Care of the Childbearing Family – NURS 420
Care of the Childbearing Family
Alterations in Hematology
Active Learning Guide – Module 6
Name: Kayla Boub
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
Instructions: Answer the following “Think Critically” questions from page 155 of Tagher, G., & Knapp, L.
(2020). Pediatric Nursing: A case-based approach. 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 that involves the presence of an abnormal Hgb sickle (Hgb
S) gene, which results in a sickle-shaped RBC. They tend to clump together, leading to occluded
blood vessels, impaired oxygen delivery, pain crises, premature RBC death and anemia. It’s
important to receive genetic counseling to understand how this disease is transmitted and the
risks of having a child with sickle cell disease.
2. What are the signs and symptoms of a vasoocclusive crisis?
A vasoocclusive crisis is often the first manifestation of sickle cell disease; hospitalization in
children and adolescents with sickle cell disease is common due to the intensity of pain.
Vasoocclusion causes lack of oxygen to tissues that leads to ischemic infarcts at the site of the
occlusion. This results in cellular death anywhere the blood flows and causes extreme pain.
Common areas in children younger than 5 include the hands and feet where the infarct of the
bone marrow spaces present edematous, erythematous extremities known as dactylitis. Dactylitis
can lead to ulcerations of tissues. Another common area for vasoocclusive crisis in toddlers and
preschoolers is the brain causing neurological tissue injury. Neurological assessments are
indicated for children with sickle cell anemia because they may have a stroke or silent cerebral
ischemia causing debilitating paralysis, aphasia, seizure, headache, facial droop and even death.
3. What are three ways patients with sickle cell disease can lower their risk of a vasoocclusive crisis?
Updated 3.28.22ev
, Care of the Childbearing Family – NURS 420
Care of the Childbearing Family
Three ways patients with sickle cell disease can lower their risk of hypoxia, infections that produce
fever and acidosis, and dehydration. Preventing hypoxia by maintaining oxygen saturation levels
at 93% or above through transfusions before procedures allows for higher concentrations of non-
sickle than sickling cells. Keeping the percentage of sickle cells in the circulatory flow below 30%
and the Hgb volume greater than 10 g/dL lowers the risk of vasoocclusive crisis. Administration of
prophylactic antibiotics and regular vaccines is a manner in which reduces the risk for infection;
infection can cause aplastic crisis. Hydration is another manner in which patients can prevent
vasoocclusion by moving RBCs through vessels interfering with sickled cells aggregating.
Maintaining the child’s fluid intake at 1,600 mL/m2 or at 1.5 times the normal fluid needs for age
and weight can help lower the risk of a vasoocclusion crisis.
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?
It is important for a patient with sickle cell disease to be seen early when a fever presents or signs
of a respiratory infection are present because these are signs of acute chest syndrome (ACS). ACS
occurs due to sickling occlusion in the pulmonary vessels, emboli from infarcted bone marrow, or
an infectious change that damages the lung tissue. It is considered a medical emergency.
Manifestations include chest pain, hypoxemia, dyspnea, tachycardia, cough, fever, wheezing,
radiographic infiltrates and respiratory distress.
5. What is the priority of care for a patient diagnosed with ACS?
Priority of care for a patient diagnosed with ACS is ensuring that their airway is patent and to
monitor respiratory status and level of consciousness. Teaching and encouraging the use of
incentive spirometry helps with maintaining respiratory function. Pain management would come
after due to its intense pain.
6. What effect can dehydration have on a patient with sickle cell disease?
Dehydration inhibits RBCs from moving through the vessels and the vascular bed causing them to
aggregate and obstruct microcirculation; hydration allows improved blood flow through the
vessels thus reducing the risk for vasoocclusion. Teaching the parents to provide the child with
1,600 mL/m2 or 1.5x the normal fluid needs for the child’s age and weight.
Tables
Instructions: Complete the tables below using information from your readings.
Table 7. Hematology
Diagnosis Etiology Clinical Presentation Nursing Interventions
Sickle Cell anemia ● abnormal sickle ● reports of ● promote rest
cell hemoglobin pain ● administer oxygen
replaces normal ● shortness of ● monitor I&O, give
adult hemoglobin breath/fatigu oral fluids,
● RBCs are “sickle e administer IV
shaped” which ● pallor, pale electrolyte
can lead to mucous replacement
increased blood membranes ● administer blood
viscosity, ● jaundice products (RBCs)
Updated 3.28.22ev
Care of the Childbearing Family
Alterations in Hematology
Active Learning Guide – Module 6
Name: Kayla Boub
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
Instructions: Answer the following “Think Critically” questions from page 155 of Tagher, G., & Knapp, L.
(2020). Pediatric Nursing: A case-based approach. 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 that involves the presence of an abnormal Hgb sickle (Hgb
S) gene, which results in a sickle-shaped RBC. They tend to clump together, leading to occluded
blood vessels, impaired oxygen delivery, pain crises, premature RBC death and anemia. It’s
important to receive genetic counseling to understand how this disease is transmitted and the
risks of having a child with sickle cell disease.
2. What are the signs and symptoms of a vasoocclusive crisis?
A vasoocclusive crisis is often the first manifestation of sickle cell disease; hospitalization in
children and adolescents with sickle cell disease is common due to the intensity of pain.
Vasoocclusion causes lack of oxygen to tissues that leads to ischemic infarcts at the site of the
occlusion. This results in cellular death anywhere the blood flows and causes extreme pain.
Common areas in children younger than 5 include the hands and feet where the infarct of the
bone marrow spaces present edematous, erythematous extremities known as dactylitis. Dactylitis
can lead to ulcerations of tissues. Another common area for vasoocclusive crisis in toddlers and
preschoolers is the brain causing neurological tissue injury. Neurological assessments are
indicated for children with sickle cell anemia because they may have a stroke or silent cerebral
ischemia causing debilitating paralysis, aphasia, seizure, headache, facial droop and even death.
3. What are three ways patients with sickle cell disease can lower their risk of a vasoocclusive crisis?
Updated 3.28.22ev
, Care of the Childbearing Family – NURS 420
Care of the Childbearing Family
Three ways patients with sickle cell disease can lower their risk of hypoxia, infections that produce
fever and acidosis, and dehydration. Preventing hypoxia by maintaining oxygen saturation levels
at 93% or above through transfusions before procedures allows for higher concentrations of non-
sickle than sickling cells. Keeping the percentage of sickle cells in the circulatory flow below 30%
and the Hgb volume greater than 10 g/dL lowers the risk of vasoocclusive crisis. Administration of
prophylactic antibiotics and regular vaccines is a manner in which reduces the risk for infection;
infection can cause aplastic crisis. Hydration is another manner in which patients can prevent
vasoocclusion by moving RBCs through vessels interfering with sickled cells aggregating.
Maintaining the child’s fluid intake at 1,600 mL/m2 or at 1.5 times the normal fluid needs for age
and weight can help lower the risk of a vasoocclusion crisis.
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?
It is important for a patient with sickle cell disease to be seen early when a fever presents or signs
of a respiratory infection are present because these are signs of acute chest syndrome (ACS). ACS
occurs due to sickling occlusion in the pulmonary vessels, emboli from infarcted bone marrow, or
an infectious change that damages the lung tissue. It is considered a medical emergency.
Manifestations include chest pain, hypoxemia, dyspnea, tachycardia, cough, fever, wheezing,
radiographic infiltrates and respiratory distress.
5. What is the priority of care for a patient diagnosed with ACS?
Priority of care for a patient diagnosed with ACS is ensuring that their airway is patent and to
monitor respiratory status and level of consciousness. Teaching and encouraging the use of
incentive spirometry helps with maintaining respiratory function. Pain management would come
after due to its intense pain.
6. What effect can dehydration have on a patient with sickle cell disease?
Dehydration inhibits RBCs from moving through the vessels and the vascular bed causing them to
aggregate and obstruct microcirculation; hydration allows improved blood flow through the
vessels thus reducing the risk for vasoocclusion. Teaching the parents to provide the child with
1,600 mL/m2 or 1.5x the normal fluid needs for the child’s age and weight.
Tables
Instructions: Complete the tables below using information from your readings.
Table 7. Hematology
Diagnosis Etiology Clinical Presentation Nursing Interventions
Sickle Cell anemia ● abnormal sickle ● reports of ● promote rest
cell hemoglobin pain ● administer oxygen
replaces normal ● shortness of ● monitor I&O, give
adult hemoglobin breath/fatigu oral fluids,
● RBCs are “sickle e administer IV
shaped” which ● pallor, pale electrolyte
can lead to mucous replacement
increased blood membranes ● administer blood
viscosity, ● jaundice products (RBCs)
Updated 3.28.22ev
