Transcript Sickle Cell Crisis

Vaso-Occlusive Crisis
By: Vicky Borger
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, vasoocclusive 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.
(DeBaun & Vichinsky, 2016), (McCance & Heuther, 2014)
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)
Triggers for VOC
 Dehydration
 Changes in elevation
 Cold temperatures
 Physical stress
 Emotional stress
 Alcohol
 Infection
 Pregnancy
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).
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
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
(DeBaun & Vichinsky, 2016), (Vichinsky, 2016).
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
(Vichinsky, 2016)
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).
(DeBaun & Vichinsky, 2016)
Prevention
 Individualized home care plan
 Hydroxyurea
 Know triggers and avoid
 Article Critique- Omega-3 Supplementation?
(DeBaun & Vichinsky, 2016), (Daak et al., 2013)
Referrals
 Sickle Cell Specialist/Hematologist
 Psychosocial Support
 Pain Management
 Genetic Counseling
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 40-50 years old.
(Papadakis & McPhee, 2016)
Questions?
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, 37-44.
 DeBaun, M. R., & Vichinsky, E. P. (2016). Vasoocclusive pain management in sickle cell disease. UpToDate.
Retrieved from http://www.uptodate.com/contents/vasoocclusive-pain-management-in-sickle-celldisease?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 https://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines
 Papadakis, M. A., & McPhee, S. J. (2016). Current medical diagnosis and treatment (55th ed.). USA: McGrawHill Education.
 Vichinsky, E. P. (2016). Overview of the clinical manifestations of sickle cell disease. UpToDate. Retrieved from
http://www.uptodate.com/contents/overview-of-the-clinical-manifestations-of-sickle-celldisease?source=search_result&search=vasoocclusive+crisis&selectedTitle=1~69