Pediatric Nursing
Alterations in Hematology
Active Learning Guide – Module 6
Name:
Instructions: Complete the module active learning guide as you work through the module content. Take
notes and answer the following questions to prepare for Exams. You will upload your completed module
active learning guide on the corresponding assignment page in Canvas. If you have questions or are
unsure about your answers; you may email your instructor for clarification.
Note: The Active Learning Guide provides a general outline of topics covered in this module; it is not all
inclusive of all information needed for the exam. You are responsible for all content in readings and
activities throughout the module.
Think Critically Chapter 12
Instructions: Answer the following “Think Critically” questions from page 163 of Tagher, G., & Knapp, L.
(2023). Pediatric Nursing: A case-based approach. (2nd ed.). Wolters Kluwer.
Chapter Questions
1. A parent asks, “What is sickle cell disease?” How would you respond?
Sickle cell disease is a group of diseases associated with the presence of an abnormal. The Hgb
gene, known as the Hgb sickle (Hgb S) gene, results in sickle-shaped RBCs that tend to clump
together, leading to occluded blood vessels, impaired oxygen delivery, pain crises, premature
RBC death, and anemia
2. What are the signs and symptoms of a vasoocclusive crisis?
This is a painful crisis caused by malocclusion. The vas occlusion robs the tissues of oxygen. It
results in ischemic infarcts at the occlusion site, ensuing in cellular death, which is extremely
painful and can occur anywhere the blood flows. In children younger than five years of age, the
Vaso occlusive crisis frequently affects the bones in the hands and feet, resulting in dactylitis.
The infarct of the bone marrow spaces produces edematous, erythematous extremities.
Dactylitis is very painful and can lead to ulcerations of the tissues, similar to venous and arterial
insufficiency ulcers.
3. What are three ways patients with sickle cell disease can lower their risk of
a vasoocclusive crisis?
One way patients with sickle cell disease can lower their risk of vaso occlusive crisis is to
maintain hydration levels. To help ensure that the child takes in the required amount, the daily
total can be divided by the number of hours the child is awake to provide an hourly intake goal.
Another way to avoid a vasoocclusive crisis is to avoid potential triggers for hypoxia, such as high
altitudes and planes that are not pressurized. A third way to prevent a vasoocclusive crisis is to
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, NURS 420
Pediatric Nursing
practice infection control. The child should be taught to use hand sanitizer and to wash hands
frequently. The family should be educated to seek medical attention promptly when the child
has a fever of 100.5°F to 101°F or more significant or demonstrates signs of infection.
4. Why is it important for a patient with sickle cell disease to be seen early when a fever or signs of
a respiratory infection are present?
Viral illnesses, many of which may be prevented with immunizations, can trigger an aplastic
crisis, an autoimmune response following a viral infection that stops bone marrow production.
Hyperhemolytic crisis is also associated with an infectious process that causes the RBCs to break
down excessively.
5. What is the priority of care for a patient diagnosed with ACS?
Acute chest syndrome (ACS) is a life-threatening pulmonary complication of sickle cell disease
triggered by an occlusion in the pulmonary blood vessels, leading to symptoms of chest pain,
fever, and difficulty breathing and typically manifesting as a new infiltrate on a chest X-ray.
Management of ACS includes pain control, IV fluids, antibiotics (if related to illness),
supplemental oxygen, and blood transfusions.
6. What effect can dehydration have on a patient with sickle cell disease?
Hydration is essential because it helps prevent vas occlusion by moving the RBCs through the
vessels and vascular bed, which prevents sickled cells from aggregating and clogging the
microcirculation. During a vasoocclusive crisis, intravenous hydration with an isotonic solution
increases the hydrostatic pressure and volume and improves blood flow. Prevent dehydration
by maintaining the child’s fluid intake at 1,600mL/m2 or 1.5 times the normal fluid needs for age
and weight. If using the Holliday-Segar formula for fluid requirements, calculate the child’s fluid
needs and multiply it by 1.5.
Tables
Instructions: Complete the tables below using information from your readings.
Table 7. Hematology
Diagnosis Etiology Clinical Presentation Nursing Interventions
Sickle Cell anemia Occurs when a Vasoocclusive IV fluids
person inherits crisis Oxygen
two abnormal Hgb Acute chest Pain
genes instead of syndrome medication
two normal adult Hydroxy urea
Hgb (Hgb A)
genes, with at
least one of the
two abnormal
genes being the
Hgb sickle (Hgb S)
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