Sickle Cell Disease Teaching Presentation

Assessment & Vulnerability Protection of Patients with Sickle Cell Disease By Tosin Ola, RN

What is Sickle Cell?
Inherited disease
Genetic mutation against malaria
Defective hemoglobin in red blood cells
Under stress, cells assume inverted C (sickle), rigid shape
This leads to blockage of veins/arteries
Blockage causes excruciating pain

Genetics and the Transmission of SCD

Who Does it Affect?
85,000 people in the United States
4 million people worldwide
Affects mostly those of African descent in the US
Also found in African, Caribbean, Middle Eastern, Indian, Latin American, Native American and Mediterranean heritage

Sickle Cell Variations
All forms of SCD can exhibit the complications associated with the disease (OHSU, 2008).
Sickle cell trait (HbAS)
Sickle cell anemia (HbSS)
Hemoglobin C disease (HbSC)
Hemoglobin E disease
Hemoglobin S-beta thallasemia

Assess Patient For
Anemia
Syncope, dizziness
Shortness of breath
Pallor, cold extremities
Headache, chest pain
Pain
Infections
Jaundice
yellowing in skin, mouth, eyes
Respiratory distress

Complications of SCD
Stroke
Gallstones
Splenic Sequestration
Avascular Necrosis
Leg ulcers
Priapism
Renal failure
Acute chest syndrome
Pulmonary hypertension
Retinal damage
Opioid tolerance, addiction or pseudoaddiction

Hospital Management
Oxygenation
Pain management
Hydration
Blood transfusions
Antibiotics
Bone marrow transplants & gene therapy
Medications given:
NSAIDS
Opioids
Hydroxyurea
Folic acid

Complementary & Alternative Therapies
Herbal remedies
Alternative therapy
Sauna/Heat therapy
Reiki
Acupuncture
Yoga
Massage
Prayer
Meditation
Breathing

Health Disparities in SCD Patients
Structural barriers
Socio-economic
Patient-provider communication problems
Provider discrimination and lack of cultural competency

Vulnerability Assessment
Demographics
Learning method
Medical history
Pain assessment
Financial barriers
Transportation
Sickle cell risk factors
Lifestyle factors
Sex and genetic counseling
Personal safety
Mental health
Please see Appendix A Vulnerability Assessment for SCD Patients

Action Plan
Do a thorough and proper assessment of all SCD patients
Develop an individualized care plan focusing on their vulnerabilities and pain issues
Communicate this plan to help increase their self-care management ability
Develop a caring relationship while focused on enhancing trust, cultural competency and support

Conclusion
Assess vulnerabilities
Manage pain
Avoid stereotyping & bias
Practice cultural competency
Provide compassionate care

References
Dorsey, C. J., & Murdaugh, C. L. (2003). The theory of self care management for vulnerable populations. The Journal of Theory Construction and Testing , 7 (2), 43-49.
Fiaskerud, J. H., Kim, S., Strehlow, A., Tullman, D., Verzemniek, I., & Koniah-Criffin, D. et al. (2002). Health disparities among vulnerable populations: Evolution of knowledge over five decades in Nursing Research publications. Nursing Research, 5 (2), 74-85.
Goldberg, J., Hayes, W., & Huntley, J. (2004, November). Understanding Health Disparities . Retrieved from Health Policy Institute of Ohio: http://healthpolicyohio.org/pdf/healthdisparities.pdf
Healthy People (n.d.). What are the Leading Health Indicators? Retrieved November 30, 2008, from http://www.healthypeople.gov/
Lusher, J., Elander, J., Bevan, D., Telfer, B., & Burton, P. (2006). Analgesic addiction and pseudoaddiction in painful chronic illness. Clinical Journal of Pain , 22 (3), 316-24.
Massers, L. J. (November 18, 2008). Willamette Falls Hospital Merges with Providence Health System. Clackamas Review . Retrieved from http:// clackamasreview.com

References cont.
National Heart, Lung and Blood Institute (2007). Sickle Cell Anemia . Retrieved November 8, 2008, from http://www.nhlbi.nih.gov/health/dci/index.html
Steiner, C., & Miller, J. (2006, December). Sickle cell disease patients in U.S. hospitals, 2004 (Agency for Healthcare Research and Quality). Rockville, MD: HCUP.
Strickland, O. I., Jackson, G., Jilead, M., Mcguire, D. B., Quarles, S., & Douglas, P. et al. (2001). Use of focus groups for pain and quality of life assessment in adults with sickle cell disease. Journal of the National Black Nurses Association , 12 (2), 36-43.
Tanabe, P., & Myers, R. (2007). Emergency department management of acute pain episodes in sickle cell disease. Emergency Medicine , 14 (2), 419-425.
U.S. Department of Health and Human Services (January, 2000). Healthy People 2010: National Health Promotion and Disease Prevention Objectives . Retrieved November 29, 2008, from http://healthpeople2010.gov
Watson, J. (1985). Nursing: The philosophy and science of caring (2nd ed.). Boulder: Colorado Associated Press.
Weissman, D. E., & Haddox, J. D. (1999). Opioid pseudoaddiction--an iatrogenic syndrome. Pain , 36 (3), 363-366.

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Sickle Cell Disease Teaching Presentation

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