Nursing discussion
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sicklecellanemia.pptxCase Study
Mr. L.P. is a 37-year-old half African and half Saudi Arabian male with a history of sickle cell anemia, HTN, acute chest syndrome and avascular necrosis of the right shoulder. Patient has no known allergies and has a surgical history of cholecystectomy. The patient reports having generalized pain and swelling noted on his bilateral knees. The swelling has progressed in the last 24 hours and has become difficult to walk without assistance causing him to have a compromised gait. He also states this is common with his sickle cell crisis symptoms and has tried to manage symptoms at home with prescribed pain medication however has no relief.
Objective Data
History of Present Illness:
Generalized pain
Knee swelling/inflammation
Difficulty ambulating
Previous Medical History
HTN, avascular necrosis of right shoulder, acute chest syndrome
Surgical History
Cholecystectomy
Allergies
None
Physical Exam:
Head and Neck: Unremarkable
Eye: Glasses
Respiratory: Clear lungs, WDL
Cardiac: Noted to be tachycardic on telemetry, heart rate sustaining 118 bpm
Skin: Intact
Musculoskeletal: Bilateral swelling of the knees, difficulty extending.
GI: Soft, Tender to touch, some guarding noted
GU: WDL
Vital Signs:
BP 144/80 HR 118 RR 18 O2 Sat 99% Temp 37.7
Labs: CBC with differential, BMP, Reticulocytes, Ferritin, Iron, hemoglobin electrophoresis
Imaging: Xray of bilateral knees, US for DVT of bilateral extremities
Description of Disease
Sickle cell anemia also can be known as sickle cell hemoglobin (HbSS) or homozygous SS disease
It is an inherited autosomal recessive disorder
African Americans, Arabian, Greek, Italian, Caribbean, Hispanics, Turkish
Resulting in qualitative mutation of the hemoglobin structure in red blood cells
The sickling of the red blood cell causes under deoxygenated cells therefore results in pain
Sickle cell crisis causes vaso-occlusive crisis that can cause abdominal, joint/bone pain accompanied by fever caused by masses of sickle cell trapped in blood vessels.
Severe sickle crisis includes:
Avascular necrosis of joints
Acute chest syndrome
Stroke
Severe Anemia
Hypoxemia
(National heart, lung and blood institute, 2020)
(MayoClinic, 2020)
Signs and Symptoms
Anemia
Swelling of joints
Joint/bone pain
Fever
Hypoxemia
Chest pain
Periods of generalized pain
Infections
This Photo by Unknown Author is licensed under CC BY-SA
This Photo by Unknown Author is licensed under CC BY-SA-NC
This Photo by Unknown Author is licensed under CC BY-SA-NC
(National heart, lung and blood institute, 2020)
Potential Barriers
Cultural – Preconceived notions that sickle cell disease patients are drug seeking
Solution: Educating the community, family and practitioners on sickle cell disease management
Socioeconomic – Majority of patients have difficulty maintaining jobs, attending school and contributing to society due to prolonged hospital stays or difficulty with ADLs
Solution: Pain management, Sickle cell clinics, Hematologist, Appointment compliance
Healthcare literacy – Adolescents are susceptible to narcotic drug dependency
Solution: Educate about sickle cell disease and offer different forms of pain management and alternative therapy. Also discuss importance of medical compliance
(William C. Shiel Jr., n.d.)
Medication Management
Opioid Analgesics
Oxycodone
Morphine Sulfate ( IV push administration or PCA)
Percocet
Fentanyl patch
Nonsteroidal Analgesics
Ketorolac
Acetaminophen
Ibuprofen
Vitamins
Folic acid, fish oil, zinc
Antimetabolites
Hydroxyurea
This Photo by Unknown Author is licensed under CC BY-SA
("Sickle cell anemia medication," 2020)
Opioid Analgesics – Morphine Sulfate
Mechanism of Action
Binds to opioid receptors in the CNS causing inhibition of ascending pathways altering the perception of and response to pain; produces generalized CNS depression
Dose
Patient controlled Analgesia
0.5 mg to 2 mg IV (1 mg/ 1ml)
Lock out every 5 to 10 minutes
Adverse Effects
Respiration depression
Hypotension
CNS depression
Constipation
Major Contraindications
Low blood pressure
Hypoxemia
Decreased lung function
Renal impairment
History of opioid abuse or overdose
Drug to drug Interactions
Benzodiazepines
Alcohol use
(Lexicomp, 2021)
Antimetabolites – Hydroxyurea
Mechanism of Action
Assists with enlarging red blood cells (RBCs) and makes then rounder or flexible preventing them from returning to their sickled state. It increases hemoglobin F. which helps RBCs and reduce pain levels.
Dose
Start 15 mg/kg/day
Titrate 5 mg/kg/day every 12 weeks
Do not exceed 35 mg/kg/day
Adverse Effects
Renal failure
Leukopenia and neutropenia
Anemia
Hepatotoxicity
Major Contraindications
Severe anemia and bone marrow suppression
WBC less than 2500 or PLTs less than 100,000
Drug to drug Interactions
Vaccines – MMR, Varicella, Smallpox, typhoid, yellow fever
Can help patients with:
Sickle Cell disease type SS
Sickle cell type sickle beta zero thalassemia
(Lexicomp, 2021)
9
Alternative Therapy and Outcome
Alternative Therapy:
Stress relieving activities
Yoga, meditation
Nutritional supplements
Zinc, Folic acid, Fish oil, joint support supplements
Oxygenation
Hydration
Heat therapy (Heating pads)
Optical Outcome for treatment
Pain relief
Reduce risks for sickle cell crisis
Improve nutritional status
Fewer hospital stays
Lessen the risk of acute chest syndrome
This Photo by Unknown Author is licensed under CC BY-ND
This Photo by Unknown Author is licensed under CC BY-NC
(MayoClinic, 2020)
Expectations for patient follow up
Patient must follow up with their hematologist and primary physicians regularly
A sickle cell clinic can be appointed to the patient
Regular scheduled laboratory work up
Educate on side effects or onset of new symptoms when prescribed new medications
This Photo by Unknown Author is licensed under CC BY-ND
References
Lexicomp. (2021). UpToDate. Retrieved from https://www.uptodate.com/contents/morphine-drug-information#F8776704
MayoClinic. (2020, January 30). Sickle cell anemia - Diagnosis and treatment - Mayo Clinic. Retrieved from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882
National heart, lung and blood institute. (2020, September). Sickle cell disease. Retrieved from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
Sickle cell anemia medication. (2020, August 21). Retrieved from https://emedicine.medscape.com/article/205926-medication
William C. Shiel Jr. (n.d.). 21 sickle cell anemia symptoms, genetics, treatment, life expectancy. Retrieved from https://www.medicinenet.com/sickle_cell/article.htm
