1. Vaso-Occlusive Crisis
By: Vicky Borger

2. Diagnosis
Sickle cell anemia is an inherited autosomal recessive disease that is predominantly found in African Americans in the United States.
Sickle cell disease is part of prenatal and neonatal screenings.
Formerly known as sickle cell crisis, vaso- occlusive crisis (VOC) is an episode of acute pain experienced by someone diagnosed with sickle cell anemia.
Acute pain from VOC is the most common reason that a sickle cell patient seeks medical attention.
Sickle cell anemia may be the result of a genetic mutation that occurred in malaria-prone regions found near the equator thousands of years ago. People with sickle cell may have been more likely to survive malaria epidemics, and because they survived, it was passed down through generations.
(DeBaun & Vichinsky, 2016), (McCance & Heuther, 2014)

3. Pathogenesis
Acute pain in patients with sickle cell disease is caused by ischemic tissue injury resulting from the occlusion of microvascular beds by sickled erythrocytes during an acute crisis.
Sickled cells are inflexible, sticky, and unable to pass through blood vessels easily
Certain triggers precipitate sickling of the hemoglobin, but a cause is not always known.
(National Heart, Lung, and Blood Institute, 2015)

4. Triggers for VOC Dehydration Changes in elevation Cold temperatures
Physical stress
Emotional stress
Alcohol
Infection
Pregnancy
Children with sickle cell anemia are very prone to infection since their spleen is often compromised by the disease. They should receive the pneumonia vaccine and yearly flu vaccine. It is also recommended that children take daily penicillin until the age of 5 to prevent infections.
Educate patients on importance of avoiding triggers and staying hydrated

5. History Pain is the #1 symptom.
Pain usually occurs in long bones, abdomen, or chest.
Infants and toddlers may present with dactylitis (acute pain and swelling in the hands and/or feet)
Assess pain using OLDCARTS
Detailed medication history, including what they are currently taking and what has worked in the past for VOC
Manifestations of previous VOCs
Exposure to triggers
Fever/chills or any other signs/symptoms of infection
Sick Contacts
Assess for depression, apathy, despair related to chronic pain and illness
(DeBaun & Vichinsky, 2016), (Vichinsky, 2016).

6. Physical Exam VOC has no specific physical exam findings
Assess the site of the pain to rule out any other causes of the pain
VOCs are sometimes accompanied by fever, swelling, tenderness, tachypnea, nausea, hypertension, or vomiting

7. Diagnostic Tests
There is no diagnostic test to confirm vaso-occlusive crisis
Gold standard is assessment of pain
Is the pain typical or atypical of previous VOCs
If atypical, rule out other causes of possible pain and treat possible triggers
Studies have demonstrated that providers misperceptions of the patient’s pain leads to inadequate pain control. There is no test to diagnose a crisis and the gold standard for assessment remains the patient’s or family’s self report of the pain.
Blood smear shows sickled cells, but the amount of sickled cells has no correlation with severity of VOC.
(DeBaun & Vichinsky, 2016), (Vichinsky, 2016).

8. Differential Diagnosis
Septic Arthritis- painful, red, swollen joint wit signs or symptoms of infection
Splenic Sequestrian-
Acute Chest Syndrome- chest pain, respiratory distress, new infiltrate on xray, and fever
Stroke- neurological deficits, headache
Multi-Organ Failure- Often occurs during VOC, important to rule out
Septic Arthritis: could result in joint destruction and/or sepsis
Splenic Sequestrian: red blood cells get caught in spleen and cause enlargement and pain
Acute Chest Syndrome: occlusion of blood vessels in lungs. Appears as new infiltrate on xray accompanied by new symptoms of fever, chest pain, or respiratory distress. Usually happens during VOC.
Multi-Organ Failure: Most often occurs during VOC, so important to rule out during VOC
Stroke needs to be ruled out if there are any neurological deficits appreciated during physical exam
(Vichinsky, 2016)

9. Treatment
Pain Management (non-opioid, oral opioids, parenteral opioids)
Medicate for side effects from opioids: pruritus, nausea, constipation, and insomnia
Massage
Heat Therapy
Do Not Use: Cold therapy
Stem Cell Transplant (used in children without organ damage, currently investigational in adults)
(DeBaun & Vichinsky, 2016), (Papakis & McPhee, 2016).

10. Flowsheet for patient presenting to the ED with VOC, reinforces need for quick pain relief while at the same time ruling out other potential causes
(DeBaun & Vichinsky, 2016)

11. Prevention Individualized home care plan Hydroxyurea
Know triggers and avoid
Article Critique- Omega-3 Supplementation?
Hydroxyurea reduces acute painful episodes, takes up to 3 months to take effect. Antineoplastic drug, monitor for myelosuppression.
Care plan should be tailored to the individual and instruct them on how to appropriately manage their pain and when to contact their provider, can help to reduce hospitalizations, length of stay, and readmission rates
The article I critiqued looked at the benefit of omega 3 supplements in patients with sickle cell. It was a small study with only 128 participants, but it demonstrated that patients who took the supplements compared to the placebo group experienced less VOC and missed less days of school during a year’s time. Further research is needed to confirm this as an effective, inexpensive treatment for the disease.
(DeBaun & Vichinsky, 2016), (Daak et al., 2013)

12. Referrals Sickle Cell Specialist/Hematologist Psychosocial Support
Pain Management
Genetic Counseling

13. Outcomes VOC usually last hours to 7 days.
If pain lasts longer than 7 days, another cause of the pain should be investigated.
Gently wean opioid medications.
Life expectancy is years old.
(Papadakis & McPhee, 2016)

14. Questions?

15. References
Daak, A. A., Ghebremeskel, K., Hassan, Z., Attallah, B., Azan, H. H., Elbashir, M. I., Crawford, M. (2013). Effect of omega-3 fatty acid supplementation in patients with sickle cell anemia: Randomized, double-blind, placebo-controlled trial. The American Journal of Clinical Nutrition, 97,
DeBaun, M. R., & Vichinsky, E. P. (2016). Vasoocclusive pain management in sickle cell disease. UpToDate. Retrieved from disease?source=search_result&search=vasoocclusive+crisis&selectedTitle=3~85
McCance, K. L., & Huether, S. E. (2014). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Elsevier.
National Heart, Lung, and Blood Institute (2014). Evidence-based management of sickle cell disease. Retrieved from
Papadakis, M. A., & McPhee, S. J. (2016). Current medical diagnosis and treatment (55th ed.). USA: McGraw- Hill Education.
Vichinsky, E. P. (2016). Overview of the clinical manifestations of sickle cell disease. UpToDate. Retrieved from disease?source=search_result&search=vasoocclusive+crisis&selectedTitle=1~69