Sickle Cell Anemia

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By Ginny Higley and Erdylle Macapinlac

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What is Sickle Cell Anemia?

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An inherited blood disorder which causes abnormal/ distorted �hemoglobin cells to form. The sickled red blood cells�are fragile and can rupture, which results in anemia. �The sickled cells can also block the blood vessels �which can cause pain and organ damage.

This disorder primarily affects African Americans �and Africans and is estimated that one in 500 �African American births will have sickle cell Anemia.

Different Sickle Cell Diseases:

Hb C & Sickle cell thalassemia:

• Heterozygous
• Less common and less severe than HbSS

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Sickle cell trait:

• One hemoglobin S and one normal hemoglobin (Hgb A)
• Mild to asymptomatic

Case Study:

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Ms. Fox, 20 year old black American comes to the emergency department with complaints of chest pain and shortness of breath.

Has a non productive cough, and low grade fever for the past two days.

Subjective data: Patient stated that her symptoms are typical of her sickle cell crisis and therefore came to the emergency department. Patient states that her pain was an 8 out of 10, and that it is “a constant burning pain”

Objective data: Vital signs; temp: 100.8 fahrenheit, BP 120/76, pulse 96, respiratory rate 22, O2 sat 94% on room air, but had difficulty breathing and placed on 2 liters via nasal cannula.

Clinical Manifestations:

Jaundice Tissue necrosis�Pallor Silent strokes�Pain �Fatigue�Eye damage�Leg ulcers��Fever, abdominal pain �and dactylitis are usually �seen within the first year of life.

Labs and Dx Imaging:

Labs:

• Blood test to see if you have Hgb S in your blood
• PKU test for newborns

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Dx imaging:

• Skeletal x-rays
• MRI
• Doppler
• Chest x-ray

Patients lab results vs. normal lab results:

Normal labs:

• WBC: 3,000-10,500 cells/mcL
• RBC: 3.9-5.03 x10^12
• MCV: 80-96 fL/red cell in adult
• RDW: 11.6-14.6%
• Hgb: 12-15.5 grams
• Hct: 34.9-44.5%
• Reticulocytes: 0.5-1.5%

Patients labs:

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• WBC: 18,000 cells/mcL
• RBC: 3 x10^6
• MCV: 70 fl/red cell in adult
• RDW: 20.4%
• Hgb: 7.5 grams
• Hct: 21.8%
• Reticulocytes: 23%

Safety Concerns:

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Avoid dehydration

Give examples of a nutritious diet

Moderate physical activity

Go to doctor when experiencing symptoms of an episode

Teach ways to cope with pain

Medications & Interventions:

Antibiotics for infection -Penicillin

Pain medications- NSAIDS, Duloxetine, Gabapentin, Amitriptyline and opiates

Hydroxyurea- oral medication to reduce or prevent sickle cell complications

RBC transfusions- to help raise the number of RBC’s and provide normal RBC that are more flexible than RBC’s with sickle hemoglobin

Immunizations to prevent infections- Pneumococcal, influenza and meningococcus

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Hematopoietic Stem Cell Transplantation

This hematopoietic stem cell transplantation (HSCT) is the only known cure for sickle cell at this time

The stem cells are taken from bone marrow of a person without sickle cell, usually the patient's brother or sister. The stem cells are matched for special proteins called HLA antigens which are inherited from parents and the siblings are most likely to have the same antigens.

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Complications and Nursing Considerations:

RBC transfusion: Requires check from two RN’s to read and confirm, monitor vital signs 15 minutes after infusion, stay with patient for first 15 minutes.

Hematopoietic stem cell transplantation: 85% success rate. Monitor for signs of infection and graft versus host disease. Monitor for seizures. The transplant has lead to death in about 5% of cases

NCLEX Question

• Why are patients who are diagnosed with sickle cell anemia more prone to infections?
• Damage to liver
• Damage to spleen
• Decreased oxygen
• Damage to kidney

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NCLEX continued

B. Damage to spleen

The abnormal sickle cells can �block the flow of blood through �the vessels into the spleen �causing organ damage or�failure, which can lead to�serious bacterial infections.

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Other Considerations:

Physical/Mental: A patient may be cured of the disease (bone marrow transplant), but it involves two major factors: One is that once they do get the transplant they need to be on immunosuppressants for at least a year, and two tissue must come from a healthy sibling.

Psychosocial: It is important to know how the patient who is diagnosed with sickle cell anemia feels. Support groups are encouraged.

Other Considerations Continued:

Prevention/Education: Teach patients to avoid high altitudes, maintain adequate fluid intake, and treat infections promptly. Teach patient and family members of symptoms sickle cell crisis: fever, stroke symptoms, problems breathing and sudden loss of vision.

Concerns for staff caring for the patient: Nurse should monitor patient for signs and symptoms of infection. Monitor for signs and symptoms of sepsis.

Nursing Diagnosis #1

• Nursing diagnosis: Risk for infection r/t alterations in splenic function
• Goal: Remain free from symptoms of infection during shift
• Int 1: Use appropriate hand hygiene.
• EBN: “Meticulous infection prevention precautions are required to prevent healthcare associated infection.” (Ackley et al, 2017, pg 530).
• Int 2: Observe and report signs of infection such as warmth and increased body temperature.
• EBN: “ Change in mental status, fever, shaking, chills and hypotension are indicators of sepsis.” ( Ackley et al, 2017, pg 529)

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Nursing Diagnosis #2

• Nursing diagnosis: Acute pain r/t tissue hypoxia
• Goal: Patient will report a 3 out 10 on a 0 to 10 scale, with 10 being the worst pain, at end of the shift.
• Intervention 1: Assess the patient's ability to provide a self-report of pain intensity, by using a 0-10 scale rating.
• EBN: “Self-report is considered the single most reliable indicator of pain presence and intensity” (Ackley et al, 2017, pg.640).
• Intervention 2: Explain to the patient the pain management approach, and the importance of prompt reporting of unrelieved pain.
• EBN: “One of the most important steps toward improved control of pain is a better client understanding of the nature of pain, its treatment, and the role the client needs to play in pain control” (Ignatavicius, 2013).

References:

Ackley, B., Ladwig, G., & Flynn Makic, M. B. (2017). Nursing Diagnosis Handbook (11th ed.). St. Louis, MO: Elsevier.

Lewis, S. L., RN, Dirksen, S. R., RN, Bucher, L., RN, & Heitkemper, M. M., RN. (2014). Medical-Surgical Nursing (9th ed., Vol. 1)

(M. M. Harding, RN, Ed.). St. Louis, MO: Elsevier.

Living With Sickle Cell Disease. (n.d.). Retrieved February 21, 2017, from

https://www.nhlbi.nih.gov/health/health-topics/topics/sca/livingwith

Mayo Clinic Staff Print. (2016, December 29). Sickle cell anemia. Retrieved February 21, 2017, from

http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/treatment/txc-20303509

Shiel, W. C., Jr. (n.d.). Sickle Cell Anemia: Learn About SCD Symptoms and Treatment. Retrieved February 21, 2017, from

http://www.medicinenet.com/sickle_cell/article.htm

Sickle Cell Society. (2015, October 30). Retrieved February 21, 2017, from

http://sicklecellsociety.org/a-new-cure-for-sickle-cell-disease/

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