
    4. Ketamine Use In Vaso-Occlusive Crisis
  13. Disposition
  14. Summary
  15. Risk Management Pitfalls In Pediatric Patients With Sickle Cell Disease
  16. Time- And Cost-Effective Strategies
  17. Case Conclusions
  18. Clinical Pathway For Pain Management In Vaso-Occlusive Crisis In Children With Sickle Cell Disease
  19. Tables And Figures
    1. Table 1. Common Genotypes Of Sickle Cell Hemoglobinopathies And Their Characteristics
    2. Table 2. Differential Diagnosis Of Acute Complications In Sickle Cell Disease
    3. Table 3. Management Of Acute Complications Of Sickle Cell Disease
    4. Figure 1. Oxygen Dissociation Curve Of Hemoglobin A Versus Hemoglobin S
    5. Figure 2. X-Ray Of Right-Sided Infiltrate In A Child With Acute Chest Syndrome
    6. Figure 3. Magnetic Resonance Imaging Scan Of Axial Fluid Attenuation Inversion Recovery
  20. References

Abstract

Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management. Patients with sickle cell trait generally have a benign course, but are also subject to serious complications. This issue provides a current review of evidence-based management of the most common acute complications of sickle cell disease seen in pediatric patients in the emergency department.

Case Presentations

An 18-year-old adolescent girl with sickle cell disease presents to the ED stating she has severe bilateral thigh and shoulder pain. On examination, she has normal vital signs and appears comfortable lying on her gurney. She reports mild pain when her shoulder and thighs are palpated. A nurse comments that she had also presented 2 days ago, and is possibly seeking drugs. How do you decide whether this is drug-seeking behavior or pain from vaso-occlusive crisis? What pain medication can you offer her? How do you decide whether she will need to be admitted for pain control?

A 12-year-old boy with sickle cell disease is brought in by EMS because he is having trouble breathing. He is coughing and complains of right-sided chest pain. On examination, he appears to be in severe respiratory distress. His vital signs are: respiratory rate, 50 breaths/ min; oxygen saturation, 89% on room air; temperature, 37°C (98.6°F); heart rate, 110 beats/min; and blood pressure, 110/70 mm Hg. On auscultation of the lungs, you hear crackles bilaterally. A chest x-ray shows a right-sided infiltrate. The resident is placing him on a nonrebreather mask. What is the diagnosis of this patient? What treatment should be started? Is there any role for a blood transfusion?

A 4-year-old boy with sickle cell disease is brought in by his father because the child developed a fever today. Prior to fever onset, the child was in school, and was his usual, active self. On examination, he has a fever of 38.9°C (102°F), but all other vital signs are normal. He appears well and is playful for the rest of the examination. Should you obtain any laboratory studies? Does he need antibiotics?

An 18-month-old toddler with sickle cell disease is brought in by his mother because he looks pale and tired. His vital signs are: respiratory rate, 30 breaths/min; oxygen saturation, 97% on room air; temperature, 37°C (98.6°F); heart rate, 142 beats/ min; and blood pressure, 88/60 mm Hg. He appears alert and comfortable. You note a large spleen on examination. What steps should you take next? Does this patient need to be admitted or can he be discharged home if all tests are normal?

Introduction And Epidemiology

Sickle cell disease (SCD) refers to a family of genetic blood disorders caused by the sickling of red blood cells secondary to atypical hemoglobin molecules, known as hemoglobin S (HbS). This disease is seen primarily in individuals of African, Indian, Mediterranean, or Saudi Arabian ancestry.1 Annually, 200,000 children are born with SCD worldwide. Ninety percent of these births are in sub-Saharan Africa. In the United States, 1 in 2474 births are children with SCD. The national prevalence is between 70,000 to 140,000 patients, mostly in those of African ancestry.2

SCD has wide-ranging implications to human physiology. Newborns inherit the autosomal recessive genes from their biological parents. The pathophysiological processes that alter the properties of the red blood cell begin in infancy, with effects seen through adulthood. The following common acute complications (listed in order or prevalence) will be discussed:

  • Vaso-occlusive crisis (VOC)3
  • Acute chest syndrome (ACS)3,4
  • Priapism5
  • Splenic sequestration6,7
  • Stroke8
  • Bacteremia9

Emergency clinicians are first-line providers in the care of patients with acute SCD complications. As such, an understanding of the pathophysiology, clinical features, and management is essential to providing high-quality care. This issue of Pediatric Emergency Medicine Practice provides an overview of SCD and recommendations for the management of some of the most common complications seen in pediatric patients with SCD.

Critical Appraisal Of The Literature

A literature search was performed in PubMed using the terms sickle cell, pain, vaso-occlusion, hemolysis, fever, sepsis, wheeze, acute chest syndrome, stroke, splenic sequestration, transient red cell aplasia, and priapism. Over 500 articles from 1961 to 2015 were reviewed. The Cochrane Database of Systematic Reviews was searched using the key words sickle cell; 52 studies were reviewed. The American College of Emergency Physicians (ACEP) and American Society of Hematology (ASH) websites were reviewed; neither have issued SCD policy statements or guidelines. The American Academy of Pediatrics (AAP) Section on Hematology/Oncology and Committee on Genetics issued a policy statement on health supervision for children with SCD that was reaffirmed in 2006 and 2011.10 The June 2014 publication by the National Institutes of Health National Heart, Lung, and Blood Institute (NHLBI) expert panel report on SCD was reviewed.11 The AAP endorsed this report in November 2014. The comprehensive report included consensus statements and evidence-based guidelines. Using a modified Grading of Recommendation, Assessment, Development, and Evaluation (GRADE) standard evidence scale, there was only 1 high-quality, strongly recommended finding for management of acute SCD complications.12 All other recommendations used moderate- to low-quality evidence. Between 1995 and 2010, only 12 randomized controlled trials were conducted for acute complications of SCD in children.13 Reasons for this lack of high-quality evidence for management of acute complications are likely due to the rarity of the genetic disorder, poor funding for research, and the discovery of successful breakthrough health main-tenance interventions (ie, immunizations, penicillin prophylaxis, regular blood transfusions, and hydroxyurea use).14,15

Risk Management Pitfalls In Pediatric Patients With Sickle Cell Disease

  1. “The patient says she is in pain, but her heart rate, respiratory rate, and blood pressure are normal. Is she malingering?”
    Patients with SCD who state that they are in pain need to be treated compassionately and with urgency to alleviate their pain. The majority of patients with VOC have normal vital signs even when severe pain is reported.
     
  2. “This SCD patient complaining of right knee pain must be having a VOC episode even though it is not his usual site for VOC pain.”
    Although patients with SCD experience VOC more commonly than other complications, there is a broad differential to consider. Extremity pain can be due to many reasons, including fracture, osteomyelitis, septic arthritis, or sports injuries.
     
  3. “I want to give a fluid bolus to this patient who is experiencing his usual VOC.”
    Fluid boluses are not recommended for VOC treatment unless the patient is volume-deficient. Oral fluids should be encouraged. If the patient is not able to tolerate oral intake, then intravenous fluids should be started at the maintenance rate.
     
  4. “He looks so well, this cough and fever must be from a viral upper respiratory tract infection.”
    Patients with SCD presenting with cough and fever need to be evaluated for ACS and should receive imaging of the chest. A new infiltrate on CXR in combination with features such as cough or fever meet the definition of ACS.
     
  5. “The headache of this SCD patient with no prior transfusions is probably a common tension headache.”
    Remember that children with SCD are at risk for stroke. Those who meet the criteria for being at high risk for stroke may be on regular transfusions to prevent this. Headaches are a feature of stroke and require detailed evaluation.
     
  6. “We should transfuse this patient with priapism since it has lasted more than 4 hours.”
    There is no role for transfusion in priapism. Past the 4-hour time point, priapism causes irreversible erectile tissue damage. After consulting urology, emergency clinicians should begin aspiration and irrigation to salvage erectile tissue.
     
  7. “There is no place for starting this patient on incentive spirometry because he is admitted for VOC, not ACS”
    Incentive spirometry should be started on patients with SCD being admitted for VOC management, as it prevents the development of ACS during the patient’s inpatient stay.
     
  8. “This 19-year-old HbSC patient has left-sided abdominal pain. It cannot be splenic sequestration.”
    In HbSC, splenic tissue is present in sufficient quantity that splenic sequestration is a possibility. Adolescents and adults can experience sickle cell sequestration and it should be included in the differential when managing these SCD genotypes.
     
  9. “Since this patient has sickle cell trait, he has no problems to worry about.”
    Individuals who carry 1 gene that codes for HbS are at risk for several medical problems, including renal medullary carcinoma, renal papillary necrosis, splenic infarcts, and exercise-related deaths.
     
  10. “He’s of Indian origin, so he does not have SCD.”
    The sickle cell genotype originated from areas endemic for Plasmodium falciparum malaria. This means people of Indian, African, Middle Eastern, and Mediterranean descent could be carriers or be homozygous for the gene.

Tables And Figures

Table 1. Common Genotypes Of Sickle Cell Hemoglobinopathies And Their Characteristics

References

Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study is included in bold type following the references, where available. The most informative references cited in this paper, as determined by the authors, are noted by an asterisk (*) next to the number of the reference.

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  69. Dunlop RJ, Bennett KC. Pain management for sickle cell disease. Cochrane Database Syst Rev. 2006(2):CD003350. (Systematic review)
  70. * Jacobson SJ, Kopecky EA, Joshi P, et al. Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet. 1997;350(9088):1358-1361. (Double-blinded randomized controlled trial; 56 patients)
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  73. Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database Syst Rev. 2007(2):CD005406. (Systematic review)
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  75. Uman LS, Birnie KA, Noel M, et al. Psychological interventions for needle-related procedural pain and distress in children and adolescents. Cochrane Database Syst Rev. 2013;10:CD005179. (Systematic review)
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