Describe the pathophysiology of a sickle cell crisis


Pediatric Class Project- Critical Thinking Case Study

Nishais a 14-year old with sickle cell anemia. She lives with her mother and grandmother in a rural neighborhood. Nisha has experienced several "sickle cell crises", however, they seem to have become more frequent since she became an adolescent. Even though her crises are more frequent than when she was younger, she hasn't had a crisis in over 8 months. Nisha is enjoying her summer break from school. She is active in a summer softball league and enjoys shopping with her girlfriends.

Nisha's mother brings her to the hematology clinic at the hospital with complaints of severe generalized pain following a softball game in which she pitched seven innings. Her vital signs are: temperature, 37.6° C (99.7° F); pulse 110 beats/minute; respirations, 30 breaths/minute; and blood pressure, 96/70. Her oxygen saturation is 89% on room air. She weighs 110 lbs and is 5'6". She states her pain is "all over," but especially in abdomen, and rates her pain at "9." Nisha takes hydroxyurea (Hydrea) 1000 mg daily. She is up to date with all immunizations.

The physician orders oxygen per nasal cannula at 2 L per minute, an IV fluid bolus of D5 ¼ NS to run over 2 hours. (Administer 10mL/kg over 2 hours). Once IV access is established, administer morphine 0.2mg/kg every 2-4 hours PRN pain.

Her complete blood count reveals: hemoglobin 9 g/dL; hematocrit 25%; white blood cell count 12,000 cells/ mm3; and platelet count 140,000 cells/ mm3. Basic metabolic panel reveals a few abnormal values with an osmolality of 299 mOsm/L, sodium 146 mEq/L, and potassium 4.7 mEq/L. A chest x-ray does not reveal any active disease and her urinalysis is normal.

Update: 30 minutes after the morphine, Nisha's pain is rated at "1," and her oxygen saturation is 99%. Her physician orders a one-time dose of ceftriaxone IV 50 mg/kg piggy bag to run over 30 minutes. She will discharged to hours after the ceftriaxone infuses, providing she has no adverse reactions.

Questions:

1. Is there sufficient evidence to draw any conclusions at this time about Nisha's condition? Please explain your answer in detail.

2. Describe the pathophysiology of a sickle cell crisis (vaso-occlusive). (A definition is unacceptable.)

3. Identify clinical manifestations (signs and symptoms) of a sickle cell (vaso-occlusive) crisis.

4. Nisha pitched 7 innings in a softball game, then developed severe pain. Did her recent activity lead to a sickle cell crisis? Please explain your answer.

5. How many total ml of D5 ¼ NS will Nisha receive? How many ml/hour will the IV pump be set at in order to deliver the prescribed amount in the time frame ordered? Round to the nearest tenth.

6. Medications: (show your calculation work)

• How many mg/dose of morphine will the nurse administer? Round to the nearest tenth.
• How many mg of ceftriaxone will the nurse administer? Round to the nearest tenth

7. Sickle cell disease can affect a child's growth. Determine Nisha's weight, height, and body mass index (BMI) percentile using the forms provided. Is Nisha's growth normal for age?

8. Create a nursing concept map for Nisha's sickle cell (vaso-occlusive) crisis: (form provided)

a. 4 priority nursing diagnoses
b. Outcome for each nursing diagnosis
c. 4 interventions for each nursing diagnosis

9. Develop a teaching plan/discharge instructions for Nisha and her mother regarding sports/activity and the risk for dehydration as they relate to the patient with sickle cell disease. (form provided)

10. Complete an Active Learning Medication sheet for hydroxyurea (Hydrea). (form provided)

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