A pediatric nurse health educator provides a teaching session to the nursing staff regarding hemophilia. Which of the following information regarding this disorder would the nurse plan to include in the discussion?
-
Solution
Hemophilia A results from a deficiency of factor VIII
Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX.
Option A: Hemophilia is inherited in a recessive manner via a genetic defect on the X-chromosome.
Options B and C: Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers.
Laboratory studies are performed for a child suspected of having iron deficiency anemia. The nurse reviews the laboratory results, knowing that which of the following results would indicate this type of anemia?
-
Solution
Red blood cells that are microcytic and hypochromic
The results of a CBC in children with iron deficiency anemia will show decreased hemoglobin levels and microcytic and hypochromic red blood cells. The red blood cell count is decreased. The reticulocyte count is usually normal or slightly elevated.
A clinic nurse instructs the mother of a child with sickle cell disease about the precipitating factors related to pain crisis. Which of the following, if identified by the mother as a precipitating factor, indicates the need for further instructions?
-
Solution
Fluid overload
Pain crisis may be precipitated by infection, dehydration, hypoxia, trauma, or physical or emotional stress. The mother of a child with sickle cell disease should encourage fluid intake of 1 ½ to 2 times the daily requirement to prevent dehydration.
A child suspected of having sickle cell disease is seen in a clinic, and laboratory studies are performed. A nurse checks the lab results, knowing that which of the following would be increased in this disease?
-
Solution
Reticulocyte count
A diagnosis is established based on a complete blood count, examination for sickled red blood cells in the peripheral smear, and hemoglobin electrophoresis. Increased reticulocyte counts occur in children with sickle cell disease because the life span of their sickled red blood cells is shortened.
Options A, B, and D: Laboratory studies will show decreased hemoglobin and hematocrit levels and a decreased platelet count, and increased reticulocyte count, and the presence of nucleated red blood cells.
Because of the risks associated with administration of factor VIII concentrate, the nurse would teach the client’s family to recognize and report which of the following?
-
Solution
Yellowing of the skin
Because factor VIII concentrate is derived from large pools of human plasma, the risk of hepatitis is always present. Clinical manifestations of hepatitis include yellowing of the skin, mucous membranes, and sclera.
Options B, C, and D: Use of factor VIII concentrate is not associated with constipation, abdominal distention, or puffiness around the eyes.
Which of the following assessments in a child with hemophilia would lead the nurse to suspect early hemarthrosis?
-
Solution
Child’s reluctance to move a body part
Bleeding into the joints in the child with hemophilia leads to pain and tenderness, resulting in restricted movement. Therefore, an early sign of hemarthrosis would be the child’s reluctance to move a body part.
Option B: If the bleeding into the joint continues, the area becomes hot, swollen, and immobile—not cool, pale, and clammy.
Option C: Ecchymosis formation around a joint would be difficult to assess.
Option D: Instability of a long bone on passive movement is not associated with joint hemarthrosis.
The physician has ordered several laboratory tests to help diagnose an infant’s bleeding disorder. Which of the following tests, if abnormal, would the nurse interpret as most likely to indicate hemophilia?
-
Solution
Partial thromboplastin time (PTT)
PTT measures the activity of thromboplastin, which is dependent on intrinsic clotting factors. In hemophilia, the intrinsic clotting factor VIII (antihemophilic factor) is deficient, resulting in a prolonged PTT.
Option A: Bleeding time reflects platelet function.
Option B: the tourniquet test measures vasoconstriction and platelet function.
Option C: Clot retraction test measures capillary fragility. All of these are unaffected in people with hemophilia.
Which of the following foods would the nurse encourage the mother to offer to her child with iron deficiency anemia?
-
Solution
Potato, peas, and chicken
Potato, peas, chicken, green vegetables, and rice cereal contain significant amounts of iron and therefore would be recommended. Milk and yellow vegetables are not good iron sources. Rice by itself also is not a good source of iron.
Which statements by the mother of a toddler would lead the nurse to suspect that the child has iron-deficiency anemia? Select all that apply.
-
Solution
Answers: A,B.
Toddlers should have between 2 and 3 cups of milk per day and 8 ounces of juice per day. If they have more than that, then they are probably not eating enough other foods, including iron-rich foods that have the needed nutrients.
A mother asks the nurse if her child’s iron deficiency anemia is related to the child’s frequent infections. The nurse responds based on the understanding of which of the following?
-
Solution
Children with iron deficiency anemia are more susceptible to infection than are other children.
Children with iron-deficiency anemia are more susceptible to infection because of marked decreases in bone marrow functioning with microcytosis.