Pediatric Nursing: Hematological Problems Flashcards
1
Q
Sickle Cell Anemia: description
A
- constitutes a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S.
- caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain.
- risk factors include parents heterozygous for hemoglobin S or being of African American descent.
- Sickledex is frequently used, performed from a fingerstick and provide accurate results in 3 min. However, if positive Hgb electrophoresis is necessary to help distinguish a trait from the disease.
- hemoglobin S is sensitive to changes in the oxygen content of 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.
- situations that precipitate sickling: fever, dehydration, and emotional or physical stress; any condition that increases the need for oxygen or alters transport of oxygen.
- acute exacerbations vary in frequency and severity; these include vaso-occlusive crisis, splenic sequestration, hyperhemolytic crisis, and aplastic crisis.
- response is reversible under conditions of adequate oxygenation and hydration; after repeated sickling, the cell becomes permanently sickled.
- Care focuses on the prevention and treatment of the crisis include hydration, oxygen, pain management, and bed rest.
2
Q
Sickle Cell Anemia: Assessment
A
-Vaso-occlusive crisis: caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infarction.
= Fever, painful swelling of hands, feet, and joints; and abdominal pain.
-Splenic sequestration: caused by pooling of blood in the spleen.
= profound anemia, hypovolemia, and shock.
-Hyperhemolytic crisis: an accelerated rate of red blood cell destruction.
= anemia, jaundice, and reticulocytosis.
-Aplastic crisis: caused by diminished production and increased destruction of red blood cells, triggered by viral infection or depletion of folic acid.
= profound anemia and pallor.
3
Q
Sickle Cell Anemia: Interventions
A
- maintain adequate hydration (OR or IV).
- adm O2, analgesics, blood transfusions and ATB as prescribed.
- assist the child into a comfortable position with extremities extended to promote venous return, and elevate head of the bed no more than 30 degrees.
- encourage high-calorie and protein diet with folic acid supplementation.
- monitor signs of complications and instruct parents on measures to prevent crisis.
- ensure child receives pneumococcal and meningococcal vaccines and an anual influenza vaccine, because of susceptibility to infections.
- splenectomy may be necessary.
- adm of meperidine for pain is avoided because of the risk of seizures.
4
Q
Hemophilia: description and assessment
A
- refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins.
- identifying the specif coagulation deficiency is important to have a definitive treatment.
- most common types are factor VIII (hemophilia A or classic) and factor IX (hemophilia B or Christmas disease).
- transmitted as an X-linked recessive disorder (or gene mutation)
- carrier females pass on the defect to males; females are rarely born with the disorder but may be if they inherit an affected gene from their mother and are offspring of a father with hemophilia.
- primary treatment is replacement of the missing clotting factor.
- assessment: abnormal bleeding in response to trauma or surgery, epistaxis, joint bleeding causing pain, tenderness, swelling, and limited range of motion. Tendency to bruise easily. Lab results show platelet function normal but clotting factor function may be abnormal.
5
Q
Hemophilia: Interventions
A
- monitor for bleeding and maintain precautions.
- prepare to adm factor VIII or plasma as prescribed.
- DDAVP (synthetic form of vasopressin) increases plasma factor VIII and may be prescribed.
- monitor for joint pain and immobilize the affected extremity.
- monitor urine for hematuria
- control joint bleeding by immobilization, elevation, and application of ice; apply pressure (15 min) for superficial bleeding.
- instruct parents regarding activities for the child and to wear protective devices.
6
Q
Von Willebrand’s Disease
A
- is a hereditary bleeding disorder that is characterized by a deficiency of or a defect in a protein termed von Willebrand factor.
- causes platelets to adhere to damaged endothelium.
- servers as a protein carrier for factor VIII.
- characterized by an increased tendency to bleed from mucous membranes.
- assessment: epistaxis, gum bleeding, easy bruising, excessive menstrual bleeding.
- interventions: treatment and care are similar to measures implemented for hemophilia, including adm of clotting factors.
7
Q
B-Thalassemia Major: description and assessment
A
- is an autosomal recessive disorder characterized by the reduced production of 1 of the globin chains in the synthesis of hemoglobin (both parents must be carriers).
- incidence is higher in Mediterranean descent, such as Italians, Greeks, Syrians, and their offspring.
- treatment is supportive and the goal is to maintain normal Hb levels by adm of blood transfusion.
- bone marrow transplant may be an alternative therapy.
- splenectomy may be performed in a child with severe splenomegaly who requires repeated transfusions.
- assessment: frontal bossing, maxillary prominence, wide-set eyes with flattened nose, greenish yellow skin tone, hepatosplenomegaly, severe anemia, microcytic, hypochromic red blood cells.
8
Q
B-Thalassemia Major: interventions
A
- blood transfusions as prescribed
- monitor for iron overload; chelation therapy with deferasirox or deferoxamine may be prescribed to treat iron overload and to prevent organ damage from the elevated levels of iron caused by the multiple transfusion therapy.
- if child has had splenectomy, instruct parents to report any signs of infection.
- ensure child receives pneumococcal and meningococcal vaccines and an anual influenza vaccine, because of susceptibility to infections.
