Pediatrics Ch. 26-28

Hemophilia

A blood disorder where the blood does not clot normally. Inherited as a sex linked recessive trait.

The nurse finds an adolescent with Hodgkins disease crying. The adolescent says, I am so scared. What is the most appropriate nursing response to this comment?

"Tell me what's got you scared."

The nurse, caring for a child receiving chemotherapy, notes that the childs abdomen is firm and slightly distended. There is no record of a bowel movement for the last 2 days. What do these assessment findings suggest?

peripheral neuropathy

Which statement by a mother may indicate a cause for her 9-month-olds iron deficiency anemia?

"Formula is so expensive. We switched to regular milk right away."

2. The statement by a mother that may indicate a cause for her 9-month-old having iron deficiency anemia is: a. "Formula is so expensive. We switched to regular milk right away." b. "She almost never drinks water." c. "She doesn't really like peaches or pears, so we stick to bananas for fruit." d. "I give her a piece of bread now and then. She likes to chew on it."

ANS: A Because cow's milk contains very little iron, infants should drink iron-fortified formula for the first year of life.

7. A child who is receiving a transfusion should be closely assessed for: a. fever. b. lethargy. c. jaundice. d. bradycardia.

ANS: A The child receiving a blood transfusion is observed for signs of a transfusion reaction including chills, itching, fever, rash, headache, and back pain.

At what age is the highest incidence for iron deficiency anemia and why?

Between 9-24 months. This is a rapid growth period where the baby outgrows the limited iron reserve in the body.

Thalassemia

Blood disorder in which the patients body cannot produce sufficient hemoglobin. RBC's are abnormal in size and shape and are destroyed rapidly.

Aplastic Crises

Bone marrow stops producing RBC's.

Signs and Symptoms of Hemophilia

Can be diagnosed at birth because the factor VIII cannot cross the placenta. Prolonged bleeding that can take up to 1 hour to clot. Hematomas after immunizations. Hemarthrosis (bleeding into the joints).

Leukemia Treatment

Chemotherapy and radiation. Adequate hydration to reduce kidney damage. Delayed immunizations, TPN.

Hemophilia B (Christmas Disease)

Deficiency in factor IX

Hemophilia A

Deficiency in factor VIII.

Tratment of Hemophilia

Delayed procedures to newborns with family history. Replacement of missing factor. DDAVP is a nasal spray that increases factor VIII in the blood. Amicar can control bleeding in dental care.

Stages of Dying

Denial, anger, bargaining, depression, acceptance.

Blood Dyscracias

Disorders where blood components fail to form correctly or when blood values exceed or fail to reach normal limits.

A newly married couple is seeking genetic counseling because they are both carriers of the sickle cell trait. How can the nurse best explain the childrens risk of inheriting this disease?

Each child has a one in four chance of having the disease and a two in four chance of being a carrier.

How has synthetic recombinant antihemophilic factor improved the management of hemophilia? (Select all that apply.)

Eliminates the need for frequent transfusions Can be administered by family at home Reduces cost of care of the hemophiliac Reduces risk of HIV and hepatitis A and B transmission

Hodgkin's treatment

Radiation and chemotherapy. Patient should avoid direct sunlight exposure on treated areas. PABA may be used to prevent burning. Patient needs emotional support and should be given a coping mechanism for anger. Side effects of sterility should be discussed.

What will the nurse teach the parents of a child with a low platelet count to avoid?

aspirin

The nurse confirms that sickle cell trait can be distinguished from sickle cell disease by a lab test called ________________.

electrophoresis

The rate of RBC production is regulated by _________________.

erythropoietin

What should the nurse closely assess in a child receiving a transfusion?

fever

A child is diagnosed with iron deficiency anemia. What will the nurse explain can occur if this disorder goes untreated?

heart failure

Vasooclusive(painful) crises

Most common, some obstruction of blood flow. Abdominal pain and painful joints and extremities.

Hodgkin's Signs and Symptoms

Painless lumps around the neck, low grade fever, anorexia, unexplained weight loss, night sweats, general malaise, rash, and itching.

Leukemia

Uncontrolled growth of immature WBC's. The count may be high but the cells do not function as they are supposed to.

A child has just been diagnosed with acute lymphoblastic leukemia. What is the result of an overproduction of immature white blood cells in the bone marrow?

increased susceptibility to infection

What will the nurse administer with ferrous sulfate drops when providing them to a child on the pediatric unit?

with orange juice

Which statement made by a parent indicates an understanding of health maintenance of a child with sickle cell disease?

"It is important for my child to drink plenty of fluids."

Idiopathic Thrombocytopenia Purpura (ITP)

An acquired platelet disorder. Platelets are perceived as foreign and the spleen will destroy them.

Sickle Cell Trait

Inherited through one parent. RBC and hemoglobin counts are normal. This does not develop into the disease.

Treatment of Iron deficiency anemia

Iron supplements and education on foods high in iron. EX: Boiled egg yolk, liver, leafy green veggies, cream of wheat, dried fruits, dry beans, crushed nuts, and whole grain bread.

The nurse is presenting information on the congentital disorder of hemophilia A. What fact will the nurse include?

It is transmitted by symptom-free females.

What should be included in the nursing care of a 12-year-old child receiving radiation therapy for Hodgkins disease? (Select all that apply.)

Application of sunblock Appetite stimulation Conservation of energy Provision for expressions of anger

On admission, a child with leukemia has widespread purpura and a platelet count of 19,000/mm3. What is the priority nursing intervention?

Assessing neurological status

Treatment of ITP

Avoid salicylates and caffeine. Prednisone. IV IgG to elevate counts. Anti-D antibody if not active bleeding is present. (must be watched for 1hr for chills, HA, and change in vitals. Patient should use soft bristle tooth brush.

Signs and Symptoms of Leukemia

Low grade fever, pallor, bruising tendency, leg and joint pain, listlessness, abdominal pain, and enlargement of lymph nodes. Spleen and liver may enlarge as disease progresses. Petechiae and lesions around mucous membranes. Anorexia, vomiting, weight loss.

Hodgkin's disease

Malignancy of the lymph nodes. Confirmed by the presence of Reed-Sternberg cells.

A 2-year-old child has been diagnosed with hemophilia A. What information should the nurse include in a teaching plan about home care?

If bleeding occurs, apply pressure, ice, elevate, and rest the extremity.

A school-aged child is living with a chronic disease process. How would the nurse anticipate chronic illness will effect growth and development? (Select all that apply.)

Impaired sense of belonging Decreased feelings of independence

Thalassemia Major (Cooley's disease)

Inherited by both parents. Severe anemia is evident by the first 6 months of life.

Thalassemia Minor (B-thalassemia trait)

Inherited by one parent and considered mild anemia. Signs and symptoms are paleness and enlargement of the spleen.

Sickle Cell Anemia

Inherited from both parents and is chronic anemia.

Sickle Cell Disease

Is inherited and is a defect in the formation of hemoglobin. Clumping of crescent shaped cells cause pile ups and can lead to a clot.

Iron-Deficiency Anemia

Most common nutritional deficiency in children. Condition where there is a reduction in the amount and size of red blood cells, the amount of hemoglobin, or both.

Thalassemia Major Signs ans Symptoms

Paleness, poor appetite, fever, jaundice that proceeds to bronze color from hemosiderosis, enlarged liver, and spleen becomes enormous. Abdominal distention, upper teeth may protrude due to overgrowth.

Sickle Cell Anemia Signs and Symptoms

Paleness, tiredness, and small appetite. Hemoglobin ranges from 6-9 g/dL or lower. May have unusual swelling of the fingers and toes.

Signs and Symptoms of Iron-deficiency anemia

Pallor, irritability, anorexia, and decrease in activity.

Anemia signs and symptoms

Pallor, weakness, tachypnea, SOB, and can result in congestive heart failure.

The nurse is caring for a child with a low platelet count. What skin assessments would alert the nurse to bleeding? (Select all that apply.)

Petichiae Purpura Ecchymosis Hematoma

The nurse notes that a 4-year-old childs gums bleed easily and he has bruising and petechiae on his extremities. Which lab value is consistent with these symptoms?

Platelet count of 25,000/mm3

Splenic Sequestration

Pooling of blood into the liver and spleen. Massive spleen enlargement, and signs of shock and circulatory collapse are present.

Hyperhemolytic

Rare. Rapid rate of hemolysis, causes spleen to reduce its function. Pt is susceptible to infection.

Erythropoietin

Regulation of RBC production.

The most recent blood count for a child who received chemotherapy last week shows neutropenia. What is the priority nursing diagnosis for this child?

Risk for infection

A 6-year-old with leukemia asks, Who will take care of me in heaven? What is the best response by the nurse?

"Who do you think will take care of you?"

Sickle Cell Crisis

Severe abdominal pain, muscle spasms, leg pain, painful swollen joints, fever, vomiting, hematuria, convulsions, stiff neck, como or paralysis can also occur.

5. A 2-year-old child has been diagnosed with hemophilia A. The information the nurse would include in a teaching plan about home care would be: a. if bleeding occurs, apply pressure, ice, elevate, and rest the extremity. b. children's aspirin in lowered doses may be given for joint discomfort. c. a firm, dry toothbrush should be used to clean teeth at least twice a day. d. do not permit interactive play with other children.

ANS: A When bleeding occurs, the traditional approach is to follow RICE—rest, ice, compression, and elevation.

Normal range for hemoglobin

12-18 g/dL

Normal Platelet Count

150,000-400,000

Normal range for RBC

4.5-11.0

Normal WBC Range

45,000-10,000

Treatment of Thalassemia

Frequent blood transfusions to maintain hemoglobin levels above 9.5 g/dL. Balancing rest and energy. Desferal may cause red discoloration of the urine.

A child with thalassemia major receives blood transfusions frequently. What is a complication of repeated blood transfusions?

Hemosiderosis

8. On admission, a child with leukemia has widespread purpura and a platelet count of 19,000/mm3. The priority nursing intervention is: a. assessing neurological status. b. inserting an intravenous line. c. monitoring vital signs during platelet transfusions. d. providing family education about how to prevent bleeding.

ANS: A When platelets are low, the greatest danger is spontaneous intracranial bleeding. Neurological assessments are therefore a priority of care.

6. The nurse would teach the parents of a child with a low platelet count to avoid: a. ibuprofen. b. aspirin. c. caffeine. d. prednisone.

ANS: B Aspirin interferes with platelet function and should be avoided to prevent the risk of prolonged bleeding.

1. When teaching the parents of a young child about iron deficiency anemia, the nurse would tell them that a rich source of iron is: a. an egg white. b. cream of Wheat. c. a banana. d. a carrot.

ANS: B Good nutritional sources of iron include boiled egg yolk, liver, green leafy vegetables, cream of Wheat, dried fruits, beans, nuts, and whole-grain breads.

11. The statement made by a parent indicating understanding of health maintenance of a child with sickle cell disease is: a. "I should give my child a daily iron supplement." b. "It is important for my child to drink plenty of fluids." c. "He needs to wear protective equipment if he plays contact sports." d. "He shouldn't receive any immunizations until he is older."

ANS: B Prevention of dehydration, which can trigger the sickling process, is a priority goal in the care of a child with sickle cell disease.

3. The nurse would instruct the parent to give ferrous sulfate drops to the child: a. with milk. b. with orange juice. c. with water. d. on a full stomach.

ANS: B Vitamin C aids in the absorption of iron, whereas food and milk interfere with the absorption of iron.

9. An adolescent is diagnosed with Hodgkin's disease. Lymph nodes on both sides of her diaphragm have been found to be involved, including cervical and inguinal nodes. The disease is in stage: a. I. b. II. c. III. d. IV.

ANS: C Lymph node regions on both sides of the diaphragm are consistent with a diagnosis of stage III Hodgkin's disease.

12. A newly married couple is seeking genetic counseling because they are both carriers of the sickle cell trait. How can the nurse best explain the children's risk of inheriting this disease? a. Every fourth child will have the disease; two others will be carriers. b. All of their children will be carriers, just as they are. c. Each child has a one in four chance of having the disease and a two in four chance of being a carrier. d. The risk levels of their children cannot be determined by this information.

ANS: C The sickle cell gene is inherited from both parents; therefore each offspring has a one in four chance of inheriting the disease.

10. A 3-year-old child with sickle cell disease is admitted to the hospital in sickle cell crisis with severe abdominal pain. The nurse recognizes that the type of crisis the child is most likely experiencing is: a. aplastic. b. hyperhemolytic. c. vaso-occlusive. d. splenic sequestration.

ANS: C Vaso-occlusive crisis, or painful crisis, is caused by obstruction of blood flow by sickle cells, infarctions, and some degrees of vasospasm.

13. A child with thalassemia major receives blood transfusions frequently. The nurse is aware that a complication of repeated blood transfusions is: a. hemarthrosis. b. hematuria. c. hemoptysis. d. hemosiderosis.

ANS: D As a result of repeated blood transfusions, excessive deposits of iron (hemosiderosis) are stored in tissues.

4. The nurse clarifies that the deficiency of factor IX results in: a. thalassemia. b. idiopathic thrombocytopenic purpura. c. hemophilia A. d. Christmas disease.

ANS: D Christmas disease, or hemophilia B, is caused by the deficiency of factor IX.

What are the classic symptoms of thalassemia major (Cooleys anemia)? (Select all that apply.)

Hepatomegaly Jaundice Protruding teeth Pathological fractures

Sickle Cell Crisis Interventions

Hydration, rest, protection from infection, pain control with analgesics, blood transfusion, and emotional support.

An adolescent is diagnosed with Hodgkins disease. Lymph nodes on both sides of her diaphragm have been found to be involved, including cervical and inguinal nodes. Which disease stage is this?

III

Hemophilia patient teaching

Children should not participate in contact sports. Parents can place padding on play outfits to prevent cuts and scrapes. Use RICE when injury occurs. ***should not receive any salicylates***

What is the result of a deficiency of factor IX?

Christmas disease

The child receiving a transfusion complains of back pain and itching. What is the best initial action by the nurse?

Clamp off blood and keep line open with normal saline.

What would the nurse include in a teaching plan about mouth care of a child receiving chemotherapy?

Clean teeth with a soft toothbrush.

Patient teaching for Leukemia

Commercial mouthwashes should be avoided because they alter normal flora. Use one part hydrogen peroxide and four parts normal saline instead.

Blood transfusions

Confirm the right pt and correct blood is being given. Administer slowly. Check site for infiltration. Check vitals q5mins for the fist 15 minutes. Signs of reaction include chills, fever, headache and back pain. Tubing should be clamped off and pt should be given normal saline.

Lymphadenopathy

Enlargement of the lymph nodes that may indicate infection or disease.

Splenomegaly

Enlargement of the spleen during infections, congenital and acquired hemolytic anemias, and liver malfunctions.

What important focus of nursing care for the dying child and the family should the nurse implement?

Families should be made aware that hearing is the last sense to stop functioning before death.

Hematopoiesis

Formation of blood. Occurs in long bones. EX: Tibia and femur

Why would the nurse urge the family of a dying 12-year-old boy to include his 8-year-old sister in care? (Select all that apply.)

She will feel less neglected by the parents. She can make amends for past hostilities to her brother. She can express her feelings through care. She can experience being supportive of her parents and brother.

Signs and Symptoms of ITP

Slow clotting time and bruising that occurs easily, and, petichiae. Platelet count is below 20,000. Confirmed by bone marrow aspiration to rule out leukemia.

Iron supplement patient teaching

Take iron with cup of orange juice because Vitamin C helps absorb the iron. If using liquid preparation, take with a straw because it stains the teeth. Stools may become tarry and green. Breast feed for the first 6 months and use iron based formula for the first year of life.

The nurse is dealing with a preschool-age child with a life-threatening illness. What should the nurse remember the childs concept of death is at this age?

That a person becomes alive again soon after death

Hemosiderosis

The deposit of iron into the tissues. Can be treated with deferoximine meslyte (Desferal).

The family of a child receiving chemotherapy for leukemia should be taught to focus on which aspect(s) of the childs care? (Select all that apply.)

Using a support group Stimulating appetite Maintaining adequate hydration Reporting exposure to infectious diseases

A 3-year-old child with sickle cell disease is admitted to the hospital in sickle cell crisis with severe abdominal pain. Which type of crisis is the child most likely experiencing?

Vaso-occlusive

The nurse explains that the COPP medical regimen for the treatment of Hodgkins disease uses a combination of which drugs? (Select all that apply.)

Vincristine Cyclophosphamide Prednisone Procarbazine hydrochloride

Place the stages of dying in the usual order as detailed by Kbler-Ross (1975). Put a comma and space between each answer choice (a, b, c, d, etc.)

c. Denial d. Anger a. Bargaining f. Depression b. Acceptance e. Reaching out to help others

To prevent ________________ ________________, the nurse warms the blood that is to be given as a transfusion through a central line.

cardiac arrhythmias

The nurse is teaching the parents of a young child with iron deficiency anemia about nutrition. What food would the nurse emphasize as being a rich source of iron?

cream of wheat

The nurse shows slides of red blood cells from a child with sickle cell disease, noting that in addition to their sickle shape, the cells contain the abnormal element of ______________ _____.

hemoglobin S