1229 Exam 7: Childhood Anemias Flashcards
CBC
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%
Sickle Cell Anemia
- 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.
Hemoglobin S
- 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.
Clinical Manifestations of Sickle Cell
Primarily occur as a result of obstruction caused by sickled red blood cells and increased red blood cell destruction.
Situations that precipitate sickling
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)
Acute Exacerbations of Sickle Cell
Vary considerably in severity and frequency
They include Vaso-Occlusive Crisis, splenic sequestration, and aplastic crisis.
Vaso-Occlusive Crisis
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
Splenic Sequestration
Caused by pooling and clumping of blood in the spleen (hypersplenism)
Manifestations: Profound anemia, hypovolemia, and shock
Aplastic Crisis
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
Interventions for Sickle Cell
- 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)
Interventions for Sickle Cell cont…
- 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
Interventions for SC cont..
- 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
Iron Deficiency Anemia
- 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
Signs and Symptoms of Iron Deficiency
Pallor Weakness and fatigue Irritability Low HGB and HCT levels Red blood cells that are microcytic and hypochromic
Interventions for Iron Deficiency
- 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
Interventions for Iron Deficiency cont…
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
Aplastic Anemia
- 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)
Causes of Aplastic Anemia
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
How is Aplastic Anemia diagnosed
Bone Marrow Aspiration (shows conversion of red bone marrow to fatty bone marrow)
Therapeutic Management
Focuses on restoring function of the bone marrow and involves immunosuppressive therapy and bone marrow transplantation (treatment of choice if a suitable donor exist)
S/S of Aplastic Anemia
Pancytopenia (deficiency of erythrocytes, leukocytes, and thrombocytes) Petechiae Pupura Bleeding Pallor Weakness Tachycardia Fatigue
Interventions for Aplastic Anemia
- 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
Interventions for Aplastic Anemia cont..
- 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
Hemophilia
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
Identifying the specific coagulation deficiency is important so that definitive treatment with the specific replacement agent can be implemented…
Aggressive replacement agent therapy is initiated to prevent the chronic crippling effects from joint bleeding
Common types of Hemophilia
They are factor VIII (8) deficiency (Hemophilia A or classic) and factor IX (9) (Hemophilia B or Christmas disease)
Treatment for Hemophilia
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
S/S of Hemophilia
- 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
Interventions for Hemophilia
- 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
Interventions for Hemophilia cont…
- 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
Interventions for Hemophilia cont…
- 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
Von Willebrand’s Disease
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
Assessment of Von Willebrand’s Disease
Epitaxis
Gum bleeding
Easy bruising
Excessive menstrual bleeding
Interventions for Von Willebrand’s Disease
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
Beta-Thalassemia Major
-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)
Risk Factors for BTM
Mediterranean descent
- Greek
- Italian
- Syrians
Treatment for Von Willebrand’s
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)
Assessment for Von Willebrand’s
Frontal bossing Maxillary prominence Wide-set eyes with a flattened nose Greenish yellow skin color (Jaundice) Hepatosplenomegaly Severe anemia Mircocytic, hypochromic red blood cells
Interventions for Von Willebrand’s
- 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
Interventions for Von Willebrand’s cont…
- 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
Kind of read over the notes! I may have missed something! I did not put the Nursing Diagnoses in the cards
GOOD LUCK!!!!
