used with permission -...

Running head: SICKLE CELL DISEASE IN AFRICAN AMERICANS 1

Sickle Cell Disease in African Americans: A Discharge Teaching Research Paper

Danielle Crich

National American University

NS4000D Transcultural Nursing

February 4, 2018

Used with Permission

SICKLE CELL DISEASE IN AFRICAN AMERICANS 2

Abstract

The understanding of culture, as it relates to African Americans and sickle cell disease, has many

variables. Trying to understand the impact that this genetically acquired disease has on a certain

population cannot be done without first understanding what culture means to the afflicted group.

This understanding is influential in being able to provide culturally competent discharge teaching.

Avoiding ethnocentrism, in discharge teaching, by focusing on the clients’ values and not ones’

own values, will create a foundation for learning and culturally competent nursing care and

teaching can take place. The ideas and decisions of cultural care allow the client and nurse to

work together to comprise a mutually agreed upon discharge plan. Incorporating the culture,

values, and beliefs in this plan will ultimately lead to the development of a strong nurse-patient

relationship, a stepping stone to culturally congruent care and a decrease in rehospitalizations in

African Americans with sickle cell disease.


Sickle Cell Disease in African Americans:

A Discharge Teaching Research Paper

The understanding of culture, as it relates to African Americans and sickle cell disease,

has many variables. Trying to understand the impact that this genetically acquired disease has on

African Americans cannot be done without first understanding what culture means to the

afflicted group. This paper will discuss cultural influences regarding the African American

community, the risk they face with sickle cell disease, culturally influenced discharge teaching

and avoidance of ethnocentrism. The African American culture emphasizes a great deal on their

religious and spiritual values. Unfortunately, this population has an increasingly large incidence

of sickle cell trait inheritance, with one in twelve African Americans being carriers (National

Human Genome Research Institute, 2016). This disease places those affected with sickle cell

disease in the African American population at a higher risk of mortality because of complications

leading to hospitalization, infections, or even death. An understanding of the African American

culture, and how it relates to their care with sickle cell disease, is influential in being able to

provide culturally competent discharge teaching.

Background

Sickle cell disease, inherited from both parents, produces abnormal red blood cells. These

red blood cells contain hemoglobin S, as opposed to the normal hemoglobin A, in non-afflicted

individuals (Appendix A). The problem arises when the hemoglobin S loses its oxygen, resulting

in a distorted shape, resembling a crescent or sickling appearance (National Heart, Lung, and

Blood Institute, 2012). When red blood cells sickle, while traveling through blood vessels, they

can clump or cause aggregation. This causes blood flow obstruction and leads to excruciating

pain, organ and joint damage in the afflicted individual know as sickle cell crisis (Center for


Disease Control and Prevention, 2017). The Center for Disease Control and Prevention goes on

to state that sickle cell crisis, and corresponding pain, is the number one reason individuals with

sickle cell disease go to the emergency room. It is impossible to determine the number one cause

of sickle cell crisis, so focus on specific triggers, including dehydration, infection, injury, cold

exposure, emotional stress, or strenuous exercise, as an integral part of discharge teaching, need

to be addressed. This will help in the proper management of sickle cell disease and prevent

hospitalization from sickle cell crisis.

Cultural Assessment of African Americans

Utilizing a cultural assessment tool allows care to be centered around a person’s culture,

to better assess the specific health needs of the individual (Boyle & Baird, 2016). According to

the National Institutes of Health’s Office of Communications and Public Liaison (NIH OCPL,

2017), cultural respect is important in reducing health disparities and enhancing access to care

that respects and responds to the needs of culturally diverse individuals. The NIH OCPL further

explains that positive health outcomes are supported and benefit the individual, community and

health care organization. Cultural assessment, with regard to African Americans, is an important

tool, because of health care professional suspicion. This suspicion tends to delay treatment and

care, often leading African Americans to choose home remedies to treat their specific conditions

(Campinha-Bacote, 2009). Cultural assessment allows nurses to truly know the client, to respect

their values, beliefs and customs and to treat them with compassion by suspending judgement

and bias.

Family and kinship systems

When one thinks about family, a sense of belonging, safety, love and caring are felt.

Hecht, Jackson and Ribeau (2003) state that family, to the African American culture, is the


principle and paramount tradition to one’s culture. When looking at African American family

structure, a primarily matriarchal relationship is manifested. African American mothers feel a

responsibility to serve others, portray a sense of strength, withhold emotion and emanate a sense

of independence regarding success (Abrams, 2012). Abrams further states that African American

women are pressured to be strong and preserve unity in the family and community dynamic.

Edwards (2013) adds that this belief system helps to classify the African American family into

an extended family unit which incorporates both family and community.

African Americans prefer to be addressed formally and by their surname, because of the

pride they have for their family name (Campinha-Bacote, 2009). Referring to these clients with

Mr., Mrs., Ms., or Miss., demonstrates respect for the African American culture. Camponha-

Bacote (2009) continues by stating that non-verbal communication is used continuously during

conversation, and that African Americans level of volume is higher that other cultures. It is

important to not misunderstand this increased volume with a negative attitude while

communicating discharge teaching to these clients.

Social life and networks

Education to the African American community is an important part of life, with an

emphasis on equality and opportunity for social mobility and economic independence (Anderson,

2016). However, Anderson continues by stating that there are breaks in the African American

educational system which result in inadequate and insufficient learning opportunities for students.

Anderson’s study provides statistics showing 96 percent of African American families feel a

sense of disappointment with the educational system. This disappointment in the educational

system provides a barrier to optimal health care and contributes to low health literacy affecting

about forty-percent of African Americans (Campinha-Bacote, 2009).


Religious beliefs and practices

As humans, reliance on cultural influences helps to guide and mold one throughout their

growth and development. African Americans are statistically more active in their religion than

any other race in the US. For example, of all Christians in the US, 53 percent are African

American protestants, and 83 percent of African Americans believe in God (Pew Research

Center, 2018). A large emphasis is placed on their religious culture as being the focal point in

one’s life. This was brought about secondary to the experiences and hardships they endured

while under slavery.

Spirituality issues, according to Adegbola (2011), including hopelessness, negative self-

talk, and pessimism, often affect African American adults with sickle cell disease. The author

continues by stating African Americans feel that little can be done to change the course of sickle

cell disease, which is the cause of much despair. This despair is important to address during

discharge of patients with sickle cell disease. When patients are hospitalized, specific guidelines

and follow-up advice must be followed to prevent rehospitalization and further complications of

sickle cell disease. Cotton (2012) reports that adults with sickle cell disease who use positive

religious coping, like prayer, report a significant decrease in the amount of hospital admissions.

There was also a noteworthy decline in pain scores when African American attended church on a

regular basis.

African American churches serve as central institutions in the community and spiritual

aspects of these Christian lives (Taylor, Chatters and Brown, 2014). The church congregation

serves as an unwavering support system with many resources. According to Ehrmin (2016),

religion is viewed as the heart of the African American culture and is utilized to help achieve and

maintain optimal health. Individuals engaging in religious activities enhance their social support


systems and networks through increased exposure to church members. This in turn helps to

develop coping mechanisms through spirituality and religious bonding (Le, Holt, Hosack, Huang,

& Clark, 2015).

Health beliefs and practices

Health, to African Americans, is a road endured with God in one hand and medicine in

the other. Both entities must be present for true spiritual, mental and physical health to be

maintained. Health can be influenced through fellowship, trusting relationships, constructive

criticism and an exchange of emotional and physical support (Le et al., 2015). The African

American church helps to understand health as being a complete balance between physical,

mental, and spiritual well-being, making health promotion through church activities ideal

(Collins, 2015). African Americans view health care in a spiritual manner, and when it comes to

pain, like in sickle cell disease, they believe that evil forces or punishment from God are

responsible and require repentance (Campinha-Bacote, 2012).

Long et al., (2011) describe common health disparities and misconceptions among the

African American community as it relates to sickle cell disease. Some of these themes include

limited prominence of sickle cell disease and trait health awareness, stigma associated with

sickle cell disease in the African American community, and a desire for compassionate and

culturally competent care from health professionals.

Research shows that African Americans number one restaurant choices usually fall in the

fast food category with a twenty-nine percent statistic, second being sit-down restaurants at

twenty-sex percent and third being soul food establishments at twenty-two percent (Mandal,

2013). A study done by Savoca (2011), on food choices of young adult African American males,

shows an “eat and run” approach to meal planning leading to increase intake of sugar and fat,


and poor intake of healthy foods. These participants had an extremely high intake of fast food

meals and infrequently cited eating healthy meals containing whole grains, nuts, seeds, or

legumes. Smoking is shown to be higher in the African American culture as opposed to any other

population and accounts for 45,000 deaths per year from smoking related diseases (Mandal,

2013). A study done by Cohen et al., (2010) indicates that active and passive cigarette smoke

exposure increase vascular inflammation, platelet aggregation and expression of endothelial

adhesion molecules that increase risk for sickle cell crisis.

Discharge Teaching for Sickle Cell Disease

Nurses can provide culturally competent care in hospital settings and community settings

regarding education and discharge teaching to help reduce risks associated with this population

(Boyle et al., 2016). Discharge teaching, for African Americans, should encompass the

community cultural assessment referenced earlier in this paper. Also, it helps to establish a

working nurse-patient relationship for mutually agreed upon discharge teaching plans and

instructions. Appendix B provides a detailed brochure, from the Indiana Hemophilia and

Thrombosis Center (2015), for managing sickle cell disease at home. Appendix G demonstrates a

sample discharge summary for a 24 y/o male with a hospital admission for sickle cell crisis and

dehydration.

Managing sickle cell disease

Discharge planning will address the most common causes that precipitate acute sickle cell

crisis including dehydration, infection, extreme changes in temperature, smoking and reduced

oxygen availability. Preventing infections, in persons with sickle cell disease, requires strict

adherence to handwashing as the best defense against pathogens (National Center on Birth

Defects and Developmental Disabilities, 2017, Appendix D). Food safety involves washing fruits


and vegetables and avoiding all raw and unpasteurized food products. Reptiles can harbor

harmful pathogens and should be avoided, included but not limited to, turtles, snakes and lizards.

Vaccinations given at regular developmental growth periods with boosters will help decrease

infection risk.

The National Heart, Lung, and Blood Institute (2014) describe coping with pain in sickle

cell crisis as a difficult endeavor. However, pain management will most likely involve prescribed

or over the counter medications. Collaboration with a health care provider to develop a pain

management plan is of the upmost importance (Appendix E). Soe et al., (2017) report about a

study done with the administration of vitamin D, showing 10 fewer days of pain when compared

to the placebo group. Other alternatives to pain medications, as mentioned by the NHLBI,

involve heating pads, warm baths, massages, acupuncture, physical therapy, chiropractic and

distracting or relaxing activities. Some of these activities include listening to music, talking on

the phone or watching TV.

Living with a chronic disease can be stressful and cause emotional problems. Support

services that may help include counseling, psychiatry, church congregations and support groups

(National Heart, Blood and Lung Institute, 2014). Visit American Sickle Cell Anemia

Association (2010) for support groups near you at http://www.ascaa.org/support-groups.php .

The National Heart, Lungs and Blood Institute (2014) advocate for the maintenance of a healthy

lifestyle to help reduce risk for infections and crisis. Attention to a nutritious and well-balanced

diet, sleep, regular physical activity and hydration is key to a healthy lifestyle.

Medication used to treat sickle cell disease

Hydroxyurea is used to decrease the number of crisis encountered by those afflicted with

sickle cell disease (Rodgers & George, 2017). It works by increasing production of Hemoglobin


F which helps to inhibit sickling (Maakaron, 2017). The medication is dosed based on weight,

initially 15mg/kg/day taken in one daily dose. After low dose initiated and titrated to reduce

crisis, blood counts are monitored every 2 weeks to prevent toxicity. Dose may be increased by 5

mg/kg/day every 12 weeks until the maximum tolerated dose of 35 mg/kg/day is achieved

(Lexicomp, n.d.). Once the dose is determined, the available form for adults in 200-500 mg

capsules are provided. Side effects to be aware of include nausea, vomiting, diarrhea, loss of

appetite, weight gain, sores in mouth, constipation, rash, pale skin, dizziness, hair loss and

darkening of skin and nails (The American Society of Health-System Pharmacists, 2015).

The U.S. Food and Drug Administration [FDA] (2017) approved Endari (L-glutamine)

Oral Powder to reduce acute complications of sickle cell disease, including sickle cell crisis.

RxList (2017) claims that L-glutamine may improve the nicotinamide adenine dinucleotide

(NAD) redox potential in sickle red blood cells by increasing the availability of reduced

glutathione. This helps reduce cellular damage and decrease chance of hemolysis and sickle cell

crisis. Adult dosage is based on weight less than 30kg (5g dose BID), 30-65kg (10g dose BID),

and greater than 65kg (15g dose BID). The powder is mixed in 8 oz of fluids or 4-6oz of pureed

food prior to ingestion. The FDA (2017) list the side effects as follows, constipation, nausea,

headache, abdominal pain, cough, pain in the extremities, back pain, and chest pain.

Activity level and restrictions

Gradual mild to moderate exercise may reduce risk of crisis, decrease pain and increase

respiratory muscle strength. Moderate exercise, 50 percent of maximal aerobic power, should be

included 2-3 days per week for 10-30 minutes at a time (Connes, Machado, Hue, & Reid, 2011).

Special consideration for one’s aerobic level in important in determining the amount of time

spent exercising, so as not to induce a sickle cell crisis. Some exercises may include strength


training, endurance exercises, gymnastics, stationary bicycle riding, games, aquatic rehab,

kinesiology, stretching, aerobics and relaxation like yoga or Pilates. It is extremely important to

rest every 20 minutes of exercise to avoid dehydration and lactic acid accumulation which can

precipitate crisis. Drinking water during and after exercising, preventing cold or heat stress with

outdoor activities and avoid any strenuous activity will help in avoiding a crisis.

Nutritional needs

Nutritional needs for those with sickle cell disease include eating a nutritious and

balanced diet (Appendix C). This includes whole fruits, varied vegetables, whole grains, varied

proteins, dairy and oils (United States Department of Agriculture [USDA], 2017). Special

attention to the vitamins and minerals, especially folic acid, can be accomplished by

incorporating extra leafy green vegetables to help counter micronutrient deficiencies (Mandese et

al, 2016). Some foods that contain folic acid include leafy greens, asparagus, broccoli, citrus

fruits, beans, peas, lentils, and brussel sprouts (National Institutes of Health, 2016). Dehydration

is a main contributor to sickle cell crisis and drinking minimum 8-10 glasses of water daily will

help prevent this complication (National Center on Birth Defects and Developmental Disabilities,

Division of Blood Disorders, 2011). Pells et al., (2005) state that a majority of the African

American participants with sickle cell disease report eating less and significantly decreasing their

intake of fats and proteins during episodes of pain.

Criteria for notifying or following-up with physician

Ongoing medical care is essential to prevention of complications with sickle cell disease,

of an adult, by maintaining a good primary care doctor, hematologist, and keeping

immunizations up to date (CDC, 2017). Health maintenance requires routine medical evaluations

every 2-6 months, yearly HIV and hepatitis testing, and routine dental exams. Important


immunizations for the adult with sickle cell disease include tetanus, influenza, hepatitis and

pneumococcal.

Follow up after hospitalization is important to make sure recurrence of crisis does not

occur. In the meantime, see a doctor right away if you have any of the following serious signs or

symptoms, Appendix F, including severe uncontrolled pain, fever, stroke symptoms, difficulty

breathing, abdominal pain, increased fullness without eating or eating little, sudden vision loss,

pale skin, weakness, fatigue, shortness of breath, dizziness, tingling or crawling of skin, coldness

in hands or feet and chest pain (National Heart, Lungs, and Blood Institute, 2017).

Avoiding Ethnocentrism in Discharge Teaching

A culturally competent nurse can reflect on one’s cultural self-assessment and clinical

experiences to learn, grow, accept, and incorporate better nursing practices into culturally

competent nursing care for African Americans. This can be achieved by being consciously aware

of one’s own cultural influences to relay a positive, caring, and advocative attitude no matter the

situation or culture present. Avoiding ethnocentrism, in discharge teaching, by focusing on the

clients’ values, and not ones’ own values, will create a foundation for learning and culturally

competent nursing care and teaching to take place. It also helps to establish a strong nurse-patient

relationship, so mutual goals can be agreed and acted upon without bias or restraint. For this

cultural competence to work, a personal cultural self-assessment of one’s relatability to various

groups must be completed. Also, the African American client should be provided with cultural

care preservation or maintenance techniques, accommodation or negotiation techniques, and

repatterning or restructuring ideas. Practicing these assessments, decisions or actions, allows one

to participate in culturally competent decision making and care.

Self-Reflection


One step in cultural competent care is cultural self-reflection influenced by one’s

commitment to compassion, healing, advocacy, integrity, altruism and professionalism. Integrity

integrates one’s cultural and professional values, concern for the well-being of others and one-

self, and allows one’s emotions to help pave the way to a therapeutic nurse-patient relationship.

Reflection from Olsen (2014) helps to understand that developing acceptance, through cultural

self-reflection, requires gaining personal meaning from nursing encounters and interactions with

clients. The evolution of culturally competent care stems from reflection on previous clinical

experiences with African American, by understanding their cultural preferences, how they

communicate and how they incorporate their religion into their health practices. This helps in the

incorporation of true cultural acceptance after evaluation of one’s values, prejudices and beliefs.

This reflection helps in the development of a trusting nurse-patient relationship to decrease the

stigma African Americans have with health care professionals.

Cultural care preservation or maintenance

Providing cultural care preservation or maintenance allows nurses to suspend judgment

and accept that other cultural practices, along with traditional medicine, may be beneficial in

healing from illness or disease (Andrews and Murray-Wright, 2016). This allows one to preserve

and honor core values regarding personal cultural beliefs and practices. It is imperative to gain an

understanding of whatever the cultural, medicinal, or religious desires of the African American

client are to provide a trusting caring environment.

Cultural care accommodation or negotiation

Accommodation or negotiation help to promote acceptance of combined cultural

practices or folk remedies with the dominant culture’s medical practices and (Andrews and

Boyle, 2016). This combination helps clients, with a designated culture, to adapt to or negotiate


with the healthcare teams approach to include personal healing techniques used to maintain

health and well-being. The care plan should be a mixture of medications and health regimens that

incorporate both these practices.

Cultural care repatterning or restructuring

Open mindedness can help clients to repattern or restructure their way of thinking, or

lifestyle choices, for new, different, and beneficial health ideas that promote health maintenance

(Andrews and Boyle, 2016). In this restructuring, the care plan is taking into consideration

cultural and health-related beliefs, while assisting in the modification of beliefs and practices for

the promotion of health. This restructuring allows one to be open-minded and change their way

of thinking while also maintaining their personal beliefs and values. The nurse can use creative

ways of incorporating change without interfering in the clients’ cultural beliefs. This change is

usually geared toward practices or behaviors that may cause harm. An example of this may be

the use of prayers and blessings as a means of healing alone, and not allowing treatment

procedures and medications to be used. A second example is the use of nicotine via cigarette

smoking, which is highly prevalent in African Americans and increases risk for sickle cell crisis.

Conclusion

Being able to provide culturally competent care and discharge teaching is the goal for any

nurse and can be accomplished first by self-reflection of one’s culture, values and beliefs. Once

this reflection is done a true acceptance for others’ beliefs can begin and discharge teaching can

be molded to the client’s individual culture. Understanding one’s strengths and weaknesses,

when it comes to acceptance of various groups, will help in bias restraint by suspending

judgement. Finally, the nurse can utilize Leininger’s culture care decisions and actions to help

create a culturally competent discharge teaching plan which incorporates preservation or


maintenance techniques, accommodation or negotiation techniques, and repatterning or

restructuring ideas (Andrews & Murray-Wright, 2016). The ideas and decisions of cultural care

allow the client and nurse to work together to comprise a mutually agreed upon discharge plan.

Incorporating the culture, values, and beliefs in this plan will ultimately lead to the development

of a strong nurse-patient relationship, a stepping stone to culturally congruent care and a

decrease in rehospitalizations in African Americans with sickle cell disease.


References

Abrams, J. (2012). Blurring the lines of traditional gender roles: Beliefs of African American

women (Master's thesis). Retrieved January 6, 2018 from

https://scholarscompass.vcu.edu/cgi/viewcontent.cgi?article=3781&context=etd

Adegbola, M. (2011). Spirituality, self-efficacy, and quality of life among adults with sickle cell

Disease. Southern Online Journal of Nursing Research, 11(1), 1-16.

American Sickle Cell Anemia Association. (2010). Support groups. Cleveland, Ohio: American

Sickle Cell Anemia Association. Retrieved January 27, 2018, from

http://www.ascaa.org/support-groups.php

Anderson, M.B.L. (2016). Building better narratives in black education. Frederick D. Patterson

Research Institute. Washington, DC: United Negro College Fund. Retrieved February 2,

2018 from http://images.uncf.org/production/reports/2016_Advocacy-

Better_Narratives_Report_FINAL_(digital).pdf

Andrews, M. M., & Murray-Wright, M. (2016). Cultural competence in the health history and

physical examination. In MM Andrews and JS Boyle (eds). Transcultural Concepts in

Nursing Care (7th ed., pp. 55-101). Philadelphia, PA: Wolters Kluwer.

Boyle, J.S., & Andrews, M.M. (2016). Andrews/Boyle transcultural nursing assessment guide

for families, groups, and communities. In MM Andrews & JS Boyle (eds.). Transcultural

concepts in nursing care (7th ed., pp. B:1- B:3). Philadephia, PA: Wolters Kluwer.

Boyle, J.S., & Baird, B.B. (2016). Culture, family, and community. In MM Andrews & JS Boyle

(eds.). Transcultural concepts in nursing care (7th ed., pp. 317-358). Philadelphia, PA:

Wolters Kluwer.


Campinha-Bacote, J. (2009). A culturally competent model of care for African Americans.

Urologic Nursing, 29(1), 49-54.

Campinha-Bacote, J. (2012). People of African American heritage. In L. Purnell (Ed.),

Transcultural health care: A culturally competent approach (pp. 91-114). Philadelphia,

PA: F.A. Davis.

Centers for Disease Control and Prevention. (2017, July 27). Sickle Cell Disease: Complications

and Treatments. Atlanta, GA: U.S. Department of Health & Human Services. Retrieved

January 6, 2018 from https://www.cdc.gov/ncbddd/sicklecell/treatments.html

Cohen, R. T., DeBaun, M. R., Blinder, M. A., Strunk, R. C., & Field, J. J. (2010). Smoking is

associated with an increased risk of acute chest syndrome and pain among adults with

sickle cell disease. Blood, 115(18), 3852-3854. doi:10.1182/blood-2010-01-265819

Collins, W. L. (2015). The role of African American churches in promoting health among

congregations. Social Work and Christianity, 42(2), 193-204.

Cotton, S., Grossoehme, D., & McGrady, M. E. (2011). Religious coping and the use of prayer in

children with sickle cell disease. Pediatric Blood & Cancer, 58(2), 244-249.

doi:10.1002/pbc.23038

Edwards, F. A. (2013). The African American family's influence on academic achievement of

urban secondary students (Doctoral dissertation). Retrieved January 6, 2018 from

https://digitalcommons.pcom.edu/cgi/viewcontent.cgi?article=1280&context=psychology

_dissertations

Ehrmin, J.T. (2016). Transcultural perscpectives in mental health nursing. In MM Andrews and

JS Boyle (eds). Transcultural Concepts in Nursing Care (7th ed., pp. 272-316).

Philadelphia, PA: Wolters Kluwer.


Hecht, M. L., Jackson, R. L., & Ribeau, S. A. (2003). Introduction. In African American

Communication: Exploring Identity and Culture. (2nd ed., pp. 1-40). Mahwah, NJ: L.

Erlbaum Associates.

Indiana Hemophilia and Thrombosis Center. (2015). All about: Sickle cell disease and sickle cell

trait. Indianapolis, IN: Indiana Hemophilia and Thrombosis Center, Inc. Retrieived

February 4, 2018 from http://www.ihtc.org/wp-content/uploads/2015/06/Sickle-Cell-

Disease-Brochure.pdf

Le, D., Holt, C. L., Hosack, D. P., Huang, J., & Clark, E. M. (2015). Religious participation is

associated with increases in religious social support in a national longitudinal study of

African Americans. Journal of Religion and Health, 55(4), 1449-1460.

doi:10.1007/s10943-015-0143-1

Lexicomp. (n.d.). Hydroxyurea (hydroxycarbamide): Drug information. UpToDate. Alphenaan

den Rijn, The Netherlands: Wolters Kluwer. Retrieved January 27, 2018, from

https://www.uptodate.com/contents/hydroxyurea-hydroxycarbamide-

druginformation?source=see_link

Long, K. A., Thomas, S. B., Grubs, R. E., Gettig, E. A., & Krishnamurti, L. (2011). Attitudes

and Beliefs of African-Americans Toward Genetics, Genetic Testing, and Sickle Cell

Disease Education and Awareness. Journal of Genetic Counseling, 20(6), 572-592.

doi:10.1007/s10897-011-9388-3

Maakaron, J. E. (2017, December 28). Sickle cell anemia. Medscape. New York, NY: WebMD

LLC Retrieved January 27, 2018, from

https://emedicine.medscape.com/article/205926/overview


Mandal, A. (2013). Factors affecting African-American health: empowering the community with

health literacy. Journal of Bioprocessing & Biotechniques, 03(01), 1-2.

doi:10.4172/2155-9821.1000e111

National Center on Birth Defects and Developmental Disabilities, Division of Blood Disorders.

(2011, September 8). Living well with sickle cell disease self-care toolkit. Document

CS226674B Atlanta, GA: U.S. Department of Health and Human Services, Centers for

Disease Control and Prevention. Retrieved January 6, 2018 from

https://stacks.cdc.gov/view/cdc/28027

National Center on Birth Defects and Developmental Disabilities. (2017, July 13). Preventing

infections. Centers for disease control and prevention. Document C5263755C Atlanta,

GA: U.S. Department of Health and Human Services, Centers for Disease Control and

Prevention. Retrieved from

https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_5.pdf

National Heart, Lung, and Blood Institute. (2014). Evidence-based management of sickle cell

disease: Expert panel report. Bethesda, MD: U.S Department of Health and Human

Services, National Institutes of Health Retrieved January 28, 2018 from

https://www.nhlbi.nih.gov/sites/default/files/media/docs/sickle-cell-disease-

report%20020816_0.pdf

National Heart, Lungs, and Blood Institute. (2012). Sickle cell disease research & care. National

Heart, Lungs, and Blood Institute. Bethesda, MD: U.S. Depatment of Health and Human

Services, National Institutes of Health. Retrieved February 2, 2018 from

https://www.nhlbi.nih.gov/news/2012/sickle-cell-disease-research-care


National Human Genome Research Institute. (2016). Learning about sickle cell disease.

Bethesda, MD: U.S. Department of Health and Human Services, National Institutes of

Health. Retrieved February 2, 2018 from https://www.genome.gov/10001219/learning-

about-sickle-cell-disease/

Office of Communications and Public Liaison. (2017). Cultural respect. Clear Communication.


Health, NIH Office of the Director. Retrieved February 2, 2018 from

https://www.nih.gov/institutes-nih/nih-office-director/office-communications-public-

liaison/clear-communication/cultural-respect

National Institutes of Health. (2016). Folate. National Institutes of Health. Bethesda, MD: U.S

Department of Health and Human Services. Retrieved January 28, 2018 from

https://ods.od.nih.gov/factsheets/Folate-HealthProfessional/

National Institute for Children’s Health Quality. (2015). Sickle cell pain in the emergency

department: A guide to improving care. Boston, MA: National Institute for Children’s

Health Quality. Retrieved January 6, 2018 from

https://www.nichq.org/sites/default/files/resource-file/Sickle-Cell-Pain-in-the-

Emergency-Department.pdf

Olsen, N.C. (2014, February 3). Self-Reflection: Foundation for Meaningful Nursing Practice.

Reflections on Nursing Leadership, 39(2). Retrieved from

https://www.reflectionsonnursingleadership.org/features/more-features/Vol39_2_self-

reflection-foundation-for-meaningful-nursing-practice

Pells, J. J., Presnell, K. E., Edwards, C. L., Wood, M., Harrison, M. O., DeCastro, L., …

Robinson, E. (2005). Moderate chronic pain, weight and dietary intake in African-


American adult patients with sickle cell disease. Journal of the National Medical

Association, 97(12), 1622–1629.

Pew Research Center. (2018). Blacks: Religious composition of Blacks. Religious Landscape

Study. Washington DC: Pew Research Center. Retrieved February 2, 2018 from

http://www.pewforum.org/religious-landscape-study/racial-and-ethnic-

composition/black/

Rodgers, G. P., & George, A. (2017). Hydroxyurea use in sickle cell disease. UpToDate.

Alphenaan den Rijn, The Netherlands: Wolters Kluwer. Retrieved January 27, 2018,

from https://www.uptodate.com/contents/hydroxyurea-use-in-sickle-cell-

disease?source=see_link#H3109840581

RxList. (2017, July 19). Endari (L-glutamine Oral Powder). Atlanta, GA: WebMD LLC.

Retrieved February 24, 2018, from https://www.rxlist.com/endari-drug.htm#medguide

Savoca, M. R., Martine, T. L., Morton, T. B., Johnson, L. T., Bell, N. M., Aronson, R. E., &

Wallace, D. C. (2011). Meal patterns and food choices of young African-American men:

Understanding eating within the context of daily life. Journal of the American Dietetic

Association, 111(9), 1335-1342. doi:10.1016/j.jada.2011.06.006

Soe, H. H., Abas, A. B., Than, N. N., Ni, H., Singh, J., Said, A. R., & Osunkwo, I. (2017).

Vitamin D supplementation for sickle cell disease. Cochrane Database of Systematic

Reviews, 010858(1), 1-32. doi:10.1002/14651858.cd010858

Taylor, R. J., Chatters, L. M., & Brown, R. K. (2014). African American religious

participation. Review of Religious Research, 56(4), 513–538. doi:10.1007/s13644-013-

0144-z


The American Society of Health-System Pharmacists. (2015). Hydroxyurea. Medline Plus


Health, National Library of Medicine. Retrieved January 28, 2018 from

https://medlineplus.gov/druginfo/meds/a682004.html

United States Department of Agriculture. (2017A). Variety. MyPlate. Alexandria, VA: USDA

Center for Nutrition Policy and Promotion. Retrieved January 28, 2018 from

https://www.choosemyplate.gov/variety

United States Department of Agriculture. (2017B). What’s MyPlate all about? MyPlate.

Alexandria, VA: USDA Center for Nutrition Policy and Promotion. Retrieved February 2,

2018 from https://choosemyplate-

prod.azureedge.net/sites/default/files/printablematerials/2013-

WhatsMyPlateAllAboutInfographic.pdf

U.S. Food and Drug Administration. (2014). The FDA encourages new treatments for sickle cell

disease. Washington, DC: U.S. Department of Health and Human Services. Retrieved

February 24, 2018 from

https://www.fda.gov/ForConsumers/ConsumerUpdates/ucm418232.htm


Appendix A

Hemoglobin A and Hemoglobin S (National Human Genome Institute, 2016)


Appendix B

Sickle Cell Disease Home Management (Indiana Hemophilia and Thrombosis Center, 2015)


Appendix C

My Plate (USDA, 2017)


Appendix D

Infection Prevention (National Center on Birth Defects and Developmental Disabilities, 2017)


Appendix E

Managing Sickle Cell Disease (National Center on Birth Defects and Developmental

Disabilities, 2017)


Appendix F

When to see a doctor (National Center on Birth Defects and Developmental Disabilities, 2017)


Appendix G

Sample Discharge Teaching

Discharge Summary

Patient Name: John Doe 24y/o M

Admission Date: 2/20/2018

Discharge Date:2/25/2018

Attending Physician: Smith, MD

Primary Care Physician: Wilke, MD

Referring Physician: Smith, MD

Consulting Physician(s): Hematologist Anderson, MD

Final Diagnosis: Acute Sickle Cell Crisis, Dehydration

Vital Signs:

Weight: 165lb; Oxygen Saturation: 93% RA

Pulse: 90; Respirations: 18

Blood Pressure: 128/84 (99); Temperature: 98.9 °F

Discharge Medications:

Hydroxyurea 1000mg- 2300mg/day capsules (blood draws every 2 weeks to determine

therapeutic dose)

Endari (L-glutamine oral powder)15g BID (Mix Endari immediately before ingestion with 8

oz of fluids or 4-6 oz of soft foods)

Ibuprofen 600mg tabs Q6 hours PRN for mild pain

Acetaminophen 500mg tabs Q4-6 hors PRN for mild pain

Oxycodone 10mg every 4-6 hrs as needed for severe pain

Discharge Instructions: Managing Sickle Cell Disease (SCD)

Prevent and Control Complications:

Prevent infections:

o Frequent handwashing is the best defense

o Food safety- no raw or unpasteurized food products, wash fruits and vegetables

o Reptiles- avoid turtle’s, snakes and lizards

o Vaccinations- get all recommended vaccines


o Prophylactic antibiotics for children 2 mo-5 years and high-risk adults

Coping with Pain:

o Collaborate with HCP to develop a pain management plan.

o Other alternative methods to help control pain include:

Heating pads

Warm baths

Massages

Physical therapy

Acupuncture

Distracting and/or relaxing activities, such as listening to music, talking

on the phone, or watching TV

Mental Health:

o Living with a chronic disease can be stressful and cause emotional problems

support services include:

Counseling

Psychiatrist

Support groups

Visit American Sickle Cell Anemia Association for support

groups near you at http://www.ascaa.org/support-groups.php

Pursue a Healthy Lifestyle:

o Eat a nourishing diet

o Get enough sleep

o Regular physical activity- avoid strenuous activities

o Hydration

Activity Level and Restrictions

Exercise

o Gradual mild to moderate exercise may reduce risk of crisis, decrease pain and

increase respiratory muscle strength

Moderate (50% of maximal aerobic power) exercise for 10-30 minutes 2-

3 times per week

strength training

endurance exercises

gymnastics

stationary bicycle riding

games

aquatic rehab

kinesiology

stretching

aerobics

relaxation (Yoga, Pilates)

Rest every 20min of exercise to avoid dehydration and lactic acid

accumulation

Drink water during and after exercising


Prevent cold or heat stress with outdoor activities

Avoid any strenuous activity

Nutritional needs

Drink at least 8-10 glasses of water daily

Eat a healthy balanced diet

o Whole fruits

fresh, frozen, dried, and canned options

o Varied Vegetables

dark-green vegetables, red and orange vegetables, legumes (beans and

peas), starchy vegetables, and other vegetables

o Whole grains

o Varied Proteins

animal (seafood, meat, poultry, and eggs) and plant sources (nuts, beans

and peas, seeds, and soy products)

o Dairy (low-fat or fat-free)

milk, yogurt, cheese, and calcium-fortified soy beverages (soymilk)

o Oils

Choose the right amount of oil to stay within your daily calorie needs

o Add extra green vegetables to help counter micronutrient deficiencies

vitamins A, B2, B6, B12, C, D, E, iron, calcium, magnesium and zinc

folic acid

Dark leafy greens

Asparagus

Broccoli

Citrus fruits

Beans, peas and lentils

Brussel sprouts

Criteria for notifying or following-up with physician

See a doctor right away if you have any of the following serious signs

o Severe uncontrolled pain

o Fever

o Stroke symptoms

Altered mentation and speech and facial droop

o Difficulty breathing

o Symptoms of spleen enlargement

Abdominal pain, increased fullness without eating or eating little

o Sudden vision loss

o Symptoms of severe anemia

Pale skin, weakness, fatigue, shortness of breath, dizziness, tingling or

crawling of the skin, coldness in hands or feet and chest pain

Ongoing Medical Care:


Maintain a good primary care and hematologist to get regular checkups

o Annual Physical

labs, immunizations, and counseling on preventive measures

Penicillin prophylaxis

prophylaxis for surgical procedures

o Adult health maintenance requires routine medicals evaluations every 2-6

months

Immunizations

Tetanus, influenza and hepatitis annually

Pneumococcal every 5years

HIV and Hepatitis testing annually

Routine Dental exams to prevent SCD complications

Follow up Appointments:

Hematology Oncology Associates: Call for an appointment within 1 week

Anderson, MD (505) 727-3040

715 Dr Martin Luther King Jr Ave NE #102, Albuquerque, NM 87102

used with permission -...

Documents