Patient Encounter 3

CC: "My right leg and lower back hurt badly"

HPI: CD is a 21-year-old African American male diagnosed with sickle cell disease at birth. He has had repeated pain crises (average of about two per year) and presents today with another. He started feeling ill yesterday and this morning woke up with increased pain and a cough.

PMH: Frequent pain crises, acute chest syndrome last year, priapism

FH: Mother with sickle cell trait, Father with sickle cell disease. Father died at age 36 from stroke. Has an older brother with sickle cell disease and a younger sister with sickle cell trait.

SH: CD is a college student. He lives in the dorms. Enjoys basketball. PE: Wt 64 kg (14 lb), ht 180 cm (6 ft), BP 130/80, HR 90, RR 30, temp 40°C (104°F) Lungs: decreased breath sounds in bases

Skin: Warm, dry, tender, and increased warmth in right leg, nailbeds dusky, poor capillary refill

HEENT: Sclera slightly yellow

Meds: Tylenol #3, two tablets orally every 4 to 6 hours as needed for pain; pseudoep-hedrine 30 mg orally twice daily

Allergies: NKDA

Lab: All within normal limits except; Hemoglobin: 6.2 g/dL; hematocrit: 30 g/dL; WBC: 20,000/mm3; Total bilirubin: 2. 2 mg/dL

Based on the information presented, create a care plan for this patient S vasoocclusive crisis. Your plan should include the following:

Statement of the drug-related needs and/or problems

Goals of therapy

Patient-specific, detailed therapeutic plan

Plan for follow-up and monitoring to determine whether the goals have been achieved and adverse effects avoided.

Table 68-5 Chronic Complications of SCD

System Complications

Auditory Cardiovascular

Dermatologic Genitourinary

Growth and development

Sensorineural hearirg loss due to sickling in cochlear vasculature with hair cell damage Cardiomegaly, myocardial ischemia, murmurs, and abnormal ECG; patients with SCD have lower BP than the normal population; normal BP values for SCD should be used for diagnosis of hyoertension ('relative" hypertension); heart failure usually is related to fluid overload Painful leg ulcers; failure to heal occurs in 50%

of patients; recurrences are common Renal papillary necrosis, hematuria, hyposthenuria, proteinuria, nephrotic syndrome, tubular dysfunction, chronic renal failure, impotence Delay in growth (weight and height) and sexual development; decreased fertility; inrreaspd rnmplir,itinn<; during prpgrwnry; depression may be more prevalent than in general population, especially in patients with unstable disease

Hepatic and biliary



Pulmonaiy Renal



Cholelithiasis, biliary sludge, aculc and chronic cholecystitis, and choirs Las is (can be progressive and life-lhreatening)

"Silent" brain lesions cm MM are associated with poor cognitive and fine motor functions; pseudotumor cerebri (rare)

Retinal or vitreous hemorrhage, retinal detachment, transient or permanent visual loss; central retinal veinocclusion

Pulmonary fibrosis, pulmonaiy hypertension, cot pulnritinale

Hematuria, hyposthenuria {inability to concentrate urine maximally), tubular dysfunction, enuresis duringearly childhood, acute renal failure can also occur

Aseptic necrosis of ball-and-socket joints {shoulder and h]p}; prostheses may be needed due to permanent damage; bone marrow hyperplasia resulting in growth disturbances of maxilla and vertebrae

Asplenia (autosplenectomy or surgical splenectomy)


SCD treatment and prevention are considered successful when complications are minimized. The major outcome parameters are a decrease in morbidity and mortality, measured by the number of hospitalizations, and the extent of end-organ damage seen over time. Today, with longer survival for SCD, chronic manifestations of the disease contribute to the morbidity later in life (Table 68-5). Thirty years ago, complications from SCD contributed to high mortality. It was estimated that approximately 50% of patients with SCD did not survive to reach adulthood.4 Since that time data suggest improvement in mortality rates for patients with SCD. The survival age for individuals with HbSS has increased to at least the fifth decade of life. Recent reports suggest 85% survival by 18 years of age.4 SCD is a chronic disease and cannot be cured, except in some patients with transplant.

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