Question

Chapter 32

Case Study: Bethany Brown

Scenario: Five-year-old Bethany is admitted to the pediatric unit with a diagnosis of hereditary spherocytosis. Bethany’s past medical history is significant for hyperbilirubinemia, intermittent anemia, and splenomegaly. The nurse is aware that spherocytosis is a hereditary disorder and notes that Bethany’s father has the disease.

The nurse notes that Bethany’s spleen is 4 centimeters below the left costal margin and no hepatomegaly is noted. Her bowel sounds are active in all four quadrants. Bethany’s sclera is slightly jaundiced. Her lungs are clear to auscultation, with respirations regular and unlabored. Bethany’s pulse rate is 120 beats per minute, with regular heart sounds and no murmur. Bethany’s skin is free from lesions or bruising, with no pallor noted. Her skin turgor is elastic and capillary refill is less than 3 seconds.

Today, a venipuncture complete blood count (CBC) reveals that Bethany’s hemoglobin level is 7 g/dL. Bethany’s immunization record is up to date. The only medication she takes is a folic acid supplement each day.

Bethany has severe hereditary spherocytosis and is now a candidate for a splenectomy. Her spectrin content, which is a protein that is important in maintenance of cell shape, is less than 80% of normal. A splenectomy should eliminate Bethany’s anemia and improve red cell survival.

Critical Thinking Questions

1. Are there any immunizations that Bethany should receive prior to her splenectomy?

2. Will Bethany require any medication once her spleen has been removed?

3. What is the priority nursing diagnosis at this time?    

4. What are the expected nursing outcomes related to this scenario?

5. Discuss the nursing interventions related to this scenario.

Answer 1

1) Patients should receive pneumococcal vaccine from 4 to 6 weeks before elective splenectomy or initiation of chemotherapy or radiotherapy. If it is not possible, vaccination should be administered at least 2 weeks pre-operatively in elective cases or at least 2 weeks post-operatively in emergency cases.

2) Post-operative antibiotics (after splenectomy)

ORAL: either (penicillin V 666mg po q12h) OR (amoxicillin 250-500mg q12h po)

OR IV Benzylpenicillin 1.2g q12h if oral route not available

In penicillin allergy: clarithromycin 250mg q12h po or IV if oral route unavailable.

3) The nursing diagnosis is Hereditary spherocytosis.

4) Anemia, hepato-splenomegaly and jaundice are commonest clinical features of hereditary spherocytosis. Post-splenectomy sepsis is uncommon.

5) The treatment for hereditary spherocytosis is individualized and may require transfusions, folic acid administration, full or partial splenectomy, and/or cholecystectomy. Complications of spherocytosis may include megaloblastic crisis, low folic acid levels, splenomegaly, and/or gallbladder problems.