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INTRODUCTION

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CASE LEARNING OBJECTIVES

  • Differentiate sickle cell-related complications

  • Evaluate the goals of therapy for sickle-cell disease (SCD)

  • Identify modifications in medication care plans due to renal impairment

  • Develop an appropriate sickle cell education, treatment and monitoring plan for a patient with complicated sickle cell disease

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PATIENT PRESENTATION

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Chief Complaint

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"I had a mosquito bite on my leg that really bothered me so I scratched it until it got angry looking and now it is all red and swollen. My leg really hurts, and my home pain medications are not helping. My pain is 6 out of 10 right now. I've noticed that I am not going to the bathroom as much as I used to. I really feel lousy."

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History of Present Illness

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Jacob Adams is an 18-year-old male with sickle cell disease, who has been admitted to the hospital with cellulitis of the lower right leg, pain and an acute decrease in urine output with a weight gain of 2 kg during the past 2 weeks. He is maintained on a chronic transfusion program secondary to sickle cell related stroke as a child. He claims to be "mostly adherent" to his physician appointments every month, but between school and work, he has missed two appointments within the past 6 months. He states that he takes his medications daily as prescribed and rarely misses doses. He had not taken his pain medication in several months before yesterday. He denies any feelings of dizziness or lightheadedness, but does feel fatigue. He also mentions that prior to admission his urine has been a dark yellow/brown color.

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Past Medical History

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Sickle cell anemia (HbSS)

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Stroke at age 4 years; walks with a limp on the right side

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Streptoccus pneumoniae sepsis at age 3 and 12 years

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Chronic transfusions monthly × 13 years

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Cholecystectomy

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Family History

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Father had SCD, died at age 42 from complications of renal failure and myocardial infarction. Mother is alive at age 34 and has SCT, type II diabetes, obesity and history of renal stones. Sister is alive at 22 and has SCT, is pre-diabetic, with a history of renal impairment.

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Social History

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He is a junior in high school and works part-time at a local steakhouse where he often eats. Has his own car and drives himself to school and work.

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Tobacco/Alcohol/Substance Use

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Social drinker (3–4 beers on weekends only); (−) tobacco or illicit drug use.

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Allergies/Intolerances/Adverse Drug Events

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Documented allergy to erythromycin (hives).

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Medications (Current)

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Penicillin VK potassium 125 mg PO BID

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Hydrocodone/acetaminophen 7.5/500 mg 1 ...

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