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UW Health,Ambulatory Education,Educational Opportunities,Grand Rounds,Nursing Grand Round Presentation Video Archive 2012,Clinical Grand Rounds 2012,12/18/2012 - Cultural Congruence,Resources

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Presentation - UW Health, Ambulatory Education, Educational Opportunities, Grand Rounds, Nursing Grand Round Presentation Video Archive 2012, Clinical Grand Rounds 2012, 12/18/2012 - Cultural Congruence, Resources



Clinical Grand Rounds:
Cultural Congruence: Caring for
Patients with Sickle Cell Anemia
and Their Families

Tracey Abitz, MS, RN, CTN-B
Sharon Frierdich, MS, RN, CPNP,CPON
Valerie Mack, BSN, RN, CEN, CPEN
Sara Moldenhauer, MS, RN, AOCNS, CTN-B
Kelly Mommaerts, BA, BSN, RN


Objectives
• Identify different populations that have a genetic predisposition to
sickle cell anemia.

• Discuss the pathophysiology of Sickle Cell Anemia

• Describe the impact on growth and development for a child with
Sickle Cell disease.

• Describe potential stigma associated with sickle cell anemia and
how it could impact quality patient care.

• Examine the lived experience of patients and families living with
Sickle Cell Anemia.

Biocultural Ecology & the
Purnell Model
Sara Moldenhauer, MS, RN, AOCNS, CTN-B



Purnell: Biocultural Ecology
– Skin color and biological variations

– Autosomal Diseases and Health
conditions
– Tay-Sachs disease
– Sickle Cell Disease
– Cystic Fibrosis
– Variations in drug metabolism


Sickle Cell Disease-SCD
• SCD is still negatively impacting the lives of millions of people
worldwide.

• It has been touted as the “Black persons” disease, affecting only
people of African ancestry. We now know that nothing could be
further from the truth.

• SCD is the most commonly occurring genetic disease in the world. It
can and does affect people from:
– Latin America, the Middle East, India, Asia, Italy and Greece. In fact, it has been
proven that SCD can exist in any area of the world where Malaria is, or has
been, present.




Sickle Cell Disease

Sickle Cell Disease
• It is the most common of humanity’s genetic
disorders:
– There is not enough funding for research for treatment or a
cure.
– There is lack of ability to advocate and unite to draw
awareness.
– Minimal advancement in care or cure.
– Lack of a social and political resources to raise awareness
and financial backing.
.

Sickle Cell
Normal red cell
Sickle cells inflexible, can’t do this


Sickle Cell Anemia: Impact on
Growth and Development and
Quality of Life
Sharon Frierdich, MS, RN, CPNP,CPON

Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Influencing Factors
 Severity of the Disease – Organs Affected
 Quality of Health Care: Prevention and Early
Detection and Treatment of Complications
 Support and Care of Family and Community


Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Challenges for
Infants/Toddlers
Pain
Infection
Splenic Sequestration
Anemia



Sickle Cell Anemia: Impact on Growth and
Development and Quality of Life
Strategies
 Newborn Screening (State
Program)
 Referral to a
Comprehensive Sickle Cell
Clinic
 Multidisciplinary Team
 Education/Counseling
 RegularHealth/
Growth/Nutritional
Screening
 Provide Community
Resources


Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Strategies (cont.)

 Prevention and Early Treatment of Infections
 Oral Penicillin Prophylaxis.
 Immunizations (pneumococcal, influenza,
haemophilus, meningococcal etc.)
 Primary stroke prevention – Transcranial Doppler
ultrasonography at age 2.
 Pain and comfort measures.


Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
• Challenges for School-age Children
Physical
Shorter Stature
Decreased Stamina
Infections
Organ Damage
Enuresis/Priaprism
Pain


Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Strategies
– Develop an Preventive and Effective
Palliative Care Program from
Infancy through Adulthood.
– Encourage Good Health Habits and
Compliance to Care Plan:
• Medications: Hydroxyurea,
ExJade etc.
• Exchange Transfusions
• Pain Care Plan.
• Good hydration, avoid cold,
infection control.
• Early reporting of health
concerns.

Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
• Challenges for School-age
Children
Academic
Frequent school absences – fall behind
in class work.
Silent strokes – cognitive/learning
difficulties.
Self Esteem
Decreased stamina/short stature.
Difficulty socializing with peers due to
restrictions, embarrassment, lost
contact due to school absences etc.
Feelings of distrust from health care
team.


Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Strategies
Stroke Prevention Program and MRI screening
Serial Neuropsychological Testing
 Annual Individual Educational Program (IEP)
Constant Communication between
Family/School/Health Care Team (Healthy
Learner Model)

Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Strategies
 Throughout childhood teach about
sickle cell disease at their
developmental level.
 Teach them early to describe their
pain using tools and “Then believe
them!”
 Praise compliance to plan of care.
 Assist child to succeed in a subject,
sport, or develop a special talent!”
 Introduce role models with sickle
cell anemia.
 Encourage them to participate in
camps and social activities with
peers (with and without sickle cell).

Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Challenges for
Adolescents and
Young Adults
Developing a realistic
career path
Difficulty obtaining
employment
Depression
Sexual Concerns

Sickle Cell Anemia: Impact on Growth
and Development and Quality of Life
Strategies
 Realistic Vocational
Counseling and Support
 Genetic Counseling
 Transition Plan to Adult
Care
 Practice self-advocacy
techniques, call
insurance, pharmacy,
make appointments, talk
with provider alone etc.
 Written Summary of Health
History
 Transition Vist
 Health Psychology
Counseling


The patient in the ED

Valerie Mack, BSN, RN, CEN, CPEN


Emergency Department
• RN Perceptions-ED
– Demanding behavior
– Underscore the
numerical pain value
of the patient.
– May feel that they are
perceived as “drug
seeking”


Emergency Department
• Presentation-ambulatory vs. ambulance
– chest pain
– abdominal pain
– lower extremity pain with or w/o venous stasis
– neurological changes
– fever-sepsis

• Diagnostic Workup
– related to chief complaint
• Treatment includes
– cause of current crisis
– treating their pain


Pain in ER

Sickle Cell Disease in the
Inpatient setting

Kelly Mommaerts, BA, BSN, RN


Treatment of SCD
• Care Team
– PCP and Attending
– Physician Extender-NP or PA
– Medical Social Worker/Case Manager
– Access to lab services
– Radiology
– 24 hour blood bank



Inpatient Treatment for SCD
• Mainstays of SCD treatment
– O2
– IV fluids
– Pain Meds
– Access to hematology consults
– Access to pain consult service


SCD
• Health Related Stigma assigned by health
care workers.
– It’s a Black person’s disease as well as a poor
person’s disease.
– Medicaid and Medicare-76%
– Private Insurance-24%
– The rate of debility r/t SCD is extremely high.



Inpatient Care Providers Perceptions of
Pts with SCD
• There is greater scope for contextual
influences in the tx of pain than in almost
any other area of medicine, and is
frequently under treated, esp in ethnic
minority pts.

Patient’s perceptions of pain
management in the hospital
• “We need to get MDs and health care
providers to understand that we do
deserve to be triages better than we are.
They always triage us badly…we need to
be moved up the triage process….we are
always the last pts to be seen. Our pain is
every bit as REAL. We got to get the MDs
to understand this thing is REAL.”

Patient & Family Panel
Tracey Abitz, MS, RN, CTN-B
Sharon Frierdich, MS, RN, CPNP,CPON

Resources
• CDC Sickle Cell Disease Resources

• Bonham, V. L. (2001). Race, ethnicity, and pain treatment: Striving to
understand the Causes and Solutions to the Disparities in Pain Treatment.
The Journal of Law, Medicine & Ethics; 29, 1; 52.

• Elander,J., Marczewska, M., Amos, R., Thomas, A., & Tangayi, S. ( 2006).
Factors Affecting Hospital Staff Judgments About Sickle Cell Disease Pain.
Journal of Behavioral Medicine, 29, 2. doi: 10.1007/s10865-005-9042-3.

• Platt, O.S. (1991). Pain in Sickle Cell Disease New England Journal of
Medicine, 325, 11-16.




• Reddin , C., Cerrentano, E., Tanabe, P. (2011). Sickle Cell Disease
Management in the Emergency Department: What every Emergency Nurse
should know. Journal of Emergency Nursing 37:341-5. doi:
10.1016/j.jen.2010.04.014.

• Smith, L. A., Oyeku, S.O., Homer, C, & Zuckerman, B. (2006) Sickle Cell
Disease: A Question of Equity and Quality. Pediatrics, 117; 1763. doi:
10.1542/peds.2005-1611.

• Telfair, J., Myers, J., Drezner, S. (1998) Does Race Influence the Provision of
Care to Persons with Sickle Cell Disease? Perceptions of Multidisciplinary
Providers. Journal of Health Care for the Poor and Underserved 9: 2.

• White Paper describing the issues facing patients and families today in Illinois
http://www.themartincenter.org/images/martincenter_whitepaper.pdf