hematologic disorders

von Willebrand disease

The primary clinical manifestations of disease are bruising and mucous membrane bleeding from the nose, mouth, and gastrointestinal tract; bleeding may be severe and lead to anemia and shock. Deep bleeding into joints and muscles, like that seen in hemophilia, is rare, except with type III von Willebrand disease.

The nurse is caring for a child in sickle cell crisis. To best promote hemodilution, the nurse would expect to administer how much fluid per day intravenously or orally?

To promote hemodilution in sickle cell crisis, the nurse would provide 150 mL/kg of fluids per day or as much as double maintenance, either orally or intravenously.

Ecchymosis

areas of bruising.

Purpura

larger areas of hemorrhage in which blood collects under the tissues and appear purple in color.

Idiopathic thrombocytopenic purpura (ITP)

primarily an autoimmune disease, which is an acquired, self-limiting disorder of hemostasis characterized by destruction and decreased numbers of circulating platelets.

You care for a 4-year-old with sickle cell anemia. A physical finding you might expect to see in him is:

slightly yellow sclerae. Explanation: Many children with sickle cell anemia develop mild scleral yellowing from excess bilirubin from breakdown of damaged cells.

In discussing the causes of iron deficiency anemia in children with a group of nurses, the following statements are made. Which of these statements is a misconception related to iron deficiency anemia?

"Milk is a perfect food, and babies should be able to have all the milk they want." Explanation: Babies with an inordinate fondness for milk can take in an astonishing amount and, with their appetites satisfied, may show little interest in solid foods. These babies are prime candidates for iron deficiency anemia.

An 8-month-old girl appears pale, irritable, and anorexic. On blood testing, the red blood cells are hypochromic and microcytic. The hemoglobin level is less than 5 g/100 mL, and the serum iron level is high. Which symptom should the nurse most expect as a result of excessive iron deposits?

An enlarged spleen Explanation: The child with thalassemia major may have both an enlarged spleen and liver due to excessive iron deposits and fibrotic scarring in the liver and the spleen's increased attempts to destroy defective RBCs

After teaching a group of students about hemophilia, the instructor determines that the students have understood the information when they identify hemophilia A as involving a problem with:

Factor VIII Explanation: In hemophilia A, the problem is with factor VIII, and in hemophilia B it is factor IX.

The child with thalassemia may be given which classification of medication to prevent one of the complications frequently seen with the treatment of this disorder?

Frequent transfusions can lead to complications and additional concerns for the child, including the possibility of iron overload. For these children, iron-chelating drugs such as deferoxamine mesylate (Desferal) may be given.

Which nursing diagnosis would be most appropriate for a child with idiopathic thrombocytopenic purpura?

Ineffective tissue perfusion related to poor platelet formation Explanation: Idiopathic thrombocytopenic purpura results in decreased platelets, so bleeding into tissue can occur.

A school-aged child is admitted to the hospital with a vaso-occlusive sickle cell crisis. Which measure in his care should be given priority?

Maintaining a fluid intravenous line Explanation: Dehydration increases sickling of cells, so maintaining fluid balance is important

petechiae

Petechiae are pinpoint hemorrhages that occur anywhere on the body and do not blanch with pressure.

β-Thalassemia major.

Severe hemolytic anemia and chronic hypoxia, icteral sclerae, jaundice of the skin, and frontal and maxillary bossing are signs and symptoms of β-thalassemia major.

A nurse is preparing a 7-year-old girl for bone marrow aspiration. Which site should she prepare?

Iliac crest Explanation: Bone marrow aspiration provides samples of bone marrow so the type and quantity of cells being produced can be determined. In children, the aspiration sites used are the iliac crests or spines (rather than the sternum, which is commonly used in adults) because performing the test at these sites is usually less frightening for children; these sites also have the largest marrow compartments during childhood. In neonates, the anterior tibia can be used as an additional site.

The nurse preparing a client for diagnostic testing for disseminated intravascular coagulation knows this is a result indicative of this disease:

Results indicative of disseminated intravascular coagulation include: increased D-dimer assay, decreased antithrombin III, increased fibrogen/fibrin degradation products, and increased fibrinopeptide A level.

A nurse is reviewing the medical records of several children who have undergone lead screening. The nurse would identify the child with which lead level as requiring no further action?

A blood lead level less than 10 mcg/dL requires no action. A level of 14 mcg/dL would need to be confirmed with a repeat test in 1 month along with parental education for decreased lead exposure and then a repeat test in 3 months. Levels of 20 mcg/dL and 26 mcg/dL need to be confirmed with a repeat test in 1 week along with parental education and a referral to the local health department for investigation of the home for lead reduction.

The nurse is caring for a 10-year-old boy with hemophilia. He asks the nurse for suggestions about appropriate physical activities. Which activity would the nurse most likely recommend?

Children with hemophilia should stay active. Good physical activities would be swimming, baseball, basketball, and bicycling (with a helmet). He would still need to be careful about falls and sliding into base. Intense contact sports like football, wrestling, and soccer should be avoided.

A toddler who is beginning to walk has fallen and hit his head on the corner of a low table. The caregiver has been unable to stop the bleeding and brings the child to the pediatric clinic. The nurse is gathering data during the admission process and notes several bruises and swollen joints. A diagnosis of hemophilia is confirmed. This child most likely has a deficiency of which blood factor?

Factor VIII Explanation: The most common types of hemophilia are factor VIII deficiency and factor IX deficiency, which are inherited as sex-linked recessive traits, with transmission to male offspring by carrier females.

A group of students are reviewing the effects of sickle cell anemia on the various parts of the body. The students demonstrate a need for additional study when they identify what as an effect?

High urine specific gravity Explanation: Low urine specific gravity (hyposthenuria) occurs with sickle cell anemia. Chest syndrome is an acute manifestation of sickle cell anemia. Pulmonary hypertension is a chronic manifestation of sickle cell anemia. Cholelithiasis is a chronic manifestation of sickle cell anemia.

The nurse treating clients with hemophilia knows that if bleeding is not treated effectively, the following body part is at greatest risk for the development of chronic, disabling disease:

Joints Explanation: Regardless of the type, if bleeding is not treated effectively, target joints are particularly at risk for deterioration and the development of chronic, disabling hemophilic arthropathy (joint disease).

The nurse is evaluating the complete blood count of a 7-year-old child with a suspected hematological disorder. Which finding is associated with an elevated mean corpuscular volume (MCV)?

Macrocytic red blood cells (RBCs) Explanation: When the MCV is elevated, the RBCs are larger and referred to as macrocytic.

A child with sickle cell disease is brought to the emergency department by his parents. He is in excruciating pain. A vaso-occlusive crisis is suspected and analgesia is prescribed. What would the nurse expect as least likely to be ordered?

Meperidine Explanation: Meperidine is contraindicated for ongoing pain management in a child with vaso-occlusive crisis because it increases the risk for seizures. Analgesics such as morphine, nalbuphine, or hydromorphone are commonly used.

When assessing a child for a possible hematologic disorder, which of the following would the nurse need to keep in mind as most important?

Multiple body sites can be affected. Explanation: The nurse needs to keep in mind that hematologic alterations can affect multiple body sites, so assessment needs to address all body systems. A child's nutritional status may be helpful in assessing certain hematologic disorders such as iron deficiency anemia, but this information is not the most important to remember. Sequelae commonly occur with hematologic alterations, especially chronic conditions such as hemophilia or sickle cell disease. The child's demographic data are important, because some hematologic diagnoses are more commonly associated with a certain age group, sex, race, or geographic location.

A nurse is preparing a teaching plan for a child with hemophilia and his parents. Which information would the nurse include to manage a bleeding episode?

Passive range-of-motion exercises help prevent the development of joint contractures. Splints and devices are used to position the limb in a functional position. Physical therapy is helpful after the bleeding episode is under control. A child with hemophilia is healthy between bleeding episodes and does not need to be on bedrest, and can participate in normal activites appropriate for the child's age

When developing the postoperative plan of care for a child with sickle cell anemia who has undergone a splenectomy, which would the nurse identify as the priority?

Risk for infection Explanation: Removal of the spleen places the child at significant risk for infection. Although the child's skin integrity is disrupted due to the surgery, this is not the priority nursing diagnosis. Loss of fluids occurs during surgery and adequate hydration is important to prevent a sickle cell crisis, but this diagnosis is not the priority in the postoperative period. Although the child is at risk for delayed growth and development, the priority postoperatively is to prevent infection.

To prevent further sickle cell crisis, you would advise the parents of a child with sickle cell anemia to

notify a health care provider if the child develops an upper respiratory infection. Explanation: Reduction of oxygen and dehydration lead to increased sickling of cells. Early prevention of these with respiratory illness is important.

The nurse is administering meperidine as ordered for pain management for a 10-year-old boy in sickle cell crisis. The nurse would be alert for:

seizures. Explanation: Repeated use of meperidine for pain management during sickle cell crisis increases the risk of seizures when used in children with sickle cell anemia. Behavioral addiction is rarely a concern in the child with sickle cell anemia if the narcotic is used for the alleviation of severe pain.