1229 Exam 7: Childhood Anemias

Question 1

CBC

Answer 1

Must be able to evaluate the CBC in order to evaluate the degree of anemia.
RBC: 4.2-5.9 million/mm3
HGB: 13-18 g/dl Male; 12-16 g/dl Female; 14.5-22.5 Newborn
HCT: 42-50% Male; 40-48% Female
MCV: 80-94 mcm3
WBC: 5-10 x 103 cells/mm3
Retic Count: 0.5-1.5%

Question 2

Sickle Cell Anemia

Answer 2

• Constitutes a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sick hemoglobin S.
• Caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain.
• Risk Factors include having parents heterozygous for hemoglobin S or being of African-American descent.

Question 3

Hemoglobin S

Answer 3

• Is sensitive to changes in the oxygen content of the red blood cell.
• Insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow.

Question 4

Clinical Manifestations of Sickle Cell

Answer 4

Primarily occur as a result of obstruction caused by sickled red blood cells and increased red blood cell destruction.

Question 5

Situations that precipitate sickling

Answer 5

Fever
Dehydration
Emotional/Physical stress
Any condition that increases need for O2 or alters the transport of O2 can result in sickle cell crisis (acute exacerbation)

Question 6

Acute Exacerbations of Sickle Cell

Answer 6

Vary considerably in severity and frequency

They include Vaso-Occlusive Crisis, splenic sequestration, and aplastic crisis.

Question 7

Vaso-Occlusive Crisis

Answer 7

Caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infraction
Manifestations: Fever, painful swelling of hands, feet, and joints; and abdominal pain

Question 8

Splenic Sequestration

Answer 8

Caused by pooling and clumping of blood in the spleen (hypersplenism)
Manifestations: Profound anemia, hypovolemia, and shock

Question 9

Aplastic Crisis

Answer 9

Caused by diminished production and increased destruction of red blood cells, triggered by viral infection or depletion of folic acid
Manifestations: Profound anemia and pallor

Question 10

Interventions for Sickle Cell

Answer 10

• Maintain adequate hydration and blood flow through oral and IV administered fluid; electrolyte replacement is also provided as needed
• Administer oxygen and blood transfusions as prescribed to increase tissue perfusion; exchange transfusions, which reduce the number of circulating sickle cells and the risk of complications may also be prescribed.
• Administer analgesics as prescribed (around the clock)

Question 11

Interventions for Sickle Cell cont…

Answer 11

• Assist the child to assume a comfortable position so that the child keeps the extremities extended to promote venous return; elevate the head of the bed no more than 30 degrees, avoid putting strain on painful joints, and do not raise the knee hatch of the bed
• Encourage consumption of high-calorie, high-protein diet, with folic acid supplementation
• Administer antibiotics as prescribed to prevent infection
• Administration of meperidine (Demerol) for pain is avoided because of the risk of normeperidine induced seizures

Question 12

Interventions for SC cont..

Answer 12

• Monitor for signs of complications, including increasing anemia, decreased perfusion, and shock (mental status changes, pallor, vital sign changes)
• Instruct the child and parents about the early S/S of crisis and the measure to prevent crisis
• Ensure that the child receives pneumococcal and meningococcal vaccines and an annual Flu vaccine because of susceptibility to infection secondary to functional asplenia
• A splenectomy may be necessary for clients who experience recurrent splenic sequestration
• Inform parents of the hereditary aspects of the disorder

Question 13

Iron Deficiency Anemia

Answer 13

• Iron stores are depleted, resulting in a decreased supply of iron for the manufacturing of hemoglobin in red blood cells
• Commonly results from blood loss, increased metabolic demands, syndromes of GI malabsorption, and dietary inadequacy

Question 14

Signs and Symptoms of Iron Deficiency

Answer 14

Pallor
Weakness and fatigue
Irritability
Low HGB and HCT levels
Red blood cells that are microcytic and hypochromic

Question 15

Interventions for Iron Deficiency

Answer 15

• Increase oral intake of iron; iron-fortified for mull is needed for an infant
• Instruct the child and parents in food choices that are high in iron (raisins, shellfish, nuts, dried fruits)
• Administer iron supplements as prescribed
• IM injections of iron (using Z-track method) or IV administration of iron may be prescribed in severe cases of anemia

Question 16

Interventions for Iron Deficiency cont…

Answer 16

Teach parents how to administer the iron supplements
-Give iron between meals for maximum absorption
-Give iron supplements with a multivitamin or fruit juice because vitamin C increases absorption
-Do not give iron supplements with milk or antacids because these items decrease absorption
Instruct the child and parents about the side effects of iron supplements (black stools, constipation, and foul aftertaste)
-Liquid iron preparation stains the teeth. Teach the parents and child that liquid iron should be taken through a straw and brush teeth afterwards

Question 17

Aplastic Anemia

Answer 17

• Deficiency of circulating erythrocytes and all other formed elements of blood, resulting from the arrested development of cells within the bone marrow
• It can be primary (at birth) or secondary (acquired)

Question 18

Causes of Aplastic Anemia

Answer 18

Chronic exposure to myelotoxic agents (if this is the cause medication may be D/C'd to improve bone marrow function)
Viruses
Infection
Autoimmune disorders
Allergic states

Question 19

How is Aplastic Anemia diagnosed

Answer 19

Bone Marrow Aspiration (shows conversion of red bone marrow to fatty bone marrow)

Question 20

Therapeutic Management

Answer 20

Focuses on restoring function of the bone marrow and involves immunosuppressive therapy and bone marrow transplantation (treatment of choice if a suitable donor exist)

Question 21

S/S of Aplastic Anemia

Answer 21

Pancytopenia (deficiency of erythrocytes, leukocytes, and thrombocytes)
Petechiae
Pupura
Bleeding
Pallor
Weakness
Tachycardia
Fatigue

Question 22

Interventions for Aplastic Anemia

Answer 22

• Prepare the child for bone marrow transplantation if planned
• Administer immunosuppressive medications as prescribed; antilymphocyte globulin or antihymocyte globulin may be prescribed to suppress the autoimmune response
• Colony-stimulating factors may be prescribed to enhance bone marrow production

Question 23

Interventions for Aplastic Anemia cont..

Answer 23

• Corticosteroids and cyclosporine (sandimmune) may be prescribed
• Administer blood transfusions if prescribed and monitor for transfusion reactions
• Monitor for signs related to the disease and to complications of the treatments and medications administered

Question 24

Hemophilia

Answer 24

A group of bleeding disorders resulting from a deficiency of specific coagulation proteins
-Transmitted as an X-linked recessive disorder (it may also occur as a result of a gene mutation)
Carrier females pass on the defect to affected males; Female offspring are rarely born with the disorder, but they may inherit an affected gene from their mother and are offspring of a father with hemophilia

Question 25

Identifying the specific coagulation deficiency is important so that definitive treatment with the specific replacement agent can be implemented…

Answer 25

Aggressive replacement agent therapy is initiated to prevent the chronic crippling effects from joint bleeding

Question 26

Common types of Hemophilia

Answer 26

They are factor VIII (8) deficiency (Hemophilia A or classic) and factor IX (9) (Hemophilia B or Christmas disease)

Question 27

Treatment for Hemophilia

Answer 27

Replacement of the missing clotting factor; additional medications, such as agents to relieve pain or corticosteroids, may be prescribed depending on the source of bleeding from the disorder

Question 28

S/S of Hemophilia

Answer 28

• Abnormal bleeding in response to trauma or surgery (sometimes detected after circumcision)
• Epistaxis (nosebleeds)
• Joint swelling causing pain, tenderness, swelling, and limited range of motion
• Tendency to bruise easily
• Results of tests that measure platelet function are normal; results of tests that measure clotting factor function maybe abnormal

Question 29

Interventions for Hemophilia

Answer 29

• Monitor for bleeding and maintain bleeding precautions
• Prepare to administer replacement factors as prescribed
• DDAVP (1-deamino-8-D-arginine vasopressin), a synthetic form of vasopressin, increases plasma factor 8 and may be prescribed to treat mild hemophilia

Question 30

Interventions for Hemophilia cont…

Answer 30

• Monitor for joint pain; immobilize the affected extremity if joint pain occurs
• Assess neurological status (child is at risk for intracranial hemorrhage)
• Monitor urine for hematuria
• Control joint bleeding by immobilization, elevation, and application of ice; apply pressure (15 minutes) superficial bleeding

Question 31

Interventions for Hemophilia cont…

Answer 31

• Instruct the child and parents about the signs of internal bleeding
• Instruct parents in how to control the bleeding
• Instruct parents regarding activities for the child, emphasizing the avoidance of contact sports and the need for protective devices while learning to walk; assist in developing an appropriate exercise plan
• Instruct the child to wear protective devices such as helmets and knee and elbow pads when participating in sports such as bicycling and skating

Question 32

Von Willebrand’s Disease

Answer 32

Hereditary bleeding disorder that occurs in males and females and is characterized by a deficiency of or a defect in protein termed von Willebrand factor

• Causes platelets to adhere to damaged endothelium; the von Willebrand factor protein also serves as a carrier protein for factor VIII (8)
• Characterized by an increased tendency to bleed from mucous membranes

Question 33

Assessment of Von Willebrand’s Disease

Answer 33

Epitaxis
Gum bleeding
Easy bruising
Excessive menstrual bleeding

Question 34

Interventions for Von Willebrand’s Disease

Answer 34

Treatment and care are similar to measures implemented for hemophilia, including administration of clotting factors

• Provide emotional support to the child and parents, especially if the child is experiencing an episode of bleeding
• A child with a bleeding disorder needs to wear a medic-alert bracelet

Question 35

Beta-Thalassemia Major

Answer 35

-Autosomal recessive disorder characterized by the reduced production of one of the glob in chains in the synthesis of hemoglobin (both parents must be carriers to produce a child with Beta-Thalassemia major)

Question 36

Risk Factors for BTM

Answer 36

Mediterranean descent

• Greek
• Italian
• Syrians

Question 37

Treatment for Von Willebrand’s

Answer 37

Treatment is supportive

• The goal of the therapy is to maintain normal hemoglobin levels by the administration of blood transfusions
• Bone marrow transfusion may be offered as an alternative therapy
• A splenectomy may be performed in a child with severe splenomegaly who requires repeated transfusions (assists in relieving abdominal pressure and may increase the life span of supplemental red blood cells)

Question 38

Assessment for Von Willebrand’s

Answer 38

Frontal bossing
Maxillary prominence
Wide-set eyes with a flattened nose 
Greenish yellow skin color (Jaundice)
Hepatosplenomegaly
Severe anemia
Mircocytic, hypochromic red blood cells

Question 39

Interventions for Von Willebrand’s

Answer 39

• Administer blood transfusions as prescribed; monitor for transfusion reactions
• Monitor for iron overload; chelation therapy with defasirox (Exjade) or deferoxamine (Desferal) may be prescribed to treat iron overload and to prevent organ damage from the elevated levels of iron caused by the multiple transfusion therapy

Question 40

Interventions for Von Willebrand’s cont…

Answer 40

• If the child has had a splenectomy, instruct parents to report any signs of infection because of the risk of sepsis
• Ensure that parents understand the importance of the child receiving pneumococcal and minigococcal vaccines in addition to an annual Flu vaccine and any regularly scheduled vaccines
• Provide genetic counseling to parents

Question 41

Kind of read over the notes! I may have missed something! I did not put the Nursing Diagnoses in the cards

Answer 41

GOOD LUCK!!!!