PrepU Ch 25: Hematologic Disorders

A 5-year-old girl with sickle-cell disease has had a temper tantrum in the office waiting room, and the mother has dealt with it inadequately. How should the nurse address the parent's apparent lack of discipline when the pair is in the examination room?

"How did feel about your daughter's behavior in the waiting room?" Explanation: It is common for parents of children with a chronic condition to spoil their children. The nurse should use an open-ended question to elicit the mother's feelings about the girl's behavior in order to open a dialogue about appropriate discipline. Commenting on the tantrum is not helpful. Asking a yes or no question is less likely to elicit the necessary information.

A health care provider has prescribed hydroxyurea 20 mg/kg to a child as part of a treatment regimen for sickle cell disease. The child weighs 27 lb (12.2 kg). How many milligrams should the nurse administer?

244 Explanation: The nurse will use the client's weight in kilograms and multiply by the prescribed milligrams per kilogram. 12.2 kg × 20 mg/kg = 244 mg

Individuals with hemophilia B have a deficiency in factor IX, which can cause excessive blood loss. What is another name for this clotting factor?

Christmas factor Explanation: Factor IX is also known as plasma thromboplastin component or Christmas factor. Its function is to activate factor X. Factor X is the Stuart factor. Stuart factor's function is to activate factor II in the clotting cascade. Factor VIII is the antihemophilic factor. It is a platelet cofactor and also helps activate factor X. Factor VII is proconvertin. It is considered a stable factor and also acts to activate factor X.

The nurse is caring for a child who had a stem cell transplant and is being monitored for engraftment. Which nursing action is priority?

Ensure neutropenic precautions are in place. Explanation: With stem cell transplants, children are at greatest risk for infection and sepsis. The nurse should ensure neutropenic precautions are used to reduce the change of infection. Monitoring laboratory values, reminding the parent to contact the school, and encouraging therapeutic play are important, but preventing infection in the immunocompromised child is a priority.

A 15-year-old client diagnosed with von Willebrand disease has reached menarche. Based on this fact, what information is most important for the nurse to convey to the client?

Expect menstrual bleeding to be heavy. Explanation: Females diagnosed with von Willebrand disease are at risk for menorrhagia. Bruising in the perineal area is not a risk unless there is some sort of trauma at the site. Von Willebrand disease does not cause intermittent periods or shorten the duration of menses.

When caring for a 7-year-old client diagnosed with sickle cell anemia, which clinical manifestation will the nurse report to the health care provider first?

Hyperactive bowel sounds Explanation: The nurse would first report an unexpected manifestation because this could indicate a complication or a different disease. Hyperactive bowel sounds are not expected in the pediatric client with sickle cell anemia. These clients are expected to have an absence of bowel sounds. Sickle cell disease causes chronic anemia, with a hemoglobin level of 6 to 9 g/dL (60 to 90 g/L) with a normal level in a child at 11 to 13 g/dL (110 to 130 g/L). The chronic anemia causes the child to have a poor appetite and severe, acute abdominal pain (caused by sludging, which leads to enlargement of the spleen), swelling of the hands and feet (dactylitis), and increased respiration.

The nurse is caring for a 6-year-old sickle-cell client in an acute care setting. A high priority for this client's plan of care is pain relief. The nurse understands that untreated acute pain can lead to which physiological effects?

Impaired mobility, anorexia, anxiety, sleep disturbances, and developmental regression Explanation: Unrelieved acute pain can lead to impaired mobility; anorexia, causing poor nutritional intake; delayed wound healing; anxiety and irritability; somatic symptoms; sleep disturbances; avoidance; developmental regression; and increased parental distress. Constipation, nausea, vomiting, nocturnal enuresis, and migraine headaches are not effects of acute pain.

A home care nurse is teaching a parent how to administer a clotting factor infusion to their child. How can the nurse best evaluate the effectiveness of the teaching?

Observe the parent set up and administer the infusion. Explanation: Observing the parent set up and administer the infusion is the best way to evaluate the nurse's teaching. Asking the parent to repeat the instructions, providing an opportunity for asking questions, or providing cues as the parent sets up the infusion does not evaluate the effectiveness of the teaching.

The nurse is reviewing the laboratory test results of a child diagnosed with disseminated intravascular coagulation (DIC). What would the nurse interpret as indicative of this disorder?

Positive fibrin split products Explanation: Laboratory test results associated with DIC include positive fibrin split products; prolonged prothrombin time, partial thromboplastin time, bleeding time, and thrombin time; decreased fibrinogen levels, platelets, clotting factors II, V, VIII, and X, and antithrombin III; and increased levels of fibrinolysin, fibrinopeptide A, and positive D-dimers.

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.

Which lab values will the nurse expect to observe in a child with a hemolytic blood transfusion reaction? Select all that apply.

bilirubin 10 mg/dL (17 µmol/L) hemoglobin 8 mg/dL (80 g/L) red blood cells 3.0 mm3 Explanation: A child with hemolysis of red blood cells (RBCs) will exhibit decreased red blood cells, increased bilirubin related to hemolyzed RBCs, and a decreased hemoglobin. An elevated white blood cell count indicates infection or other hematologic disorder. An increased prothrombin time (PT) indicates difficulty forming a clot.

The nurse is performing a respiratory assessment on a black adolescent experiencing a sickle-cell crisis. Where is the best place for the nurse to check for cyanosis on this client?

mucous membranes Explanation: Assessing for cyanosis is more difficult in dark-skinned children than fair-skinned children. The mucous membranes is the best place to do this.

The nurse is collecting data from the caregivers of a child brought to the clinic setting. The parents tell the nurse that the child's skin seems to be an unusual color. The nurse notes that the child's skin appears bronze-colored and jaundiced. This observation alerts the nurse to the likelihood that this child has which disorder?

Thalassemia Explanation: In the child with thalassemia the skin may appear bronze-colored or jaundiced. The child with hemophilia may have bruised areas on the skin. The skin color in children with sickle cell disease may be pale in color, and with Kawasaki disease the child may have a rash on the trunk and extremities.

In caring for a child with sickle cell disease, the highest priority goal is:

The child's fluid intake will improve. Explanation: The highest priority goals for this child are maintaining comfort and relieving pain. The child is prone to dehydration because of the kidneys' inability to concentrate urine, so increasing fluid intake is the next highest priority. Other goals include improving physical mobility, maintaining skin integrity, reducing the caregiver's anxiety, and increasing the caregiver's knowledge about the causes of crisis episodes — but these goals are not the highest priority.

The nurse is caring for a 4-year-old with sickle cell anemia. A physical finding the nurse 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.

The nurse is teaching an in-service program to a group of nurses on the topic of children diagnosed with sickle cell anemia. The nurses in the group make the following statements. Which statement is most accurate regarding sickle cell anemia?

"If the trait is inherited from both parents the child will have the disease." Explanation: When the trait is inherited from both parents (homozygous state), the child has sickle cell disease, and anemia develops. The trait does not skip generations. The trait occurs most commonly in black clients. Either sex can have the trait and disease.

A nurse is providing teaching to the parents of a child diagnosed with sickle cell anemia. The discussion is focused on precipitating factors for sickle cell crisis. Which statement by the parents requires the nurse to reinforce the teaching?

"Our family is taking a fun hiking trip up in the mountains next week." Explanation: High altitudes are a contributing factor for sickle cell crisis and should be avoided, as should flights in planes that are not pressurized. Extreme temperatures (hot or cold) are also triggers for a crisis so keeping warm during the winter is important. Dehydration and exposure to infection or other illness are precipitating factors for sickle cell crisis. Adequate hydration and keeping up with immunizations are imperative for health and wellness in a child diagnosed with sickle cell anemia.

A couple is expecting a child. The fetus undergoes genetic testing and the couple discover the fetus has sickle cell anemia. The couple ask the nurse how this happened. Which statement is accurate for the nurse to provide?

"Sickle cell anemia is passed to a fetus when both parents have the gene." Explanation: Sickle cell anemia is an inherited disease. The recessive gene is passed from both parents who either have the disease or the trait. There is no need for further testing to determine the cause. There are no other ways to pass the disease other than through genetics. Informing the parents that the gene was passed from both parents is most informative. Sickle cell anemia is not a dominant disease, which is passed when only one parent has the gene, nor is caused by a random mutation.

The nurse is providing family education for the prevention or early recognition of vaso-occlusive events in sickle cell anemia. Which response by a family member indicates a need for further teaching?

"We should call the doctor for any fever over 100°F (37.8°C)." Explanation: The nurse must emphasize that any febrile illness requires immediate attention. Fever causes dehydration, which can trigger problems in a child with sickle cell anemia. Seeking medical attention for abdominal pain; watching for unusual headache, loss of feeling, or sudden weakness; and compliance with vaccinations are appropriate.

The mother of a 10-year-old who had a febrile reaction following a transfusion, asks the nurse: "Why did this happen to my child?" What response by the nurse is best?

"Your child's blood has developed antibodies to leukocyte, platelet, or plasma protein antigens in the donor blood." Explanation: A febrile reaction is not associated with hemolysis and generally occurs when the recipient has developed antibodies to leukocyte, platelet, or plasma protein antigens in the donor blood. In a hemolytic reaction, the blood product is not compatible with the recipient's blood. An allergic reaction is a nonhemolytic reaction that occurs when the donor blood contains plasma proteins or antigens to which the recipient is hypersensitive.

The caregiver of a child with sickle cell disease asks the nurse how much fluid her child should have each day after the child goes home. In response to the caregiver's question, the nurse would explain that for the child with sickle cell disease, it is best that the child have:

1,500 to 2,000 mL of fluid per day. Explanation: Prevention of crises is the goal between episodes. Adequate hydration is vital; fluid intake of 1,500 to 2,000 mL daily is desirable for a child weighing 20 kg and should be increased to 3,000 mL during the crisis.

A nurse is providing care to a child who is to receive a blood transfusion. The health care provider has prescribed the infusion to run at a rate of 5 mL/kg/hour. The child weighs 55 lb (25 kg). At what rate should the nurse set the infusion pump? Record your answer using a whole number.

125 Explanation: The nurse will use the child's weight in kilograms, and multiply weight by the prescribed milligrams per hour. 25 kg × 5 mL = 125 mL/hour

A child is hospitalized with complications related to hemophilia. The health care provider has discussed the child's plan of care with the parents, but they continue to ask questions. What action will the nurse take?

Answer the parents' questions as completely as possible. Explanation: Because the health care provider has discussed the child's care, the nurse should answer the parents' questions as completely as possible. Telling the parents that they have been fully briefed negates their concerns and is inappropriate. Encouraging the parents to focus on their child also negates their concerns. Unless the parents ask specifically for the health care provider, the nurse can answer the parents' questions.

A group of newly hired nurses who will be working on the pediatric unit are attending an in-service program about sickle cell disease. During the program, the nurse manager describes the steps for managing sickle cell pain. Place these steps in the sequence in which the nurse manager would describe them.

Assess the pain. Believe the child's report of pain. Look for complications or cause of pain. Give medications and use distraction. Provide rest in a quiet area. Administer fluids. Explanation: The ABCs of managing sickle cell pain are assess the pain (use a pain assessment tool); believe the child's report of pain; complications or cause of pain (look for complications); drugs and distraction: pain medication (opiates and nonsteroidal anti-inflammatory drugs (NSAIDs), if no contraindications); use fixed dosing; give on a timed schedule; no PRN dosing for pain medications; distraction with music, TV, and relaxation techniques; environment (rest in quiet area with privacy); and fluids.

A child with hemophilia A is scheduled for surgery. Which precautions would the nurse institute with this client?

Handle the child gently when transferring to a stretcher. Explanation: Hemophilia is a group of X-linked recessive disorders that prevent clot formation. The best care for the child is to prevent any bruising or bleeding so gentle handling when moving the child from the strecher is necessary. Because the child is having surgery, infusion of clotting agents will be necessary. Analgesia will be needed postoperatively as will surgical dressings. IM injections are contraindicated because of potential bleeding. Brushing the teeth is part of normal daily hygiene.

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?

Iron-chelating drugs Explanation: 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 may be given. Vitamin and potassium supplements would not be given to treat the iron overload. Factor VIII preparations are given to the child with hemophilia.

The nurse is presenting a program on HIV and children for a nursing students. The nurse determines that more teaching is needed when the students identify which of the following as a mode of transmission?

Skin contact Explanation: Modes of transmission for HIV include sexual contact, mother-to-infant transmission before or around the time of birth, and contamination by blood or body fluids.

The nurse is caring for a 13-year-old girl with von Willebrand disease. After teaching the adolescent and her parents about this disorder and care, which response by the parents indicates a need for additional teaching?

We should administer desmopressin as often as needed." Explanation: The parents need to know that desmopressin spray Stimate is used for controlling bleeding; the other brands are used for homeostasis and enuresis. Additionally, Stimate should only be used for 3 days in a row as lessening of the response (tachyphylaxis) occurs with frequent use. Stimate should be used before dental work. Menorrhagia and nosebleeds may occur.

A nurse is providing care for a child with disseminated intravascular coagulation (DIC). What would alert the nurse to possible neurologic compromise?

Widely fluctuating blood pressure Explanation: A key aspect of the nurse's role is to assess the child for signs and symptoms of impaired tissue perfusion in the various body systems that may be affected by DIC. Unstable or abnormal blood pressure (such as wide BP fluctuations) or unequal pupil size may suggest neurologic compromise. Hematuria would suggest renal compromise. Petechiae would be indicative of bleeding into the skin.

The nurse is teaching an 8-year-old child and his family how to manage cancer pain using nonpharmacologic methods. Which parent statement signifies successful client teaching?

"I will begin using the technique before he experiences pain." Explanation: The parents should begin using the technique chosen before the child experiences pain or when the child first indicates he is anxious about, or beginning to experience, pain. The parents should use descriptive terms like pushing, pulling, pinching, or heat and avoid overly descriptive or judgmental statements such as, "This will really hurt a lot" or "This will be terrible." They should offer praise, positive reinforcement, hugs, and support for using the technique even when it was not effective.

A 3-year-old child is hospitalized with a diagnosis of sickle cell anemia. The child's condition has improved, and the child is much more active and eager to play. Which toy should the nurse offer the child?

Large piece puzzle Explanation: An appropriate toy for a 3-year-old child is a large piece puzzle. Board games are more appropriate for preschool and school-aged children; fabric books and squeaky toys are more appropriate for older infants and younger toddlers (10 to 18 month of age).

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.

The nurse is caring for a child with aplastic anemia. The nurse is reviewing the child's blood work and notes the granulocyte count is about 500, platelet count is over 20,000, and the reticulocyte count is over 1%. The parents ask if these values have any significance. Which response by the nurse is appropriate?

"These values will help us monitor the disease." Explanation: This response answers the parent's questions. In the nonsevere form, the granulocyte count remains about 500, the platelets are over 20,000, and the reticulocyte count is over 1%. The other responses do not address what the parents are asking and would block therapeutic communication.

An 11-year-old child is being prepared for discharge after experiencing a vasoocclusive crisis secondary to sickle cell disease. The child has been prescribed hydroxyurea. After teaching the child and parents about this medication, the nurse determines that the teaching was successful when the parents identify that they will notify the health care provider about which condition?

Infection Explanation: The adverse effect of hydroxyurea is infection. The drug can cause neutropenia. Therefore, the parents should report signs and symptoms of infection to the health care proivder as soon as possible. Gastric upset, constipation, or headache are not siginificant.

To prevent further sickle cell crisis, the nurse 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: Infections caused by the Streptococcus pneumoniae can be lethal to a child with sickle cell, because they can cause overwhelming sepsis or meningitis. By 2 months of age the child should be started on Penicillin V as prophylaxis against pneumococcal infections. The child should receive the 7 valent pneumococcal series in infancy. After 2 years of age the child should receive the 23 valent pneumococcal vaccine. He or she should also be immunized against meningitis. Participating in strenuous activities such as running and limiting the amount of fluids leads to a reduction of oxygen and dehydration. This can lead to the increased sickling of cells. The anemia of sickle cell disease is not the result of iron deficiency. It is the result of the abnormal shape of the red blood cell. Administering iron will not correct the anemia.

The nurse is assessing a child and notices pinpoint hemorrhages appearing on several different areas of the body. The hemorrhages do not blanch on pressure. The nurse documents this finding as:

petechiae. Explanation: Petechiae are pinpoint hemorrhages that occur anywhere on the body and do not blanch with pressure. Purpura are larger areas of hemorrhage in which blood collects under the tissues and appear purple in color. Ecchymosis refers to areas of bruising. Poikilocytosis refers to the variation in the size and shape of the red blood cells commonly found in children with thalassemia.

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. Priapism is a complication of sickle cell anemia unrelated to meperidine administration. Leg ulcers are a complication of sickle cell anemia unrelated to meperidine administration.

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

Apply heat to the site of bleeding. Explanation: Ice or cold compresses, not heat, would be applied to the site of bleeding. Direct pressure is applied until the bleeding stops. The injured part is elevated unless elevating would contribute to further injury. Factor VIII replacement is given to replace the missing clotting factor.

A 5-year-old girl with sickle cell disease has had a temper tantrum in the office waiting room, and the mother has dealt with it inadequately. How should the nurse address the girl's apparent lack of discipline when the pair is in the examination room?

"How did feel about your daughter's behavior in the waiting room?" Explanation: It is common for parents of children with a chronic condition to spoil their children. The nurse should use an open-ended question to elicit the mother's feelings about the girl's behavior in order to open a dialogue about appropriate discipline. Commenting on the tantrum is not helpful. Asking a yes or no question is less likely to elicit the necessary information.

The parents of a 2-month-old infant have learned that their infant has hemophilia. The parents are visibly upset and ask how this could have happened to them. What is the nurse's best response?

"News like this is difficult to hear. Let's talk about what this means for your child." Explanation: The nurse's best response is to therapeutically acknowledge the parents' concerns and the fact that it is upsetting to them. Next the nurse should give the parents information about what the diagnosis means for them and their child. While the diagnosis is not the parents' fault, saying that things like this happen sometimes is non-therapeutic. Telling the parents that there is no need to worry or that the nurse understands how they feel is untrue and non-therapeutic.

A parent calls the pediatric clinic and tells the nurse "I think my child is having a sickle cell crisis. Should I bring the child to the office?" What is the nurse's best response?

"Tell me about the symptoms your child is experiencing" Explanation: The best response is for the nurse to ask about the symptoms the child has, which will help confirm that the child is in crisis. Once the nurse is sure that the child is in crisis, the parent can be advised to take the child to the emergency department or to call 911. Giving the child water may not be appropriate depending on the child's level of consciousness. Asking the parent what makes him or her think the child is in crisis may not elicit the needed information right away. Asking specifically about the child's symptoms is more to the point.

While administering a blood transfusion to a child with a hematologic disorder, the nurse notes the child develops urticaria and wheezing. Which collaborative interventions will the nurse begin? Select all that apply.

Give an antihistamine. Apply oxygen as needed. Discontinue the transfusion. Explanation: The child is experiencing signs of an allergic reaction to the blood transfusion and would require discontinuation of the blood transfusion, administration of an antihistamine, and oxygen as needed. Diuretics would be needed for an anaphylactic or circulatory overload reaction related to the blood transfusion, whereas a blood culture would be needed if a contaminant in the blood was suspected.

A nurse is providing care for a child diagnosed with beta-thalassemia who is receiving a blood transfusion. The child reports being bored and asks to go to the playroom. What is the best action for the nurse to take?

Have a child-life specialist find an appropriate activity to occupy the child during the transfusion. Explanation: The best action the nurse can take is to have the child-life specialist work with child to find something interesting the child can do while in the room. There is a safety issue involved if the nurse tries to administer the transfusion in the playroom. The nurse can deny the request or explain the need for rest but if the child is feeling well it will likely not be enough to satisfy the child. Finding an appropriate activity for the child to engage in is the best option.

A 3-year-old child is hospitalized with a diagnosis of sickle cell anemia and is experiencing a pain crisis. Using the FACES scale, the nurse assesses the child's pain to be a 10 on a scale of 1 to 10. The child is receiving intravenous fluids and oxygen at 2 L/min via nasal cannula. The parent is at the bedside holding the child's hand and has a concerned look. What is the nurse's priority in caring for the child?

Implement strategies to address the child's pain. Explanation: In this case, the nurse's priority is to address the child's pain. The child is already receiving IV fluids and oxygen. That in combination with analgesia will assist in resolving the crisis. Asking the parent if he or she has questions, asking the health care provider to meet with the parent, and providing distraction for the child are all appropriate interventions, but the priority is to address the child's pain.

A nurse is providing teaching on safety to a group of parents whose children are diagnosed with hemophilia. Which statement made by a parent requires follow-up by the nurse?

Our child always wears a helmet and body padding when playing football." Explanation: Contact sports such as football and soccer are safety issues for children diagnosed with hemophilia. There is more chance of sustaining an injury resulting in severe bleeding. Safer sports include swimming and golf. Toddlers who are just learning to walk may have frequent falls, so a soft helmet and knee pads can help prevent injuries. Children diagnosed with hemophilia should wear a medical alert bracelet at all times. Jumping on a trampoline can result in a serious fall resulting in extensive bleeding.

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?

150 mL/kg of fluids Explanation: 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.

The nurse is working with a child who is in sickle cell crisis. Treatment and nursing care for this child include which actions? Select all that apply.

Administering oxygen Administering analgesics Maintaining fluid intake Explanation: A vaso-occlusive crisis occurs when sickle-shaped cells are clumped together in a joint or organ. This causes severe pain and hypoxia to the tissues. The management for a vaso-occlusive crisis is to provide adequate pain relief, oxygen to correct the hypoxemia, and increased IV fluids to thin out viscosity and allow the cells to flow in the vascular system. Platelets administration is not indicated as part of the treatment. Children and adults experiencing a sickle cell crisis experience a high degree of pain, so exercise and activity is postponed until the crisis is over. Activity is encourgaed when the child is not in crisis as it promotes growth and a positive self-image.

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?

Baseball Explanation: 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 health care provider has prescribed hydroxyurea 650 mg for a child diagnosed with sickle cell anemia. The child weighs 65 lb (29.5 kg). The normal recommended dose is 20 mg/kg/day. What action should the nurse take?

Contact the health care provider to lower the dose. Explanation: The nurse should perform the needed calculations to check the dosage is correct for the client. The nurse will use the client's weight in kilograms and multiply by the prescribed milligrams per day. 29.5 kg x 20 mg = 590 mg Therefore, the nurse should contact the health care provider about lowering the dose.

A nurse caring for an 8-year-old child with a bleeding disorder documents the following nursing diagnosis: Ineffective tissue perfusion related to intravascular thrombosis and hemorrhage. This nursing diagnosis would be most appropriate for a child diagnosed with which condition?

Disseminated intravascular coagulation Explanation: Disseminated intravascular coagulation (DIC) is an acquired coagulopathy that, paradoxically, is characterized by both thrombosis and hemorrhage. The outcome for this client is: The child will maintain adequate tissue perfusion of all body systems affected by DIC and regain adequate laboratory values for hemostasis. von Willebrand disease and hemophilia involve hemorrhage but not thrombosis. Iron deficiency anemia does not involve either hemorrhage or thrombosis.

A nurse is caring for a child diagnosed with von Willebrand disease who is receiving desmopressin as part of the treatment regimen. In relation to desmopressin administration, what assessment finding(s) is mportant for the nurse to monitor? Select all that apply.

Electrolyte balance Intake/output Heart rate Explanation: Desmopressin is a synthetic antidiuretic hormone that can cause water retention, hyponatremia, and congestive heart failure. It is important for the nurse to closely monitor a client's electrolytes, intake and output, and for signs of congestive heart failure (increased heart rate, wheezing, etc.). While monitoring the level of consciousness and temperature are important assessments for a client with von Willebrand disease, they are not the most important assessments related to desmopressin administration.

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 nurse is caring for a newborn whose screening test result indicates the possibility of sickle cell anemia (SCA) or sickle cell trait. The nurse would expect the test result to be confirmed by which lab tests?

Hemoglobin electrophoresis Explanation: If the screening test result indicates the possibility of SCA or sickle cell trait, hemoglobin (Hgb) electrophoresis is performed promptly to confirm the diagnosis. While Hgb electrophoresis is the only definitive test for diagnosis of the disease, other laboratory testing that assists in the assessment of the disease include reticulocyte count (greatly elevated), peripheral blood smears (presence of sickle-shaped cells and target cells), and erythrocyte sedimentation rate (elevated).

A child diagnosed with hemophilia presents with warm, swollen, painful joints. Which action will the nurse take first?

Prepare to administer factor replacement medication Explanation: Many clients with hemophilia have repeated episodes of hemarthrosis or bleeding into the joints, and develop functional impairment of the joints, despite careful treatment. To assist in limiting impairment, the nurse would prepare to administer factor replacement medications, such as plasma, recombinant clotting factor VIII, or a clotting promotor medication. The nurse would document the finding, notify the health care provider, and assess the client for additional symptoms after limiting the amount of blood loss.

A nurse is providing teaching on the medication regimen for beta-thalassemia to an adolescent. What is the best way for the nurse to determine if the teaching was successful?

Request that the adolescent teach the information to the nurse. Explanation: The best way for the nurse to determine if teaching has been successful is to ask the client to "teach back" the information taught. Using this method, the nurse can correct any misconceptions. Providing written materials to reinforce teaching, having the client verbalize understanding the instructions and providing an opportunity to ask questions are all appropriate client education strategies, but they do not evaluate the effectiveness of the teaching.

The nurse is caring for a 12-year-old boy with sickle-cell disease. During a routine wellness exam, his mother tells the nurse that the boy is becoming rebellious. He responds by telling the nurse that it's because "she never lets me out of her sight!" What would be the best intervention for this family?

Tell them about a camp for children with sickle-cell disease and their parents. Explanation: The camp for children with sickle-cell disease would be the best suggestion. Both mother and son can attend together and gain valuable knowledge from medical experts and counselors. It will also give them the opportunity to meet other families just like them. The mother will be less resistant because she can attend, yet it will allow the boy much-needed freedom. Encouraging the mother to give him freedom and the other reminders are helpful, but this situation needs to be addressed immediately.

The nurse is caring for a child with thalassemia who is receiving chelation therapy at home using a battery-operated pump. After teaching the parents about this treatment, which statement by the mother indicates a need for additional teaching?

"I can have the nurse administer the chelation therapy if I am uncomfortable." Explanation: The nurse needs to emphasize to the mother that therapy must be maintained at home to continuously decrease the iron levels in the child's body. Family members need to be taught to administer deferoxamine subcutaneously with a small battery-powered infusion pump over a several-hour period each night (usually while the child is sleeping).

A nurse is providing care for a child diagnosed with beta-thalassemia major, and is preparing the family for discharge. The nurse notes that the family is overwhelmed with the challenges of their child's diagnosis. What is the best way for the nurse to ensure that the family is supported after discharge?

Have the case manager meet with the family prior to discharge. Explanation: The best way for the nurse to ensure the family has the support they need after discharge is to have a case manager meet with the family prior to discharge. The case manager can assess the family for specific needs and coordinate necessary services. Individually, making weekly calls to the family, providing information on support groups, and providing follow-up appointments would be helpful. However, the case manager could coordinate all of those services and provide the family with one person to contact with questions or concerns.

A nurse is teaching the parents of a child with sickle cell disease about factors that predispose the child to a sickle cell crisis. The nurse determines that the teaching was successful when the parents identify what as a factor?

Infection Explanation: Factors that may precipitate a sickle cell crisis include: fever, infection, dehydration, hot or humid environment, cold air or water temperature, high altitude, or excessive physical activity. Respiratory distress and pallor are general signs and symptoms of a sickle cell crisis.

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

Maintaining fluids through an intravenous line Explanation: Sickle cells clump together and prevent normal blood flow. This leads to tissue hypoxia. With a vaso-occlusive crisis, the cells are clumped together and prevent blood flow to the joint or organ. The blood with the clumped sickled cells is very viscous. Adequate hydration is crucial in relieving the problems of a vaso-occlusive crisis. The hydration dilutes the blood and decreases the viscosity. During a crisis the recommended fluid intake (IV and PO) is 150 ml/kg/day. During a vaso-occlusive crisis, the child has severe pain. The goal is to get the pain under control and increase blood flow. Range-of-motion excercises will add to the increased pain during this period of time, so should not be started until crisis in under control. The diet and hourly deep breaths are important, but they are not crucial to correcting the crisis.