Sickle Cell
Diane Bernaldo, RN
Kalani Faustina, RN
Megan Bentley, RN
Morgan James, RN
Zainab Kamara, RN What is Sickle Cell Disease (SCD)? • Inherited and not contagious
• Hemoglobin SS is called sickle
cell anemia- the most common
and most severe kind of SCD
• Hemoglobin SC disease and
hemoglobin Sβ thalassemia
are two common forms of SCD Image curtesy of http://gudhealth.com/sickle-cell-anemia.html • Abnormal hemoglobin called
hemoglobin S or sickle
hemoglobin (National Heart,
Lung, and Blood Institute,
2016). Pathophysiology
• Normal hemoglobin are disc shaped; sickle hemoglobin are
shaped like a crescent, or sickle shape.
• Sickle-shaped cells cause blockage in vessels slowing or
stopping blood flow, decreasing oxygenation of tissues.
• Normal RBC’s live 90-120 days; sickle
cells last only 10-20 days.
• Lack of tissue oxygenation causes pain
and organ damage.
• Life-long illness
• Severity varies from person to person
Image curtesey of http://oncofertility.northwestern.edu/resources/sickle-cellanemia • In 1910- First published literature of sickle cell
• In 1915- Suggested that SCD is an inherited condition
• In 1922- common link between first four recorded casesAfrica
• In 1973- average lifespan was 14 years.
• Life expectancy today is 40-60 years.
• Hematopoietic stem cell transplant (HSCT) in the only cure
• Well-matched donor is needed to have successful transplant
• Effective treatments can reduce symptoms The History of Sickle Cell Disease Impact of Sickle Cell on Health Care
System
Image curtesy of https://www.ubyssey.ca/science/sickle-cell-project2017/ • Health care have seen a rise in
the treatment of Sickle Cell
Patients.
• The cost is higher among adult
patients versus adolescent
patients.
• Many Inpatients are not
economically able to pay for
treatment which causes the
health care system to rely on
government aid and research
grants to help subsidize the
cost. Impact of Sickle Cell on Children
• The effect of Sickle Cell on
Children can cause many
issues and prevent them from
living a regular childhood.
• Increase likelihood of
bacterial infections and can
even experience strokes.
• Pain Crises has the biggest
impact because it can be
brought on by any stress to
the body and turn a good day
to a long painful one. Image curtesy of https://www.youtube.com/watch?v=rDN2_qccS3k Impact of Sickle Cell on Adults Sickle Cell does
not have a cure as
of yet so it will
carry to
adulthood from Patient Receiving Blood Transfusion Face same
symptoms of
children with
increase
experience with Image curtesy of
http://ourweekly.com/news/2014/jul/1
0/partial-transplant-reverses-sicklecell/ Adult feet with Sickle
Image curtesy
of https://mshealth.wikispaces.com/EFFECTSCell
SICKLE+CELL+ANEMIA+BY+ROGER Psychological
issues develop
from living with
the disease since
childhood. Sickle Cell Statistics
• SCD affects approximately 100,000 Hand of Infant with Sickle
Cell
Americans.
• SCD occurs among about 1 out of
every 365 Black or African-American
births.
• SCD occurs among about 1 out of
every 16,300 Hispanic-American
births.
• About 1 in 13 Black or AfricanAmerican babies is born with sickle
Image curtesy of http://gotstemcells.blogspot.com/2013/07/get-informed-sickle-celldisease.html Mental and Emotional Impact on Adults
• Lower age of
mortality
• Fear of transfer to
offspring
• Fear of impact on
love ones
• Fear of onset of pain
crisis Image curtesy of https://g.co/kgs/DZgw7G Physical Effects & Sexual Effects
Physical
• Anemia
• Pain
• Stroke
• Blindness
• Leg Ulcers
• Organ Damage
• Acute Chest Syndrome
• Gallstones
• Pulmonary Hypertension Sexual
• Delayed Sexual Maturation
• Sex Hormone Disturbances
• Priapism Prenatal Care and Childbearing
• Increase in Sickle Cell
Crisis
• Pre-term Labor
• Low Birth Weight
• Genetic Testing
• Recessive Gene Substance Abuse vs Pseudoaddiction
• What may seem like a substance abuse issue may
actually be pseudoaddiction for those who suffer with
sickle cell disease.
• Pseudoaddicion is a condition resembling drug
addiction but caused by under prescription of drugs to
treat pain in the patient, causing them to seek more. Economical Effects
• Health Care Cost (Undiscounted)
• 30 to 39 years old
• $2,853.00 monthly per patient
• $34,266.00 annually per patient
• 45+ years old
• $953,640.00 annually per patient Occupational Considerations and
Hazards
& Ability to cope with Stress
Occupational
Considerations and
Hazards
• Avoid situations that can
trigger a crisis
• Abrupt changes in
temperature
• Extreme heat or cold • Low energy, easily tired Ability to Cope With
Stress
• Sickle Cell Disease can
be stressful
• Pain
• Decreased energy levels
• Fear of the future Care Plan
Expected Outcomes
• Patient will verbalize understanding of
sickle cell disease, prevention of crisis and
appropriate treatment
• Patient identifies appropriate resources
• Patient verbalizes intention to follow
prescribed regimen
• Patient demonstrates ongoing adherence to
treatment plan Care Plan (cont)
Health Screening
• Assess patient for crisis
episode
• Lab work
• Assess factors that
might negatively affect
success in following
prescribed regimen Health Promotion
• Infection
• Emotional stress
• Smoking and alcohol
ingestion
• Cold exposure
• Extreme fatigue
• High altitudes Care Plan (cont)
Health
Interventions
• Take prescribed
medications
• Drink 4 to 6
liters of fluid
daily
• Keep follow up
appointments Health
Education • Genetic counseling
needed when
planning a family
• Refer to support Sickle Cell Anemia Resources
The American Sickle Cell Anemia Association
http://www.ascaa.org/index.php Sickle Cell Association of Houston
http://sicklecellhouston.comcastbiz.net/ U.S. Department of Health and Human ServicesNational Heart, Lung and Blood Institute
https://www.nhlbi.nih.gov/health/health-topics/topics/sca References
• American Journal of Hematology. (2009, July 03). Sickle cell disease healthcare
costs high in U.S. Retrieved April 26, 2017, from http://www.reuters.com/article/ussickle-cell-idUSTRE5623EL20090703
• Center for Disease Control. (August 31, 2016). Sickle Cell Disease. Data &
Statistics. Retrieved April 23, 2017. https://www.cdc.gov/ncbddd/sicklecell/data.html
• Centers for Disease Control and Prevention. (2015, September 14). Sickle Cell
Disease and Pregnancy. Retrieved April 26, 2017, from
https://www.cdc.gov/ncbddd/sicklecell/pregnancy.html
• Duffy, T. (2014, July 7). Sexual Experiences in Men with Sickle Cell Disease – a
Phenomenological Enquiry. Retrieved April 26, 2017, from
http://www.sicklecellsociety.org/resources/sexual-experiences-in-men-with-sicklecell-disease-a-phenomenological-enquiry/
• Elander, J., Lusher, J., Bevan, D., Telfer, P., & Burton, B. (2004). Understanding the
causes of problematic pain management in sickle cell disease: evidence that
pseudoaddiction plays a more important role than genuine analgesic
dependence. Journal Of Pain & Symptom Management, 27(2), 156-169. References (cont)
• Gil KM, Wilson JJ, Edens JL. (1997). The stability of pain coping strategies in
young children adolescents, and adults with sickle cell disease over an 18-month
period. Clin J Pain. ;13:110–115. doi: 10.1097/00002508-199706000-00005.
• Gulanick, M., & Myers, J. L. (2011). Nursing care plans: diagnoses, interventions,
and outcomes (7th ed.). pgs. 777-779. St. Louis, MO: Elsevier Mosby.
• Images retrieved from https://www.google.com/search?
q=sickle+cell+disease&source=lnms&tbm=isch&sa=X&ved=0ahUKEwiksOm53
MjTAhUBRGMKHS2bCH8Q_AUICygC&biw=1366&bih=638
• Kanter, J., & Kruse-Jarres, R. (2013). Review: Management of sickle cell disease
from childhood through adulthood. Blood Reviews, 27279-287.
doi:10.1016/j.blre.2013.09.001
• Mayo Clinic. (2016, December 29). Sickle Cell Anemia. Retrieved April 26, 2017,
from http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptomscauses/dxc-20303269 References (cont)
• Miller, R. (2015). Sickle Cell Diesease. Retrieved April 23, 2017.
http://kidshealth.org/en/parents/sickle-cell-anemia.html#
• National Heart, Lung, and Blood Institute. (2016). What is sickle cell disease?
Retrieved from https://www.nhlbi.nih.gov/health/health-topics/topics/sca
• Rajinder P., Hasan, S., Castro, O., Perlin, E., Kim, K. . (2003) Long-Term
Outcomes in Patients with Sickle Cell Disease and Frequent Vaso-Occlusive
Crises. The American Journal of the Medical Sciences 325:3, 107-109.
• Rodgers, G., Walker, E,. Podgor, M. J., (1994). Mortality in Sickle Cell Disease.
New England Journal of Medicine. http://www.nejm.org/toc/nejm/331/15/
• Sickle cell disease healthcare costs high in U.S. (June, 2009). American
Journal of Hematology. Retrieved April 21, 2017
• Smith, Y. (2015). Sickle cell history. News medical. Retrieved from
http://www.news-medical.net/health/Sickle-Cell-Disease-History.aspx

 

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